KEGG   DISEASE: Vulvar cancerHelp
Entry
H00029                      Disease                                

Name
Vulvar cancer
Description
Vulvar cancer is a relatively uncommon malignancy, occurring at a rate of 2.2 per 100,000 women per year. Squamous cell carcinoma (SCC) is the most common type of vulvar cancer and is observed in 80% to 90% of cases. There are at least two quite different types of SCC of the vulva. The less common, accounting for about one-third of cases, occurs in relatively young women, is usually preceded by the undifferentiated form of vulvar squamous intraepithelial neoplasia (VIN) and is associated with high-risk human papillomavirus (HPV) infection; the more frequent form develops in elderly patients, is not commonly associated with undifferentiated VIN but is frequently associated with differentiated VIN, lichen sclerosus or squamous hyperplasia, and is generally not linked to HPV infection. Relatively little is known about molecular changes in the genesis of vulvar cancer. Some studies suggest that early p53 mutation may be a defining step only in HPV-negative tumors. A rather small study suggests that mutation in phosphatase and tensin homologue (PTEN) is an early change in a substantial subset of vulvar carcinomas.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of the breast and female genital organs
   H00029  Vulvar cancer
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 female genital organs
     2C70  Malignant neoplasms of vulva
      H00029  Vulvar cancer
BRITE hierarchy
Gene
PTEN (mutation) [HSA:5728] [KO:K01110]
p53 (mutation) [HSA:7157] [KO:K04451]
Carcinogen
Azathioprine [CPD:C06837]
Tobacco smoking and tobacco smoke
Pathogen
Human papillomavirus type 16 [GN:T40087]
Human papillomavirus type 18 [GN:T40088]
Drug
Imiquimod [DR:D02500]
Recombinant adsorbed quadrivalent human papillomavirus virus-like particle vaccine [DR:D10192]
Comment
ICD-O: 8070/3, Tumor type: Squamous cell carcinoma
Other DBs
ICD-11: 2C70
ICD-10: C51
MeSH: D014846
Reference
PMID:12605639 (description, gene, carcinogen, tumor type)
  Authors
Fox H, Wells M
  Title
Recent advances in the pathology of the vulva.
  Journal
Histopathology 42:209-16 (2003)
DOI:10.1046/j.1365-2559.2003.01578.x
Reference
PMID:19286150 (description, carcinogen)
  Authors
Lanneau GS, Argenta PA, Lanneau MS, Riffenburgh RH, Gold MA, McMeekin DS, Webster N, Judson PL
  Title
Vulvar cancer in young women: demographic features and outcome evaluation.
  Journal
Am J Obstet Gynecol 200:645.e1-5 (2009)
DOI:10.1016/j.ajog.2009.01.014
Reference
PMID:10955808 (gene, tumor type)
  Authors
Holway AH, Rieger-Christ KM, Miner WR, Cain JW, Dugan JM, Pezza JA, Silverman ML, Shapter A, McLellan R, Summerhayes IC.
  Title
Somatic mutation of PTEN in vulvar cancer.
  Journal
Clin Cancer Res 6:3228-35 (2000)
Reference
PMID:11156383 (gene, tumor type)
  Authors
Brooks LA, Tidy JA, Gusterson B, Hiller L, O'Nions J, Gasco M, Marin MC, Farrell PJ, Kaelin WG Jr, Crook T.
  Title
Preferential retention of codon 72 arginine p53 in squamous cell carcinomas of the vulva occurs in cancers positive and negative for human papillomavirus.
  Journal
Cancer Res 60:6875-7 (2000)
Reference
PMID:15203746 (marker, tumor type)
  Authors
Marjoniemi VM.
  Title
Immunohistochemistry in gynaecological pathology: a review.
  Journal
Pathology 36:109-19 (2004)
DOI:10.1080/00313020410001671948
Reference
PMID:11281434 (carcinogen)
  Authors
Severson J, Evans TY, Lee P, Chan T, Arany I, Tyring SK.
  Title
Human papillomavirus infections: epidemiology, pathogenesis, and therapy.
  Journal
J Cutan Med Surg 5:43-60 (2001)
DOI:10.1177/120347540100500110
Reference
PMID:1311142 (carcinogen)
  Authors
Daling JR, Sherman KJ, Hislop TG, Maden C, Mandelson MT, Beckmann AM, Weiss NS.
  Title
Cigarette smoking and the risk of anogenital cancer.
  Journal
Am J Epidemiol 135:180-9 (1992)
DOI:10.1093/oxfordjournals.aje.a116270
Reference
PMID:16062089 (carcinogen)
  Authors
Patel P, Moazzam N, Potti A, Mehdi SA.
  Title
Azathioprine induced hodgkin lymphoma: a case report and review of literature.
  Journal
Am J Clin Oncol 28:427-8 (2005)
DOI:10.1097/01.coc.0000168670.28659.8d
Reference
PMID:12605088 (marker)
  Authors
Nucci MR, Castrillon DH, Bai H, Quade BJ, Ince TA, Genest DR, Lee KR, Mutter GL, Crum CP.
  Title
Biomarkers in diagnostic obstetric and gynecologic pathology: a review.
  Journal
Adv Anat Pathol 10:55-68 (2003)
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