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Diseases of the Vulva

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Practical Gynecologic Pathology

Part of the book series: Practical Anatomic Pathology ((PAP))

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Abstract

This chapter reviews the clinical and pathological features of common vulvar lesions and provides some diagnostic pearls focusing on the differential diagnosis and challenges encountered. Non-neoplastic diseases include genital infections, inflammatory dermatoses, benign pigmented lesions, etc. Squamous lesions are most frequently seen in the vulvar specimens and represent a broad spectrum of conditions. As elsewhere, neoplastic squamous lesions in the vulva may present as intraepithelial, noninvasive lesions in the early stages and, if left untreated, may progress to frankly invasive tumors. Vulvar intraepithelial lesions are known to develop along two distinct pathways, one is driven by human papillomavirus infection (HPV-associated) and the other has no association with HPV (HPV-independent). They have some different clinical and histological features. Vulvar squamous cell carcinoma is a rare and heterogeneous disease developed from intraepithelial neoplasia. Regardless of the tumor histology and etiology, surgery is the mainstay of staging and treatment for patients with resectable disease. Mesenchymal tumors of the vulva can be primarily divided into two groups: one group is relatively site-specific, including fibroepithelial stromal polyp, deep “aggressive” angiomyxoma, angiomyofibroblastoma, and cellular angiofibroma; the other group includes the tumors that more commonly occur at other body parts. Among the latter group, vulvar smooth muscle tumors have a unique set of criteria for predicting behavior. Despite its relatively low incidence, vulvar melanoma carries a poor prognosis since it is usually diagnosed at a later stage.

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Abbreviations

AGN:

Atypical genital nevi

AJCC:

The American Joint Committee on Cancer

CD34:

Cluster of designation 34

CDC:

Centers for disease control and prevention

CEA:

Carcinoembryonic antigen

CIN:

Cervix intraepithelial neoplasia

CK:

Cytokeratin

CMV:

Cytomegalovirus

CTLA-4:

Cytotoxic T-lymphocyte-associated protein 4

DNA:

Deoxyribonucleic acid

dVIN:

Differentiated vulvar intraepithelial neoplasia

EMA:

European Medicines Agency

EMPD:

Extramammary Paget disease

ER:

Estrogen receptor

FDA:

Food and Drug Administration

FFPE:

Formalin-fixed paraffin-embedded

FIGO:

The International Federation of Gynecology and Obstetrics

GCDFP15:

Gross cystic disease fluid protein 15

HMB45:

Human Melanoma Black 45

HMGA2:

High mobility group protein A2

HPV:

Human papillomavirus

HSIL:

High-grade squamous intraepithelial lesion

HSV:

Herpes simplex virus

ISH:

In situ hybridization

ISSVD:

International Society for the Study of Vulvovaginal Disease

LAST:

Lower Anogenital Squamous Terminology

LP:

Lichen planus

LS:

Lichen sclerosus

LSC:

Lichen simplex chronicus

LSIL:

Low-grade squamous intraepithelial lesion

MART-1:

Melanoma antigen recognized by T-cells-1

NOS:

Not otherwise specified

PD-1:

Programmed cell death protein 1

PR:

Progesterone receptor

Rb:

Retinoblastoma

RNA:

Ribonucleic acid

SATB2:

Special AT-rich sequence-binding protein 2

SCC:

Squamous cell carcinoma

SFT:

Solitary fibrous tumor

SMA:

Smooth muscle actin

SOX10:

SRY-related HMG-box 10

uVIN:

Usual-type vulvar intraepithelial neoplasia

VaIN:

Vagina intraepithelial neoplasia

VIN:

Vulvar intraepithelial neoplasia

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Authors and Affiliations

  1. Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA

    Gloria Zhang & Bin Yang

  2. Department of Obstetrics and Gynecology, Peking University People’s Hospital, Beijing, China

    Yun Zhao

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  1. Gloria Zhang
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    Jian-Jun Wei

  2. Department of Pathology, Yale School of Medicine, New Haven, CT, USA

    Pei Hui

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Zhang, G., Zhao, Y., Yang, B. (2021). Diseases of the Vulva. In: Wei, JJ., Hui, P. (eds) Practical Gynecologic Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-68608-6_1

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