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 primary neoplasms 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

Related
pathway

hsa05165

Human papillomavirus infection

Gene

PTEN (mutation) [HSA:5728] [KO:K01110]
p53 (mutation) [HSA:7157] [KO:K04451]

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]

Other DBs

ICD-11:

2C70

ICD-10:

C51

MeSH:

D014846

Reference

PMID:12605639

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

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

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

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

Authors

Marjoniemi VM.

Title

Immunohistochemistry in gynaecological pathology: a review.

Journal

Pathology 36:109-19 (2004)
DOI:10.1080/00313020410001671948

Reference

PMID:11281434

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

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

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

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)
DOI:10.1097/00125480-200303000-00001

DISEASE: Vulvar cancer