Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso / Surgical treatment of advanced facial Merkel cell carcinoma. A case report

Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

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 Metastático: Relato de Caso / Metastatic Merkel cell Carcinoma: Case Report / Carcinoma de Células de Merkel Metastásico: Relato de Caso

Introdução: O carcinoma de células de Merkel é um raro tumor neuroendócrino cutâneo, que se origina das células responsáveis pela sensibilidade tátil, possui caráter agressivo, evolução rápida e difícil tratamento. Relato do caso: Paciente do sexo masculino, 49 anos, caucasiano, que, ao atendimento dermatológico, apresentou nódulo indolor, infiltrando tecidos profundos, não ulcerado e localizado na região do braço esquerdo. O resultado da biópsia incisional foi positivo para carcinoma de células de Merkel. Após ressecção da lesão, os exames complementares evidenciaram doença metastática na axila e parede torácica. Com o tratamento quimioterápico, houve um benefício inicial com redução tumoral, porém, não durável, uma vez que foram reveladas novas áreas com metástases tumorais em regiões superiores do corpo, sendo submetido a novo procedimento cirúrgico, o qual, após novo regime quimioterápico, não obteve sucesso. Conclusão: Na ocasião do tratamento desse paciente, os anticorpos monoclonais, como o avelumab, não estavam disponíveis. O diagnóstico precoce com cirurgia de exérese da lesão imediata, antes do acometimento de outras regiões, permanece sendo a melhor opção para um prognóstico favorável ao paciente. Contudo, a despeito disso, com as limitações à época do tratamento, o paciente evoluiu a óbito.

Introduction: The Merkel cell carcinoma is a rare cutaneous neuroendocrine tumor that originates from cells responsible for tactile sensitivity, it has an aggressive character, fast evolution and difficult treatment. Case report: 49 years Caucasian male patient, with a painless nodule, infiltrating deep tissue, not ulcerated and located in left arm identified during the dermatological consultation. The result of the incisional biopsy was positive for Merkel cell carcinoma. After resection of the lesion, complementary exams revealed metastatic disease in the axilla and chest wall. The chemotherapy treatment brought an initial improvement with tumor reduction, however, it was not durable, because new areas with tumor metastases in upper regions of the body were revealed, the patient was submitted to an another surgical procedure, after which a new chemotherapy regimen failed. Conclusion:At the time of the treatment of this patient, monoclonal antibodies, such as avelumab, were not available. Early diagnosis with immediate lesion excision surgery, before the involvement of other regions, remains the best option for a better prognosis. However, regardless of this, because of the limitations at the time of the treatment, the patient died.

Introducción: El carcinoma de células de Merkel es un tumor neuroendocrino cutáneo raro, que se origina en células responsables de la sensibilidad táctil, tiene un carácter agresivo, una evolución rápida y un tratamiento difícil. Relato del caso: Paciente masculino, de 49 años, caucásico, que en atención dermatológica encontró nódulo indoloro, infiltrando tejidos profundos, no ulcerados y ubicados en la región del brazo izquierdo. El resultado de la biopsia incisional fue positivo para el carcinoma de células de Merkel. Después de la resección de la lesión, los exámenes complementarios mostraron enfermedad metastásica en la axila y la pared torácica. Con el tratamiento de quimioterapia, hubo un beneficio inicial con la reducción del tumor, sin embargo, no es duradero, ya que se revelaron nuevas áreas con metástasis tumorales en las regiones superiores del cuerpo, que se sometieron a un nuevo procedimiento quirúrgico, que después de un nuevo régimen de quimioterapia no tuvo éxito. Conclusión: En el momento del tratamiento de este paciente, los anticuerpos monoclonales, como avelumab, no estaban disponibles. El diagnóstico temprano con cirugía para la escisión de la lesión inmediata, antes de la participación de otras regiones, sigue siendo la mejor opción para un pronóstico favorable para el paciente. Sin embargo, a pesar de esto, con las limitaciones al momento del tratamiento, el paciente falleció.

Merkel cell carcinoma metastatic to the testis: report of a rare diagnosis and review of the literature

Merkel cell carcinoma is an aggressive malignancy that frequently recurs/disseminates, but metastases to the genitourinary tract are rare. Only eight cases of Merkel cell carcinoma metastatic to the testis are reported. We describe the ninth case of this event and provide a review of the literature. A 58-year-old man diagnosed with Merkel cell carcinoma of the wrist, presented, 37 months later, a recurrence in the form of a testicular metastasis. The tumor consisted of a monotonous proliferation of small, blue, round cells, with immunoexpression of neuroendocrine markers and the typical dot-like paranuclear immunostaining for cytokeratin 20, in the absence of immunostaining for cytokeratin 7. The patient is alive with no evidence of disease. Clinicians should be aware of the possibility of metastatic dissemination to the testis since genital examination/imaging is not part of routine follow-up for these patients, but timely orchiectomy may be curative.

Carcinoma de células de Merkel de región glútea. Reporte de un caso / Merkel cell carcinoma of the gluteal region. Report of a case / Carcinoma de células de Merkel de região glútea. Relato de caso

Resumen: Introducción: el carcinoma de células de Merkel es un tumor primario maligno de piel que afecta fundamentalmente regiones expuestas a las radiaciones solares. Pocos casos han sido comunicados en una región no expuesta al factor de riesgo mencionado, como es la glútea. El objetivo de este trabajo es comunicar un caso clínico de carcinoma de células de Merkel de región glútea. Caso clínico: paciente de 63 años, sexo masculino, que consultó por tumoración de 2 cm de diámetro en región glútea derecha, de rápido crecimiento, que se operó de coordinación con anestesia, realizándose su resección completa. El resultado del estudio anatomopatológico informó carcinoma de células de Merkel. No se evidenció diseminación a distancia, por lo que de acuerdo con la clasificación TNM se determinó como estadio I. Se completó el tratamiento con radioterapia local. Actualmente el paciente sigue en seguimiento y asintomático. Discusión: los carcinomas de células de Merkel son tumores altamente agresivos. Además de la radiación solar como factor de riesgo, se mencionan la inmunodeficiencia y un nuevo poliomavirus, el poliomavirus de células de Merkel. Se presentan como tumoraciones de color violáceo y rápido crecimiento, y es frecuente el compromiso ganglionar sincrónico o metacrónico. La resección quirúrgica con márgenes suficientes y vaciamiento ganglionar, en caso de haber compromiso o estudio de ganglio centinela en caso de no haberlo, es lo indicado. El pronóstico depende del estadio y se ha reportado hasta 30% de recidiva a dos años.

Summary: Introduction: Merkel cell carcinoma is a malign primary tumour that mainly affects regions that are exposed to solar radiation. Few cases have been reported in a region that is not exposed to the above mentioned risk factor, as the gluteal regions. The study aims to communicate the clinical case of a Merkel cell carcinoma of the gluteal region. Clinical case: 63-year-old male patient who consulted for a tumour with a 2cm diameter in the right gluteal region, rapidly growing, being completely resected in a cordinaterd surgery under anesthesia. Pathology study revealed that it was Merkel cell carcinoma. No distance dissemination was seen, and thus as per the TNM staging system it was classified as stage I. Therapy was completed with local radiotherapy, It is currently under follow up and asymptomatic. Discussion: Merkel cell carcinoma are highly aggressive tumours. Apart from solar radiation as a risk factor, it is worth mentioning immunodeficiency and a new polyomavirus, the Merkel cell polyomavirus. This condition can be seen as purple, rapidly growing tumours and they frequently involve synchronic or metachronic lymph node compromise. Surgical resection with sufficient margins and lymph node emptying, in the event of lymph node compromise or sentinel node biopsy is recommended. Prognosis depends on the stage and a 30% relapse has been reported after 2 years.

Resumo: Introdução: o carcinoma de células de Merkel é um tumor primário maligno de pele que afeta fundamentalmente regiões expostas às radiações solares. São poucos os casos relatados em uma região não exposta a esse fator de risco, como é a glútea. O objetivo desta comunicação é apresentar o caso clínico de um carcinoma de células de Merkel de região glútea. Caso clínico: paciente de 63 anos, sexo masculino que consultou por tumoração de 2 cm de diâmetro, na região glútea direita, com rápido crescimento que foi ressecada completamente em uma cirurgia eletiva com anestesia. O laudo anatomopatológico foi: carcinoma de células de Merkel. Não se evidenciou disseminação a distância por isso foi classificado como estádio I de acordo com a classificação TNM. O tratamento foi completado com radioterapia local. Atualmente em seguimento e assintomático. Discussão: os carcinomas de células de Merkel são tumores altamente agressivos. Além da radiação solar, a imunodeficiência e um novo poliomavirus, o poliomavirus de células de Merkel, são mencionados como fator de risco. Apresentam-se como tumorações de cor violácea e crescimento rápido e frequentemente se observa compromisso ganglionar sincrônico ou metacrônico. A ressecção cirúrgica com margens suficientes e esvaziamento ganglionar, quando há compromisso ou estudo de gânglio sentinela quando não há, é a conduta indicada. O prognóstico depende do estádio e há registros de até 30% de recidiva a 2 anos.

Virus polioma en carcinoma de células de Merkel / Polyoma virus in Merkel cell carcinoma

Introducción: El carcinoma de células de Merkel (MCC) es un tumor cutáneo maligno agresivo y de mal pronóstico. La incidencia es mayor en adultos hombres, caucásicos, con edad promedio de 70 años. Feng et al, lograron aislar un nuevo virus en muestras de este tumor, que denominaron virus polioma de células de Merkel (MCPyV). Se ha intentado establecer una relación causal entre el virus y MCC. El virus está integrado al genoma y produciría mutaciones específicas. En muestras de MCC, se ha detectado expresión de oncoproteinas virales (antígenos T) que promueven la replicación viral y tumorogénesis

Introduction: Merkel cell carcinoma (MCC) is an aggressive malignant cutaneous tumor with poor prognosis. Most cases affect elder patient with an average of 70 years of age. Feng et al isolated a new virus, the Merkel cell carcinoma polyoma virus (MCPyV). A causal relationship between MCPyV y MCC has been established. The virus is integrated in the genome and pro-duces specific mutations. MCC samples show ex-pression of viral oncoproteins (T antigens) that promote viral replication and tumorogenesis.

Rapidly Growing Merkel Cell Carcinoma after Biopsy / 대한피부과학회지

No abstract available.

A case of Merkel cell carcinoma of the head and neck

Merkel cell carcinoma (MCC) is a relatively rare and aggressive cutaneous neuroendocrine malignancy. It is characterized by high rates of recurrence and metastasis, both to regional lymph nodes and to distant locations. Its characteristic clinical manifestation is a single, painless, hard, erythematous nodule on a sun-exposed area, particularly in older men. Surgical management of both the primary site and the sentinel lymph node is the standard of care. In this article, we describe the diagnosis and treatment of a case of MCC in the left cheek.

Merkel cell carcinoma: A series of seven cases

BACKGROUND: Merkel cell carcinoma (MCC) is a rare neuroendocrine malignancy affecting the skin, for which timely diagnosis and aggressive treatment are essential. MCC has most often been reported in Caucasians, and case reports in Asians are rare. This study presents our experiences with the surgical treatment and radiotherapy of MCC in Asian patients. METHODS: We retrospectively reviewed the records of seven MCC patients between 2000 and 2018 from a single institution, and analyzed patient characteristics, tumor characteristics, surgical treatment, sentinel lymph node evaluation, reconstruction, adjuvant radiation therapy, and prognosis. RESULTS: Eight MCC lesions occurred in seven patients, most commonly in the head and neck region. All patients underwent surgical excision with reconstruction. The final surgical margin was 1.0 cm in most cases, and reconstruction was most commonly performed with a split-thickness skin graft. Five patients received adjuvant radiotherapy, and two patients received sentinel lymph node biopsy. During the follow-up period, three patients remained well, two died from other causes, one experienced recurrence, and one was lost to follow-up. CONCLUSIONS: We treated seven Asian MCC patients and our series confirmed that MCC is a very dangerous cancer in Asians as well. Based on our experiences, thorough surgical excision of MCC with histopathological clearance should be considered, with sentinel lymph node evaluation if necessary, followed by appropriate reconstruction and careful postoperative observation. Adjuvant radiation therapy is also recommended for all Asian MCC patients. The results of this case series may provide guidance for the treatment of Asian MCC patients in the future.

Two separate nodules of Merkel cell carcinoma occurring concomitantly on one cheek

Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroectodermal carcinoma arising from mechanoreceptor Merkel cells. Multiple MCCs are even rarer. We report a case of two independent MCCs simultaneously present in the cheek of a patient, which were effectively and esthetically treated using a cheek flap. Punch biopsy performed in a 60-year-old woman admitted with a chief complaint of two skin-colored hard nodules in her left cheek, accompanied by an itching sensation, was suggestive of MCC. Accordingly, we performed sentinel lymph node biopsy through the modified Blair incision under general anesthesia, in cooperation with the head and neck surgery department. The defect was covered with a cheek flap by slightly extending the existing incision following wide excision with a safety margin of 1 cm. This paper is significant in that it introduces an effective reconstruction technique that maintains function using a cheek flap for the management of this rare case. In addition, this paper is the first to classify multiple MCCs according to the time of onset. We believe that this paper presents an effective alternative reconstruction technique with sentinel node biopsy through the modified Blair incision.

Upper eyelid Merkel cell carcinoma treated with neoadjuvant chemotherapy and surgical excision

Merkel cell carcinoma is a rare cutaneous carcinoma, featured by an aggressive clinical course and a mortality rate of 28% at 2 years. A 71-year-old female was affected by a 4.1-cm-wide locally advanced Merkel cell carcinoma of the upper eyelid, previously misdiagnosed as chalazion, with involvement of the extraocular muscles. Although the tumor showed a macroscopic spontaneous regression in size after the incisional biopsy, the mass was treated with neoadjuvant chemotherapy and surgical excision. Good functional and aesthetic result with preservation of the eyeball and absence of tumor recurrence were achieved at 3-year follow-up. In our experience, the combination of the inflammatory cascade due to the incisional biopsy and neoadjuvant chemotherapy led to the regression of a locally advanced large Merkel cell carcinoma of the eyelid.

A Case of Merkel Cell Carcinoma on the Finger

No abstract available.

Merkel Cell Carcinoma of Cheek: 2 Case Reports / 대한이비인후과학회지

Merkel cell carcinoma (MCC) is a rare aggressive skin cancer with the mortality rate of 33%. MCC was first described by Toker in 1972 as a trabecular cell carcinoma and is classified as a neuroendocrine tumor similar to small cell lung carcinoma and melanoma. The pathogenesis of MCC remains largely unknown. However, ultraviolet radiation and immunosuppression are likely to play a significant pathogenic role. The primary skin lesion is usually asymptomatic and it typically presents as a red or purple dome-shaped nodule. The management of MCC is controversial, however, local wide excision followed by radiotherapy is accepted as the primary treatment modality. The regional draining nodal basin is the most common site for recurrence. Therefore, sentinel lymph node biopsy is recommended in all cases, except for the clinically node-negative cases. We herein report two cases of MCC on the left cheek with different clinical manifestations.

Merkel Cell Carcinoma Originating in a Setting of Pre-existing Bowen's Disease / 대한피부과학회지

No abstract available.

Poorly Differentiated Neuroendocrine Carcinoma of the Eyelid

PURPOSE: To report a case of poorly differentiated neuroendocrine carcinoma of the eyelid. CASE SUMMARY: A 70-year-old male presented with a 5-month history of a right upper eyelid mass. The mass appeared as 1.2 × 1.2 cm on the right upper eyelid. A mass excision was performed under frozen section control. The tumor was completely excised with a safety margin clearance and an upper eyelid reconstruction was performed. Histopathological examination revealed a tumor composed of small atypical cells which showed a high nuclear/cytoplasm ratio, nuclear molding, and increased mitotic activity. Immunohistochemical examination revealed positive reactivity for Ki-67, synaptophysin, CD56, and negative reactivity for chromogranin, cytokeratin 20, and thyroid transcription factor-1. CONCLUSIONS: Primary neuroendocrine carcinoma of the eyelid is extremely rare, but the tumor has high malignancy and readily metastasizes. Poorly differentiated neuroendocrine carcinoma should be considered in the differential diagnosis of a rapidly growing eyelid mass.