Mucosal malignant melanoma - a clinical, oncological, pathological and genetic survey
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Mucosal malignant melanoma - a clinical, oncological, pathological and genetic survey. / Mikkelsen, Lauge H; Larsen, Ann-Cathrine; von Buchwald, Christian; Drzewiecki, Krzysztof T; Prause, Jan U; Heegaard, Steffen.
I: APMIS - Journal of Pathology, Microbiology and Immunology, Bind 124, Nr. 6, 06.2016, s. 475-486.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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T1 - Mucosal malignant melanoma - a clinical, oncological, pathological and genetic survey
AU - Mikkelsen, Lauge H
AU - Larsen, Ann-Cathrine
AU - von Buchwald, Christian
AU - Drzewiecki, Krzysztof T
AU - Prause, Jan U
AU - Heegaard, Steffen
N1 - © 2016 APMIS. Published by John Wiley & Sons Ltd.
PY - 2016/6
Y1 - 2016/6
N2 - Mucosal melanomas constitute 1.3% of all melanomas and they may develop in any mucosal membrane. Conjunctival melanomas (0.5/million/year) and melanomas in the sinonasal cavity (0.5/million/year) are the most common, followed by anorectal melanomas (0.4/million/year) and melanomas in the oral cavity (0.2/million/year). Anorectal melanoma occurs slightly more often in females, whereas oral melanoma has a male predilection. Mucosal melanoma most commonly develops in a patient's sixth or seventh decade of life, and no differences between races have been found except for sinonasal melanoma and conjunctival melanoma, which are very rare in Black people. The symptoms are not tumour-specific and are related to the organ system affected, and the disease is most often diagnosed at an advanced clinical stage. The diagnosis of a primary tumour is difficult, and metastatic cutaneous melanoma and choroidal melanoma must be excluded. Mutations in KIT are frequently found, while BRAF and NRAS mutations are rarely found - except in conjunctival melanomas that carry BRAF mutations. Mutations in the TERT promotor region are also found in mucosal melanomas. Complete surgical resection with free margins is the treatment of choice. The prognosis is poor, with the 5-year survival rate ranging from 0% (gastric melanoma) to 80% (conjunctival melanoma).
AB - Mucosal melanomas constitute 1.3% of all melanomas and they may develop in any mucosal membrane. Conjunctival melanomas (0.5/million/year) and melanomas in the sinonasal cavity (0.5/million/year) are the most common, followed by anorectal melanomas (0.4/million/year) and melanomas in the oral cavity (0.2/million/year). Anorectal melanoma occurs slightly more often in females, whereas oral melanoma has a male predilection. Mucosal melanoma most commonly develops in a patient's sixth or seventh decade of life, and no differences between races have been found except for sinonasal melanoma and conjunctival melanoma, which are very rare in Black people. The symptoms are not tumour-specific and are related to the organ system affected, and the disease is most often diagnosed at an advanced clinical stage. The diagnosis of a primary tumour is difficult, and metastatic cutaneous melanoma and choroidal melanoma must be excluded. Mutations in KIT are frequently found, while BRAF and NRAS mutations are rarely found - except in conjunctival melanomas that carry BRAF mutations. Mutations in the TERT promotor region are also found in mucosal melanomas. Complete surgical resection with free margins is the treatment of choice. The prognosis is poor, with the 5-year survival rate ranging from 0% (gastric melanoma) to 80% (conjunctival melanoma).
KW - Aged
KW - Conjunctival Neoplasms
KW - Female
KW - GTP Phosphohydrolases
KW - Humans
KW - Male
KW - Melanoma
KW - Membrane Proteins
KW - Middle Aged
KW - Mouth Neoplasms
KW - Mucous Membrane
KW - Mutation
KW - Nose Neoplasms
KW - Promoter Regions, Genetic
KW - Proto-Oncogene Proteins B-raf
KW - Proto-Oncogene Proteins c-kit
KW - Rectal Diseases
KW - Sex Factors
KW - Surgical Procedures, Operative
KW - Surveys and Questionnaires
KW - Survival Analysis
KW - Telomerase
U2 - 10.1111/apm.12529
DO - 10.1111/apm.12529
M3 - Journal article
C2 - 27004972
VL - 124
SP - 475
EP - 486
JO - A P M I S. Acta Pathologica, Microbiologica et Immunologica Scandinavica
JF - A P M I S. Acta Pathologica, Microbiologica et Immunologica Scandinavica
SN - 0903-4641
IS - 6
ER -
ID: 173940716