1983
DOI: 10.1001/archderm.119.5.409
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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 tendenc… Show more

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Cited by 11 publications
(10 citation statements)
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“…Merkel cell carcinomas (MCC), originally described as trabecular carcinomas in 1972 [40], are rare neuroendocrine malignancies of the skin that arise particularly in elderly individuals [14,18,35] and only exceptionally in children [7,8,31,44]. About half of all MCC are located in the head and neck region, and the eylids including the periorbital region are affected by almost one fifth of them [14,18,35,37].…”
Section: Introductionmentioning
confidence: 99%
“…Merkel cell carcinomas (MCC), originally described as trabecular carcinomas in 1972 [40], are rare neuroendocrine malignancies of the skin that arise particularly in elderly individuals [14,18,35] and only exceptionally in children [7,8,31,44]. About half of all MCC are located in the head and neck region, and the eylids including the periorbital region are affected by almost one fifth of them [14,18,35,37].…”
Section: Introductionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…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.…”
Section: Discussionmentioning
confidence: 99%
“…multiple sites, [6][7][8] or who subsequently developed multifocal cutaneous tumors during the course of their disease. 6,[8][9][10][11][12][13][14][15] We report a case of a 59-year-old white male who presented with abrupt onset of multiple small bluish papules on his frontal scalp. On low power, the tumor had the overall histological silhouette of a nodular basal cell carcinoma (BCC).…”
mentioning
confidence: 99%