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Introduction: Metanephric adenoma (MA) is an uncommon benign tumor accounting for 0.2–0.7% of adult renal epithelial neoplasms. The clinical course is often indolent, but diagnosis should not be delayed since clinical symptoms (hematuria, fever, palpable abdominal mass, and flank pain) may be non-specific and overlap with those of a malign renal neoplasm. We report on 4 cases of AM, for which morphological and mutational analysis were performed. Material and Methods: Immunohistochemical staining was performed on sections cut from paraffin blocks to assess expression of WT1, vimentin, racemase, CK7, CD10 and RCC. Testing for the BRAF gene mutation V600 was carried out using real-time PCR (Cobas® 4800). Results: In all four cases, tumors were visible as well-circumscribed, non encapsulated masses located in the renal cortex and extending towards the medulla. At immunohistochemical examination, tumor cells stained negative for CK7, CD10 and RCC and positive for both WT1 (nuclear, intense) and vimentin (cytoplasmic, intense, and diffuse). Molecular analysis revealed the BRAF gene mutation V600E in three cases and wild-type BRAF in the fourth. Conclusions: BRAF molecular mutation analysis may aid diagnosis in cases with atypical histological features, especially in small incisional biopsies when reassessment of surgical treatment may be considered.

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, This study has been developed with GETHI group (Spanish group of orphan and infrequent tumours, from the Spanish Grupo Espanol ˜ de Tumores Hu´erfanos e Infrecuentes) as promotor. This research was financially supported by Merck KGaA, Darmstadt, Germany, as part of an alliance between Merck KGaA and Pfizer. Merck KGaA, Darmstadt, Germany and Pfizer reviewed the manuscript for medical accuracy only before journal submission. The authors are fully responsible for the content of this manuscript, and the views and opinions described in the publication reflect solely those of the authors

Objective: High-grade squamous intraepithelial lesions (HSIL) are the precursors of invasive cervical carcinomas and are generally associated with the integration of mucosotropic human papillomavirus (HPV) DNA into the host cell genome. Detection of HPV is easy to perform nowadays, even in laboratories with limited technological capacity, and follow-up procedures for patients with HSIL are well established. Study Design: HPV detection was performed in a large group of patients with HSIL, and results were correlated with cytological, histological, and colposcopic findings. Discrepancies were examined and discussed. Results: Conventional Papanicolaou (Pap) screening detected 446 HSIL (0.20%) in 218,906 cervical smears. HPV detection by PCR was positive in 339/358 (94.7%) patients. The strains involved were: HPV 16 in 180 patients (53.1%), HPV 18 in 35 (10.3%), HPV 31/33 in 27 (8%), HPV 6/11 in 10 (2.96%), and an unidentified type in 73 (30%). For the last 97 patients (2006–2007), HPV typing was expanded with the following results: HPV 52 was detected in 9 patients (9.2%), HPV 58 in 6 (6.1%), HPV 51 in 4 (4.1%), HPV 68 in 2 (2.0%), and HPV 39 in 1 (1.0%). The number of nonidentified patients dropped to 9 (9.4%); in addition, 14/97 (14.4%) patients were infected with 2 or more viral types. Finally, 19 (5.3%) patients were HPV negative. Colposcopy revealed minor changes in 59 patients (17.3%), major changes in 264 (77.6%), and normal findings in 17 (5.1%). A biopsy was taken in 331/446 patients, and the diagnosis of HSIL or overt malignancy was histologically confirmed in 281 (84.9%) patients: CIN II in 46, CIN III in 224, and histologically upgraded in 11 (6 microinvasive squamous carcinomas, 1 squamous carcinoma, 2 in situ endocervical adenocarcinomas, and 2 microinvasive endocervical adenocarcinomas). Thirty-five patients (10.6%) were downgraded to CIN I and 15 (4.5%) patients had a negative biopsy. Follow-up in the negative-biopsy patients confirmed the existence of SIL in 11 patients [1 HSIL and 10 low-grade squamous intraepithelial lesions (LSIL)] while 4 were considered false positives (atrophic changes, 2; reactive changes, 2). After treatment, 31/331 (9.36%) patients displayed recurrence (HSIL in 29 and LSIL in 2). The viral strains involved in patients with recurrence were HPV 16 in 16 patients (51.6%); HPV 18 in 4 (12.9%); HPV 16 and 18 in 1 (3.2%); HPV 31 in 1 (3.2%); HPV 52 in 1 (3.2%); HPV 18, 31, and 58 in 1 (3.2%); HPV 68 in 1 (3.2%); HPV 51 and 73 in 1 (3.2%), and an unidentified type in 5 (16.1%). Follow-up in 14/19 HSIL and HPV-negative patients confirmed the existence of cervical pathology. Conclusions: HPV detection improves diagnostic sensitivity and provides an ideal tool for monitoring the response to treatment in HSIL patients. The pathogenic relevance of HPV strain 18 may be greater than previously assumed.

Case report The Leishmania genus is formed by parasitic protozoa which are transmitted by the bite of infected female sand flies. Cases of sexual, vertical or transfusional transmission or via infected needles have also been described. In humans, 4 forms of this disease have been described: localised cutaneous (LC), diffuse cutaneous, mucocutaneous and visceral (1). LC counts for 50–75% of all cases (2), it is the mildest form of the disease and can be caused by any species of Leishmania. In Spain, the most frequent form is the oriental sore caused by L. infantum (2). Most cases resolve spontaneously within one year. In United States and Europe, the incidence is increasing due to tourism and co-infection with HIV. The morphological spectrum of LC is very wide; multiple forms of clinical presentation have been described, although the most characteristic one is the nodular ulcerative lesion, characterised by painless crater-like ulcers with a necrotic base and covered by an adhesive crust. The main complication of LC is its progression in some strains towards the other 3 forms of the disease (3). In patients with AIDS and other diseases associated with immunosuppression the risk of dissemination is much higher than in the immunocompetent. We present a case of LC with clinical and histopathological features similar to a pyogenic granuloma. Yes

Case Reports; Journal Article; Review; Superficial Acral Fibromyxoma is a rare tumor of soft tissues. It is a relatively new entity described in 2001 by Fetsch et al. It probably represents a fibrohistiocytic tumor with less than 170 described cases. We bring a new case of SAF on the 5th toe of the right foot, in a 43-year-old woman. After surgical excision with safety margins which included the nail apparatus, it has not recurred (22 months of follow up). We carried out a review of the location of all SAF published up to the present day. Yes

Resumen El hemangioendotelioma retiforme (HR) es una neoplasia vascular rara y con rasgos morfologicos distintivos que por su elevada tasa de recidivas locales y ocasionales metastasis fue descrita inicialmente como angiosarcoma de bajo grado de malignidad. En la literatura revisada hemos encontrado un total de 23 casos bien documentados. Describimos dos nuevos casos de HR y realizamos una revision de la literatura. Una paciente de 82 anos se presento con una placa indurada, mal definida y de crecimiento lento, situada en el dorso de la mano derecha; la segunda paciente tenia 31 anos y consulto por una lesion en la planta del pie. En el estudio histologico ambas lesiones presentaban las caracteristicas propias del HR con una proliferacion dermohipodermica infiltrativa de canales vasculares elongados, arborescentes de paredes finas, tapizados por celulas endoteliales con morfologia en tachuela y sin atipia ni figuras de mitosis. El diagnostico diferencial se establece fundamentalmente con el tumor de Dabska, el hemangioma en tachuela y el angiosarcoma convencional. La aportacion de nuevos casos de este infrecuente tumor vascular puede ayudar a definir con mayor precision el espectro clinico y morfologico del HR.

Apoptosis may play a major role in determining tumor growth and aggressiveness. The aim of this study was to examine the relationship between apoptosis, expression of bcl-2 and p53 proteins, proliferation index, and other clinicopathological features of breast carcinoma. Sixty-five formalin-fixed paraffin-embedded tissue sections from invasive ductal breast carcinomas were studied for the presence of apoptosis by the terminaldeoxynucleotidyl-transferase-mediated dUTP-FITC nick end-labeling (TUNEL) method. Immunohistochemical methods were also used to determine the expression of estrogen receptor, Ki67, bcl-2 and p53 proteins. The number of apoptotic cells ranged from 2.0 to 236.0/10HPF (mean 36.26, median 28.0). The observation of 30 apoptotic cells/10HPF was more common in tumors > 3 cm, of histological grade III, with a high mitotic index, Ki67 index > or = 300, and p53 positivity; however, statistical significance was found only for the histological grade. Grade I and III tumors displayed an inverse association between the apoptotic index and bcl-2 and p53 protein expressions; grade I tumors frequently expressed bcl-2 (19/28), lacked p53 (20/28), and presented a low number of apoptotic cells (18/28), whereas grade III tumors tended to express p53 (12/17), lacked bcl-2 (13/17), and displayed a high number of apoptotic cells/10HPF (12/17). Multivariate analysis for survival revealed that estrogen receptors and apoptosis were independent variables. These data suggest that apoptosis, rather than proliferation index or expression of bcl-2 or p53 proteins, is an independent factor for the prognosis of survival.

To identify markers of response to therapy in neuroblastic tumors.A total of 58 patients with neuroblastic tumor (38 neuroblastomas, 13 ganglioneuroblastomas and 7 ganglioneuromas) were included in the study. TP53, BCL-2, p21Waf1/Cip1 and metallothionein were included as a biologic approach to tumor differentiation, response to therapy and prognosis.Patients who died of disease had the following immunophenotype: BCL-2 (9 of 10), nuclear TP53 (7 of 10) and metallothionein (7 of 10). TP-53 expression was related to clinical stage (p = 0.062) and disease outcome (p = 0.0218). All patients in whom treatment failed expressed metallothionein (3 of 3).TP53, BCL-2, p21Waf1/Cip1 and metallothionein had limited value reflecting tumor maturation (differentiation) or predicting response to therapy. Only nuclear TP53 accumulation may be relevant in patient's prognosis.

Myofibrosarcoma is a controversial neoplasm composed of cells with differentiation toward myofibroblasts. The authors report an unusual case of myofibrosarcoma in which, in addition to the characteristic features reported, tumor cells contained intracytoplasmic hyaline (fibroma-like) inclusion bodies. A 66-year-old man complained of a painless enlarged mass in his right shoulder over the previous 6 months. The tumor recurred 2 years after operation. On histologic examination, the tumor displayed diffusely infiltrative growth with isolation of individual skeletal muscle fibers. The tumor cells were most often arranged in an intersecting (herringbone) fascicular pattern but a vaguely storiform pattern was also observed. Cellularity varied from one area to another. The cells were spindle-shaped, with ill-define pale eosinophilic cytoplasm, and a wavy or tapering nucleus with fine chromatin and small nucleoli. Intracytoplasmic hyaline inclusion bodies of variable size, often located adjacent to the nucleus, were visible in numerous spindle and stellate cells. The mitotic index was 3/10 HPF. The tumor was scored as grade 1. Immunohistochemistry revealed positive staining to muscle-specific actin, desmin and vimentin in most tumor cells. Intracytoplasmic hyaline inclusion bodies did not react specifically to any one antibody, but showed a marked ring-like immune reaction, particularly to muscle-specific actin. Electron microscopy showed tumor cells with indented nuclei and small nucleoli, abundant rough endoplasmic reticulum, micropinocytotic vesicles, and longitudinally arranged fine filaments with focal electron-dense patches and subplasmalemmal plaques. The most striking feature was the presence of large, globular or ball-like, non-membrane-bound, randomly scattered clusters of fine filament, usually adjacent to the nucleus.