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Vulva basal cell carcinoma

MedGen UID:: 234481
•Concept ID:: C1336977
•: Neoplastic Process

Synonyms:	Basal Cell Carcinoma of the Vulva; basal cell carcinoma of the vulva; Basal cell carcinoma of vulva; Basal Cell Carcinoma of Vulva; basal cell carcinoma of vulva; mammalian vulva skin basal cell carcinoma; skin basal cell carcinoma of mammalian vulva; vulva basal cell carcinoma; vulvar basal cell cancer; Vulvar Basal Cell Carcinoma; vulvar basal cell carcinoma; Vulvar basal cell carcinoma
SNOMED CT:	Basal cell carcinoma of vulva (717731002)

Monarch Initiative:	MONDO:0002955
Orphanet:	ORPHA494451

Definition

A rare vulvar carcinoma characterized by a slowly growing ulcer or nodule which is histologically composed of demarcated nests of palisaded basal cells originating at the epidermal-dermal junction. Occasionally, the tumor may be extensively pigmented. Patients most commonly present with pruritus. The lesion is usually treated by local excision, although groin metastases have been reported. [from ORDO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVVulva basal cell carcinoma

Disease
- Neoplasm
  - Epithelial neoplasm
    - Carcinoma
      - Basal cell carcinoma
        Skin basal cell carcinoma
        Vulva basal cell carcinoma

Professional guidelines

PubMed

Vulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology.

Abu-Rustum NR, Yashar CM, Arend R, Barber E, Bradley K, Brooks R, Campos SM, Chino J, Chon HS, Crispens MA, Damast S, Fisher CM, Frederick P, Gaffney DK, Gaillard S, Giuntoli R II, Glaser S, Holmes J, Howitt BE, Kendra K, Lea J, Lee N, Mantia-Smaldone G, Mariani A, Mutch D, Nagel C, Nekhlyudov L, Podoll M, Rodabaugh K, Salani R, Schorge J, Siedel J, Sisodia R, Soliman P, Ueda S, Urban R, Wethington SL, Wyse E, Zanotti K, McMillian N, Espinosa S
J Natl Compr Canc Netw 2024 Mar;22(2):117-135. doi: 10.6004/jnccn.2024.0013. PMID: 38503056

Diagnosis and management of vulvar cancer: A review.

Tan A, Bieber AK, Stein JA, Pomeranz MK
J Am Acad Dermatol 2019 Dec;81(6):1387-1396. Epub 2019 Jul 23 doi: 10.1016/j.jaad.2019.07.055. PMID: 31349045

Vulvar basal cell carcinoma: clinical features and treatment outcomes from a tertiary care centre.

Namuduri RP, Lim TY, Yam PK, Gatsinga R, Lim-Tan SK, Chew SH, Koh MJ, Mansor S
Singapore Med J 2019 Sep;60(9):479-482. Epub 2019 Feb 18 doi: 10.11622/smedj.2019014. PMID: 30773599 Free PMC Article

See all (9)

Recent clinical studies

Etiology

Vulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology.

Classification of Vulvar Squamous Cell Carcinoma and Precursor Lesions by p16 and p53 Immunohistochemistry: Considerations, Caveats, and an Algorithmic Approach.

Yang H, Almadani N, Thompson EF, Tessier-Cloutier B, Chen J, Ho J, Senz J, McConechy MK, Chow C, Ta M, Cheng A, Karnezis A, Huvila J, McAlpine JN, Gilks B, Jamieson A, Hoang LN
Mod Pathol 2023 Jun;36(6):100145. Epub 2023 Feb 22 doi: 10.1016/j.modpat.2023.100145. PMID: 36828360

Vulvar Basal Cell Carcinoma: Clinical and Histopathologic Features.

Flipo R, Bani MA, Rejaibi S, Talhi N, Sastre-Garau X
Int J Gynecol Pathol 2022 Jan 1;41(1):86-92. doi: 10.1097/PGP.0000000000000771. PMID: 33770062

Major p53 immunohistochemical patterns in in situ and invasive squamous cell carcinomas of the vulva and correlation with TP53 mutation status.

Tessier-Cloutier B, Kortekaas KE, Thompson E, Pors J, Chen J, Ho J, Prentice LM, McConechy MK, Chow C, Proctor L, McAlpine JN, Huntsman DG, Gilks CB, Bosse T, Hoang LN
Mod Pathol 2020 Aug;33(8):1595-1605. Epub 2020 Mar 20 doi: 10.1038/s41379-020-0524-1. PMID: 32203095

Pigmented lesions of the vulva.

Venkatesan A
Dermatol Clin 2010 Oct;28(4):795-805. doi: 10.1016/j.det.2010.08.007. PMID: 20883921

See all (49)

Diagnosis

Vulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology.

Classification of Vulvar Squamous Cell Carcinoma and Precursor Lesions by p16 and p53 Immunohistochemistry: Considerations, Caveats, and an Algorithmic Approach.

Cancer of the Vulva: A Review.

Michalski BM, Pfeifer JD, Mutch D, Council ML
Dermatol Surg 2021 Feb 1;47(2):174-183. doi: 10.1097/DSS.0000000000002584. PMID: 32947298

Pigmented lesions of the vulva.

Venkatesan A
Dermatol Clin 2010 Oct;28(4):795-805. doi: 10.1016/j.det.2010.08.007. PMID: 20883921

Pigmented vulvar lesions.

Edwards L
Dermatol Ther 2010 Sep-Oct;23(5):449-57. doi: 10.1111/j.1529-8019.2010.01349.x. PMID: 20868400

See all (89)

Therapy

Cancer of the Vulva: A Review.

Michalski BM, Pfeifer JD, Mutch D, Council ML
Dermatol Surg 2021 Feb 1;47(2):174-183. doi: 10.1097/DSS.0000000000002584. PMID: 32947298

Ultraviolet radiation and cutaneous malignant melanoma.

Moan JE, Baturaite Z, Dahlback A, Porojnicu AC
Adv Exp Med Biol 2014;810:359-74. doi: 10.1007/978-1-4939-0437-2_20. PMID: 25207376

Retinoids in cancer prevention and therapy.

Bollag W, Holdener EE
Ann Oncol 1992 Jul;3(7):513-26. doi: 10.1093/oxfordjournals.annonc.a058252. PMID: 1498071

A review of lower genital intraepithelial neoplasia and the use of topical 5-fluorouracil.

Sillman FH, Sedlis A, Boyce JG
Obstet Gynecol Surv 1985 Apr;40(4):190-220. doi: 10.1097/00006254-198504000-00002. PMID: 2984615

Treatment of cancer of the vulva with high energy electrons.

Frischbier HJ, Thomsen K
Am J Obstet Gynecol 1971 Oct 1;111(3):431-5. doi: 10.1016/0002-9378(71)90790-3. PMID: 4999391

See all (12)

Prognosis

Classification of Vulvar Squamous Cell Carcinoma and Precursor Lesions by p16 and p53 Immunohistochemistry: Considerations, Caveats, and an Algorithmic Approach.

Major p53 immunohistochemical patterns in in situ and invasive squamous cell carcinomas of the vulva and correlation with TP53 mutation status.

Vulvar malignancies: an interdisciplinary perspective.

Wohlmuth C, Wohlmuth-Wieser I
J Dtsch Dermatol Ges 2019 Dec;17(12):1257-1276. Epub 2019 Dec 12 doi: 10.1111/ddg.13995. PMID: 31829526 Free PMC Article

Diagnosis and management of vulvar cancer: A review.

Tan A, Bieber AK, Stein JA, Pomeranz MK
J Am Acad Dermatol 2019 Dec;81(6):1387-1396. Epub 2019 Jul 23 doi: 10.1016/j.jaad.2019.07.055. PMID: 31349045

BASAL CELL CANCER OF THE VULVA.

SCHUELLER EF
Am J Obstet Gynecol 1965 Sep 15;93:199-208. doi: 10.1016/0002-9378(65)90659-9. PMID: 14336649

See all (44)

Clinical prediction guides

Interobserver Agreement Across Subspecialties for Diagnosis of Differentiated Vulvar Intraepithelial Neoplasia and Predictive Values of 20 Histologic Features.

Schechter SA, Chan MP, Muthusamy S, Skala SL, Wang GY
Arch Pathol Lab Med 2023 Dec 1;147(12):1431-1437. doi: 10.5858/arpa.2022-0088-OA. PMID: 36821250

Vulvar Basal Cell Carcinoma: Clinical and Histopathologic Features.

Flipo R, Bani MA, Rejaibi S, Talhi N, Sastre-Garau X
Int J Gynecol Pathol 2022 Jan 1;41(1):86-92. doi: 10.1097/PGP.0000000000000771. PMID: 33770062

Major p53 immunohistochemical patterns in in situ and invasive squamous cell carcinomas of the vulva and correlation with TP53 mutation status.

Basal cell carcinoma of the vulva with lymph node and skin metastasis--report of a case and review of 20 Japanese cases.

Mizushima J, Ohara K
J Dermatol 1995 Jan;22(1):36-42. PMID: 7897022

Retinoids in cancer prevention and therapy.

Bollag W, Holdener EE
Ann Oncol 1992 Jul;3(7):513-26. doi: 10.1093/oxfordjournals.annonc.a058252. PMID: 1498071

See all (21)