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Vulvar Merkel Cell Carcinoma

MedGen UID:
925535
•Concept ID:
C4288003
•
Neoplastic Process

Definition

A Merkel cell carcinoma that arises from the vulva. It usually presents as a cutaneous nodular lesion. Most small cell neuroendocrine carcinomas of the vulva are Merkel cell carcinomas. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVulvar Merkel Cell Carcinoma

Professional guidelines

PubMed

The management of small-cell carcinomas of the gynecologic tract.
Cohen JG, Chan JK, Kapp DS
Curr Opin Oncol 2012 Sep;24(5):572-9. doi: 10.1097/CCO.0b013e3283565ed6. PMID: 22820410
Clinical applications of sentinel lymph-node biopsy for the staging and treatment of solid neoplasms.
Gipponi M
Minerva Chir 2005 Aug;60(4):217-33. PMID: 16166921

Recent clinical studies

Etiology

High-grade Neuroendocrine Carcinomas of the Vulva: A Clinicopathologic Study of 16 Cases.
Chen PP, Ramalingam P, Alvarado-Cabrero I, Euscher ED, Nagarajan P, Lawson BC, Malpica A
Am J Surg Pathol 2021 Mar 1;45(3):304-316. doi: 10.1097/PAS.0000000000001558. PMID: 32826525
Human oncoviruses: Mucocutaneous manifestations, pathogenesis, therapeutics, and prevention: Papillomaviruses and Merkel cell polyomavirus.
Haley CT, Mui UN, Vangipuram R, Rady PL, Tyring SK
J Am Acad Dermatol 2019 Jul;81(1):1-21. Epub 2018 Nov 28 doi: 10.1016/j.jaad.2018.09.062. PMID: 30502418
Oncogenic Papillomavirus and Polyomavirus in Water Environments: Is There a Potential for Waterborne Transmission?
Fratini M, Di Bonito P, La Rosa G
Food Environ Virol 2014 Mar;6(1):1-12. Epub 2013 Nov 30 doi: 10.1007/s12560-013-9134-0. PMID: 24293168
The management of small-cell carcinomas of the gynecologic tract.
Cohen JG, Chan JK, Kapp DS
Curr Opin Oncol 2012 Sep;24(5):572-9. doi: 10.1097/CCO.0b013e3283565ed6. PMID: 22820410
Bartholin's gland carcinoma, malignant melanoma and other rare tumours of the vulva.
Finan MA, Barre G
Best Pract Res Clin Obstet Gynaecol 2003 Aug;17(4):609-33. doi: 10.1016/s1521-6934(03)00039-7. PMID: 12965135

Diagnosis

High-grade Neuroendocrine Carcinomas of the Vulva: A Clinicopathologic Study of 16 Cases.
Chen PP, Ramalingam P, Alvarado-Cabrero I, Euscher ED, Nagarajan P, Lawson BC, Malpica A
Am J Surg Pathol 2021 Mar 1;45(3):304-316. doi: 10.1097/PAS.0000000000001558. PMID: 32826525
Human oncoviruses: Mucocutaneous manifestations, pathogenesis, therapeutics, and prevention: Papillomaviruses and Merkel cell polyomavirus.
Haley CT, Mui UN, Vangipuram R, Rady PL, Tyring SK
J Am Acad Dermatol 2019 Jul;81(1):1-21. Epub 2018 Nov 28 doi: 10.1016/j.jaad.2018.09.062. PMID: 30502418
The management of small-cell carcinomas of the gynecologic tract.
Cohen JG, Chan JK, Kapp DS
Curr Opin Oncol 2012 Sep;24(5):572-9. doi: 10.1097/CCO.0b013e3283565ed6. PMID: 22820410
Bartholin's gland carcinoma, malignant melanoma and other rare tumours of the vulva.
Finan MA, Barre G
Best Pract Res Clin Obstet Gynaecol 2003 Aug;17(4):609-33. doi: 10.1016/s1521-6934(03)00039-7. PMID: 12965135
Merkel cell (neuroendocrine) carcinoma of the vulva. A case report with immunohistochemical and ultrastructural findings and review of the literature.
Hierro I, Blanes A, Matilla A, Muñoz S, Vicioso L, Nogales FF
Pathol Res Pract 2000;196(7):503-9. doi: 10.1016/S0344-0338(00)80052-7. PMID: 10926328

Therapy

The management of small-cell carcinomas of the gynecologic tract.
Cohen JG, Chan JK, Kapp DS
Curr Opin Oncol 2012 Sep;24(5):572-9. doi: 10.1097/CCO.0b013e3283565ed6. PMID: 22820410
Primary vulvar Ewing's sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: a case report.
Boldorini R, Riboni F, Cristina S, Allegrini S, Valentini S, Muscarà M, Ruspa G
Pathol Res Pract 2010 Jul 15;206(7):476-9. Epub 2009 Aug 4 doi: 10.1016/j.prp.2009.07.006. PMID: 19656640

Prognosis

Human oncoviruses: Mucocutaneous manifestations, pathogenesis, therapeutics, and prevention: Papillomaviruses and Merkel cell polyomavirus.
Haley CT, Mui UN, Vangipuram R, Rady PL, Tyring SK
J Am Acad Dermatol 2019 Jul;81(1):1-21. Epub 2018 Nov 28 doi: 10.1016/j.jaad.2018.09.062. PMID: 30502418
The management of small-cell carcinomas of the gynecologic tract.
Cohen JG, Chan JK, Kapp DS
Curr Opin Oncol 2012 Sep;24(5):572-9. doi: 10.1097/CCO.0b013e3283565ed6. PMID: 22820410
Merkel cell (neuroendocrine) carcinoma of the vulva. A case report with immunohistochemical and ultrastructural findings and review of the literature.
Hierro I, Blanes A, Matilla A, Muñoz S, Vicioso L, Nogales FF
Pathol Res Pract 2000;196(7):503-9. doi: 10.1016/S0344-0338(00)80052-7. PMID: 10926328
Merkel cell carcinoma of the vulva.
Gil-Moreno A, Garcia-Jiménez A, González-Bosquet J, Esteller M, Castellví-Vives J, Martínez Palones JM, Xercavins J
Gynecol Oncol 1997 Mar;64(3):526-32. doi: 10.1006/gyno.1996.4575. PMID: 9062165
Merkel's cell (neuroendocrine) carcinoma of the vulva.
Chen KT
Cancer 1994 Apr 15;73(8):2186-91. doi: 10.1002/1097-0142(19940415)73:8<2186::aid-cncr2820730825>3.0.co;2-q. PMID: 8156524

Clinical prediction guides

High prevalence of p16 staining in malignant tumors.
De Wispelaere N, Rico SD, Bauer M, Luebke AM, Kluth M, Büscheck F, Hube-Magg C, Höflmayer D, Gorbokon N, Weidemann S, Möller K, Fraune C, Bernreuther C, Simon R, Kähler C, Menz A, Hinsch A, Jacobsen F, Lebok P, Clauditz T, Sauter G, Uhlig R, Wilczak W, Steurer S, Burandt E, Krech R, Dum D, Krech T, Marx A, Minner S
PLoS One 2022;17(7):e0262877. Epub 2022 Jul 21 doi: 10.1371/journal.pone.0262877. PMID: 35862385Free PMC Article
High-grade Neuroendocrine Carcinomas of the Vulva: A Clinicopathologic Study of 16 Cases.
Chen PP, Ramalingam P, Alvarado-Cabrero I, Euscher ED, Nagarajan P, Lawson BC, Malpica A
Am J Surg Pathol 2021 Mar 1;45(3):304-316. doi: 10.1097/PAS.0000000000001558. PMID: 32826525
Oncogenic Papillomavirus and Polyomavirus in Water Environments: Is There a Potential for Waterborne Transmission?
Fratini M, Di Bonito P, La Rosa G
Food Environ Virol 2014 Mar;6(1):1-12. Epub 2013 Nov 30 doi: 10.1007/s12560-013-9134-0. PMID: 24293168
Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study.
Albores-Saavedra J, Batich K, Chable-Montero F, Sagy N, Schwartz AM, Henson DE
J Cutan Pathol 2010 Jan;37(1):20-7. Epub 2009 Jul 23 doi: 10.1111/j.1600-0560.2009.01370.x. PMID: 19638070
Clinical applications of sentinel lymph-node biopsy for the staging and treatment of solid neoplasms.
Gipponi M
Minerva Chir 2005 Aug;60(4):217-33. PMID: 16166921

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