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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

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
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
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

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
Light JG, Muñoz G, Council ML
Arch Dermatol Res 2023 Oct;315(8):2423-2425. Epub 2023 Apr 7 doi: 10.1007/s00403-023-02617-2. PMID: 37024690
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
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
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

Diagnosis

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
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
Michalski BM, Pfeifer JD, Mutch D, Council ML
Dermatol Surg 2021 Feb 1;47(2):174-183. doi: 10.1097/DSS.0000000000002584. PMID: 32947298
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
Allbritton JI
Obstet Gynecol Clin North Am 2017 Sep;44(3):339-352. doi: 10.1016/j.ogc.2017.04.002. PMID: 28778635

Therapy

Michalski BM, Pfeifer JD, Mutch D, Council ML
Dermatol Surg 2021 Feb 1;47(2):174-183. doi: 10.1097/DSS.0000000000002584. PMID: 32947298
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
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
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
Bollag W, Holdener EE
Ann Oncol 1992 Jul;3(7):513-26. doi: 10.1093/oxfordjournals.annonc.a058252. PMID: 1498071

Prognosis

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
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
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
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
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

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
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
Pleunis N, Schuurman MS, Van Rossum MM, Bulten J, Massuger LF, De Hullu JA, Van der Aa MA
Gynecol Oncol 2016 Sep;142(3):440-5. Epub 2016 Jun 24 doi: 10.1016/j.ygyno.2016.04.021. PMID: 27126004
Bollag W, Holdener EE
Ann Oncol 1992 Jul;3(7):513-26. doi: 10.1093/oxfordjournals.annonc.a058252. PMID: 1498071
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

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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