137 results on '"B, Llombart"'
Search Results
2. Treatment of extramammary Paget disease with imiquimod in a real-life setting: a multicentre retrospective analysis in Spain.
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Escolà H, Llombart B, Escolà-Rodríguez A, Barchino-Ortiz L, Marcoval J, Alcaraz I, Beà-Ardébol S, Toll A, Miñano-Medrano R, Rodríguez-Jiménez P, López-Nuñez M, Ferrándiz-Pulido C, Jaka A, Masferrer E, Aguayo-Ortiz RS, Yébenes M, Arandes-Marcocci J, Ruiz-Salas V, Turrión-Merino L, Just M, Sánchez-Schmidt J, Leal L, Mayo-Martínez F, Haya-Martínez L, Sandoval-Clavijo A, Greta Dradi G, Delgado Y, Verdaguer-Faja J, López-Castillo D, Pujol RM, and Deza G
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- Humans, Retrospective Studies, Female, Male, Spain, Aged, Aged, 80 and over, Middle Aged, Treatment Outcome, Skin Neoplasms drug therapy, Skin Neoplasms pathology, Imiquimod therapeutic use, Imiquimod administration & dosage, Paget Disease, Extramammary drug therapy, Paget Disease, Extramammary pathology, Antineoplastic Agents therapeutic use
- Abstract
Background: Topical imiquimod has been shown to be an effective treatment for extramammary Paget disease (EMPD), although available evidence supporting its use is based on case reports and small series of patients., Objectives: To investigate the therapeutic outcomes and analyse potential clinicopathological factors associated with the imiquimod response in a large cohort of patients with EMPD., Methods: Retrospective chart review of 125 patients with EMPD treated with imiquimod at 20 Spanish tertiary-care hospitals., Results: During the study period, patients received 134 treatment regimens with imiquimod, with 70 (52.2%) treatments achieving a complete response (CR), 41 (30.6%) a partial response and 23 (17.2%) no response. The cumulative CR rates at 24 and 48 weeks of treatment were 46.3% and 71.8%, respectively, without significant differences between first-time and previously treated EMPD. Larger lesions (≥ 6 cm; P = 0.04) and EMPD affecting > 1 anatomical site (P = 0.002) were significantly associated with a worse treatment response. However, the CR rate did not differ significantly by the number of treatment applications (≤ 4 vs. > 4 times per week; P = 0.112). Among patients who achieved CR, 30 of 69 (43%) treatments resulted in local recurrences during a mean follow-up period of 36 months, with an estimated 3- and 5-year recurrence-free survival of 55.7% and 36.4%, respectively., Conclusions: Imiquimod appears as an effective therapeutic alternative for both first-line and previously treated EMPD lesions. However, a less favourable therapeutic response could be expected in larger lesions and those affecting > 1 anatomical site. Based on our results, a three to four times weekly regimen of imiquimod with a treatment duration of at least 6 months could be considered an appropriate therapeutic strategy for patients with EMPD., Competing Interests: Conflicts of interest The authors declare no conflicts of interest., (© The Author(s) 2024. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)
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- 2024
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3. Slow Mohs Micrographic Surgery for the Treatment of Genital Dermatofibrosarcoma Protuberans: A Single-Center Series of 5 Cases.
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Algarra-Sahuquillo J, Llombart B, Serra-Guillen C, and Sanmartin O
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- Humans, Male, Middle Aged, Female, Adult, Treatment Outcome, Aged, Mohs Surgery, Dermatofibrosarcoma surgery, Dermatofibrosarcoma pathology, Skin Neoplasms surgery, Skin Neoplasms pathology
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- 2024
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4. Long-Term Improvement of Different Types of Acne Vulgaris Using a Mild Photodynamic Therapy Protocol with BF-200 ALA Gel: A Series of Cases.
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Serra-Guillén C, Llombart B, and Sanmartín O
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Background : Photodynamic therapy (PDT) can be a promising alternative for patients with acne vulgaris. Our study aimed to evaluate the efficacy and safety of red light photodynamic therapy with BF-200 ALA gel in the treatment of different types of acne vulgaris. Methods : We performed a retrospective, observational study of a series of 22 cases. All patients were treated according to a mild PDT protocol. After a careful wash of the affected skin areas, BF-200 ALA gel was applied to the skin in a thin layer and incubated for 30 min, followed by illumination using narrow-spectrum red light (635 nm) at a dose of 4 J/cm
2 . Most patients received one (36.4%), two (27.3%), or three (22.7%) PDT sessions. About a third of the patients received concomitant acne treatment with topical retinoids. Results : Patients of 25.1 ± 8.9 years suffering from papulopustular (45.5%), nodular (27.3%), and comedonal acne (27.3%) in the face were included. Irrespective of acne type or severity, 95.5% of patients had good or excellent responses to the treatment with PDT (≥60% lesion clearance). We found no association between concomitant acne medication and the favorable results achieved by PDT. Most patients reported no adverse events (72.7%), except for six patients who experienced erythema. The good efficacy results were maintained over a follow-up period of 12.5 ± 10.8 months. Conclusions : In this study, we show that PDT with BF-200 ALA gel and low light dose is an effective and long-lasting option for the treatment of different acne types.- Published
- 2024
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5. Avelumab to treat Merkel cell carcinoma: real-life experience in a dedicated oncology center.
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Ríos-Viñuela E, García-Vázquez M, Juan MJ, Nagore E, Requena C, Sanmartín O, and Llombart B
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The arrival of immunotherapy has revolutioned the management of patients with metastatic Merkel cell carcinoma (MCC). We conducted an observational, retrospective study of 14 cases treated with avelumab. The response rate was 57%: complete response was reached in 29% of patients, and partial responses in 29%. The drug proved effective in 83% (5/6) of the patients with a single metastatic site. However, the disease progressed in 75% (3/4) of the patients with bone metastases. PD1-L expression, MCC polyomavirus (MCPyV) positivity, and an impaired neutrophil-to-lypmhocyte ratio (NLR) could not be associated with responses to the therapy. Avelumab is an effective and safe drug for the management of advanced MCC, and its effectiveness appears to be impacted by the number and location of metastases., (Copyright © 2024. Publicado por Elsevier España, S.L.U.)
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- 2024
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6. Combined Merkel Cell Carcinoma and Squamous Cell Carcinoma: A Systematic Review.
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Ríos-Viñuela E, Mayo-Martínez F, Nagore E, Millan-Esteban D, Requena C, Sanmartín O, and Llombart B
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Combined Merkel cell carcinoma (MCC) and squamous cell carcinoma (SCC) have classically been regarded as more aggressive than conventional, pure, Merkel cell polyomavirus (MCPyV)-positive MCC. It is still unknown whether combined MCC and SCC are more aggressive than pure, MCPyV-negative MCC, and the origin of both the SCC and MCC elements of these combined tumors has not been elucidated. The main objective of this systematic review was to assess whether combined MCC and SCC tumors are associated with a worse prognosis than pure MCC; the secondary goals were the characterization of the clinical and histopathological features of these combined neoplasms. A total of 38 studies, including 152 patients, were selected for review. In total, 76% of the cases were MCPyV-negative, whereas 4% were MCPyV-positive. The most frequent histopathological pattern was that of an SCC in situ combined with a dermal MCC (36%), followed by both an in situ and invasive SCC combined with a dermal MCC (20%). Forty-seven percent of all cases fitted in the morphology of the so-called "collision tumors". Three combined MCC cases that would fit in the morphological category of collision tumors presented both squamous and neuroendocrine elements in their respective nodal metastases. The mean overall survival was 36 months, comparable to that of pure, MCPyV-negative MCC. This review found similarly aggressive behavior for combined MCC and SCC and pure, MCPyV-negative MCC. Preliminary data strongly suggest that all MCPyV-negative MCC tumors, whether combined or pure, are part of a common spectrum.
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- 2024
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7. Therapeutic outcomes and survival analysis of Extramammary Paget's disease: A multicentre retrospective study of 249 patients.
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Escolà H, Llombart B, Escolà-Rodríguez A, Barchino-Ortiz L, Marcoval J, Alcaraz I, Beà-Ardébol S, Toll A, Miñano-Medrano R, Rodríguez-Jiménez P, López-Nuñez M, Ferrándiz-Pulido C, Jaka A, Masferrer E, Aguayo-Ortiz RS, Yébenes M, Arandes-Marcocci J, Ruiz-Salas V, Turrión-Merino L, Just M, Sánchez-Schmidt J, Leal L, Mayo-Martínez F, Haya-Martínez L, Sandoval-Clavijo A, Dradi GG, Delgado Y, Verdaguer-Faja J, López-Castillo D, Pujol RM, and Deza G
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- Humans, Retrospective Studies, Mohs Surgery, Survival Analysis, Margins of Excision, Treatment Outcome, Neoplasm Recurrence, Local epidemiology, Neoplasm Recurrence, Local therapy, Neoplasm Recurrence, Local pathology, Paget Disease, Extramammary surgery
- Abstract
Background: Evidence regarding long-term therapeutic outcomes and disease-specific survival (DSS) in Extramammary Paget's disease (EMPD) is limited., Objectives: To assess the DSS and outcomes of surgical and nonsurgical therapeutic modalities in a large cohort of EMPD patients., Methods: Retrospective chart review of EMPD patients from 20 Spanish tertiary care hospitals., Results: Data on 249 patients with a median follow-up of 60 months were analyzed. The estimated 5-, 10-, and 15-year DSS was 95.9%, 92.9%, and 88.5%, respectively. A significantly lower DSS was observed in patients showing deep dermal invasion (≥1 mm) or metastatic disease (P < .05). A ≥50% reduction in EMPD lesion size was achieved in 100% and 75.3% of patients treated with surgery and topical therapies, respectively. Tumor-free resection margins were obtained in 42.4% of the patients after wide local excision (WLE). The 5-year recurrence-free survival after Mohs micrographic surgery (MMS), WLE with tumor-free margins, WLE with positive margins, radiotherapy, and topical treatments was 63.0%, 51.4%, 20.4%, 30.1%, and 20.8%, respectively., Limitations: Retrospective design., Conclusions: EMPD is usually a chronic condition with favorable prognosis. MMS represents the therapeutic alternative with the greatest efficacy for the disease. Recurrence rates in patients with positive margins after WLE are similar to the ones observed in patients treated with topical agents., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2023. Published by Elsevier Inc.)
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- 2024
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8. Topical Imiquimod in Primary Cutaneous Extramammary Paget's Disease: A Systematic Review.
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Mayo-Martínez F, Moro R, Millán-Esteban D, Ríos-Viñuela E, Bautista IJ, Nagore E, Sanmartín O, and Llombart B
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Extramammary Paget's disease (EMPD) is subclinical in extent and multifocal in nature. There is no global consensus for treatment, so its management represents a challenge in clinical practice. Therefore, we conducted a systematic review through the main electronic databases to assess the effectiveness of topical imiquimod in cutaneous EMPD and to discuss its management. Finally, 24 studies involving a total of 233 EMPD patients treated with topical imiquimod were selected. The topical imiquimod response rate was 67%, and the complete response (CR) rate was 48%. Patients were treated with a three-four times a week regimen in most cases, ranging between 2 to 52 weeks. In addition, imiquimod was applied as an adjunctive treatment in 21 patients, achieving a CR rate of 71%. Consequently, imiquimod therapy could achieve a good response ratio as a first-line treatment, as adjuvant and neo-adjuvant therapy, and as a treatment for recurrent disease. The heterogeneity between studies and the lack of a control arm made it impossible to conduct a meta-analysis. To improve the quality of evidence on EMPD, multicenter studies are essential to collect a larger number of patients and, consequently, obtain high-quality evidence to standardize treatment. The Prospero registration number is CRD42023447443.
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- 2023
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9. Tumor Microenvironment and Its Clinicopathologic and Prognostic Association in Cutaneous and Noncutaneous Angiosarcomas.
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Machado I, Requena C, López-Reig R, Fernández-Serra A, Giner F, Cruz J, Traves V, Lavernia J, Claramunt R, Llombart B, López-Guerrero JA, and Llombart-Bosch A
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- Humans, B7-H1 Antigen, Prognosis, Tumor Microenvironment, Hemangiosarcoma genetics, Skin Neoplasms genetics, Skin Neoplasms pathology
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Objectives: We explored features of the angiosarcoma (AS) tumor microenvironment to discover subtypes that may respond to immunotherapy., Methods: Thirty-two ASs were included. Tumors were studied by histology, immunohistochemistry (IHC), and gene expression profile using the HTG EdgeSeq Precision Immuno-Oncology Assay., Results: Comparing cutaneous and noncutaneous ASs, the second group showed 155 deregulated genes, and unsupervised hierarchical clustering (UHC) delineated two groups: the first mostly cutaneous AS and the second mainly noncutaneous AS. Cutaneous ASs showed a significantly higher proportion of T cells, natural killer cells, and naive B cells. ASs without MYC amplification revealed a higher immunoscore in comparison with ASs with MYC amplification. PD-L1 was significantly overexpressed in ASs without MYC amplification. UHC showed 135 deregulated genes differentially expressed when comparing ASs from the non-head and neck area with patients who had AS in the head and neck area. ASs from the head and neck area showed high immunoscore. PD1/PD-L1 content was significantly more highly expressed in ASs from the head and neck area. IHC and HTG gene expression profiling revealed a significant correlation between PD1, CD8, and CD20 protein expression but not PD-L1., Conclusions: Our HTG analyses confirmed a high degree of tumor and microenvironment heterogeneity. Cutaneous ASs, ASs without MYC amplification, and ASs located in the head and neck area seem to be the most immunogenic subtypes in our series., (© The Author(s) 2023. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
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- 2023
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10. Lymphovascular Invasion and High Mitotic Count Are Associated With Increased Risk of Recurrence in Pleomorphic Dermal Sarcoma.
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Pons Benavent M, Ríos-Viñuela E, Nagore E, Monteagudo C, Aguerralde M, Mata Cano D, Llombart B, Serra-Guillén C, Pinazo Canales I, Requena C, and Sanmartín O
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- Humans, Necrosis complications, Neoplasm Recurrence, Local epidemiology, Prognosis, Retrospective Studies, Bone Neoplasms complications, Sarcoma pathology, Skin Neoplasms pathology
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Background and Objective: Atypical fibroxanthoma and pleomorphic dermal sarcoma (PDS) are rare mesenchymal tumors. Due to the low incidence of PDS and a historically confusing nomenclature, little is known about the true aggressiveness of this tumor. The aim of this study was to investigate clinical and histologic risk factors for recurrence in PDS., Material and Methods: Retrospective, observational, bicentric study of 31 PDSs diagnosed and treated at Hospital Clínico Universitario de Valencia and Instituto Valenciano de Oncología in Valencia, Spain, between 2005 and 2020. We described the clinical and histologic features of these tumors and performed univariate analysis and multivariate Cox regression analysis., Results: In the univariate analysis, tumor recurrence (P<.001), necrosis (P=.020), lymphovascular invasion (P=.037), perineural invasion (P=.041), and mitotic count (<18 vs ≥18 mitoses per 10 high-power fields) (P=.093) were associated with worse disease-free survival. In the multivariate Cox regression analysis, mitotic count and lymphovascular invasion retained their significance as predictors of worse disease-free survival (P<.05)., Conclusions: PDS is an aggressive tumor in which a high mitotic count (≥18) and lymphovascular invasion are associated with a higher risk of recurrence and worse disease-free survival. Necrosis and perineural invasion are also probably linked to increased tumor aggressiveness., (Copyright © 2023 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
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- 2023
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11. Combined Merkel cell carcinoma and cutaneous squamous cell carcinoma with lymph node metastases: Report of two cases.
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Ríos-Viñuela E, Traves V, Cruz J, Machado I, López-Guerrero JA, Requena C, and Llombart B
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- Humans, Aged, Lymphatic Metastasis, Skin pathology, Carcinoma, Merkel Cell pathology, Carcinoma, Squamous Cell pathology, Skin Neoplasms pathology, Merkel cell polyomavirus, Polyomavirus Infections, Tumor Virus Infections
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Merkel cell carcinoma (MCC) is an infrequent, aggressive cutaneous neoplasm, that typically affects the photodamaged skin of elderly individuals, and immunosuppressed patients. Because a subset of MCC is closely related to UV radiation, MCC can develop concurrently with other tumors, most commonly, as a combined tumor with squamous cell carcinoma (SCC). These combined tumors appear to represent a distinct disease process from pure MCC, as they are mostly Merkel cell polyomavirus (MCPyV) negative, and show a more aggressive behavior. We present two additional cases of combined MCC and SCC with nodal metastases, one of which was MCPyV positive. Two different subtypes of MCC have been proposed based on their origin: a true neuroendocrine carcinoma, that is MCPyV positive and has a dermal origin, and a UV-related SCC with neuroendocrine differentiation. This theory could explain why MCC can develop concurrently with SCC, and why these combined cases are generally MCPyV negative. However, it fails to explain the minority of combined MCC and SCC tumors that are MCPyV positive. Because both our patients had a history of chronic UV exposure, we hypothesize that UV radiation probably played a major role in the pathogenesis of these tumors, while MCPyV integration probably acted as an additional trigger., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
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- 2023
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12. Trichoepithelioma on the Finger: A Rare Location and Dermoscopic-Histopathologic Correlation.
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Mayo-Martínez F, Moro R, Traves V, Llombart B, and Requena C
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- Male, Humans, Aged, Hair pathology, Upper Extremity pathology, Fingers pathology, Skin Neoplasms pathology, Hair Diseases pathology
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Abstract: Trichoepithelioma is a benign adnexal neoplasm of follicular germinative cells, with bulbs, papillae, and sheaths of perifollicular connective tissue as signs of follicular differentiation. Accordingly, trichoepithelioma may arise in any hair-bearing location, mostly on the face. That is why trichoepithelioma cannot appear in glabrous skin, and, although the dorsum of the hands and feet are a hair-bearing area, acral location is exceptional. We report the first case of trichoepithelioma localized in the finger of a 79-year-old man. It was a brown-pink, firm, 7-mm diameter, solid papule on the dorsal aspect of his left index finger. The lesion was completely asymptomatic, and he remembered to have it for many years. We describe this case highlighting its rare anatomical location and correlate its dermoscopic features with the histopathological appearance., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)
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- 2023
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13. Primary cutaneous leiomyosarcoma: a single institution study treated with modified Mohs surgery.
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Vargas-Mora P, Llombart B, Castro JR, Cruz J, Serra C, Requena C, Traves V, and Sanmartín O
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- Humans, Mohs Surgery, Neoplasm Recurrence, Local surgery, Skin, Leiomyosarcoma surgery, Skin Neoplasms surgery
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- 2023
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14. Clinicopathological features, MCPyV status and outcomes of Merkel cell carcinoma in solid-organ transplant recipients: a retrospective, multicentre cohort study.
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Ferrándiz-Pulido C, Gómez-Tomás A, Llombart B, Mendoza D, Marcoval J, Piaserico S, Baykal C, Bouwes-Bavinck JN, Rácz E, Kanitakis J, Harwood CA, Cetkovská P, Geusau A, Del Marmol V, Masferrer E, Orte Cano C, Ricar J, de Oliveira WR, Salido-Vallejo R, Ducroux E, Gkini MA, López-Guerrero JA, Kutzner H, Kempf W, and Seçkin D
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- Humans, Retrospective Studies, TOR Serine-Threonine Kinases, Carcinoma, Merkel Cell pathology, Merkel cell polyomavirus, Organ Transplantation adverse effects, Polyomavirus Infections, Skin Neoplasms pathology, Tumor Virus Infections complications
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Background: The proportion of Merkel cell carcinomas (MCCs) in solid-organ transplant recipients (SOTR) harbouring Merkel cell polyomavirus (MCPyV) is unknown, as are factors affecting their outcomes., Objective: To describe clinicopathological features of MCC in SOTR, investigate the tumoral MCPyV-status and identify factors associated with tumour outcomes., Methods: Retrospective, international, cohort-study. MCPyV-status was investigated by immunohistochemistry and polymerase chain reaction., Results: A total of 30 SOTR and 44 consecutive immunocompetent patients with MCC were enrolled. SOTR were younger at diagnosis (69 vs. 78 years, P < 0.001). Thirty-three percent of SOTR MCCs were MCPyV-positive vs. 91% of immunocompetent MCCs (P = 0.001). Solid-organ transplantation was associated with an increased cumulative incidence of progression (SHR: 3.35 [1.57-7.14], P = 0.002), MCC-specific mortality (SHR: 2.55 [1.07-6.06], P = 0.034) and overall mortality (HR: 3.26 [1.54-6.9], P = 0.002). MCPyV-positivity and switching to an mTOR inhibitor (mTORi) after MCC diagnosis were associated with an increased incidence of progression (SHR: 4.3 [1.5-13], P = 0.008 and SHR: 3.6 [1.1-12], P = 0.032 respectively) in SOTR., Limitations: Retrospective design and heterogeneity of SOTR cohort., Conclusions: MCPyV appears to play a less prominent role in the aetiopathogenesis of MCC in SOTR. SOTR have a worse prognosis than their immunocompetent counterparts and switching to an mTORi after the diagnosis of MCC does not improve progression., (© 2022 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)
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- 2022
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15. Cutaneous Ossifying Amyloidoma.
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Requena C, Diago A, Traves V, Llombart B, Nagore E, and Sanmartin O
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- Aged, Amyloid analysis, Female, Humans, Immunoglobulin A, Immunoglobulin Light Chains, Osteogenesis, Amyloidosis pathology, Soft Tissue Neoplasms
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Abstract: Amyloidoma, otherwise known as tumoral amyloidosis, is a localized deposition of amyloid (AL-type or AA type) without systemic amyloidosis. It is the rarest form of tissue amyloid deposition, and up to 7% of amyloidomas develop systemic amyloidosis.Cutaneous AL-type amyloidoma is considered by many authors as an unusual variant of primary cutaneous marginal zone lymphoma. Although cutaneous amyloidoma can form calcifications, ossification is extremely unusual, with only 1 case previously published to date.We report the case of a 75-year-old woman with voluminous and strikingly ossifying AL-type amyloidoma in the left pretibial skin. Her medical history included excision of hepatic hydatidic cysts 25 years prior and diffuse large B-cell lymphoma of the left parotid gland 8 years prior treated with chemotherapy and radiotherapy with complete response. After the diagnosis of amyloidoma, an extension study with cervical, chest, abdominal, and pelvic TC was performed, with no additional lesions found. Serum and protein electrophoresis revealed elevations in kappa light chain and IgA immunoglobulin levels but did not reveal monoclonal bands. In situ hybridization for immunoglobulin light chains showed monotypic kappa expression in plasma cells infiltrating the amyloidoma.Extensive ossification in amyloidomas can make diagnosis difficult; therefore, we describe an interesting case of this histopathologically peculiar amyloidoma., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)
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- 2022
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16. Cemiplimab in Advanced Cutaneous Squamous Cell Carcinoma: Real-World Experience in a Monographic Oncology Center.
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Ríos-Viñuela E, Álvarez P, Lavernia J, Serra-Guillén C, Requena C, Bernia E, Diago A, Llombart B, and Sanmartín O
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- Antibodies, Monoclonal, Humanized adverse effects, Humans, Immunotherapy, Carcinoma, Squamous Cell drug therapy, Carcinoma, Squamous Cell pathology, Skin Neoplasms drug therapy, Skin Neoplasms pathology
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Management of advanced cSCC is challenging, and many available systemic medications have modest efficacy. Cemiplimab has demonstrated efficacy in the treatment of advanced cSCC in clinical trials, but real-world data are still limited. With the objective of evaluating the efficacy of cemiplimab in a real-world clinical setting, we conducted a prospective observational study of 13 patients with advanced cSCC. Six patients (46%) had locally advanced disease, while 7 (54%) had metastatic disease. A total of 8 patients (62%) responded to cemiplimab. Five (38%) showed a partial response, while 3 (23%) showed a complete response. Four patients with an initial partial response presented subsequent disease progression during follow-up. Six patients (46%) developed AEs, most of which were mild (G1). PFS was 5.9 months, with a median follow-up was 9 months. In conclusion, cemiplimab demonstrated its utility in the treatment of advanced cSCC, with acceptable response rates, a remarkable number of complete responses, and a very good safety profile., (Copyright © 2022. Publicado por Elsevier España, S.L.U.)
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- 2022
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17. Cemiplimab in Advanced Cutaneous Squamous Cell Carcinoma: Real-World Experience in a Monographic Oncology Center.
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Ríos-Viñuela E, Álvarez P, Lavernia J, Serra-Guillén C, Requena C, Bernia E, Diago A, Llombart B, and Sanmartín O
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- Antibodies, Monoclonal, Humanized adverse effects, Humans, Immunotherapy, Carcinoma, Squamous Cell drug therapy, Carcinoma, Squamous Cell pathology, Skin Neoplasms drug therapy, Skin Neoplasms pathology
- Abstract
Management of advanced cSCC is challenging, and many available systemic medications have modest efficacy. Cemiplimab has demonstrated efficacy in the treatment of advanced cSCC in clinical trials, but real-world data are still limited. With the objective of evaluating the efficacy of cemiplimab in a real-world clinical setting, we conducted a prospective observational study of 13 patients with advanced cSCC. Six patients (46%) had locally advanced disease, while 7 (54%) had metastatic disease. A total of 8 patients (62%) responded to cemiplimab. Five (38%) showed a partial response, while 3 (23%) showed a complete response. Four patients with an initial partial response presented subsequent disease progression during follow-up. Six patients (46%) developed AEs, most of which were mild (G1). PFS was 5.9 months, with a median follow-up was 9 months. In conclusion, cemiplimab demonstrated its utility in the treatment of advanced cSCC, with acceptable response rates, a remarkable number of complete responses, and a very good safety profile., (Copyright © 2022 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
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- 2022
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18. Clinical and pathological features of Merkel cell carcinoma: A 4-year follow-up observational retrospective study in Spain.
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Ríos-Martín JJ, Rodriguez-Salas N, Vázquez-Doval FJ, Llombart B, Rojas-Ferrer N, González-Vela MC, Zulueta T, Monteagudo C, Aneiros-Fernández J, Beato MJ, Carrillo R, Silva-Carmona MY, Ayala M, Gallego E, Rodríguez-Peralto JL, Fraga-Fernández J, Fernández-Figueras MT, Barranco C, Córdoba A, Sanz-Zorrilla A, Ferrer B, Fúnez R, Santonja C, Saus C, Idoate MA, Santos-Briz A, Onrubia J, Pinedo F, and de Las Peñas R
- Subjects
- Follow-Up Studies, Humans, Male, Retrospective Studies, Spain epidemiology, Carcinoma, Merkel Cell epidemiology, Carcinoma, Merkel Cell therapy, Merkel cell polyomavirus, Skin Neoplasms epidemiology, Skin Neoplasms therapy
- Abstract
Background: Merkel cell carcinoma (MCC) is a malignant skin cancer with a 5-year survival rate of approximately 50%. Knowledge of MCC has increased in recent years mostly due to improved diagnosis techniques. In Spain there is lack of information regarding the incidence and tumour characteristics, and the treatment approaches are not standardised. The objective of this study was to provide information of the clinical and epidemiological characteristics of MCC patients in Spain., Methods: Retrospective, observational study involving 192 patients from 25 Spanish hospitals. Evaluated variables included overall survival and incidence rate of Merkel cell polyomavirus, in patients diagnosed from 2012 to 2016., Results: The Spanish incidence rate was estimated 0.32/100,000 inhabitants/year, with variations according to geographical regions, being slightly higher in areas with greater sunlight exposure. In total, 61.5% of tumours showed expansive growth (progressive growth of the tumour), 78.6% showed localisation in UV-exposed skin. 97.4% of patients were diagnosed by excisional biopsy. Surgery was the first line treatment in 96.6% of patients, radiotherapy in 24.6%, and chemotherapy in 6.3%. These treatments were not mutually exclusive. Median overall survival was 38.3 months (78.4% at 12 months and 60% at 24 months). MCPyV was present in 33.8% of patients., Conclusion: The incidence of MCC in Spain is one of the highest in Europe, with a slight predominance in men. The sample has shown that a biopsy is available for diagnosis in most cases. Moreover, the treatment is surgical when the tumour is localized and is associated with lymphadenectomy, and/or it is radiotherapy if widespread., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)
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- 2022
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19. Determination of Margins for Tumor Clearance in Dermatofibrosarcoma Protuberans: A Single-Center Study of 222 Cases Treated With Modified Mohs Surgery.
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Serra-Guillén C, Llombart B, Nagore E, Guillén C, and Sanmartín O
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- Adolescent, Adult, Dermatofibrosarcoma diagnosis, Dermatofibrosarcoma pathology, Female, Follow-Up Studies, Head and Neck Neoplasms diagnosis, Head and Neck Neoplasms pathology, Humans, Longitudinal Studies, Male, Margins of Excision, Neoplasm Recurrence, Local pathology, Neoplasm Recurrence, Local prevention & control, Skin pathology, Skin Neoplasms diagnosis, Skin Neoplasms pathology, Treatment Outcome, Young Adult, Dermatofibrosarcoma surgery, Head and Neck Neoplasms surgery, Mohs Surgery methods, Neoplasm Recurrence, Local epidemiology, Skin Neoplasms surgery
- Abstract
Background: Dermatofibrosarcoma protuberans (DFSP) is an invasive skin tumor traditionally associated with very high recurrence rates when treated with conventional surgery (CS)., Objective: To calculate the minimum margin that would have been required to achieve complete tumor clearance with hypothetical CS. To analyze DFSP characteristics and Mohs micrographic surgery (MMS) effectiveness in treatment of this tumor., Materials and Methods: Minimum margin was calculated by measuring the largest distance from the visible edge of the tumor to the edge of the surgical defect. Tumor variables (age, sex, size, time since onset, and location) were correlated with surgical variables (number of stages and minimum margin)., Results: We studied 222 cases of DFSP treated with MMS. A mean of 1.47 MMS stages and a mean minimum margin of 1.23 cm were required to achieve tumor clearance. Tumors on the head and neck required significantly more stages and a significantly wider margin. Tumor size was positively correlated with time to diagnosis, age, and number of MMS stages., Conclusion: Tumors located on the head and neck have greater subclinical extension. Tumor size was also a predictor of surgical difficulty, but time to diagnosis was not., (Copyright © 2021 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)
- Published
- 2022
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20. Usefulness of ultrasound in dermatofibrosarcoma protuberans and correlation with histopathological findings: A series of 30 cases.
- Author
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Diago A, Llombart B, Serra-Guillen C, Arana E, Guillén C, Requena C, Traves V, Bancalari B, Bernia E, Ríos-Viñuela E, and Sanmartín O
- Subjects
- Humans, Retrospective Studies, Subcutaneous Tissue, Ultrasonography, Dermatofibrosarcoma diagnostic imaging, Skin Neoplasms diagnostic imaging
- Abstract
Background: Small series of ultrasound findings in dermatofibrosarcoma protuberans (DFSP) have been published, but the usefulness of this technique as a preoperative planning tool for tumor resection has not been studied., Materials and Methods: We retrospectively reviewed patients with DFSP at our hospital that underwent ultrasound examination. Depth of invasion was evaluated by ultrasound and histopathology. Accuracy of ultrasound for assessing depth of tumor invasion was estimated., Results: Thirty histopathologically confirmed DFSPs were studied. Classic finger-like projections were observed in 73.3% of cases. A posterior hyperechoic area extending deep into the subcutaneous tissue correlated with the honeycomb DFSP pattern and was observed in 53.3% of patients. Concordance between ultrasound and histopathologic depth measurements was excellent. Lateral tumor extension and Doppler activity were not evaluated in our series., Conclusion: Ultrasound showed excellent prediction of depth of invasion. Further studies are required to define the usefulness of ultrasound for determining lateral tumor extension., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2021
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21. Cutaneous intralymphatic histiocytosis associated with breast and orthopedic surgery.
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Ríos-Viñuela E, Bernia E, Diago A, Traves V, Requena C, Llombart B, and Sanmartín O
- Subjects
- Antigens, CD metabolism, Antigens, Differentiation, Myelomonocytic metabolism, Biopsy, Diagnosis, Differential, Erythema pathology, Female, Humans, Mastectomy adverse effects, Membrane Glycoproteins metabolism, Middle Aged, Orthopedic Procedures adverse effects, Shoulder Prosthesis adverse effects, Skin pathology, Histiocytes pathology, Histiocytosis diagnosis, Lymphatic Vessels pathology, Skin Neoplasms pathology
- Published
- 2021
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22. The role of 18 F-FDG PET/CT in the management of Merkel cell carcinoma: The experience of 51 studies in our institution.
- Author
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López Prior V and Llombart Cussac B
- Abstract
Objectives: Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor with limited evidence on the role of
18 F-FDG PET/CT. The aim of this study was to assess the impact of the18 F-FDG PET/CT in the management of MCC., Methods: Fifty-one studies of18 F-FDG PET/CT of 35 patients (19 men [54.30%]; 72.17±14.63years) with histologic diagnosis of MCC were retrospectively evaluated. The change in tumor staging and the impact on the treatment were analysed., Results: There were 23 PET/CT positive studies (45.10%) and 28 (54.90%) negative. Thirty four (66.7%) studies were performed for assessment of stage at initial presentation and 17 (33.3%) were performed during the follow up: 6 (35.29%) for suspected recurrence; 7 (41.18%) for restaging; 4 (23.53%) as a part of ongoing surveillance. On the basis of PET/CT results, there was a change in disease stage (SC) in 20 studies (39.20%) and impact in the management (MI) in 28 (54.90%): 11 (32.40%) SC and 12 (35.30%) MI in the initial staging; 5 (71.43%) SC and 7 (100%) MI in the restaging; 3 (50.00%) SC and 6 (100%) MI in suspected recurrence; 1 (25.00%) SC and 3 (75.00%) MI in the surveillance.18 F-FDG PET/CT incidentally detected one additional histologically confirmed cancer. The presence of nodal involvement in the beginning (0.0098; HR 3.82; 95%CI: 1.38-10.6), chemotherapy treatment (6e-04; HR 7.06; 95%CI: 2.30-21.60), size of primary tumor >2cm (6e-04; HR 7.06; 95%CI: 2.30-21.60) and positive resection margin (0.00069; HR 4.01; 95%CI: 1.46-11.00) were statistically significant prognostic factors for overall survival. There was a trend towards significance for worse overall survival with initial positive18 F-FDG PET/CT but the trend did not reach statistical significance., Conclusion:18 F-FDG PET/CT altered the stage in 2 out of 5 studies and changed the treatment in more than half of the studies performed. The study confirms the important impact of18 F-FDG PET/CT on the management of MCC patients., (Copyright © 2020 Sociedad Española de Medicina Nuclear e Imagen Molecular. Publicado por Elsevier España, S.L.U. All rights reserved.)- Published
- 2021
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23. Localized Injection-site Toxic Erythema of Chemotherapy: An Under-recognized Acquaintance Revisited.
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Ríos-Viñuela E, Bernia E, Toledo-Pastrana T, Requena C, Diago A, Serra-Guillén C, Llombart B, Traves V, Nagore E, Guillén C, and Sanmartin O
- Subjects
- Erythema chemically induced, Erythema diagnosis, Friends, Humans, Erythema Multiforme, Stevens-Johnson Syndrome
- Published
- 2021
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24. Sonidegib in the Treatment of Locally Advanced Basal Cell Carcinoma.
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Sanmartín O, Llombart B, Carretero Hernández G, Flórez Menéndez Á, Botella-Estrada R, Herrera Ceballos E, and Puig S
- Subjects
- Biphenyl Compounds, Hedgehog Proteins therapeutic use, Humans, Pyridines, Antineoplastic Agents adverse effects, Carcinoma, Basal Cell drug therapy, Skin Neoplasms drug therapy
- Abstract
Sonidegib is an antagonist of the transmembrane protein Smoothened in the Hedgehog signaling pathway. It is indicated for the treatment of locally advanced basal cell carcinoma (BCC) that is not amenable to curative surgery or radiotherapy. Sonidegib's efficacy and safety were demonstrated in the phase 2 BOLT trial, where 61% (95% CI, 48-72%) of patients with locally advanced BCC treated with sonidegib 200 mg achieved an objective response to treatment with a mean time to response of 4 months. The median duration of response was 26.1 months and the median progression-free survival was 22.1 months. The most common adverse events were muscle spasms (54.4%), hair loss (49.4%), and loss of taste (44.3%); most events were grade 1 or 2. In this review, we summarize the main findings on the efficacy, safety, and tolerability of sonidegib and discuss the management of locally advanced BCC with this drug., (Copyright © 2020 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2021
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25. Angiosarcomas: histology, immunohistochemistry and molecular insights with implications for differential diagnosis.
- Author
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Machado I, Giner F, Lavernia J, Cruz J, Traves V, Requena C, Llombart B, López-Guerrero JA, and Llombart-Bosch A
- Subjects
- Biomarkers, Tumor analysis, Breast Neoplasms diagnosis, Breast Neoplasms metabolism, Breast Neoplasms pathology, Cell Differentiation, Diagnosis, Differential, Female, Hemangiosarcoma metabolism, Humans, Sarcoma pathology, Skin Neoplasms diagnosis, Skin Neoplasms metabolism, Skin Neoplasms pathology, Soft Tissue Neoplasms metabolism, Tumor Microenvironment, Vascular Neoplasms diagnosis, Vascular Neoplasms metabolism, Vascular Neoplasms pathology, Hemangiosarcoma diagnosis, Hemangiosarcoma pathology, Immunohistochemistry methods, Soft Tissue Neoplasms diagnosis, Soft Tissue Neoplasms pathology
- Abstract
Angiosarcomas (AS) represent a heterogenous group of tumors with variable clinical presentation. AS share an important morphologic and immunohistochemical overlap with other sarcomas, hence the differential diagnosis is challenging, especially in poorly-differentiated tumors. Although molecular studies provide significant clues, especially in the differential diagnosis with other vascular neoplasms, a thorough hematoxylin and eosin analysis remains an essential tool in AS diagnosis. In this review, we discuss pathological and molecular insights with emphasis on implications for differential diagnosis in cutaneous, breast, soft tissue and visceral AS.
- Published
- 2021
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26. Erythema Papulosa Semicircularis Recidivans: A New Entity or a Subtype of Erythema Annulare Centrifugum?
- Author
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Bernia E, Requena C, and Llombart B
- Subjects
- Erythema diagnosis, Humans, Ascomycota, Skin Diseases, Genetic
- Published
- 2020
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27. Pleomorphic dermal sarcoma: a retrospective study of 16 cases in a dermato-oncology centre and a review of the literature.
- Author
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Ríos-Viñuela E, Serra-Guillén C, Llombart B, Requena C, Nagore E, Traves V, Guillén C, Vázquez D, and Sanmartín O
- Subjects
- Aged, Aged, 80 and over, Female, Humans, Male, Margins of Excision, Mohs Surgery, Neoplasm Metastasis, Neoplasm Recurrence, Local pathology, Neoplasm Staging, Prognosis, Retrospective Studies, Sarcoma pathology, Sarcoma surgery, Skin Neoplasms pathology, Skin Neoplasms surgery
- Abstract
Background: Relatively little is known about the true aggressive potential of pleomorphic dermal sarcoma (PDS) or optimal management strategies., Objective: To describe the outcomes of 16 cases of PDS treated at our hospital (14 with modified Mohs micrographic surgery [M-MMS] and two with conventional surgery) and establish an adequate plan for management., Materials & Methods: We reviewed 16 PDS cases treated at our hospital between October 2007 and June 2019 and compared our results with the available evidence., Results: In total, 69% of cases had recurred after initial conventional surgery, M-MMS led to local disease control in 83% of cases, and 19% of patients developed metastasis. Combining all published PDS cases with ours, we calculated an overall metastasis rate of 12%, and an overall recurrence rate of 35% after conventional surgery and 17% after M-MMS., Conclusion: PDS is more aggressive than previously estimated, with an overall metastatic rate of 12%. Despite high recurrence rates with previous conventional surgery (69%), M-MMS achieved a good rate of local disease control (83%). Given the potential aggressivity of PDS and the importance of clear surgical margins, M-MMS appears to be more adequate than conventional excision. Staging studies and close monitoring are warranted in PDS patients, for which we propose a management algorithm.
- Published
- 2020
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28. Conventional Photodynamic Therapy for Necrobiosis Lipoidica: Successful Treatment in a Series of 4 Cases.
- Author
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Bernia E, Llombart B, Bancalari B, Diago A, and Serra-Guillén C
- Subjects
- Female, Humans, Photosensitizing Agents therapeutic use, Necrobiosis Lipoidica drug therapy, Photochemotherapy
- Abstract
Necrobiosis lipoidica is a rare chronic granulomatous disease. Multiple treatment approaches are available, but results are generally minimal and inconsistent. Some publications report variable results with photodynamic therapy (PDT) as a second line of treatment for refractory cases. We report 4 cases of necrobiosis lipoidica treated satisfactorily with conventional PDT using methyl aminolevulinate or 5-aminolevulinic acid BF-200 as the photosensitizing agent. All 4 patients were women with diabetes mellitus who had undergone treatment at least twice in the past, with little improvement. The lesions resolved completely with PDT, leaving only residual atrophy after a mean of 3.2 sessions per lesion., (Copyright © 2020 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2020
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29. Predictive Value of Sentinel Lymph Node Biopsy in Cutaneous Squamous Cell Carcinoma Based on the AJCC-8 and Brigham and Women's Hospital Staging Criteria.
- Author
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Tejera-Vaquerizo A, Cañueto J, Llombart B, Martorell-Calatayud A, and Sanmartín O
- Subjects
- Humans, Predictive Value of Tests, Carcinoma, Squamous Cell pathology, Neoplasm Staging methods, Sentinel Lymph Node Biopsy, Skin Neoplasms pathology
- Abstract
Background: Recommendations on when to perform sentinel lymph node (SLN) biopsy in cutaneous squamous cell carcinoma (cSCC) are lacking despite the tumor's clear predilection for lymphatic spread., Objective: To analyze the frequency of SLN metastasis in published series of cSCC in the context of the eighth edition of the American joint Committee on Cancer (AJCC-8) and the Brigham and Women's Hospital (BWH) staging criteria., Methods: Systematic review of studies of patients with cSCC who underwent SLN biopsy that described biopsy results., Results: In total, 153 patients with 24 positive SLN biopsies (15.7%) were included. Based on the AJCC-8 criteria positivity rates in the T2 and T3 categories were 8.3% (1/12 patients) and 25% (8/32), respectively. Using the BWH system there were, 2/33 in category T2a (6.5%), and 5/17 in category T2b (29.8%). On applying the same criteria to tumors of the trunk and extremities the results were similar., Conclusion: It would seem reasonable to recommend SLN biopsy for patients with AJCC-8 Stage T3+ disease or BWH Stage T2b/T3 disease. Both the AJCC-8 and the BWH systems would appear to be useful for staging cSCC of the trunk and extremities.
- Published
- 2020
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30. Oral Contraceptives in Dermatology.
- Author
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Requena C and Llombart B
- Subjects
- Ethinyl Estradiol adverse effects, Female, Humans, Levonorgestrel, Pregnancy, Progesterone, Contraceptives, Oral, Dermatology
- Abstract
Oral contraceptives combine estrogen and progesterone to suppress ovulation. Synthetic forms are usually used. In dermatology, oral contraceptives are prescribed for 2 main reasons: to prevent pregnancy when teratogenic drugs must be taken and to treat skin manifestations of hyperandrogenism. Most oral contraceptives improve both acne and hirsutism, but the androgenic effect of progestogens - particularly if the contraceptive contains first- or second-generation progestogens- can trigger or exacerbate acne. One of the most serious side effects of oral contraceptives, thrombosis, is mainly caused by the estrogen component and its dose. If we mainly consider a contraceptive's thrombotic profile when prescribing, the choice would be to have 30μg or less of ethinyl estradiol combined with levonorgestrel. On the other hand, if our main objective is to treat signs of androgenization, we would prefer contraceptives containing progestogens with antiandrogenic effects., (Copyright © 2020. Publicado por Elsevier España, S.L.U.)
- Published
- 2020
- Full Text
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31. Functions of Dermatology Residents' Supervisors.
- Author
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Medina S, Díaz R, Llombart B, García-Patos V, and Jaén P
- Subjects
- Clinical Competence, Dermatology education, Internship and Residency
- Published
- 2020
- Full Text
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32. Coding and noncoding somatic mutations in candidate genes in basal cell carcinoma.
- Author
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Maturo MG, Rachakonda S, Heidenreich B, Pellegrini C, Srinivas N, Requena C, Serra-Guillen C, Llombart B, Sanmartin O, Guillen C, Di Nardo L, Peris K, Fargnoli MC, Nagore E, and Kumar R
- Subjects
- DNA Methylation, Epigenesis, Genetic, Gene Expression Regulation, Neoplastic, Humans, Promoter Regions, Genetic, Skin Neoplasms genetics, Biomarkers, Tumor, Carcinoma, Basal Cell genetics, Mutation, Open Reading Frames, Untranslated Regions
- Abstract
Basal cell carcinoma (BCC) represents the most commonly diagnosed human cancer among persons of European ancestry with etiology mainly attributed to sun-exposure. In this study we investigated mutations in coding and flanking regions of PTCH1 and TP53 and noncoding alterations in the TERT and DPH3 promoters in 191 BCC tumors. In addition, we measured CpG methylation within the TERT hypermethylated oncological region (THOR) and transcription levels of the reverse transcriptase subunit. We observed mutations in PTCH1 in 58.6% and TP53 in 31.4% of the tumors. Noncoding mutations in TERT and DPH3 promoters were detected in 59.2% and 38.2% of the tumors, respectively. We observed a statistically significant co-occurrence of mutations at the four investigated loci. While PTCH1 mutations tended to associate with decreased patient age at diagnosis; TP53 mutations were associated with light skin color and increased number of nevi; TERT and DPH3 promoter with history of cutaneous neoplasms in BCC patients. Increased reverse transcriptase subunit expression was observed in tumors with TERT promoter mutations and not with THOR methylation. Our study signifies, in addition to the protein altering mutations in the PTCH1 and TP53 genes, the importance of noncoding mutations in BCC, particularly functional alterations in the TERT promoter.
- Published
- 2020
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33. Parotid Fistula After Skin Biopsy: Treatment With Botulinum Toxin.
- Author
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Bancalari B, Llombart B, Requena C, and Vendrell JB
- Subjects
- Aged, Biopsy adverse effects, Cutaneous Fistula etiology, Female, Fistula etiology, Humans, Parotid Diseases etiology, Photography, Sialorrhea drug therapy, Sialorrhea etiology, Botulinum Toxins, Type A therapeutic use, Cutaneous Fistula drug therapy, Fistula drug therapy, Parotid Diseases drug therapy, Parotid Gland pathology
- Published
- 2019
- Full Text
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34. Histologic Changes During Treatment With Vismodegib in Locally Advanced Basal Cell Carcinoma: A Series of 19 Cases.
- Author
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Bancalari B, Llombart B, Serra-Guillén C, Bernia E, Requena C, Nagore E, Traves V, Calomarde L, Diago A, Guillén C, and Sanmartín O
- Subjects
- Adult, Aged, Aged, 80 and over, Carcinoma, Basal Cell pathology, Female, Humans, Male, Middle Aged, Retrospective Studies, Skin Neoplasms pathology, Anilides therapeutic use, Antineoplastic Agents therapeutic use, Carcinoma, Basal Cell drug therapy, Pyridines therapeutic use, Skin Neoplasms drug therapy
- Abstract
Background: There are no large series describing cutaneous histologic changes during treatment with vismodegib in locally advanced basal cell carcinoma (BCC)., Objective: To analyze histologic changes in skin biopsy specimens from patients with locally advanced BCC treated with vismodegib., Methods: A descriptive, retrospective study of patients with locally advanced BCC treated with vismodegib between June 2012 and December 2017 at the Instituto Valenciano de Oncología, Spain. Nineteen patients were biopsied before and during the treatment with vismodegib, and we compared histologic changes observed., Results: Seven patients (37%) achieved complete response, which was characterized by replacement of tumor stroma with a hyaline scar, lymphocytic inflammatory infiltrate, keratin formation, and infundibular cysts. Twelve patients (63%) achieved partial response; 5 showed no phenotypic changes, whereas 7 showed histologic changes; 5 cases showed metatypical differentiation; and 2 cases presented squamous differentiation. We observed no cases of squamous cell carcinoma arising at vismodegib treatment sites and no association between initial histologic subtype and clinical response., Limitations: Many biopsy specimens were obtained by punch biopsy and may not be representative of the full tumors. We studied histologic changes only in complete and partial responses., Conclusion: Vismodegib can induce histologic changes toward metatypical or squamous differentiation of BCC in patients with partial response. Keratinizing phenomena were frequent, both in partial and complete response groups.
- Published
- 2019
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35. Dusky erythema secondary to anti-MEK therapy.
- Author
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Bancalari B, Algarra MA, Llombart B, Nagore E, Soriano V, Sanmartín O, and Requena C
- Subjects
- Humans, Erythema chemically induced, Mitogen-Activated Protein Kinase Kinases adverse effects, Mitogen-Activated Protein Kinase Kinases antagonists & inhibitors, Molecular Targeted Therapy adverse effects
- Published
- 2019
- Full Text
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36. Diagnosis and Treatment of Merkel Cell Carcinoma in Specialized Dermatology Units: A Clinical Practice Guideline of the Spanish Academy of Dermatology and Venereology.
- Author
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Vázquez Doval J, Llombart Cussac B, Pérez Bustillo A, Paradela de la Morena S, Fuente González MJ, Fernández Figueras MT, Villanueva MJ, Rodríguez Salas N, Descalzo-Gallego MÁ, García-Doval I, and Ríos-Buceta L
- Subjects
- Carcinoma, Merkel Cell pathology, Dermatology organization & administration, Evidence-Based Medicine, Hospital Departments, Hospital Units, Humans, Neoplasm Staging, Skin Neoplasms pathology, Spain, Carcinoma, Merkel Cell diagnosis, Carcinoma, Merkel Cell therapy, Skin Neoplasms diagnosis, Skin Neoplasms therapy
- Abstract
Background and Objective: Merkel cell carcinoma is a rare, aggressive skin cancer that is managed in a great variety of ways. However, international clinical practice guidelines give only partial coverage to issues considered major problems.The recommendations presented here aim to provide Spanish dermatologists with a guide to improving disputed aspects of diagnosis, staging, and treatment of localized Merkel cell carcinomas., Material and Methods: The ADAPTE process was used. Members of the Spanish Group of Oncologic Dermatology and Surgery (GEDOC) with experience in treating Merkel cell carcinoma and interest in drafting these guidelines were selected. The group described the care process and listed the most important clinical questions. They then searched for guidelines and assessed them with the AGREE II (Appraisal of Guidelines for Research and Evaluation) tool. After consulting the guidelines for answers to their clinical questions, the group drafted the present statementand sent it for external review., Results: The guidelines that scored highest in the AGREE II assessment step were the consensus-based interdisciplinary guideline of the European Association of Dermato-Oncology and the European Organization of Research and Treatment of Cancer, and those of the Comprehensive Cancer Network, the Alberta Health Services in Canada, the American Cancer Society, and the Cutaneous Oncology Group of the French Society of Dermatology. A total of 9 clinical questions were answered based on these guidelines., Conclusions: The guidelines presented here answer clinical questions that arise in routine practice. They can provide dermatologists with a starting point for decision-making, although available resources and patient preferences must always be borne in mind., (Copyright © 2019 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2019
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37. The Rising Incidence of Merkel Cell Carcinoma.
- Author
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Llombart B
- Subjects
- Humans, Incidence, Carcinoma, Merkel Cell epidemiology, Skin Neoplasms epidemiology
- Published
- 2019
- Full Text
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38. Multiple facial plaque variant of trichoblastoma.
- Author
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Requena C, Requena L, Kazakov DV, Traves V, Nagore E, Llombart B, Serra C, Guillén C, and Sanmartín O
- Subjects
- Aged, Face pathology, Female, Hair Diseases surgery, Humans, Male, Middle Aged, Mohs Surgery, Skin Neoplasms surgery, Hair Diseases pathology, Hair Follicle pathology, Skin Neoplasms pathology
- Abstract
Background: The plaque variant of trichoblastoma has been described as a solitary tumor with diffuse infiltration of the lower dermis and hypodermis, with poorly defined borders. Herein, we report a new variant of multiple centrofacial trichoblastoma., Object: To describe clinical and pathological features of a new multiple kind of plaque variant of centrofacial trichoblastoma., Methods: Case series of patients with a multiple-plaque variant of centrofacial trichoblastoma treated in our department between 2005 and 2017. We identified eight patients with the centrofacial plaque variant of trichoblastoma treated in our department from 2005 to 2017., Results: The final study sample comprised 13 trichoblastomas from four patients. All patients also developed at least one basal cell carcinoma. Mohs surgery was the method of treatment in the majority of the cases of trichoblastoma and in all the cases of basal cell carcinoma. We needed between 2 and 6 stages to obtain free margins in our cases of facial plaque trichoblastomas treated by Mohs surgery., Conclusion: To the best of our knowledge, a multiple-plaque variant of trichoblastoma has not been described in the literature. We suggest a genetic origin of this variant of trichoblastoma and describe its remarkable infiltrative nature, with poorly defined surgical margins., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
- Published
- 2019
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39. Postirradiation Morphea in Patients With Breast Cancer: Possible Association With Other Autoimmune Diseases.
- Author
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Diago A, Llombart B, Requena C, Sanmartín O, and Guillén C
- Subjects
- Aged, Female, Humans, Middle Aged, Autoimmune Diseases complications, Breast Neoplasms radiotherapy, Radiation Injuries complications, Scleroderma, Localized etiology
- Abstract
Postirradiation morphea is an uncommon entity that has been mostly described in women with breast cancer. The increasing use of radiotherapy to treat breast cancer and the clinical similarities between morphea and other conditions, such as radiodermatitis, postirradiation fibrosis, and tumor recurrence, highlights the need for dermatologists to be familiar with this entity. We present a series of 6 women with a mean age of 64.2 years and a mean latency of 9.5 years between radiotherapy for breast cancer and onset of morphea. Four of the patients had a history of autoimmune disease: rheumatoid arthritis, Sjögren syndrome, vitiligo, and Crohn disease. No specific risk factors for postirradiation morphea have been identified to date, although it would appear that a history of autoimmune disease could be associated with an increased risk of morphea in patients treated with radiation therapy., (Copyright © 2018 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2019
- Full Text
- View/download PDF
40. Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment.
- Author
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Llombart B, Serra-Guillén C, Requena C, Alsina M, Morgado-Carrasco D, Machado I, and Sanmartín O
- Subjects
- Algorithms, Humans, Practice Guidelines as Topic, Leiomyosarcoma diagnosis, Leiomyosarcoma therapy, Sarcoma diagnosis, Sarcoma therapy, Skin Neoplasms diagnosis, Skin Neoplasms therapy
- Abstract
There are 3 types of leiomyosarcoma of the skin: dermal, subcutaneous, and metastatic cutaneous. Dermal leiomyosarcoma arises from smooth muscle fibers in arrector pili muscles, genital dartos muscles, and the nipple-areola complex. It is an intermediate-grade tumor associated with a tendency for local recurrence (24%) and low metastatic potential (4%). Subcutaneous leiomyosarcoma originates from smooth muscle in blood vessel walls and has higher rates of local recurrence (37%) and metastasis (43%). Plemorphic dermal sarcoma typically affects elderly patients and arises in sun-exposed areas (e.g., the scalp). Its histologic and immunohistochemical characteristics are similar to those of atypical fibroxanthoma, but it is more aggressive (metastasis rate of 10-20%). Histologically, it can be distinguished from atypical fibroxanthoma by the observation of subcutaneous tissue invasion, perineural invasion, and foci of necrosis., (Copyright © 2018 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2019
- Full Text
- View/download PDF
41. Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.
- Author
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Requena C, Alsina M, Morgado-Carrasco D, Cruz J, Sanmartín O, Serra-Guillén C, and Llombart B
- Subjects
- AIDS-Related Opportunistic Infections diagnosis, AIDS-Related Opportunistic Infections therapy, Age Distribution, Combined Modality Therapy, Female, Hemangiosarcoma pathology, Herpesvirus 8, Human isolation & purification, Humans, Lymphangiosarcoma diagnosis, Lymphangiosarcoma pathology, Lymphangiosarcoma therapy, Male, Neoplasms, Second Primary diagnosis, Neoplasms, Second Primary etiology, Neoplasms, Second Primary therapy, Radiotherapy adverse effects, Sarcoma, Kaposi classification, Sarcoma, Kaposi virology, Skin Neoplasms pathology, Skin Neoplasms virology, Hemangiosarcoma diagnosis, Hemangiosarcoma therapy, Practice Guidelines as Topic, Sarcoma, Kaposi diagnosis, Sarcoma, Kaposi therapy, Skin Neoplasms diagnosis, Skin Neoplasms therapy
- Abstract
Kaposi sarcoma is a vascular sarcoma with 4 clinical variants: classic Kaposi sarcoma, which mainly affect the extremities of elderly patients and follows a chronic, generally indolent course; African Kaposi sarcoma; immunosuppression-associated Kaposi sarcoma; and AIDS-associated Kaposi sarcoma. Type8 human herpesvirus is the etiologic agent in all 4variants. Cutaneous angiosarcoma is a cutaneous neoplasm with a very poor prognosis. It carries a high probability of local relapse and has a 10% to 15% survival rate at 5years. There are 3 main variants of cutaneous angiosarcoma: idiopathic angiosarcoma of the face and scalp; Stewart-Treves syndrome; and postradiation angiosarcoma. The only potentially curative treatment is surgery with or without radiotherapy. However, its indistinct borders and multicentric nature mean that treatment is often palliative with chemotherapy, radiotherapy, or both., (Copyright © 2018 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2018
- Full Text
- View/download PDF
42. A randomized intraindividual comparative study of methyl-5-aminolaevulinate vs. 5-aminolaevulinic acid nanoemulsion (BF-200 ALA) in photodynamic therapy for actinic keratosis of the face and scalp.
- Author
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Serra-Guillén C, Nagore E, Bancalari E, Kindem S, Sanmartín O, Llombart B, Requena C, Serra-Guillén I, Calomarde L, Diago A, Bernia E, and Guillén C
- Subjects
- Administration, Cutaneous, Aged, Aminolevulinic Acid administration & dosage, Aminolevulinic Acid adverse effects, Erythema diagnosis, Erythema etiology, Face, Female, Humans, Keratosis, Actinic pathology, Male, Pain diagnosis, Pain etiology, Patient Satisfaction, Photochemotherapy adverse effects, Photosensitizing Agents adverse effects, Prospective Studies, Scalp, Skin drug effects, Skin pathology, Skin radiation effects, Treatment Outcome, Visual Analog Scale, Aminolevulinic Acid analogs & derivatives, Keratosis, Actinic drug therapy, Photochemotherapy methods, Photosensitizing Agents administration & dosage
- Published
- 2018
- Full Text
- View/download PDF
43. Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans.
- Author
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Llombart B, Serra C, Requena C, Alsina M, Morgado-Carrasco D, Través V, and Sanmartín O
- Subjects
- Antineoplastic Agents therapeutic use, Combined Modality Therapy, Dermatofibrosarcoma genetics, Dermatofibrosarcoma pathology, Humans, Imatinib Mesylate therapeutic use, Mohs Surgery, Neoplasm Grading, Neoplasm Staging, Oncogene Proteins, Fusion genetics, Radiotherapy, Adjuvant, Sarcoma classification, Sarcoma diagnosis, Sarcoma pathology, Sarcoma therapy, Skin Neoplasms genetics, Skin Neoplasms pathology, Dermatofibrosarcoma diagnosis, Dermatofibrosarcoma therapy, Practice Guidelines as Topic, Skin Neoplasms diagnosis, Skin Neoplasms therapy
- Abstract
Sarcomas comprise a broad group of tumors, many of whose biological behavior and aggressiveness differ from one type to another. The therapeutic approach is generally multidisciplinary and often complex. Developments in surgical and oncological dermatology during the last few decades have positioned dermatologists as specialists in the diagnosis and treatment of skin cancer. The aim of this article is to review the main soft tissue sarcomas that typically affect the skin. Dermatofibrosarcoma protuberans is a low-grade malignant sarcoma. It exhibits slow-growth, is locally invasive, and has low metastatic potential (<3%). Mohs micrographic surgery is the treatment of choice. The COL1A1-PDGFB translocation should be analyzed in cases of unclear diagnosis and when it is necessary to identify candidates for tyrosine kinase inhibitors. Imatinib is indicated for the treatment of locally advanced and metastatic dermatofibrosarcoma protuberans., (Copyright © 2018 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2018
- Full Text
- View/download PDF
44. Experience With Vismodegib in the Treatment of Advanced Basal Cell Carcinoma at a Cancer Center.
- Author
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Bernia E, Llombart B, Serra-Guillén C, Bancalari B, Nagore E, Requena C, Calomarde L, Diago A, Lavernia J, Traves V, Guillén C, and Sanmartín O
- Subjects
- Adult, Aged, Aged, 80 and over, Carcinoma, Basal Cell pathology, Female, Humans, Male, Middle Aged, Neoplasm Staging, Retrospective Studies, Skin Neoplasms pathology, Anilides therapeutic use, Carcinoma, Basal Cell drug therapy, Pyridines therapeutic use, Skin Neoplasms drug therapy
- Abstract
Introduction and Objectives: Vismodegib is the first selective Hedgehog inhibitor approved for the treatment of locally advanced and metastatic basal cell carcinoma (BCC). In this article, we describe our experience with the use of this drug to treat advanced and/or multiple BCCs at a cancer center over 5 years., Material and Methods: We analyzed the following variables: patient age and sex; tumor location, size, type, and characteristics; time since onset; primary or recurrent status; duration of treatment; response to treatment (complete, partial, stabilization, or absence of response); adverse effects; and recurrences., Results: We treated 22 patients, of whom 20 had locally advanced BCCs and 2 had metastatic BCCs with lymph node involvement. The treatment was administered over a mean of 11.8 months. Nine patients (41%) achieved complete response and 10 (45%) partial response. The disease was stabilized in 3 (14%). Two patients relapsed after a median of 21 months. The main adverse effects were dysgeusia, alopecia, and muscle cramps, all of which were mild. None of the patients developed squamous cell carcinoma in an area treated with vismodegib, although metatypical changes were observed after treatment., Conclusions: With a response rate of 96%, vismodegib is a safe and effective treatment for locally advanced BCC. Adverse effects are generally mild but they need to be taken into account owing to their high frequency., (Copyright © 2018 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2018
- Full Text
- View/download PDF
45. Systematic review of the prevalence of nodal metastases and the prognostic utility of sentinel lymph node biopsy in cutaneous squamous cell carcinoma.
- Author
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Tejera-Vaquerizo A, García-Doval I, Llombart B, Cañueto J, Martorell-Calatayud A, Descalzo-Gallego MA, and Sanmartín O
- Subjects
- Carcinoma, Squamous Cell pathology, Carcinoma, Squamous Cell secondary, Humans, Lymphatic Metastasis, Neoplasm Recurrence, Local pathology, Prevalence, Prognosis, Risk Factors, Skin Neoplasms pathology, Carcinoma, Squamous Cell epidemiology, Lymph Nodes pathology, Neoplasm Recurrence, Local epidemiology, Sentinel Lymph Node Biopsy statistics & numerical data, Skin Neoplasms epidemiology
- Abstract
Cutaneous squamous cell carcinoma (cSCC) is the second most common skin cancer and its incidence is rising. The prognosis is mostly good but patients with high-risk cSCC have a greater risk of recurrence and death. The aim of the present study was to conduct a systematic review analyzing the prevalence, predictors and prognostic utility of sentinel lymph node (SLN) involvement in cSCC. We performed a published work search in Ovid MEDLINE and reviewed the reference lists of selected studies. Based on the 23 studies included in the systematic review, the proportion of patients with cSCC and positive SLN biopsy findings was 8% (95% confidence interval, 5.1-10.8%; I
2 = 44.51%). We found no studies reporting on predictors of SLN involvement in cSCC or on the prognostic utility of this finding following adjustment for confounders. The rate of positive SLN in cSCC is less than previously reported. Criteria for recommending SLN biopsy as a staging tool for cSCC vary considerably from study to study, and none of the studies were large enough to reliably identify predictors of SLN positivity. No randomized controlled trials have yet analyzed whether SLN biopsy may improve the prognosis of cSCC. More studies are required on the prognostic value of SLN positivity and the associated risk factors in cSCC., (© 2018 Japanese Dermatological Association.)- Published
- 2018
- Full Text
- View/download PDF
46. Cutaneous metastatic breast cancer simulating angiosarcoma on the face and neck of a woman.
- Author
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Calomarde-Rees L, Llombart-Cussac B, Requena-Caballero C, Traves-Zapata V, Bancalari-Simon B, and Guillén-Barona C
- Subjects
- Diagnosis, Differential, Female, Humans, Middle Aged, Breast Neoplasms pathology, Head and Neck Neoplasms diagnosis, Head and Neck Neoplasms secondary, Hemangiosarcoma diagnosis, Skin Neoplasms diagnosis, Skin Neoplasms secondary
- Published
- 2018
- Full Text
- View/download PDF
47. Kutan metastasiertes Mammakarzinom bei einer Frau simuliert ein Angiosarkom am Gesicht und Hals.
- Author
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Calomarde-Rees L, Llombart-Cussac B, Requena-Caballero C, Traves-Zapata V, Bancalari-Simon B, and Guillén-Barona C
- Published
- 2018
- Full Text
- View/download PDF
48. Immunohistochemical and Fluorescence In Situ Hybridization Analysis of MYC in a Series of 17 Cutaneous Angiosarcomas: A Single-Center Study.
- Author
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Requena C, Rubio L, Lavernia J, Machado I, Llombart B, Sanmartín O, Traves V, Guillén C, and Cruz J
- Subjects
- Adult, Aged, Aged, 80 and over, Female, Gene Amplification, Hemangiosarcoma mortality, Hemangiosarcoma pathology, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Male, Middle Aged, Skin Neoplasms mortality, Skin Neoplasms pathology, Hemangiosarcoma genetics, Neoplasms, Radiation-Induced genetics, Proto-Oncogene Proteins c-myc genetics, Skin Neoplasms genetics
- Abstract
Cutaneous angiosarcoma (AS) is an uncommon, aggressive sarcoma whose incidence is rising because of the increasing use of radiation therapy, especially in breast cancer. The few studies on the relevance of prognostic factors, such as MYC status in cutaneous AS, have reported inconclusive findings, with some authors reporting MYC amplification only in postirradiation and lymphedema-associated AS and others reporting evidence of MYC amplification in idiopathic AS. We analyzed 17 cases of cutaneous AS (6 idiopathic AS, 10 postirradiation AS, and 1 lymphedema-associated AS) treated at our institute between 2000 and 2015. Follow-up data were available in all cases. We compared the presence/absence of MYC amplification by fluorescence in situ hybridization (FISH) and immunohistochemical (IHC) MYC overexpression in the different AS subtypes. We also investigated potential associations between MYC amplification and prognosis. MYC amplification was observed by FISH in 6 of 14 informative cases. The positive cases were all secondary AS (5 postirradiation AS and 1 lymphedema-associated AS). IHC detected MYC overexpression in 8 of 15 informative cases (7 secondary AS and 1 idiopathic AS). In conclusion, MYC amplification and MYC overexpression were detected almost exclusively in secondary AS. No associations were found between MYC amplification/overexpression and prognosis. We found MYC amplification or overexpression in a similar proportion of the patients who died and who were still alive at the end of the study. In the group of 9 patients who died, MYC was detected by FISH in 4 cases and by IHC in 5. The corresponding figures in the group of 6 patients still alive were 2 by FISH and 3 by IHC.
- Published
- 2018
- Full Text
- View/download PDF
49. A 12-Day Course of Imiquimod 5% for the Treatment of Actinic Keratosis: Effectiveness and Local Reactions.
- Author
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Serra-Guillén C, Nagore E, Llombart B, Sanmartín O, Requena C, Calomarde L, and Guillén C
- Subjects
- Aged, Aged, 80 and over, Female, Humans, Imiquimod adverse effects, Interferon Inducers adverse effects, Male, Time Factors, Treatment Outcome, Imiquimod administration & dosage, Interferon Inducers administration & dosage, Keratosis, Actinic drug therapy
- Abstract
Introduction and Objectives: Imiquimod is an excellent option for patients with actinic keratosis, although its use may be limited by the long course of treatment required (4 weeks) and the likelihood of local skin reactions. The objectives of the present study were to demonstrate the effectiveness of a 12-day course of imiquimod 5% for the treatment of actinic keratosis and to examine the association between treatment effectiveness and severity of local reactions., Patients and Methods: We included patients with at least 8 actinic keratoses treated with imiquimod 5% cream for 12 consecutive days. Local reactions were classified as mild, moderate, or severe. The statistical analysis of the association between local reactions and clinical response was based on the Pearson χ
2 test and the Spearman rank correlation test., Results: Sixty-five patients completed the study. Complete response was recorded in 52.3% and partial response in 75.4%. We found a statistically significant association between severity of the local reaction and response to treatment in both the Pearson χ2 test and the Spearman rank correlation test., Conclusions: A 12-day course of imiquimod 5% proved effective for the treatment of actinic keratosis. Severity of local reactions during treatment was correlated with clinical response., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)- Published
- 2018
- Full Text
- View/download PDF
50. Cutaneous Leiomyosarcoma: On the Importance of Histologic Location.
- Author
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Llombart B
- Subjects
- Humans, Prognosis, Leiomyosarcoma, Skin Neoplasms
- Published
- 2018
- Full Text
- View/download PDF
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