Porocarcinoma ecrino metastásico / Metastatic eccrine porocarcinoma

Presentamos el caso de un paciente varón de 69 años, con lesión tumoral en región inguinal izquierda, asintomática. El estudio histopatológico e inmunohistoquímico fue consistente con porocarcinoma ecrino. A la realización de imágenes se evidenció metástasis ganglionares en región iliaca femoral, retroperitoneal y supraclavicular izquierda. Se confirmó infiltración de porocarcinoma ecrino en ganglio supraclavicular al estudio histopatológico. El porocarcinoma ecrino metastásico es de muy rara presentación. El tratamiento quirúrgico no es posible en estos casos. No existe un tratamiento estándar sistémico, solo series de casos con respuestas variables. Nuestro paciente recibió tratamiento sistémico con docetaxel con buena tolerancia.

We present the case of a 69-year-old male patient with tumor lesion in the left inguinal region, asymptomatic. The histopathological and immunohistochemical study was consistent with ecrine porocarcinoma. Upon imaging, nodal metastases in the iliac femoral, retroperitoneal and supraclavicular left region are evident. Ecrine porocarcinoma infiltration into supraclavicular ganglion is confirmed at histopathological study. Metastatic eccrine porocarcinoma is very rare. Surgical treatment is not possible in these cases. There is no standard systemic treatment, only case series with variable responses. Our patient received chemotherapy treatment with doxetacel with good clinical response to follow-up.

Porocarcinoma Ecrino / Ecrine Porocarcinoma

Resumen:El porocarcinoma ecrino es un tipo muy poco frecuente de cáncer de piel, originado en la porción epidérmica de las glándulas sudoríparas ecrinas. Representa no más del 0.01% de los tumores cutáneos. El 20% de los porocarcinomas ecrinos son recurrentes y el 20% producen metástasis a ganglios linfáticos. Se ha reportado escasos casos de metástasis a distancia. Tiene un índice de mortalidad del 67% de los pacientes con metástasis.El diagnóstico es basado en los hallazgos histopatológicos y los estudios complementarios de inmunohistoquímica, a veces necesarios para el diagnóstico diferencial con otros tipos más frecuentes de cáncer de piel.No existen pruebas de que este tipo de carcinoma responda a la quimioterapia ni la radioterapia. Se considera que el manejo principal debe ser la resección quirúrgica y la electroquimioterapia.

Abstract:Eccrine porocarcinoma is a rare type of skin cáncer arising from the intraepidermal portion of eccrine sweat glands, representing no more tan 0.01% of all cutaneous tumors. 20% of the Eccrine porocarcinoma will recur and 20% will metastasize to regional lymph nodes. Few cases of distant metastases has been reported . There is a mortality rate of 67% in patients with metastases. The diagnosis is primarily based on histopathologic findings and complementary immunohistochemistry for differential diagnosis mainly with more frequent skin cáncer.Neither chemotherapy nor radiation therapy has been proven to be of clinical benefit in treating this type of carcinoma. It is considered that the management should be based on surgical resection and electrochemotherapy.

Tumores cutâneos raros em cabeça e pescoço: experiência de 4 anos em uma instituição terciária / Head and neck rare skin tumors: 4 years of experience in a tertiary institution

Introdução: O câncer de pele é o mais incidente dentre as neoplasias. Os tipos histológicos mais comuns são Carcinoma Basocelular (CBC), Carcinoma Espinocelular (CEC) e, menos frequente, o Melanoma. Geralmente acometem segmentos corporais expostos aos raios ultravioletas, principalmente a região de cabeça e pescoço. Porém, há outros tipos mais de tumores que acometem esta área e que fazem parte da prática da oncologia cutânea encaminhada aos cirurgiões de cabeça e pescoço. Objetivo: O objetivo deste trabalho é descrever estas neoplasias percutâneas raras que foram operadas no Instituto do Câncer do Estado de São Paulo (ICESP) nos últimos 4 anos. Resultados: As entidades Carcinoma de células de Merkel, Dermatofibrossarcoma protuberans, Angiossarcoma, Fibroxantoma atípico, Porocarcinoma, Carcinoma triquilemal, Carcinoma de células claras, Carcinoma sarcomatoide e Carcinoma apócrino compuseram 4,4% dos atendimentos de oncologia cutânea realizada pela Cirurgia de Cabeça e Pescoço. Uma breve revisão de cada entidade foi realizada. Discussão: A compreensão dos tipos histológicos raros é importante no tratamento de tumores cutâneos na prática da cirurgia de cabeça e pescoço.

Introduction: Skin cancer is the most frequent cancer among neoplasms. The most common histological types are Basal Cell Carcinoma (BCC), Squamous Cell Carcinoma (SCC) and, less frequent, Melanoma. They generally affect body segments exposed to ultraviolet rays, especially the head and neck region. However, there are other types of tumors involving this area and they are part of the practice of cutaneous oncology referred to head and neck surgeons. Objective: The objective of this paper is to describe these rare cutaneous neoplasms, which were treated at the Instituto do Câncer do Estado de São Paulo (ICESP) in the last 4 years. Results: Merkel cell carcinoma, Dermatofibrosarcoma protuberans, Angiosarcoma, Atypical Fibroxanthoma, Porocarcinoma Trichilemmal carcinoma, Sarcomatoid carcinoma and Apocrine carcinoma comprised 4.4 % of cases of cutaneous oncology conducted by Head and Neck Surgery. A brief review of each neoplasm was performed. Discussion: understanding of the rare histological types is important in the treatment of skin tumors in the practice of head and neck surgery.

Porocarcinoma ecrino: estudio clínico-patológico de 19 casos en el instituto nacional de enfermedades neoplásicas, Lima - Perú / Eccrine porocarcinoma: clinical-pathological study of 19 cases in the instituto nacional de enfermedades neoplásicas, Lima - Peru

Con el objetivo de describir las características clínico-patológicas de los casos de porocarcinoma ecrino (PE) registrados en el Instituto Nacional de Enfermedades Neoplásicas (INEN), se realizó un estudio trasversal que incluyó todos los casos entre 1998-2009. Para ello se realizó una revisión de fichas clínico-patológicas y una nueva lectura de las láminas de patología de los casos encontrados. Se hallaron 19 casos de PE; la mediana de edad fue de 64 años (rango: 37-98); con mayor frecuencia en el sexo masculino (57,9%); la localización más frecuente fue en el pie (21,1%), seguida de cabeza, tórax y muslo (15,8%, en todos los casos). Se describe un caso de localización glútea que cursó con una conducta inusualmente invasiva y con compromiso rectal. Se concluye que el PE es una entidad poco frecuente en el Perú, su localización más común es en el pie, y afecta predominantemente al sexo masculino.

In order to describe the clinical-pathological characteristics of the cases of Eccrine Porocarcinoma (EP) found in the Instituto Nacional de Enfermedades Neoplásicas (INEN), a cross-sectional study was carried out, including all EP cases recorded between 1998 and 2009. A revision of the clinical-pathological records indentified and a new reading of the pathology slides of the cases found were performed. 19 EP cases were found, the median age of which was 64 years (range: 37-98). EP was primarily found on male patients (57.9%) and was most commonly located on the foot (21.1%), followed by the head, thorax and thigh (15.8%, respectively). In one case, EP was located on the gluteal region, and showed an unusually invasive behavior and rectal involvement. We conclude that EP is a rather uncommon entity in Peru. Its most common location is on the foot and it affects mostly male patients for the most part.