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Vulvar Intraepithelial Neoplasia, HPV-Independent

MedGen UID:
233445
•Concept ID:
C1335968
•
Neoplastic Process
Synonyms: Differentiated VIN (vulvar intraepithelial neoplasia); Differentiated Vulvar Intraepithelial Neoplasia; Differentiated vulvar intraepithelial neoplasia; Simplex (Differentiated) Vulvar Intraepithelial Neoplasia; Vulvar Carcinoma In Situ of Simplex Type; Vulvar Intraepithelial Neoplasia, Differentiated; Vulvar Intraepithelial Neoplasia, Differentiated (Simplex); Vulvar Intraepithelial Neoplasia, Differentiated (Simplex) Type; Vulvar Intraepithelial Neoplasia, Differentiated Type; Vulvar Intraepithelial Neoplasia, Simplex
SNOMED CT: Differentiated vulvar intraepithelial neoplasia (816979008); Differentiated VIN (vulvar intraepithelial neoplasia) (816979008)

Definition

HPV-negative vulvar squamous intraepithelial proliferation characterized by atypia of the basal and parabasal keratinocytes in an otherwise well-differentiated epithelium. Despite its subtle appearance, it has a high risk of malignant transformation. (WHO 2020) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVulvar Intraepithelial Neoplasia, HPV-Independent

Professional guidelines

PubMed

Molecular Analysis of HPV-independent Primary Endometrial Squamous Cell Carcinoma Reveals TP53 and CDKN2A Comutations : A Clinicopathologic Analysis With Re-evaluation of Diagnostic Criteria.
Hopkins MR, Palsgrove DN, Ronnett BM, Vang R, Lin J, Murdock TA
Am J Surg Pathol 2022 Dec 1;46(12):1611-1622. Epub 2022 Sep 5 doi: 10.1097/PAS.0000000000001970. PMID: 36069815
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

Recent clinical studies

Etiology

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
HPV-independent, p53-wild-type vulvar intraepithelial neoplasia: a review of nomenclature and the journey to characterize verruciform and acanthotic precursor lesions of the vulva.
Parra-Herran C, Nucci MR, Singh N, Rakislova N, Howitt BE, Hoang L, Gilks CB, Bosse T, Watkins JC
Mod Pathol 2022 Oct;35(10):1317-1326. Epub 2022 Apr 18 doi: 10.1038/s41379-022-01079-7. PMID: 35437330
DEVIL, VAAD and vLSC constitute a spectrum of HPV-independent, p53-independent intra-epithelial neoplasia of the vulva.
Roy SF, Wong J, Le Page C, Tran-Thanh D, Barkati M, Pina A, Trinh VQ, Rahimi K
Histopathology 2021 Dec;79(6):975-988. Epub 2021 Sep 20 doi: 10.1111/his.14451. PMID: 34265101
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 and Anal Intraepithelial Neoplasia: Terminology, Diagnosis, and Ancillary Studies.
Yang EJ, Kong CS, Longacre TA
Adv Anat Pathol 2017 May;24(3):136-150. doi: 10.1097/PAP.0000000000000149. PMID: 28398952

Diagnosis

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
HPV-independent, p53-wild-type vulvar intraepithelial neoplasia: a review of nomenclature and the journey to characterize verruciform and acanthotic precursor lesions of the vulva.
Parra-Herran C, Nucci MR, Singh N, Rakislova N, Howitt BE, Hoang L, Gilks CB, Bosse T, Watkins JC
Mod Pathol 2022 Oct;35(10):1317-1326. Epub 2022 Apr 18 doi: 10.1038/s41379-022-01079-7. PMID: 35437330
Vulval squamous cell carcinoma and its precursors.
Singh N, Gilks CB
Histopathology 2020 Jan;76(1):128-138. doi: 10.1111/his.13989. PMID: 31846523
Human Papillomavirus-Independent Squamous Lesions of the Vulva.
Watkins JC
Surg Pathol Clin 2019 Jun;12(2):249-261. Epub 2019 Apr 5 doi: 10.1016/j.path.2019.01.001. PMID: 31097103
Vulvar and Anal Intraepithelial Neoplasia: Terminology, Diagnosis, and Ancillary Studies.
Yang EJ, Kong CS, Longacre TA
Adv Anat Pathol 2017 May;24(3):136-150. doi: 10.1097/PAP.0000000000000149. PMID: 28398952

Therapy

A Mimicker of Differentiated Vulvar Intraepithelial Neoplasia: Reactive Atypia From Noncompliance With Lichen Sclerosus Therapy.
Chaudhari AS, McFadden JR, Bentz J, Evans RH, Selim MA, Sriharan A
Am J Dermatopathol 2024 Aug 1;46(8):519-522. Epub 2024 Apr 23 doi: 10.1097/DAD.0000000000002712. PMID: 38648048
Is Vulvovaginal Lichen Planus Associated With Squamous Cell Carcinoma?
Day T, Otton G, Jaaback K, Weigner J, Scurry J
J Low Genit Tract Dis 2018 Apr;22(2):159-165. doi: 10.1097/LGT.0000000000000384. PMID: 29470358Free PMC Article

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
Vulval squamous cell carcinoma and its precursors.
Singh N, Gilks CB
Histopathology 2020 Jan;76(1):128-138. doi: 10.1111/his.13989. PMID: 31846523
Human Papillomavirus-Independent Squamous Lesions of the Vulva.
Watkins JC
Surg Pathol Clin 2019 Jun;12(2):249-261. Epub 2019 Apr 5 doi: 10.1016/j.path.2019.01.001. PMID: 31097103
Pathways of vulvar intraepithelial neoplasia and squamous cell carcinoma.
Del Pino M, Rodriguez-Carunchio L, Ordi J
Histopathology 2013 Jan;62(1):161-75. Epub 2012 Nov 27 doi: 10.1111/his.12034. PMID: 23190170

Clinical prediction guides

High-grade vulvar intraepithelial neoplasia: comprehensive characterization and long-term vulvar carcinoma risk.
Thuijs NB, van Beurden M, Duin S, Heideman DAM, Berkhof J, Steenbergen RDM, Bleeker MCG
Histopathology 2024 Jan;84(2):301-314. Epub 2023 Sep 19 doi: 10.1111/his.15050. PMID: 37726173
DEVIL, VAAD and vLSC constitute a spectrum of HPV-independent, p53-independent intra-epithelial neoplasia of the vulva.
Roy SF, Wong J, Le Page C, Tran-Thanh D, Barkati M, Pina A, Trinh VQ, Rahimi K
Histopathology 2021 Dec;79(6):975-988. Epub 2021 Sep 20 doi: 10.1111/his.14451. PMID: 34265101
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
HPV-independent Vulvar Squamous Cell Carcinoma is Associated With Significantly Worse Prognosis Compared With HPV-associated Tumors.
Allo G, Yap ML, Cuartero J, Milosevic M, Ferguson S, Mackay H, Kamel-Reid S, Weinreb I, Ghazarian D, Pintilie M, Clarke BA
Int J Gynecol Pathol 2020 Jul;39(4):391-399. doi: 10.1097/PGP.0000000000000620. PMID: 31274700
Differentiated Vulvar Intraepithelial Neoplasia-like and Lichen Sclerosus-like Lesions in HPV-associated Squamous Cell Carcinomas of the Vulva.
Rakislova N, Alemany L, Clavero O, Del Pino M, Saco A, Quirós B, Lloveras B, Alejo M, Halec G, Quint W, de Sanjosé S, Ordi J; VVAP study group
Am J Surg Pathol 2018 Jun;42(6):828-835. doi: 10.1097/PAS.0000000000001047. PMID: 29505429

Recent systematic reviews

Precursor lesions of vulvar squamous cell carcinoma - histology and biomarkers: A systematic review.
Dasgupta S, Ewing-Graham PC, Swagemakers SMA, van der Spek PJ, van Doorn HC, Noordhoek Hegt V, Koljenović S, van Kemenade FJ
Crit Rev Oncol Hematol 2020 Mar;147:102866. Epub 2020 Jan 15 doi: 10.1016/j.critrevonc.2020.102866. PMID: 32058913

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