The role of 18F-FDG PET/CT in the management of Merkel cell carcinoma: The experience of 51 studies in our institution. / Papel de la 18F-FDG PET/TC en el manejo del carcinoma de células de Merkel: experiencia de 51 estudios en nuestra institución.

OBJECTIVES: Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor with limited evidence on the role of 18F-FDG PET/CT. The aim of this study was to assess the impact of the 18F-FDG PET/CT in the management of MCC. METHODS: Fifty-one studies of 18F-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. 18F-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 positive 18F-FDG PET/CT but the trend did not reach statistical significance. CONCLUSION: 18F-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 of 18F-FDG PET/CT on the management of MCC patients.

Carcinoma de células de Merkel: diagnóstico y tratamiento en atención especializada dermatológica. Guía de práctica clínica de la Academia Española de Dermatología y Venerología / Diagnosis and Treatment of Merkel Cell Carcinoma in Specialized Dermatology Units: A Clinical Practice Guideline of the Spanish Academy of Dermatology and Venereology

Antecedentes y objetivo: El carcinoma de células de Merkel es un tipo de cáncer de piel infrecuente y agresivo. Hay una gran variación en su manejo y las diferentes guías extranjeras que existen cubren parcialmente los problemas identificados como principales. El objetivo de la presente guía es servir de referencia a los dermatólogos españoles para mejorar aspectos controvertidos del diagnóstico, estadificación y tratamiento del carcinoma de células de Merkel. Materiales y métodos: Se empleó el método ADAPTE: se escogió a miembros del Grupo Español de Dermato-Oncología y Cirugía (GEDOC) con experiencia en el tratamiento de estos tumores y con interés en participar en la elaboración de la guía. Tras resumir el proceso de atención y elaborar las preguntas clínicas relevantes, se hizo una búsqueda de guías, que se seleccionaron según su puntuación mediante el instrumento Appraisal of Guidelines for Research and Evaluation (AGREE II). Tras la búsqueda de las respuestas en dichas guías, se elaboraron posteriormente las recomendaciones. Por último, se sometió la guía a revisión externa. Resultados: Las guías con mejor puntuación fueron las de National Comprehensive Cancer Network, la European consensus-based interdisciplinary guideline, Alberta Healthservices Clinical practice guideline, American Cancer Society y Cutaneous Oncology Group of the French Society of Dermatology. Se obtuvieron en total 9 preguntas clínicas, contestadas a partir de estas guías. Conclusiones: Esta guía responde a preguntas habituales en la práctica clínica diaria y sirve a los dermatólogos como referencia en la toma de decisiones, siempre teniendo presentes los recursos y preferencias del paciente

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

Diagnosis and Treatment of Merkel Cell Carcinoma in Specialized Dermatology Units: A Clinical Practice Guideline of the Spanish Academy of Dermatology and Venereology. / Carcinoma de células de Merkel: diagnóstico y tratamiento en atención especializada dermatológica. Guía de práctica clínica de la Academia Española de Dermatología y Venerología.

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.

Carcinoma anexial microquístico: la cirugía micrográfica de Mohs como tratamiento de elección / Microcystic Adnexal Carcinoma: Mohs Micrographic Surgery as the Treatment of Choice

Introducción y objetivos. El carcinoma anexial microquístico (CAM) es un tumor raro y agresivo que clínicamente se manifiesta como un nódulo subcutáneo localizado en las regiones de cabeza y cuello. Esta tumoración puede ser clínica e histológicamente confundida con otras lesiones cutáneas benignas o malignas, lo que con frecuencia conduce a un tratamiento inicial erróneo. La principal complicación del CAM es la alta morbilidad y la elevada tasa de recurrencia tras escisión local amplia. Estudios preliminares recientes han mostrado unas mayores tasas de curación mediante cirugía micrográfica de Mohs (CMM). Material y métodos. Se revisaron las historias clínicas de 6 pacientes consecutivos con CAM tratados mediante CMM en nuestro Servicio de Dermatología entre 1995 y 2007. Resultados. Todos los casos se localizaron en la cabeza y el 100 % fue tumor primario. En el 70 % de los casos el tumor fue inicialmente mal diagnosticado de carcinoma basocelular. No se detectó invasión perineural en ninguno de los casos y la recurrencia estuvo ausente en todos los pacientes tras un periodo de seguimiento comprendido entre 1 y 12 años posteriores a la CMM. Conclusiones. La ausencia de afectación perineural y de una importante atipia celular puede deberse al carácter primario de la tumoración. Ello justificaría la necesidad de un tratamiento definitivo radical inicial del tumor primario para evitar las complicaciones que implica una recidiva tumoral. La localización y la ausencia de recurrencia en todos nuestros casos tratados mediante CMM apoyan el uso de esta técnica como el tratamiento de elección para el CAM (AU)

Introduction and objectives. Microcystic adnexal carcinoma is a rare and aggressive tumor that manifests clinically as a subcutaneous nodule located on the head or neck. The tumor can be confused clinically and histologically with other benign and malignant skin lesions, often leading to inappropriate initial treatment. The chief concern with microcystic adnexal carcinoma is the elevated morbidity and the high rate of recurrence after wide local excision. Recent preliminary studies point to higher cure rates with Mohs micrographic surgery. Material and methods. We reviewed the medical histories of 6 consecutive patients with microcystic adnexal carcinoma who underwent Mohs micrographic surgery in our dermatology department between 1995 and 2007. Results. In all cases, lesions were located on the head and were primary tumors. Seventy percent of the tumors were wrongly diagnosed initially as basal cell carcinoma. Perineural invasion was not detected in any patient, and all were free of recurrence after between 1 and 12 years of postoperative follow-up. Conclusions. The absence of perineural involvement and substantial cell atypia can be attributed to the lesions being primary tumors. This would provide a rationale for definitive radical treatment of the primary tumor from the outset to avoid the complications associated with recurrence. The site and the absence of recurrence in all our patients who underwent Mohs micrographic surgery support the use of this technique as the treatment of choice in microcystic adnexal carcinoma (AU)

Dactilitis crónica indolora como hallazgo inicial de un adenocarcinoma pulmonar diseminado / Chronic Painless Dactylitis as the Initial Finding in Disseminated Lung Adenocarcinoma

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[Microcystic adnexal carcinoma: Mohs micrographic surgery as the treatment of choice]. / Carcinoma anexial microquístico: la cirugía micrográfica de Mohs como tratamiento de elección.

INTRODUCTION AND OBJECTIVES: Microcystic adnexal carcinoma is a rare and aggressive tumor that manifests clinically as a subcutaneous nodule located on the head or neck. The tumor can be confused clinically and histologically with other benign and malignant skin lesions, often leading to inappropriate initial treatment. The chief concern with microcystic adnexal carcinoma is the elevated morbidity and the high rate of recurrence after wide local excision. Recent preliminary studies point to higher cure rates with Mohs micrographic surgery. MATERIAL AND METHODS: We reviewed the medical histories of 6 consecutive patients with microcystic adnexal carcinoma who underwent Mohs micrographic surgery in our dermatology department between 1995 and 2007. RESULTS: In all cases, lesions were located on the head and were primary tumors. Seventy percent of the tumors were wrongly diagnosed initially as basal cell carcinoma. Perineural invasion was not detected in any patient, and all were free of recurrence after between 1 and 12 years of postoperative follow-up. CONCLUSIONS: The absence of perineural involvement and substantial cell atypia can be attributed to the lesions being primary tumors. This would provide a rationale for definitive radical treatment of the primary tumor from the outset to avoid the complications associated with recurrence. The site and the absence of recurrence in all our patients who underwent Mohs micrographic surgery support the use of this technique as the treatment of choice in microcystic adnexal carcinoma.

[Adenoid cystic carcinoma]. / Carcinoma adenoide quístico.

Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although it is mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists in extensive local excision with free margins. Radiotherapy is not curative and should be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma.

Carcinoma adenoide quístico / Adenoid cystic carcinoma

El carcinoma adenoide quístico es un tumor poco común de cabeza y cuello, que si bien se localiza preferentemente en la glándula salivar, también se ha descrito su localización cutánea. Las metástasis son raras, pero un 50 % de los casos recidivan. Un paciente de 65 años de edad presentó una lesión en el labio superior, que tras la resección, el diagnóstico histopatológico fue de carcinoma adenoide quístico. El tratamiento se completó con radioterapia. Diez años más tarde se detectó un nódulo en el cuello cuyo diagnóstico histopatológico fue de metástasis linfática por carcinoma adenoide quístico. El carcinoma adenoide quístico, primariamente cutáneo, es un tumor muy poco frecuente en el que el tratamiento consiste en la exéresis local amplia con márgenes libres. La radioterapia no es curativa y debería reservarse para tratamientos paliativos. Se necesitan estudios multicéntricos prospectivos para determinar el tratamiento óptimo y, especialmente, el tratamiento adjuvante del carcinoma adenoide quístico

Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although it is mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists in extensive local excision with free margins. Radiotherapy is not curative and should be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma