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Background: Large prospective studies on the safety of Mohs micrographic (MMS) surgery are scarce, and most focus on a single type of surgical adverse event. Mid-term scar alterations and functional loss have not been described. Objectives: To describe the risk of MMS complications and the risk factors for them. Methods: A nationwide prospective cohort collected all adverse events on consecutive patients in 22 specialised centres. We used multilevel mixed-effects logistic regression to find out factors associated with adverse events. Results: 5,017 patients were included, with 14,421 patient-years of follow-up. 7.0% had some perioperative morbidity and 6.5% had mid-term and scar-related complications. The overall risk of complications was mainly associated with use of antiaggregant/anticoagulant and larger tumours, affecting deeper structures, not reaching a tumour-free border, and requiring complex repair. Age and outpatient setting were not linked to the incidence of adverse events. Risk factors for haemorrhage (0.9%) were therapy with antiaggregant/anticoagulants, tumour size, duration of surgery, and unfinished surgery. Wound necrosis (1.9%) and dehiscence (1.0%) were associated with larger defects and complex closures. Immunosuppression was only associated with an increased risk of necrosis. Surgeries reaching deeper structures, larger tumours and previous surgical treatments were associated with wound infection (0.9%). Aesthetic scar alterations (5.4%) were more common in younger patients, with larger tumours, in H-area, and in flap and complex closures. Risk factors for functional scar alterations (1.7%) were the need for general anaesthesia, larger tumours that had received previous surgery, and flaps or complex closures. Conclusions: MMS shows a low risk of complications. Most of the risk factors for complications were related to tumour size and depth, and the resulting need for complex surgery. Antiaggregant/anticoagulant intake was associated with a small increase in the risk of haemorrhage, that probably does not justify withdrawal. Age and outpatient setting were not linked to the risk of adverse events.

Resumen: Sonidegib es un inhibidor del receptor transmembrana Smoothened (SMO), de la vía de señalización de Hedgehog, indicado para el tratamiento del carcinoma basocelular localmente avanzado (CBCla), no susceptible a cirugía curativa ni a radioterapia. Sonidegib ha demostrado su eficacia y seguridad en pacientes con CBCla en el ensayo de fase II (BOLT), donde el 61% (IC 95%: 48; 72) de los pacientes tratados con 200 mg de sonidegib tuvo una respuesta objetiva al tratamiento, con un tiempo medio hasta la respuesta de cuatro meses. La mediana de duración de respuesta fue de 26,1 meses y la mediana de supervivencia libre de progresión fue de 22,1 meses. Los eventos adversos más frecuentes fueron los espasmos musculares (54,4%), alopecia (49,4%) y disgeusia (44,3%), siendo principalmente de grado 1 y 2. Esta revisión proporciona un resumen sobre la eficacia, seguridad y tolerabilidad de sonidegib, así como consideraciones sobre su uso en el manejo de pacientes con CBCla. 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.

Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. It also has a wide variety of causes, including thrombi, which we recently addressed in partI of this review. In this second part, we look at additional causes of vascular occlusion.

Familiarity with predictors of more aggressive behavior is crucial to the management of basal cell carcinoma (BCC). Risk factors for aggressive BCC are essentially divided into clinical and histopathologic factors. In this review we examine histopathologic features predictive of aggressiveness in BCC. The morpheaform, infiltrative, micronodular, metatypical, and basosquamous subtypes and BCC with sarcomatoid differentiation are classically considered predictive of aggressive behavior. However, 2 other features associated with aggressive BCC are perineural invasion (invasion of nerves below the dermis or nerves larger than 0.1mm in caliber) and subgaleal extension. While the former is well known and widely described in the literature, the latter is not generally recognized as a risk factor, even though it is predictive of highly aggressive behavior. In this review, we draw attention to its importance.

Familiarity with predictors of more aggressive behavior is crucial to the management of basal cell carcinoma (BCC). Risk factors for aggressive BCC are essentially divided into clinical and histopathologic factors. In this review we examine histopathologic features predictive of aggressiveness in BCC. The morpheaform, infiltrative, micronodular, metatypical, and basosquamous subtypes and BCC with sarcomatoid differentiation are classically considered predictive of aggressive behavior. However, 2 other features associated with aggressive BCC are perineural invasion (invasion of nerves below the dermis or nerves larger than 0.1mm in caliber) and subgaleal extension. While the former is well known and widely described in the literature, the latter is not generally recognized as a risk factor, even though it is predictive of highly aggressive behavior. In this review, we draw attention to its importance.

Cutaneous squamous cell carcinoma is sometimes characterized by an increased risk of locoregional recurrence and occasionally distant metastasis. Several clinical and pathological factors, including perineural invasion, have been shown to have prognostic value in this setting. Perineural invasion, that is, the spread of tumor cells into the space surrounding a nerve, is usually an incidental finding. In the presence of symptoms or radiographic evidence of perineural spread, the diagnosis is clinical perineural invasion, which is associated with an increased risk of local recurrence and mortality. (C) 2018 AEDV. Published by Elsevier Espana, S.L.U. All rights reserved.

Resumen En ocasiones el carcinoma epidermoide cutaneo se caracteriza por tener un mayor riesgo de desarrollar recurrencia locorregional y ocasionalmente metastasis a distancia. Existen diversos factores clinico-patologicos de reconocido valor pronostico, entre ellos la presencia de infiltracion perineural. Esta consiste en la diseminacion de celulas tumorales a traves de los nervios, siendo en la mayoria de los casos un hallazgo incidental. Cuando aparecen sintomas y/o evidencia radiologica de la extension de la infiltracion perineural, se clasifica como infiltracion perineural clinica y se asocia a un mayor riesgo de recurrencia local y de mortalidad.

Resumen La morfea postradioterapia es una entidad poco frecuente descrita mayoritariamente en mujeres con cancer de mama. El uso creciente de la radioterapia en el tratamiento del cancer de mama, asi como la similitud clinica de la morfea con otros cuadros tales como la radiodermitis, la fibrosis postradiacion o la recidiva tumoral, obligan al dermatologo al conocimiento de esta entidad. Presentamos una serie de 6 mujeres, con una media de edad de 64,2 anos y un tiempo medio de latencia entre la radioterapia y la morfea de 9,5 anos. Cuatro de las 6 pacientes presentaban algun trastorno de base autoinmune (artritis reumatoide, sindrome de Sjogren, vitiligo y enfermedad de Crohn) entre sus antecedentes. Hasta el momento actual no se han encontrado factores de riesgo que predispongan al desarrollo de morfea tras el tratamiento con radioterapia, si bien parece que presentar algun trastorno de base autoinmune puede condicionar la aparicion de morfea en pacientes tratados con radioterapia.

Resumen El leiomiosarcoma de la piel se clasifica en tres grupos: dermico, hipodermico y cutaneo metastasico. El dermico se origina de las fibras musculares lisas del musculo erector del pelo, dartos genital o de la areola mamaria. Se considera un tumor de malignidad intermedia, con tendencia a la recidiva local (24%) y un bajo riesgo de metastasis (4%). El leiomiosarcoma hipodermico se origina de las paredes musculares de los vasos, y se caracteriza por presentar una mayor tasa de recidiva local (37%) y metastasis (43%). El sarcoma pelomorfico dermico aparece habitualmente en pacientes ancianos y se localiza caracteristicamente en zonas de piel fotoexpuesta (cuero cabelludo). Comparte caracteristicas histologicas e inmunohistoquimicas con el fibroxantoma atipico, pero con un comportamiento mas agresivo (metastasis en el 10-20%). Los criterios histologicos que lo diferencian son la infiltracion del tejido celular subcutaneo, la infiltracion perineural y la presencia de focos de necrosis.

This series of 2 articles on dermatopathologic diagnoses reviews conditions in which granulomas form. Part 1 clarifies concepts, discusses the presentation of different types of granulomas and giant cells, and considers a large variety of noninfectious diseases. Some granulomatous diseases have a metabolic origin, as in necrobiosis lipoidica. Others, such as granulomatous mycosis fungoides, are related to lymphomas. Still others, such as rosacea, are so common that dermatologists see them nearly daily in clinical practice.

Characterization of patients, surgery procedures and the risk factors for dermatofibrosarcoma protuberans (DFSP) recurrences is poorly defined. In this study, we aimed to describe the demographics, tumor characteristics and interventions of DFSP treated with Mohs micrographic surgery (MSS) to determine the rate and risk factors for recurrence. Data were collected from REGESMOHS, a nationwide prospective cohort study of patients treated with MMS in Spain. From July 2013 to February 2020, 163 patients with DFSP who underwent MMS were included. DFSP was mostly located on trunk and extremities. Recurrent tumors had deeper tumor invasion and required higher number of MMS stages. Paraffin MMS was the most frequently used technique. Overall recurrence rate was 0.97 cases/100 person-years (95% IC = 0.36-2.57). No differences were found in epidemiological, tumor, surgery characteristics or surgical technique (frozen or paraffin MMS [p = 0.6641]) in terms of recurrence. Median follow-up time was 28.6 months with 414 patient-years of follow-up. In conclusion, we found an overall low recurrence rate of DFSP treated with MMS. None of the studied risk factors, including MMS techniques, was associated with higher risk for recurrence.

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.

Background The use of Mohs micrographic surgery (MMS) for rare cutaneous tumors is poorly defined. We aim to describe the demographics, tumor presentation and topography, surgery characteristics and complications of MMS for rare cutaneous tumors in a national registry. Methods Prospective cohort study of patients treated with MMS in Spain between July 2013 and June 2018. The inclusion criteria were patients with cutaneous tumors with final diagnosis different from basal cell carcinoma, squamous cell carcinoma, dermatofibrosarcoma protuberans, or any kind of melanoma. Results Five thousand and ninety patients were recorded in the registry, from which only 73 tumors (1.4%) fulfilled the inclusion criteria: atypical fibroxanthoma (18), microcystic adnexal carcinoma (10), extramammary Paget's disease (7), Merkel cell carcinoma (5), dermatofibroma (4), trichilemmal carcinoma (4), desmoplastic trichoepithelioma (4), sebaceous carcinoma (3), leiomyosarcoma (2), porocarcinoma (2), angiosarcoma (2), trichoblastoma (1), superficial acral fibromyxoma (1), and others (10). No intra-surgery morbidity was registered. Postsurgery complications appeared in six patients (9%) and were considered mild. Median follow-up time was 0.9 years during which three Merkel cell carcinomas, one angiosarcoma, one microcystic adnexal carcinoma, and four others recurred (12.3%). Conclusion This national registry shows that rare cutaneous tumors represent a negligible part of the total MMS performed in our country with a low complication rate.

Resumen Los sarcomas constituyen un grupo amplio de tumores, muchos de ellos con comportamiento biologico y agresividad diferentes entre si, que habitualmente requieren un tratamiento multidisciplinario, frecuentemente complejo. El desarrollo en las ultimas decadas de la dermatologia quirurgica y oncologica ha permitido que los dermatologos se conviertan en los especialistas responsables del diagnostico y tratamiento del cancer cutaneo. El proposito de este articulo es revisar los principales sarcomas de partes blandas de localizacion tipicamente cutanea. El dermatofibrosarcoma protuberans es un sarcoma de bajo grado de malignidad, con un crecimiento lento e infiltrativo localmente y escasa capacidad metastasica (

Resumen Antecedentes El registro Regesmohs es un registro de ambito nacional, de pacientes evaluados y sometidos a una cirugia de Mohs, en 17 centros espanoles, desde julio de 2013. Como la cirugia de Mohs es el tratamiento que mejores resultados da para el manejo del carcinoma de celulas basales (CCB) de alto riesgo y otros tumores de la piel, queriamos describir los motivos por los que algunos pacientes fueron considerados no aptos para ser sometidos a este tratamiento y que tratamientos alternativos recibieron. Estos datos pueden ser utiles para evitar excluir a pacientes aptos para ser sometidos a una cirugia de Mohs, para calcular la demanda que estos pacientes generan a nivel sanitario, asi como la demanda que hay de tratamientos de inhibidores de la via de Hedgehog en dicho grupo de pacientes. Objetivo Describir a aquellos pacientes que fueron considerados no aptos para ser sometidos a una cirugia de Mohs tras valoracion prequirurgica y los tratamientos que recibieron. Metodos Regesmohs incluye a todos los pacientes consecutivos para ser sometidos a una cirugia de Mohs en los centros participantes, recogiendo datos sobre las caracteristicas de los pacientes, las intervenciones y los resultados a corto y largo plazo. Se hizo una descripcion de los pacientes considerados no aptos para ser sometidos a una cirugia de Mohs tras valoracion prequirurgica. Resultados Tres mil once pacientes fueron incluidos en el registro Regesmohs entre julio de 2013 y octubre de 2016. En 85 pacientes no se realizo cirugia de Mohs porque se consideraron candidatos inadecuados. Sesenta y siete pacientes presentaban CCB. Las razones para ser considerado paciente no apto fueron: contraindicaciones medicas (27,1%, n = 23), tumores de bajo riesgo (18,8%, n = 16) y tumores gigantes e invasion osea (15,3%, n = 13). Solo un paciente (1,2%) revelo compromiso de ganglios linfaticos y ningun paciente metastasis visceral. De los 85 pacientes considerados no aptos 29 (34,1%) fueron sometidos a cirugia convencional, 24 (28,3%) a radioterapia, 4 (4,7%) a inhibidores de la via de Hedgehog (solo indicado para el CCB) y 2 (2,4%) a tratamiento paliativo. No hubo datos de seguimiento de 14 pacientes (16,5%). Conclusion Las comorbilidades medicas fueron la razon mas habitual para retener la cirugia de Mohs. Retener un tratamiento en funcion de una propagacion a lugares distantes no es algo habitual. La mayoria de los pacientes considerados no aptos recibieron tratamientos mas sencillos: cirugia convencional o radioterapia, siendo los inhibidores de la via de Hedgehog una opcion novedosa.

Introduction The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. Material and methods Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. Results Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. Conclusion The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo maligna and dermatofibrosarcoma protuberans accounted for a higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low.

Resumen Introduccion y objetivos El angiosarcoma primario cutaneo es uno de los tumores mas agresivos y de peor pronostico de la piel. Su clinica inicialmente indolente justifica frecuentes diagnosticos tardios, lo que sumado a su caracter muchas veces multifocal y a su mala delimitacion suele dificultar la cirugia. Debido a su baja frecuencia existen pocas series largas de casos tratados en un mismo centro. Revisamos las caracteristicas clinico-patologicas de los angiosarcomas cutaneos tratados en nuestro centro en busqueda de factores pronosticos, asi como de posibles rasgos que faciliten un diagnostico precoz. Material y metodos Se realizo un estudio observacional retrospectivo de todos los pacientes diagnosticados de angiosarcoma cutaneo atendidos en nuestro hospital entre enero de 2000 y diciembre de 2015. Se recogieron 16 parametros clinicos incluidos —entre otros— edad, sexo, tipo de angiosarcoma, localizacion, tamano, tiempo de evolucion y ademas 8 parametros histopatologicos. Resultados Se recogieron 16 pacientes con angiosarcoma cutaneo —11 mujeres y 5 varones—, la media de edad fue de 67 anos y la mediana de 71 anos. La localizacion mas frecuente fue el tronco con 10 casos, seguida de la cabeza y el cuello con 5 casos. La media del tamano tumoral fue de 10 cm y la mediana de 6,5 cm. Se realizo escision quirurgica del tumor a 14 pacientes. Tras una media de seguimiento de 42,5 meses, 6 de los 16 pacientes seguian vivos al finalizar el estudio. Conclusiones La supervivencia de los pacientes con angiosarcoma cutaneo viene determinada principalmente por el tamano tumoral y la edad. Otros rasgos asociados a peor pronostico en nuestros pacientes fueron la infiltracion a planos mas profundos (musculo), un patron histologico predominantemente solido y un mayor numero de mitosis.

Progress in the understanding of many tumors has enabled the development of new therapies, such as those targeted at specific molecules involved in cell growth (targeted therapies) or intended to modulate the immune system (immunotherapy). However, along with the clinical benefit provided by these new treatments, new adverse effects have also appeared. Dermatological toxicities such as papulopustular eruptions, xerosis, and pruritus are common with EGFR inhibitors. Other adverse effects have also been described with PDGFR, BCR-ABL, and MAPK tyrosine kinase inhibitors, antiangiogenic drugs, and inhibitors at immune checkpoints such as CTLA-4 and PD-1/PD-L1. Onset of these adverse effects often causes dose reductions and/or delays in administering the prescribed therapy, which can affect patient survival and quality of life. It is, therefore, important to prevent the occurrence of these adverse effects, or to treat unavoidable ones as soon as possible. This requires cooperation between medical oncologists and dermatologists. This article reviews the various dermatological toxicities associated with targeted therapies and immunotherapies, along with their diagnosis and therapeutic management.

Resumen Introduccion El dermatofibrosarcoma protuberans (DFSP) es un raro tumor cutaneo de crecimiento lento e infiltrativo que alcanza el tejido celular subcutaneo, el tejido muscular e incluso el hueso. Objetivos Buscar las caracteristicas histologicas asociadas a una mayor agresividad local en los DFSP, en forma de afectacion en profundidad. Material y metodos Se relaciono las caracteristicas histologicas propias del DFSP (forma de infiltrar el tejido celular subcutaneo, patron histologico, tipo celular, areas de fibrosarcoma) con la presencia o ausencia de afectacion de la fascia muscular. Resultados Se incluyeron 155 casos de DFSP. Las caracteristicas histologicas asociadas significativamente con la afectacion de la fascia muscular fueron: el patron histologico en sabana, un alto grado de pleomorfismo celular y la presencia de mas de una mitosis. En la mayoria de los casos (62,6%) el tumor se limito al tejido celular subcutaneo, en 17 casos (11%) contacto con la fascia muscular o con la galea aponeurotica, y en 36 casos (23,2%) afecto al tejido muscular. Conclusiones Es importante tener en cuenta el patron histologico, el pleomorfismo y el numero de mitosis en los DFSP para predecir su afectacion en profundidad (fascia o musculo), que puede llegar al 30% de los casos.

Although the arrival of new chemotherapy drugs and combinations has brought progress in terms of cancer patient survival, they entail many adverse effects that can compromise treatment, and hence prognosis, of the disease. Cytostatic agents can cause dermatological toxicity, among other side effects. The most familiar adverse effect of chemotherapy is alopecia. Although not serious, this changes the outward appearance of cancer patients. Other adverse effects include hypersensitivity and photosensitivity reactions, hand-foot syndrome, epidermal necrolysis, recall reactions, scleroderma-like reactions, Raynaud's phenomenon, eccrine squamous syringometaplasia, neutrophilic eccrine hidradenitis, nail abnormalities, pigmentation changes and extravasation injuries. Onset of these adverse effects often causes dose reduction and/or delayed treatment, which can affect patient survival and quality of life. It is therefore important to prevent their occurrence and treat them promptly, which requires cooperation between medical oncologists and dermatologists. This article reviews chemotherapy-associated dermatological toxicity, along with its diagnosis and therapeutic management.

Cutaneous squamous cell carcinoma is sometimes characterized by an increased risk of locoregional recurrence and occasionally distant metastasis. Several clinical and pathological factors, including perineural invasion, have been shown to have prognostic value in this setting. Perineural invasion, that is, the spread of tumor cells into the space surrounding a nerve, is usually an incidental finding. In the presence of symptoms or radiographic evidence of perineural spread, the diagnosis is clinical perineural invasion, which is associated with an increased risk of local recurrence and mortality.

BackgroundThe two main tumors treated with Mohs micrographic surgery (MMS) are basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). There are no studies analyzing whether MMS is different when treating these two types of tumors. ObjectiveWe aim to compare the characteristics of the patients, the tumors, and MMS, and first-year follow-up of MMS in BCC and SCC. MethodsREGESMOHS is a prospective cohort study of patients treated with MMS. The participating centers are 19 Spanish hospitals where at least one MMS is performed per week. Data on characteristics of the patients, tumors, and surgery were recorded. The follow-up was done with two visits: the first visit within 1 month after surgery and the second one within the first year. ResultsFrom July 2013 to April 2017, a total of 2,669 patients who underwent MMS were included in the registry. Of them, 2,448 (93%) were diagnosed with BCC, and 181 (7%) were diagnosed with SCC. Patients with SCC were older than those with BCC (median age 73 years vs. 68 years) and presented immunosuppression more frequently. The tumor size was significantly larger in the SCC group. Regarding surgery, deeper invasion was more frequent in SCC, resulting in larger defects. Despite this, SCC did not require more stages to get clear margins or more time in the operating room. Incomplete Mohs was more frequent in the SCC group (6%) than in the BCC group (2%). The incidence of perioperative complications was higher when treating SCC. There were more relapses in the first-year follow-up in the SCC group. ConclusionThere are significant differences when comparing MMS in BCC and SCC. Knowledge of these differences can help to prepare the patient and plan the surgery, optimizing results.

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.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.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.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.

Resumen Introduccion En julio de 2013 se inicio la recogida de datos del registro espanol de cirugia micrografica de Mohs, que describe la aplicacion y los resultados de esta tecnica en Espana. En este articulo se describen las caracteristicas del paciente y de los tumores tratados. Material y metodos Se trata de un estudio de cohortes prospectivo en el que participan centros en los que se practica al menos una intervencion semanal de cirugia micrografica de Mohs. En cada centro se incluyen todos los pacientes que son valorados para realizar cirugia de Mohs, excepto los declarados judicialmente incapaces. En este articulo describimos las caracteristicas de los pacientes y los tumores incluidos en la cohorte. Resultados El numero de pacientes incluidos desde julio de 2013 hasta octubre de 2014 es de 655. La mayoria de los tumores cutaneos intervenidos correspondieron a carcinoma basocelular, siendo el infiltrante el subtipo histologico mas frecuente. La mayoria de las cirugias se practicaron en tumores localizados en la cara y el cuero cabelludo, siendo la localizacion mas frecuente la nariz. Casi el 40% de los tumores operados son recurrentes o persistentes, y el tamano tumoral prequirurgico es similar en nuestro medio al descrito en otros estudios australianos o europeos. Hasta el 45,5% de los pacientes habia recibido algun tratamiento quirurgico previo. Conclusion Los datos observados son similares a los de otras series publicadas, y son relevantes para poder valorar la aplicabilidad en nuestro contexto de estudios realizados en otros medios.

Introduction The Spanish registry of Mohs micrographic surgery started collecting data in July 2013. The aim of the registry is to report on the use of this technique in Spain and the outcomes achieved. In the present article, we describe the characteristics of patients and the tumors treated. Material and methods This is a prospective cohort study of patients treated with Mohs micrographic surgery. The participating centers are hospitals where at least one intervention of this type is performed each week. All patients considered for Mohs micrographic surgery in participating centers are included in the registry except those who have been declared legally incompetent. Results Between July 2013 and October 2014, data from 655 patients were included in the registry. The most common tumor involved was basal cell carcinoma, and the most common histological subtype was infiltrative basal cell carcinoma. Most of the tumors treated were located on the face or scalp, and the most common site was the nose. Almost 40% of the tumors treated were recurrent or persistent, and preoperative tumor size was similar to that reported in other European studies and in Australia. In total, 45.5% of patients had received previous surgical treatment. Conclusion The findings are similar to those reported in other studies, and the data collected are useful for assessing whether the results of studies carried out elsewhere are applicable in Spain.

Background Regesmohs registry is a nationwide registry including patients evaluated for Mohs surgery in 17 Spanish centres since July 2013. Given that Mohs surgery is the therapy with best results for high risk basal cell carcinoma (BCC) and other skin tumours, we wanted to describe the reasons that lead to some patients being excluded from this therapy and the alternative treatments that they received. These data may be useful to avoid excluding patients for Mohs surgery use, to estimate the healthcare demand of these patients and the demand for Hedgehog inhibitors therapy in this group. Objective To describe patients excluded for Mohs surgery after pre-surgical assessment, and the treatments that they received. Methods Regesmohs includes all consecutive patients assessed for Mohs surgery in the participating centres, collecting data on patient characteristics, intervention, and short and long-term results. Patients excluded for Mohs surgery after pre-surgical evaluation were described. Results 3011 patients were included in Regesmohs from July 2013 to October 2016. In 85, Mohs surgery was not performed as they were considered inadequate candidates. 67 had BCC. Reasons for exclusion were: medical contraindication (27.1%, n = 23) low-risk tumour in (18.8%, n = 16) and giant tumour and bone invasion (15.3%, n = 13). Only 1 patient (1.2%) showed lymph node involvement and no patients had visceral metastases. Of the 85 excluded patients, 29 (34.1%) were treated with conventional surgery, 24 (28.3%) with radiotherapy, 4 (4.7%) with inhibitors of the Hedgehog pathway (only indicated for BCC), and 2 (2.4%) received palliative care. We had no follow-up data on 14 patients (16.5%). Conclusion Medical comorbidities were the most common reason for withholding Mohs surgery. Withholding therapy on the basis of distant extension is uncommon. Most excluded patients received simpler therapies: conventional surgery or radiotherapy, with hedgehog inhibitors being a new option.

Background The elderly population is increasing and more patients in this group undergo Mohs micrographic surgery. The few publications investigating MMS in elderly people conclude that it is a safe procedure; however, these are single centre studies without a comparison group. Objective To compare the characteristics of patients, tumours, MMS, and one-year follow-up in patients younger than 80 years, with patients older than 80 years at the time of surgery. Methods Data was analysed from REGESMOHS, a prospective cohort study of patients treated with MMS. The participating centres were 19 Spanish hospitals where at least one MMS is performed per week. Data on characteristics of the patient, tumour, and surgery were recorded. Follow-up data was collected from two visits; the first within one month post-surgery and the second within the first year. Results From July 2013 to October 2016, 2575 patients that underwent MMS were included in the registry. Of them, 1942 (75,4%) were aged < 80 years and 633 (24,6%) were ≥80 years-old. In the elderly, the tumour size was significantly higher with a higher proportion of squamous cell carcinoma. Regarding surgery, elderly more commonly had tumours with deeper invasion and required a higher number of Mohs surgery stages, leaving larger defects, and requiring more time in the operating room. Despite this, the incidence of post-operative complications was the same in both groups (7%) and there were no significant differences in proportion of relapses in the first-year follow-up. Conclusion The risk of short term complications and relapses were similar in elderly and younger groups. MMS is a safe procedure in the elderly. This article is protected by copyright. All rights reserved.

Cutaneous apocrine adenocarcinoma (CAA) is a rare adnexal neoplasm that histologically can mimic breast carcinoma metastatic to the skin or apocrine carcinoma arising in ectopic breast tissue. It can present with a wide range of clinical modalities and can often simulate many benign processes, which delays its diagnosis and hinders its prognosis. We describe a case of a 33-year-old man who had a short-evolution small nodule in the right axilla with local lymph node metastases. The immunohistochemical characterization was closer to that of breast adenocarcinoma than to an adnexal neoplasm. This was established as the main differential diagnosis. Diagnosis of cutaneous apocrine adenocarcinoma may be difficult and immunomarkers are not specific. The anatomical criteria and systemic investigation are mandatory to establish the correct diagnosis.

Resumen Introduccion y objetivos La dermatomiositis del adulto es paraneoplasica hasta en una cuarta parte de los casos. Hasta la fecha no existe ningun rasgo clinico, histologico o analitico absolutamente especifico de paraneoplasia en dermatomiositis. Ademas, los estudios sobre dermatomiositis del adulto no suelen hacer referencia a la frecuencia de aparicion de los distintos signos cutaneos propios de la dermatomiositis en los casos asociados a cancer. Por todo ello decidimos revisar nuestros casos de dermatomiositis paraneoplasicas. Material y metodos Estudiamos 12 casos de dermatomiositis paraneoplasicas en los cuales se recogio la edad, el sexo, el cancer asociado, el tiempo entre el inicio de la dermatomiositis y el cancer, 9 signos cutaneos, afectacion muscular, disfagia, enfermedad pulmonar, niveles de creatinfosfocinasa y de autoanticuerpos circulantes. Resultados La media de edad fue de 61 anos y los 2 canceres asociados mas frecuentes fueron el de ovario y el de vejiga. El tiempo medio transcurrido entre el diagnostico del cancer y el de la dermatomiositis fue de 7 meses, y en la mayoria el diagnostico de cancer precedio al de dermatomiositis. Siete pacientes tuvieron dermatomiositis amiopatica. De los signos cutaneos, los mas frecuentes fueron una erupcion violacea fotodistribuida que respetaba la region interescapular y el rash heliotropo, seguidos de las papulas de Gottron y la afectacion de cuticulas. Se encontro necrosis cutanea superficial en 3 casos. Los autoanticuerpos especificos de miositis resultaron negativos en los 6 casos estudiados. Conclusiones La dermatomiositis paraneoplasica es muchas veces amiopatica. No existe ningun rasgo cutaneo especifico de paraneoplasia en la dermatomiositis. Los anticuerpos especificos de miositis no se asocian a la dermatomiositis paraneoplasica.

Resumen El carcinoma epidermoide cutaneo, con una incidencia en la poblacion general de aproximadamente un 10% a lo largo de la vida, es la segunda neoplasia mas frecuente dentro del grupo del cancer cutaneo no melanoma. La mayoria de los carcinomas epidermoides cutaneos muestran un comportamiento benigno y pueden ser completamente erradicados mediante cirugia y otros procedimientos dermatologicos. Sin embargo, existe un subgrupo de esta entidad que se asocia con una mayor capacidad de desarrollar metastasis nodal y, por tanto, con una elevada morbimortalidad. En el presente articulo se analizan los diferentes factores que definen al carcinoma epidermoide cutaneo de comportamiento agresivo. Proponemos un metodo de definicion del carcinoma epidermoide de alto riesgo basado en el establecimiento de una serie de criterios mayores y menores. Este hecho supondra una mejor evaluacion pronostica y un manejo personalizado de este grupo de enfermos, que puede resultar en un aumento de la supervivencia global.