Multifocal Merkel's cell tumors associated with a cutaneous dysplasia syndrome

Abstract

Multifocal Merkel's cell tumors developed in a 24-year-old patient with an unusual cutaneous dysplasia syndrome. The neoplasms behaved in an aggressive fashion, with the development of multiple lymph node metastases. In this case, the dermatologic syndrome had features of both dyshidrotic ectodermal dysplasia and the basal cell nevus syndrome. In addition to Merkel's cell tumors, basal cell carcinomas and actinic elastosis were seen in sun-exposed skin sites. These findings, in conjunction with a known tendency for Merkel's cell tumors to arise in solar-damaged areas, suggest a pathogenetic relationship between these neoplasms and the underlying cutaneous syndrome in this patient.

Synchronous onset of multiple cutaneous neuroendocrine (Merkel cell) carcinomas localized to the scalp

“…23 They examined 30 cases of MCC that had BCC-like features and in all cases the MCC was surrounded by a mucinous stroma and/or Table 1. Immunohistochemical staining pattern of small round blue cell tumors of the skin 4,9,10,16,18,25,26 the tumor showed an artifactual retraction between the tumor cells and stroma. Furthermore, 27% of the cases displayed focal areas of peripheral palisading.…”

“…1,16 The majority of MCCs are solitary; however, there are a few case reports of multiple cutaneous lesions developing either in the local region or at distant sites during the course of the disease. 6,[8][9][10][11][12][13][14][15] An even more uncommon presentation, is when multiple tumors arise synchronously at onset; therefore, metastases from a visceral neuroendocrine carcinoma must be excluded. [6][7][8] The case present in this report is akin to the latter scenario.…”

Basal cell carcinoma with progression to metastatic neuroendocrine carcinoma

“…9,14,17,18 The association of BCC with MCC is a rarer occurrence; however, it is documented. 14,17–19,21,22 Cerroni et al 18 reported a case presenting with MCC, SCC and BCC arising at the same site. Another case report 22 noted the combination of MCC with BCC in a young patient diagnosed with hypohidrotic ectodermal dysplasia that is known to predispose towards actinic alterations.…”

“…14,17–19,21,22 Cerroni et al 18 reported a case presenting with MCC, SCC and BCC arising at the same site. Another case report 22 noted the combination of MCC with BCC in a young patient diagnosed with hypohidrotic ectodermal dysplasia that is known to predispose towards actinic alterations. The patient developed multiple neuroendocrine tumors in the sun-damaged skin, concomitantly with multiple BCC of the face.…”

Cytomorphological evaluation of a small round blue cell tumour of the head and neck

“…Rare associations include chronic lymphocytic leukaemia, 8 chronic arsenicism, 9 and ectodermal dysplasia. 10 Its usual locations are head, neck and the extremities. 11 The tumour is often indistinguishable from other skin cancers and may present clinically as an ulcerated nodule resembling basal cell or squamous cell carcinoma or an amelanotic form of malignant melanoma.…”