Fewer than 100 cases of Paget's disease located in the perianal skin have been reported since the extramammary location was first described in 1893. Two patients seen and treated in the authors' institutions with disease limited to the epidermis and its adnexae are described to illustrate the usual presentation and pathobiology of the disease. A staging classification based on the disease pathology has been developed from the cases reported in the literature and correlated with appropriate surgical treatment. Stage I disease treated with wide local excision promises unlimited survival, whereas the prognosis worsens for Stage II, with synchronous localized malignancies, and for Stages III and IV, with regional and distant metastatic disease, respectively.

Perianal Paget's disease. Classification and review of management: report of two cases.

A case of vulvar adenocarcinoma seen in a 42-year-old woman is reported. The tumor involved the right labium majus and diffusely spread into the surrounding soft tissues as well as the inguinal lymph nodes and was histologically composed of nests, cords, and tubular formations recalling an aggressive duct carcinoma of breast. Likewise, tumor cells exhibited positivity for common breast tumor markers, such as epithelial membrane antigen, carcinoembryonic antigen, and glandular keratins. The possible origin of the neoplasm from mammary ectopic tissue in vulva or from vulvar skin adnexa is briefly discussed.

Aggressive “Breast-like” Adenocarcinoma of Vulva

Primary vulvar adenocarcinomas are rare tumors, and their histogenesis is not fully understood. They are classified into extramammary Paget's disease, sweat gland carcinomas, and "breast-like" adenocarcinomas of the vulva. The latter resemble adenocarcinomas arising in the breast morphologically and immunophenotypically. Rare cases of adenocarcinoma with apocrine features have been reported, and whether these neoplasms originate from the "native apocrine" sweat glands or from "anogenital mammary-like" glands are still debatable. The presence of normal mammary-like glands in the vicinity of the tumor, the transitional malignant morphological features from normal mammary-like glands and the tumor, the breast-like histological features of the tumor, and the expression of estrogen and progesterone receptors generally suggest an origin from anogenital mammary-like glands. Absence of these features points toward native apocrine sweat glands as the source of these neoplasms. In this report, we present a patient who was initially diagnosed with Paget's disease of the right vulva, which was treated by hemi-vulvectomy, and who later presented with primary vulvar apocrine adenocarcinoma with metastasis to the inguinal lymph nodes and intranodal mucinous/colloidal differentiation: a feature, to the best of our knowledge, not reported before. We also reviewed the histogenesis of the vulvar adenocarcinomas, with emphasis on the morphological features that separate the tumors arising from the anogenital mammary-like glands in the vulva from those arising from the native vulvar sweat glands.

Vulvar apocrine adenocarcinoma: a case with nodal metastasis and intranodal mucinous differentiation.

Invasive Cancer of the Vulva

8 – Invasive Cancer of the Vulva

The cytologic features of a fine needle aspiration biopsy of lymph node metastases from a vulvar adenocarcinoma with apocrine differentiation are documented. Cytologic findings that suggested apocrine differentiation included extreme nuclear eccentricity, punctate eosinophilic cytoplasmic granules and moundlike protrusion of apical cytoplasm. The cytologic findings correlated well with the histologic and histochemical features of the primary vulvar adenocarcinoma and its lymph node metastases.

Belcik R

Papers

Apocrine adenocarcinoma of the vulva with associated Paget's disease. Diagnosis of lymph node metastases by fine needle aspiration biopsy.