Cases reported "Vulvar Neoplasms"

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1/14. Pigmented hidrocystoma of the eccrine secretory coil in the vulva: clinicopathologic, immunohistochemical and ultrastructural studies.

    A case of pigmented hidrocystoma of eccrine secretory coil is presented. A 47-year-old woman had developed a bluish black small nodule in the anterior portion of the labium minor a few years before entry. Microscopically, the cyst was lined by eosinophilic columnar epithelium with abundant brownish granules. There was a vague suggestion of decapitation secretion focally in the epithelial layer of cuboidal cells. This layer expressed distinct reactivity against CA19-9 with no reactivity for human milk fat globule-1 (HMFG-1). These features demonstrated that the cyst was not of apocrine nature but of eccrine derivation. In addition, positive immunoreaction for cytokeratin (CK)7, CK8 and CK19 defined the cyst as originating from the secretory coil of the sweat gland. Ultrastructurally, melanosomes in various stages were identified in most of the epithelial cells. These findings suggest that the present case was a hidrocystoma of eccrine secretory coil with abnormal melanin accumulation.
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2/14. Microcystic adnexal carcinoma of the vulva.

    BACKGROUND: Microcystic adnexal carcinoma (MAC) is a subset of sweat gland carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr, and D. J. Santa Cruz (Cancer 1982;50:566-72). We report the first case of MAC occurring on the vulva and review the literature pertaining to this rare tumor. CASE: A 43-year-old multiparous black woman presented initially to Kings County Hospital Medical Center with a chief complaint of a vulvar lesion arising on the left labia majora which she had noted for 4 years prior to presentation. Aside from increasing paresthesia in the area, she denied any constitutional symptoms. Her past medical history was significant only for hyperthyroidism and mild hypertension and surgical history was noncontributory. Gynecologic history was unremarkable, with sporadic care over the last 20 years. physical examination revealed a 1.5 x 2.0-cm raised, well-circumscribed, firm mobile lesion on the left labia majora. It was noted to be yellow in color with the surrounding tissue being unremarkable in character. The remainder of her gynecologic examination and lymph node survey was unremarkable. Preoperative chest X ray was negative as was the CAT scan of the abdomen and pelvis. All laboratory values were within normal limits. A Pap smear done preoperatively was significant for atypical squamous and glandular cells of undetermined significance. Subsequent colposcopic examination of the cervix was remarkable for cervicitis and was adequate, with the entire transformation zone visualized. Both endocervical curettage and endometrial biopsy were normal. Initially, an excisional biopsy was performed with final pathology demonstrating microcystic adnexal carcinoma with positive surgical margins. She subsequently underwent a left radical hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time of surgery, the left labia majora was noted to be well healed, with a residual surgical scar easily discernible. No areas of discoloration were noted and digital palpation of the area was unremarkable. Microscopic residual tumor was noted; however, all surgical margins and lymph nodes were negative for tumor. Her postoperative course was unremarkable. The patient has continued to do well since the time of her surgery and is being followed conservatively. CONCLUSION: Radical vulvectomy should be performed when MAC occurs in the vulva to secure negative margins of resection. groin dissection should be reserved for cases in which the inguinal lymph nodes are clinically suspicious or in cases of tumor recurrence.
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3/14. diagnosis of a vulvar granular cell tumor by fine needle aspiration biopsy. A case report.

    BACKGROUND: granular cell tumor (GCT) (granular cell "myoblastoma") is an uncommon neoplasm that may mimic carcinoma both clinically and morphologically. Fine needle aspiration diagnosis of vulvar GNT has been described on only one prior occasion. CASE: A 74-year-old, black female presented with a mass in the left labia. Fine needle aspiration biopsy revealed rare intact cells; abundant, granular, cytoplasmic fragments; and bland, ovoid, stripped nuclei. The intact cells were arranged in loose aggregates. Each sampling was exquisitely painful to the patient despite the use of local anesthesia. CONCLUSION: Cytologists should be aware of the distinctive clinical and morphologic appearance of GCT. The cytologic findings of vulvar, GCT are identical to those described at other body sites. Definitive diagnosis before extirpation permits definitive therapy.
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4/14. Squamous cell vulvar carcinoma associated with Fanconi's anemia: a case report.

    Fanconi's anemia (FA) is a rare autosomal recessive syndrome associated with a strong predisposition to cancer, particularly squamous cell carcinoma (SCC) of various organs. A few cases of lower genital tract neoplasia have been described. We present a 14-year-old black girl with an advanced squamous cell vulvar carcinoma treated with cisplatin chemotherapy plus radiation therapy. The patient died because of fungal sepsis. polymerase chain reaction (PCR) was positive to human papillomavirus (HPV)-16. Vulvar carcinoma is a very rare condition in teenagers, but the association of Fanconi's anemia and SCC of many sites is common. Vulvar carcinoma when associated with Fanconi's anemia is a great treatment challenge.
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5/14. Successful treatment of bowenoid papulosis in a 9-year-old girl with vertically acquired human immunodeficiency virus.

    A 9-year-old black girl with vertically acquired human immunodeficiency virus (hiv) and no history of condyloma acuminata presented with a 4-year history of enlarging and spreading dark brown flat papules in the perineum. Some of the lesions were confluent and extended from the clitoris to the labia majora and posteriorly to the buttocks and perianal region. A biopsy of one of the lesions showed bowenoid features. Our patient had a normal Pap smear, but vaginal and cervical biopsy specimens revealed human papillomavirus type 16. Therapy with topical imiquimod cream every other day was started, but little improvement was noted after 2 months. Application of 25% podophyllin every 4 to 8 weeks was added, and improvement was noted within 1 month. After 1 year of treatment, the patient had complete resolution of all lesions, and she has had no further appearance of lesions. Our case emphasizes the need for increased awareness of the potential for development of bowenoid papulosis in hiv-positive children as well as the successful treatment of our patient with topical therapy alone.
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6/14. Elevated amount of human papillomavirus 31 dna in a squamous cell carcinoma developed from bowenoid papulosis.

    A 50-year-old woman presented with asymptomatic, multiple black macules on the genitalia for 6 months. She presented multiple, round to irregularly shaped, black macules on her labia major and perineum. A keratotic reddish nodule was found on one of the macules. The results of laboratory investigations showed pancytopenia and a decreased CD4/CD8 ratio. A lymphocyte stimulation test showed a decreased response. The histological examination of a biopsy specimen led us to the diagnosis of bowenoid papulosis (BP) for the macules and squamous cell carcinoma (SCC) for the nodule. The macules and nodule were removed successfully. An elevated amount of HPV dna was demonstrated in SCC compared with that of BP. The HPV dna in SCC and BP was shown to be HPV-31. These observations indicated that the elevated amount of HPV-31 dna could have led to the emergence of SCC from BP under the condition of decreased cellular immunity.
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7/14. Granular-cell tumors of the vulva: a report of three cases.

    Three black women were found to have granular-cell tumors on the vulva; in one, there were several additionally in other cutaneous sites. Although this benign neoplasm is uncommon on the vulva, it should be considered in the differential diagnosis of nodular lesions on the vulva. Granular-cell tumors in multiplicity occur frequently and are not a sign of metastatic disease. This lesion has predilection for blacks. Pseudocarcinomatous hyperplasia occurs typically in overlying squamous epithelium and may be misinterpreted as squamous-cell carcinoma.
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8/14. Kaposi's sarcoma associated with acquired immunodeficiency syndrome presenting as a vulval papilloma. A case report.

    A patient with acquired immunodeficiency syndrome (AIDS)-associated Kaposi's sarcoma presenting with a vulval neoplasm is reported. Although more common in black than white people, Kaposi's sarcoma infrequently presents with gynaecological symptoms or lesions. In this case report the management of Kaposi's sarcoma presenting as a vulval neoplasm in a young woman with a positive antibody test to hiv is discussed and the need to screen young women with vulval neoplasms for this infection is highlighted.
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9/14. Localized vulvar syringomas.

    Syringomas are benign appendageal tumors with differentiation toward the intraepidermal portion of the eccrine sweat duct. A 35-year-old black woman with lesions confined to the labia majora is presented. Syringomas involving the genitalia are rare, and usually the genital tumors are part of a more generalized distribution. A review of English literature showed only four reported cases of syringomas limited to the vulva. Vulvular syringomas present as multiple, bilateral, skin-colored papules over the labia majora that may or may not be pruritic. The lesions should therefore be considered in the differential diagnosis of multiple papular lesions of the vulva and are probably more common than is generally recognized.
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10/14. Extramammary Paget's disease of the vulva in a young, black woman. A case report with histogenic confirmation by immunostaining.

    Since the initial report, in 1901, by W. Dubreuilh of a vulvar lesion with the same histopathology as the eczematoid lesion found on the nipple and areola described seven years previously by Sir James Paget, fewer than 100 cases of this uncommon vulvar neoplasia have been reported. This disease process, with and without an accompanying adenocarcinoma, is usually found in postmenopausal Caucasian women. A 24-year-old, black woman gave a subjective history of having had a pruritic vulvar lesion for 18 months. Histologically this neoplasm proved to be extensive extramammary Paget's disease of the vulva with an accompanying adenocarcinoma involving the vulva and, unilaterally, the inguinal-femoral, pelvic and aortocaval lymphatics. Immunoperoxidase staining for carcinoembryonic antigen is valuable for differentiating this disease from similar vulvar lesions plus assessing surgical, chemotherapeutic and roentgenologic treatments for local, regional and distant disease.
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