A 73-year-old man with a Merkel cell carcinoma.
ABSTRACT A 73-year-old man had a firm node on his right upper arm, which was caused by a Merkel cell carcinoma (MCC). The diagnosis was made on the basis of characteristic histopathological findings and a surgical excision with wide margins followed. Twenty months later a considerable swelling manifested in the right armpit due to a metastasis. During a CT scan of the thorax and abdomen, 2 suspicious abnormalities were seen in the mesenterial adipose tissue of the left lower abdomen and left perirenal adipose tissue. A tissue sample of the last abnormality taken under CT guidance confirmed this to be a metastasis of the MCC. The patient was irradiated but chose not to have chemotherapy. He died 2 years after the diagnosis. Merkel cell carcinoma is a rare and aggressive malignant skin neoplasm. Early recognition facilitates cure of the disease. Treatment is multidisciplinary, but surgery, either alone or in combination with radiotherapy, forms the basis of treating both the localised and regionalized forms of the disease. Chemotherapy may be used in case of disseminated disease and has a reasonable, albeit temporary, effect. The 10-year survival rate varies from 20-70%, dependent on the stage of the disease.
SourceAvailable from: Masahiro Shuda[Show abstract] [Hide abstract]
ABSTRACT: Merkel cell carcinoma (MCC) is a rare but aggressive human skin cancer that typically affects elderly and immunosuppressed individuals, a feature suggestive of an infectious origin. We studied MCC samples by digital transcriptome subtraction and detected a fusion transcript between a previously undescribed virus T antigen and a human receptor tyrosine phosphatase. Further investigation led to identification and sequence analysis of the 5387-base-pair genome of a previously unknown polyomavirus that we call Merkel cell polyomavirus (MCV or MCPyV). MCV sequences were detected in 8 of 10 (80%) MCC tumors but only 5 of 59 (8%) control tissues from various body sites and 4 of 25 (16%) control skin tissues. In six of eight MCV-positive MCCs, viral DNA was integrated within the tumor genome in a clonal pattern, suggesting that MCV infection and integration preceded clonal expansion of the tumor cells. Thus, MCV may be a contributing factor in the pathogenesis of MCC.Science 03/2008; 319(5866):1096-100. DOI:10.1126/science.1152586 · 31.48 Impact Factor
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ABSTRACT: The purpose of this study was to determine the predictive value of lymphatic mapping with selective lymphadenectomy in patients with Merkel's cell carcinoma. Eight patients with biopsy proven Merkel's cell carcinoma underwent sentinel node biopsy. Lymphoscintigraphy was performed the day before surgery following intradermal injection of 74-111MBq of 99mTc-nanocolloid divided into four doses around the biopsy scar. Dynamic and static images were obtained. At least one sentinel node was visualized in all patients. The sentinel node was intra-operatively identified with the aid of a hand-held gamma probe in all cases and patent blue dye in six out of eight cases. During surgery, all sentinel nodes were successfully harvested. Metastatic cell deposits were subsequently identified in three patients (37.5%) and they underwent regional lymphadenectomy. No additional involved lymph nodes were identified. No recurrence has been reported in a median follow-up of 4.6 years (range: 8 months-10 years). In conclusion, sentinel node biopsy in patients with Merkel's cell carcinoma appears to be a reliable staging technique.European Journal of Surgical Oncology 03/2007; 33(1):119-22. DOI:10.1016/j.ejso.2006.09.004 · 2.89 Impact Factor
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ABSTRACT: Merkel cell carcinoma (MCC) is an aggressive neuroendocrine skin cancer with poorly characterized genetics. We performed high resolution comparative genomic hybridization on 25 MCC specimens using a high-density oligonucleotide microarray. Tumors frequently carried extra copies of chromosomes 1, 3q, 5p, and 6 and lost chromosomes 3p, 4, 5q, 7, 10, and 13. MCC tumors with less genomic aberration were associated with improved survival (P=0.04). Tumors from 13 of 22 MCC patients had detectable Merkel cell polyomavirus DNA, and these tumors had fewer genomic deletions. Three regions of genomic alteration were of particular interest: a deletion of 5q12-21 occurred in 26% of tumors, a deletion of 13q14-21 was recurrent in 26% of tumors and contains the well-characterized tumor suppressor RB1, and a previously unreported focal amplification at 1p34 was present in 39% of tumors and centers on L-Myc (MYCL1). L-Myc is related to the c-Myc proto-oncogene, has transforming activity, and is amplified in the closely related small cell lung cancer. Normal skin showed no L-Myc expression, whereas 4/4 MCC specimens tested expressed L-Myc RNA in relative proportion to the DNA copy number gain. These findings suggest several genes that may contribute to MCC pathogenesis, most notably L-Myc.Journal of Investigative Dermatology 12/2008; 129(6):1547-55. DOI:10.1038/jid.2008.365 · 6.37 Impact Factor