Background

These two areas of the women's genital tract are uncommon sites for disease.  The vulva is continuous with the skin and therefore, diseases of the skin often find a counterpart within the vulva.  The vagina is remarkably resistant to most diseases.  In fact, diseases that may affect the vulva and spread to the cervix, may completely bypass the vagina.  Pathologists, however, first identified the link with DES and a rare form of vaginal cancer. 

Extramammary Paget's Disease
Vagina-Carcinoma
Vestibular Papillae of the Vulva
Vulva-Adenocarcinoma
Vulva-Squamous Cell Carcinoma

OUTLINE

Histopathological Features and Variants
Commonly Used Terms
Internet Links

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
Inflammatory dermatoses of the vulva. Hammock LA, Barrett TL. Division of Dermatopathology, Johns Hopkins Hospital, Baltimore, MD, USA.	J Cutan Pathol. 2005 Oct;32(9):604-11. Abstract quote   Inflammatory, non-neoplastic epidermal alterations of the vulva can be correctly diagnosed using classification schemes applied to skin elsewhere on the body. A wide range of inflammatory disorders may occur on the vulva, and they may have a similar clinical presentation to HPV lesions. However, HPV is incurable and often is treated surgically. Accordingly, as inflammatory dermatoses commonly occur on the vulva and are often curable with topical therapy, an awareness of these entities and an ability to distinguish them from HPV are imperative.
VARIANTS
CHILDHOOD ASYMMETRIC LABIUM MAJUS ENLARGEMENT (CALME)
Childhood Asymmetric Labium Majus Enlargement: Mimicking a Neoplasm. Vargas SO, Kozakewich HP, Boyd TK, Ecklund K, Fishman SJ, Laufer MR, Perez-Atayde AR. From the Departments of *Pathology, daggerRadiology, daggerSurgery, and section signGynecology, Children's Hospital and Harvard Medical School, Boston, MA.	Am J Surg Pathol. 2005 Aug;29(8):1007-1016. Abstract quote   We report a distinctive lesion of the labium majus resected in 14 girls from 3.9 to 13.2 years of age. All presented with enlargement of 1 or occasionally both labia majora. Radiographic imaging and surgical exploration showed expansion of the labium majus without definable borders. Grossly, specimens consisted of fibro-fatty tissue from 2 to 8 cm in greatest dimension. Microscopic examination revealed the usual constituents of vulvar soft tissue, with expansion of the fibrous component. Sparsely to moderately cellular interconnected bands encircled lobules of fat, blood vessels, and nerves. The bands consisted of plump and occasionally stellate or round fibroblasts immersed in an abundant pale myxoid matrix containing thin collagen fibers. These fibrous bands merged with thinner denser fibrous septa simlar to those seen in the vulva from age-matched controls. Elastic stains showed variably abundant thin parallel elastic fibers. Fibroblasts were immunohistochemically positive for estrogen and progesterone receptors. Electron microscopy showed fibroblasts with dilated rough endoplasmic reticulum cisternae and prominent nuclear fibrous laminae; extracellular matrix contained precollagen, collagen, elastic fibers, and numerous proteoglycan granules. Cytogenetic analysis of 3 lesions revealed a normal karyotype. Recurrence was observed in 7 (50%) patients, and regression was observed in 1 whose recurrence was not reexcised. Over an 11-year period at Children's Hospital (Boston, MA), these lesions represented 22% of all pediatric vulvar soft tissue masses and 3% of all vulvar lesions biopsied. We conclude that "childhood asymmetric labium majus enlargement" is a distinctive clinicopathologic entity of pre- and early puberty. Recognition of this fairly common lesion is important, since it may clinically, radiographically, and histologically mimic an infiltrative neoplasm. Its occurrence at an age roughly coincident with the time of breast budding, capacity for spontaneous regression, histologic architecture and composition of elements native to the vulva, expression of hormone receptors, and normal karyotype suggest that it is an asymmetric physiologic enlargement in response to hormonal surges of pre- and early puberty.
PREPUBERTAL VULVAR FIBROMA
Distinctive Prepubertal Vulval Fibroma: A Hitherto Unrecognized Mesenchymal Tumor of Prepubertal Girls: Analysis of 11 Cases. Iwasa Y, Fletcher CD. From the daggerDepartment of Pathology, Brigham and Women's Hospital and Harvard University Medical School, Boston, MA; and *Kyoto University Hospital, Kyoto University, Kyoto, Japan.	Am J Surg Pathol. 2004 Dec;28(12):1601-1608. Abstract quote   Eleven cases of a distinctive previously unrecognized mesenchymal tumor that arises in the vulvar region in the prepubertal years are described. The tumors presented in the vulva (8 cases arising from labia majora) of prepubertal girls (range, 4-12 years; median, 8 years). The preoperative diagnoses were labial mass or swelling, not otherwise specified in 3 cases, hemangioma in 2 cases, lipoma in 2 cases, and lymphangioma and Bartholin cyst/lymphedema in 1 case each. The tumors were unilateral, ill defined, located in the submucosa or subcutaneous tissue, and ranged in size from 2.0 to 8.0 cm in maximum dimension. Microscopically, they were poorly marginated, hypocellular neoplasms composed of bland spindle-shaped cells in a variably collagenous to edematous or myxoid stroma, diffusely infiltrating between preexisting normal vascular, adipose, and neural tissues. No cytologic atypia was identified. Mitotic activity was minimal with no abnormal mitotic figures. The tumor cells were immunoreactive for CD34 (8 of 9 cases) but not for smooth muscle actin, desmin, and S-100 protein. Treatment was by local excision. From the follow-up data available for 9 patients, 3 patients showed locally recurrent tumor after 4, 6, and 13 months, respectively, of which the second case showed a second recurrence 18 months after the first excision. The term "prepubertal vulval fibroma" is suggested because it reflects the distinct features of this seemingly unique, previously uncharacterized, site- and age-specific mesenchymal tumor.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

DES-Diethylstilbestrol.  This hormone was used between 1938-1971 in pregnant women who had a threatened miscarriage.   Unfortunately, daughters of mothers who had been exposed to this drug are at increaed risk (although overall <0.14% of all DES exposed women) for developing a rare form of vaginal cancer termed clear cell adenocarcinoma. 

VIN/VAIN-Vulvar Intrapithelial Neoplasia (VIN) or Vaginal Intraepithelial Neoplasia (VAIN).  This is a grading system similar to CIN in the cervix.  It is graded low grade to high grade and is considered a significant risk factor for the development of invasive carcinoma. 

Basic Principles of Disease
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Last Updated April 27, 2006

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