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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

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVulva basal cell carcinoma

Professional guidelines

PubMed

Diagnostic Criteria for Differentiated Vulvar Intraepithelial Neoplasia and Vulvar Aberrant Maturation.
Heller DS, Day T, Allbritton JI, Scurry J, Radici G, Welch K, Preti M; ISSVD Difficult Pathologic Diagnoses Committee
J Low Genit Tract Dis 2021 Jan 1;25(1):57-70. doi: 10.1097/LGT.0000000000000572. PMID: 33105449Free 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
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: 30773599Free PMC Article

Recent clinical studies

Etiology

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
Non-human-papillomavirus-related malignancies of the vulva: A clinicopathological study.
Furlan K, Rohra P, Mir F, Gattuso P
J Cutan Pathol 2020 Oct;47(10):917-922. Epub 2020 Jul 29 doi: 10.1111/cup.13768. PMID: 32511773
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
Vulvar basal cell carcinoma: retrospective study and review of literature.
de Giorgi V, Salvini C, Massi D, Raspollini MR, Carli P
Gynecol Oncol 2005 Apr;97(1):192-4. doi: 10.1016/j.ygyno.2004.12.008. PMID: 15790457
Basal cell carcinoma of the vulva: clinical features and treatment results in 28 patients.
Benedet JL, Miller DM, Ehlen TG, Bertrand MA
Obstet Gynecol 1997 Nov;90(5):765-8. doi: 10.1016/S0029-7844(97)00416-X. PMID: 9351761

Diagnosis

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
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
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: 31829526Free 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
Vulvar Neoplasms, Benign and Malignant.
Allbritton JI
Obstet Gynecol Clin North Am 2017 Sep;44(3):339-352. doi: 10.1016/j.ogc.2017.04.002. PMID: 28778635

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
Basal cell carcinoma of the vulva.
Piura B, Rabinovich A, Dgani R
J Surg Oncol 1999 Mar;70(3):172-6. doi: 10.1002/(sici)1096-9098(199903)70:3<172::aid-jso5>3.0.co;2-i. PMID: 10102347
Ki67 expression in lichen sclerosus of vulva in patients with and without associated squamous cell carcinoma.
Scurry J, Beshay V, Cohen C, Allen D
Histopathology 1998 May;32(5):399-404. doi: 10.1046/j.1365-2559.1998.00397.x. PMID: 9639113
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

Prognosis

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
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: 31829526Free 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
Vulvar cancer: a review for dermatologists.
Chokoeva AA, Tchernev G, Castelli E, Orlando E, Verma SB, Grebe M, Wollina U
Wien Med Wochenschr 2015 Apr;165(7-8):164-77. Epub 2015 May 1 doi: 10.1007/s10354-015-0354-9. PMID: 25930015
Mammary and extramammary Paget's disease.
Lopes Filho LL, Lopes IM, Lopes LR, Enokihara MM, Michalany AO, Matsunaga N
An Bras Dermatol 2015 Mar-Apr;90(2):225-31. doi: 10.1590/abd1806-4841.20153189. PMID: 25830993Free PMC Article

Clinical prediction guides

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
Clinicopathologic Diagnosis of Differentiated Vulvar Intraepithelial Neoplasia and Vulvar Aberrant Maturation.
Day T, Marzol A, Pagano R, Jaaback K, Scurry J
J Low Genit Tract Dis 2020 Oct;24(4):392-398. doi: 10.1097/LGT.0000000000000569. PMID: 32976294Free PMC Article
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
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

Recent systematic reviews

Basal cell carcinoma of the vulva: a case report and systematic review of the literature.
Renati S, Henderson C, Aluko A, Burgin S
Int J Dermatol 2019 Aug;58(8):892-902. Epub 2018 Dec 2 doi: 10.1111/ijd.14307. PMID: 30506682

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