Merkel cell carcinoma: A clinicopathological study of 11 cases

Department of Dermatology, Hospital de Cruces, Barcaldo, Vizcaya, Spain.
Journal of the European Academy of Dermatology and Venereology (Impact Factor: 2.83). 10/2005; 19(5):546-51. DOI: 10.1111/j.1468-3083.2005.01224.x
Source: PubMed


To report our 12-year experience with Merkel cell carcinomas (MCCs) from a clinical and pathological point of view.
Eleven MCCs were diagnosed at our institution between 1991 and 2002.
A retrospective clinical, histopathological and immunohistochemical study was performed. Age, gender, location, size, stage, treatment and follow-up data were collected. Histopathological pattern and immunohistochemical study with CAM 5.2, cytokeratin 20 (CK20), CK7, Ber EP4, neurofilaments, synaptophysin, chromogranin, S100 protein, p53 protein, CD117, leucocyte common antigen (LCA) and Ki-67 were accomplished.
Six females and five males with a mean age of 82 years were identified. Tumours were located on the face (n = 6), extremities (n = 3) and trunk (n = 1). At diagnosis, one patient was in stage Ia, six in stage Ib, three in stage II and one in stage III. All but one patient experienced wide surgical excision of the tumour. Additional treatment consisted of lymph node dissection in two patients, radiotherapy in four patients and systemic chemotherapy in one patient. Local recurrence developed in five patients. Three patients died because of MCC after 14 months of follow-up. Intermediate-size round cell proliferation was found in all cases. Additional small-size cell pattern and trabecular pattern were observed in seven and six cases, respectively. Eccrine and squamous cell differentiation were found in three cases. A dot-like paranuclear pattern was observed in all cases with CAM 5.2 and neurofilaments, and in 89% of cases with CK20. Seventy-five per cent of cases reacted with Ber EP4, chromogranin and synaptophysin, 70% with p53, 22% with S100 protein, 55% with CD117 and none with LCA. Ki-67 was found in 75% of tumoral cells on average. Fifty per cent of MCCs reacted with CK7 and showed eccrine differentiation areas.
MCC is an aggressive neuroendocrine tumour of the elderly. Wide surgical excision is the recommended treatment. Lymph node dissection, adjuvant radiotherapy and chemotherapy decrease regional recurrences but have not been demonstrated to increase survival. Immunohistochemically, MCC is an epithelial tumour with neuroendocrine features.

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    • "Occasional studies have shown that patients with MCC treated with radiotherapy alone experience a high likelihood of obtaining infield disease control [21]. The role of chemotherapy is unclear [7] [8] [9] [10] [11]. "
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    ABSTRACT: Merkel cell carcinoma (MCC) is a rare, clinically aggressive primary cutaneous neuroendocrine carcinoma. The present series describes clinicopathological features of 16 MCCs diagnosed at a tertiary cancer referral center. Sixteen MCCs occurred in 10 men and 6 women (M/F = 1.6:1), between the ages 37 and 74 years (mean, 58.3; median, 58.6), commonly in lower extremities (7) (43.7%) and head and neck sites (5) (31.2%), followed by upper extremities (3) (18.7%) and abdominal wall (1). Tumor size varied from 0.5 to 9.9 cm. Histopathologically, most tumors were composed of round to oval cells, mostly arranged diffusely with hyperchromatic nuclei, including “sudden” pleomorphism in some tumors. Variable features included coexisting Bowen disease (2/16), along with squamous, pseudoglandular, and rhabdomyoblastic dedifferentiation, all in a single tumor. Immunohistochemically, tumor cells were positive for at least a single epithelial marker in all 16 cases (100%) cases, including CK20, mostly paranuclear “dot-like” (12/13, 92.3%); CK (8/9, 88.8%), AE1/AE3 (3/3, 100%), and CK7 (1/6, 16.6%), along with neuroendocrine markers (16/16, 100%), including synaptophysin (11/13, 84.6%), chromogranin (12/15, 80%), and CD56 (4/4, 100%). Among other immunohistochemical markers, positive CKIT/CD117 was positive in 3 of 3 tumors. Surgical resection was performed in 11 (100%) of 11 cases, with adjuvant chemotherapy offered in a single case. Two cases with large-sized tumors, along with another case developed lymph node metastasis, including 1 who later developed pulmonary metastasis. Two patients were free of disease and 2 were alive with disease. Merkel cell carcinomas exhibit a diverse histopathological spectrum, including coexisting Bowen disease and, rarely, rhabdomyoblastic dedifferentiation, in some cases. Optimal immunohistochemical markers include CK20, synaptophysin, chromogranin, and CD56 for a timely diagnosis. Surgical resection is the treatment mainstay. Large-sized tumors and MCCs showing dedifferentiation portend a relatively more aggressive clinical course. Other recent developments in this tumor are discussed herewith.
    Full-text · Article · Jul 2015 · Annals of Diagnostic Pathology
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    ABSTRACT: RESUMEN Antecedentes: el carcinoma de células de Merkel es un tumor cutáneo neuroendocrino agresivo, que aparece en áreas fotoexpuestas de pacientes ancianos como neoformaciones nodulares o placas. Objetivo: reportar la frecuencia y las características clínicas e histológicas del carcinoma de células de Merkel en la Unidad de Dermato-Oncología y del Departamento de Dermatopatología del Hospital General de México, en un periodo de 10 años. Material y método: estudio retrospectivo de tipo descriptivo. Se revisaron los reportes histopatológicos de la Unidad de Dermato-Onco-logía y del servicio de Dermatopatología entre 1999 y 2009. Para cada paciente se elaboró una ficha de registro. Se realizaron análisis descriptivos. Resultados: se diagnosticaron siete carcinomas de células de Merkel en el periodo estudiado, todos tenían fototipo cutáneo IV, la mayoría eran mujeres. Lo tumores se encontraron principalmente en el segmento cefálico y la mayor parte de las lesiones correspondió clínica-mente a nódulos eritematosos. El patrón histológico intermedio fue el más observado en los estudios histológicos. El tratamiento que se realizó en la mayoría de los pacientes fue resección local amplia. Conclusiones: en nuestra experiencia, el carcinoma de células de Merkel es un tumor raro, que aparece en ancianos. Se considera un tumor agresivo, aunque al momento del diagnóstico la mayoría se encuentre como enfermedad localizada. No se sospecha el diagnóstico de inicio. Se requieren estudios de inmunohistoquímica para diagnosticar los casos. No hay un consenso sobre el tratamiento. ABSTRACT Background: Merkel cell carcinoma is a very aggressive neuroedocrine skin tumor that occurs in areas involving elderly patients as nodular neoformations, or indurated plaques.
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    ABSTRACT: Merkel cell carcinoma is an uncommon but aggressive primary cutaneous neuroendocrine (small cell) carcinoma. There is ongoing debate regarding the optimal treatment of this disease. The early literature comprised small institutional studies with inherent weaknesses. Recent data have emerged from larger studies, including those from Australian institutions, that adds support to a multimodality approach as best practice. Despite this, the outcome for patients with unfavourable disease remains poor and in most series 25-30% of patients die as a direct result of Merkel cell carcinoma. The head and neck is the commonest site for presentation (50-60%) and wide excision (2-3 cm) of the primary lesion is usually recommended, although achieving this is often difficult within functional and cosmetic constraints. All clinically node-negative patients should be considered candidates for elective nodal treatment and those with clinical nodal disease should undergo nodal dissection and adjuvant radiotherapy. Recent evidence suggests that patients treated with surgery and adjuvant locoregional radiotherapy experience a better disease-free survival compared with those undergoing surgery alone. The role of platinum-based chemotherapy is evolving. The aim of this article is to discuss relevant issues in the management of a patient with Merkel cell carcinoma.
    No preview · Article · Sep 2006 · Australasian Journal of Dermatology
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