KEGG   DISEASE: Merkel cell carcinomaHelp
Entry
H01555                      Disease                                

Name
Merkel cell carcinoma
Description
Merkel cell carcinoma (MCC) is a cutaneous neuroendocrine malignancy that exhibits clinically aggressive features and is associated with a poor prognosis. Ultraviolet (UV) radiation may be a cause, and corresponds with the sites of these tumours, often on sun-exposed skin, mostly (94%) in the White population. UV light may exert local immunomodulating action as it decreases amount of epidermal T cells and Langerhans cells, inducing hapten tolerance. Additionally, UV- specific mutations in TP53 and Harvey (Ha)-RAS genes are present in some MCC cell lines. Recently, the polyomavirus group has been linked with MCC. The MCC polyomavirus (MCPyV) has been isolated from MCC tissue and thought to be present in up to 80% of MCC. Tumorigenesis likely is caused by a number of sequential steps from viral integration into host DNA, mutagenic events, and specific immune responses.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Skin cancers
   H01555  Merkel cell carcinoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of skin
     2C34  Cutaneous neuroendocrine carcinoma
      H01555  Merkel cell carcinoma
BRITE hierarchy
Gene
TP53 (mutation) [HSA:7157] [KO:K04451]
Ha-RAS (mutation) [HSA:3265] [KO:K02833]
Pathogen
Merkel cell polyomavirus (MCPyV) [GN:T40051]
Drug
Avelumab [DR:D10817]
Other DBs
ICD-11: 2C34
ICD-10: C44.3
MeSH: D015266
Reference
PMID:24811434 (description, gene, pathogen, marker)
  Authors
Czapiewski P, Biernat W
  Title
Merkel cell carcinoma - recent advances in the biology, diagnostics and treatment.
  Journal
Int J Biochem Cell Biol 53:536-46 (2014)
DOI:10.1016/j.biocel.2014.04.023
Reference
PMID:26009549 (description, pathogen)
  Authors
Prewett SL, Ajithkumar T
  Title
Merkel Cell Carcinoma: Current Management and Controversies.
  Journal
Clin Oncol (R Coll Radiol) 27:436-44 (2015)
DOI:10.1016/j.clon.2015.04.007
Reference
PMID:26716756 (description, pathogen, marker, drug)
  Authors
Miles BA, Goldenberg D
  Title
Merkel cell carcinoma: Do you know your guidelines?
  Journal
Head Neck 38:647-52 (2016)
DOI:10.1002/hed.24359
Reference
PMID:22234254 (drug)
  Authors
Schrama D, Ugurel S, Becker JC
  Title
Merkel cell carcinoma: recent insights and new treatment options.
  Journal
Curr Opin Oncol 24:141-9 (2012)
DOI:10.1097/CCO.0b013e32834fc9fe
Reference
PMID:23476782 (pathogen, drug)
  Authors
Desch L, Kunstfeld R
  Title
Merkel cell carcinoma: chemotherapy and emerging new therapeutic options.
  Journal
J Skin Cancer 2013:327150 (2013)
DOI:10.1155/2013/327150
Reference
PMID:27163912 (description, pathogen, marker)
  Authors
Oram CW, Bartus CL, Purcell SM
  Title
Merkel cell carcinoma: a review.
  Journal
Cutis 97:290-5 (2016)
LinkDB All DBs

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