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WHO Classification of tumors of the vulva*

WHO Classification of tumors of the vulva*
Epithelial tumors
Squamous cell tumors and precursors  
Squamous intraepithelial lesions  
Low-grade squamous intraepithelial lesion 8077/0
High-grade squamous intraepithelial lesion 8077/2
Differentiated-type vulvar intraepithelial neoplasia 8071/2Δ
Squamous cell carcinoma 8070/3
Keratinizing 8071/3
Non-keratinizing 8072/3
Basaloid 8083/3
Warty 8051/3
Verrucous 8051/3
Basal cell carcinoma 8090/3
Benign squamous lesions  
Condyloma acuminatum  
Vestibular papilloma 8052/0
Seborrheic keratosis  
Keratoacanthoma  
Glandular tumors  
Paget disease 8542/3
Tumors arising from Bartholin and other specialized anogenital glands  
Bartholin gland carcinomas  
  • Adenocarcinoma
8140/3
  • Squamous cell carcinoma
8070/3
  • Adenosquamous carcinoma
8560/3
  • Adenoid cystic carcinoma
8200/3
  • Transitional cell carcinoma
8120/3
Adenocarcinoma of mammary gland type 8500/3
Adenocarcinoma of Skene gland origin 8140/3
Phyllodes tumor, malignant 9020/3
Adenocarcinomas of other types  
Adenocarcinoma of sweat gland type 8140/3
Adenocarcinoma of intestinal type 8140/3
Benign tumors and cysts  
Papillary hidradenoma 8405/0
Mixed tumor 8940/0
Fibroadenoma 9010/0
Adenoma 8140/0
Adenomyoma 8932/0
Bartholin gland cyst  
Nodular Bartholin gland hyperplasia  
Other vestibular gland cysts  
Other cysts  
Neuroendocrine tumors  
High-grade neuroendocrine carcinoma  
Small cell neuroendocrine carcinoma 8041/3
Large cell neuroendocrine carcinoma 8013/3
Merkel cell tumor 8247/3
Neuroectodermal tumors
Ewing sarcoma 9364/3
Soft tissue tumors
Benign tumors  
Lipoma 8850/0
Fibroepithelial stromal polyp  
Superficial angiomyxoma 8841/0Δ
Superficial myofibroblastoma 8825/0
Cellular angiofibroma 9160/0
Angiomyofibroblastoma 8826/0
Aggressive angiomyxoma 8841/0Δ
Leiomyoma 8890/0
Granular cell tumor 9580/0
Other benign tumors  
Malignant tumors  
Rhabdomyosarcoma  
Embryonal 8910/3
Alveolar 8920/3
Leiomyosarcoma 8890/3
Epithelioid sarcoma 8804/3
Alveolar soft part sarcoma 9581/3
Other sarcomas  
Liposarcoma 8850/3
Malignant peripheral nerve sheath tumor 9540/3
Kaposi sarcoma 9140/3
Fibrosarcoma 8810/3
Dermatofibrosarcoma protuberans 8832/1Δ
Melanocytic tumors
Melanocytic naevi  
Congenital melanocytic naevus 8761/0
Acquired melanocytic naevus 8720/0
Blue naevus 8780/0
Atypical melanocytic naevus of genital type 8720/0
Dysplastic melanocytic naevus 8727/0
Malignant melanoma 8720/3
Germ cell tumors
Yolk sac tumor 9071/3
Lymphoid and myeloid tumors
Lymphomas  
Myeloid neoplasms  
Secondary tumors
WHO: World Health Organization; IARC: International Agency for Research on Cancer.
* The morphology codes are from the International Classification of Diseases for Oncology (ICD-O).[1] Behavior is coded /0 for benign tumors; /1 for unspecified, borderline, or uncertain behavior; /2 for carcinoma in situ and grade III intraepithelial neoplasia; and /3 for malignant tumors.
¶ The classification is modified from the previous WHO classification of tumors,[2] taking into account changes in our understanding of these lesions.
Δ These new codes were approved by the IARC/WHO Committee for ICD-O in 2013.
References:
  1. Fritz A, Percy C, Jack A, et al. International Classification of Diseases for Oncology (ICD-O), 3rd ed, World Health Organization, Geneva 2000.
  2. Tavassoli FA, Devilee P. Pathology and Genetics of Tumours of the Breast and Female Genital Organs (IARC WHO Classification of Tumours). International Agency for Research on Cancer, Lyon 2003.
Reprinted from: World Health Organization Classification of Tumours of the Female Reproductive Organs, International Agency for Research on Cancer, Vol. 6, Kurman RJ, Carcangiu ML, Herrington S, Young RH, Tumours of the vulva, p. 230, Copyright © 2014.
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