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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

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: 30773599Free PMC Article

Recent clinical studies

Etiology

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
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
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

Diagnosis

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
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
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
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

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
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

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
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
Vulvar basal cell carcinoma: an infrequently metastasizing neoplasm.
Perrone T, Twiggs LB, Adcock LL, Dehner LP
Int J Gynecol Pathol 1987;6(2):152-65. doi: 10.1097/00004347-198706000-00007. PMID: 3692670

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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