Carcinoma neuroendocrino de la piel: Carcinoma de células de Merkel. Revisión de la Literatura, a propósito de un caso / Neuroendocrine skin carcinoma: Merkel cell carcinoma. Review of literature, about a case

El carcinoma de células de Merkel, también llamado carcinoma neuroendocrino de la piel, es un tipo de cáncer de piel muy poco frecuente que generalmente aparece como un nódulo de color carne o rojo azulado, más frecuentemente en región facial, cabeza y cuello. El carcinoma de células de Merkel se desarrolla principalmente en personas mayores ya que la exposición al sol a largo plazo o un sistema inmunitario débil pueden aumentar el riesgo de desarrollarlo. Las células de Merkel se encuentran en la base de la capa más externa de la piel (epidermis) y están conectadas a las terminaciones nerviosas que son responsables del sentido del tacto. Tiende a crecer rápido y diseminarse a otras partes del cuerpo. Por tanto, las opciones de tratamiento para el carcinoma de células de Merkel dependen de si el cáncer se ha diseminado más allá de la piel

Merkel cell carcinoma, also called neuroendocrine skin of the skin, is a very rare type of skin cancer that generally appears as a bluish meat or red color nodule, more frequently in the facial, head and neck region. Merkel cell carcinoma develops mainly in older people since long -term exposure or a weak immune system can increase the risk of developing it. Merkel cells are at the base of the outermost layer of the skin (epidermis) and are connected to nerve endings that are responsible for the sense of touch. It tends to grow quickly and spread to other parts of the body. Therefore, the treatment options for Merkel cell carcinoma depend on whether cancer has spread beyond the skin

Carcinoma de células de Merkel em extremidade inferior / Merkel cell carcinoma in lower end

Merkel Cells Carcinoma is a rare cutaneous neoplasia. Studies revealed an increase in the occurrence from 0.15 to 0.44 cases for every 100.000 inhabitants between 1986 and 2001. Around 50% of the patients, eventually, develop systemic disease, being the most common sites the liver, the bones and the brain. The etiology is still unknown. The present study presents a new case of Merkel cells carcinoma in extremity, treated with local resection and adjuvant radiotherapy.

A importância da linfocintilografia no tratamento do carcinoma de células de Merkel / The role of lymphoscintigraphy in the treatment of Merkel´s cells carcinoma

O carcinoma de Células de Merkel constitui neoplasia cutânea maligna, rara e grave, de origem neuroendócrina, com comprometimento linfonodal em metade dos pacientes e metástases disseminadas em 20 por cento quando do diagnóstico anatomopatológico. Seu tratamento não está completamente estabelecido, entretanto a pesquisa do linfonodo sentinela vem sendo considerada indispensável e pode trazer benefícios na condução terapêutica dos pacientes.

The Merkel cell carcinoma is a rare, serious, neuroendocrine, malignant skin cancer with lymph node involvement in half of patients and disseminated metastases in 20 percent of cases at the time of pathological diagnosis. Its treatment is not fully established, though the sentinel lymph node has been considered essential and may bring benefits to the therapeutic armamentarium of patients.

A review of the epidemiology and treatment of Merkel cell carcinoma

Merkel cell carcinoma is a very rare and aggressive neoplasm. Due to its rarity, therapeutic guidelines are not well established, especially for regionally advanced disease. Articles in English, French, Italian, Portuguese, and Spanish from the last 20 years were identified in MEDLINE and reviewed. The key word "Merkel" was used for the search, relevant articles were selected, and their references were examined. The most important articles related to epidemiology, genesis and treatment were reviewed. The incidence of Merkel cell carcinoma is increasing due to the advancing age of the population, higher rates of sun exposure and an increasing number of immunocompromised individuals. With regard to etiology, the recently described Merkel Cell polyomavirus is thought to play a role. Either local or regional surgical intervention remains the standard of care, but adjuvant radiotherapy or radiotherapy as a primary treatment have been discussed as reasonable therapeutic options. An update on this rare neoplasia is essential because of its increasing incidence and changing treatment options.

Carcinoma de células de Merkel de labio: presentación de un caso con evolución especial y diagnóstico diferencial inmunohistoquímico / Lip Merkel cell carcinoma: presentation of a case with special evolution and immunohistochemic differential diagnosis

El carcinoma de células de Merkel es un tumor neuroendócrino infrecuente y muy agresivo a la piel. Las lesiones se presentan en zonas expuestas al sol tal como cabeza y cuello, extremidades y tronco; se han comunicado localizaciones intraorales y en labios. El diagnóstico diferencial clínico y por histología convencial, con otros tumores, es muy dificultoso. La inmunohistoquímica resulta útil para un correcto diagnóstico dado el inmunofenotipo específico de este tumor. El conocimiento por parte del clínico y del cirujano de la clínica y del inmunofenotipo diagnóstico por parte del patólogo, es esencial para un diagnóstico de certeza, y la clave para el planeamiento del tratamiento adecuado.

Carcinoma de células de Merkel: comunicación de cuatro casos / Merkel cell carcinoma: report of 4 cases

We report four cases of Merkel cell carcinoma, a rare cutaneous neuroendocrine neoplasm with a high malignant potencial. The first patient is a 90 years old male presenting with a tumor in the left superior eyelid. Surgical excision was the only treatment and seven months later, a local and regional tumoral relapse caused the death of this patient. The second patient is a 83 years old female with a tumor in her left ear. She was treated surgically and with radiotherapy, being free of disease after five years of follow up. The third patient is a 45 years old male with a tumor in the left forearm. Treatment was surgical excision of the primary tumor and axillary lymph nodes. He received post operative radiotherapy and is free of disease after three years of follow up. The last case is a 76 years old male, who was subjected only to an excisional biopsy of a lesion located in the left knee. He had a tumor relapse with inguinal and crural lymph node involvement that caused his death 12 months later

Tumores de Merkel en cabeza y cuello / Merkel cell tumors in head and neck

Hasta la fecha han sido comunicados poco más de 400 casos de carcinoma a células de Merkel en la literatura médica mundial. Es un tumor raro, de presumible origen neuroendócrino, que se presenta habitualmente en áreas expuestas de cabeza y cuello, luego de la sexta década de vida. El diagnóstico correcto es sólo posible a través de la microscopia electrónica y la inmunohistoquímica. Es importante tenerlo en cuenta en los diagnósticos diferenciales de los tumores cutáneos primarios o metastásicos. Se presentan dos casos, ambos de sexo femenino, mayores de 70 años, con lesiones ubicadas en la cara, y diseminación ganglionar. Una de ellas murió por la enfermedad, y la otra sin evidencia de recidiva a las 26 meses de seguimiento. De acuerdo con lo aconsejado por la mayoría de los autores y la evolución de estos pacientes, se propone la exéresis amplia del tumor primario, y la disección apropiada del cuello hemolateral. Debe considerarse la radio terapia del área del primario y de los cuellos con ganglios hitológicamente positivos