Merkel cell carcinoma in situ

Ferringer, Tammie; Rogers, Hudson C.; Metcalf, John S.

doi:10.1111/j.0303-6987.2005.00270.x

Cited by 60 publications

(58 citation statements)

References 23 publications

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“…Recent findings from multiple authors have showed all combined Merkel cell carcinoma to be negative for Merkel cell polyomavirus by PCR and immunohistochemistry. 1,2,13,17,19 Occasional reports document unusual features of these variants such as poor paranuclear globular staining with CK20 47,56,57 and neurofilament negativity, 56,58 as seen in our cases, as well as ultrastructural and/or immunohistochemical absence of neurosecretory granules in the squamous components of the tumors. 39,41,42,44 Interestingly CK20 was diminished in two histopathologically undocumented cases reported by Bhatia, which had ‘lower viral abundance’.…”

Section: Discussionsupporting

confidence: 58%

Cutaneous squamous and neuroendocrine carcinoma: genetically and immunohistochemically different from Merkel cell carcinoma

et al. 2015

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Cutaneous neuroendocrine (Merkel cell) carcinoma most often arises de novo in the background of a clonally integrated virus, the Merkel cell polyomavirus, and is notable for positive expression of retinoblastoma 1 (RB1) protein and low expression of p53 compared with the rare Merkel cell polyomavirus-negative Merkel cell carcinomas. Combined squamous and Merkel cell tumors are consistently negative for Merkel cell polyomavirus. Little is known about their immunophenotypic or molecular profile. Herein, we studied 10 combined cutaneous squamous cell and neuroendocrine carcinomas for immunohistochemical expression of p53, retinoblastoma 1 protein, neurofilament, p63, and cytokeratin 20 (CK20). We compared mutation profiles of five combined Merkel cell carcinomas and seven ‘pure’ Merkel cell carcinomas using targeted next-generation sequencing. Combined tumors were from the head, trunk, and leg of Caucasian males and one female aged 52–89. All cases were highly p53- and p63-positive and neurofilament-negative in the squamous component, whereas RB1-negative in both components. Eight out of 10 were p53-positive, 3/10 p63-positive, and 3/10 focally neurofilament-positive in the neuroendocrine component. Six out of 10 were CK20-positive in any part. By next-generation sequencing, combined tumors were highly mutated, with an average of 48 mutations per megabase compared with pure tumors, which showed 1.25 mutations per megabase. RB1 and p53 mutations were identified in all five combined tumors. Combined tumors represent an immunophenotypically and genetically distinct variant of primary cutaneous neuroendocrine carcinomas, notable for a highly mutated genetic profile, significant p53 expression and/or mutation, absent RB1 expression in the context of increased RB1 mutation, and minimal neurofilament expression.

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Section: Discussionsupporting

confidence: 58%

Cutaneous squamous and neuroendocrine carcinoma: genetically and immunohistochemically different from Merkel cell carcinoma

et al. 2015

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“…Despite multiple theories, the exact cell of origin of MCC is not yet clearly defined. Hence, it was later hypothesised that the cell of origin might be pluripotent dermal stem cells 9. Some studies have shown the presence of B cell antigens, including TdT and PAX5, in MCC cells, and a new theory was raised that MCC was probably B cell derived 10…”

Section: History and Cell Of Originmentioning

confidence: 99%

Molecular characteristics and potential therapeutic targets in Merkel cell carcinoma

et al. 2016

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Merkel cell carcinoma (MCC) is an aggressive neuroendocrine skin tumour occurring preferentially in elderly and immunosuppressed individuals. Multiple studies have provided insight into the molecular alterations of MCC, leading to the design of several ongoing clinical trials testing chemotherapy, targeted therapy and immunotherapy in patients with recurrent or metastatic disease. The results of some of these studies are available, whereas others are eagerly awaited and will likely shed light on the understanding of MCC biology and potentially improve the clinical outcomes of patients with this rare disease.

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“…The Merkel cell was first described by Sigmund Merkel in 1875 as tastzellen in the skin with putative mechanoreceptor function, which are found in the basal layer of the epidermis 11 . It is not yet clarified, whether the malignant tumor originates from the Merkel cell itself or from a pluripotent dermal stem cell 12 . The tumors are composed of small basophilic, uniformly sized, highly mitotic malignant cells having round to oval nuclei and scanty cytoplasm.…”

mentioning

confidence: 99%

Prevalence of MCPyV in Merkel cell carcinoma and non‐MCC tumors

et al. 2009

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Merkel cell carcinoma in situ

Abstract: We report the third case of MCC in situ. These lesions have only been reported in association with squamous neoplasms on the extremities.

Cited by 60 publications

References 23 publications

Cutaneous squamous and neuroendocrine carcinoma: genetically and immunohistochemically different from Merkel cell carcinoma

Cutaneous squamous and neuroendocrine carcinoma: genetically and immunohistochemically different from Merkel cell carcinoma

Molecular characteristics and potential therapeutic targets in Merkel cell carcinoma

Prevalence of MCPyV in Merkel cell carcinoma and non‐MCC tumors

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