Cases reported "Vulvar Diseases"

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1/9. Giant condyloma acuminatum or buschke-lowenstein tumor: review of the literature and report of three cases treated by CO2 laser surgery. A long-term follow-up.

    Giant condyloma acuminatum or buschke-lowenstein tumor (TBL) is a rare disease that arises on the male and female external genitalia. TBL arises from the confluence of multiple condylomata acuminata and it is induced a Human Papillomavirus infection. TBL presents a clinical malignancy, but it is known to be a histologically benign tumor, even if it carries a risk of malignant transformation. It is advisable to treat it early. After a review of the literature, three cases of giant condyloma acuminatum, treated by combined CO2 Laser surgery excision-vaporization and which underwent a long-term follow-up are described.
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2/9. thalidomide in refractory vulvar ulcerations associated with Crohn's disease.

    We report a case of recurrent vulvar ulcerations developed on vegetations associated with Crohn's disease. Lesions responded to low-dose thalidomide treatment. A biopsy revealed a neutrophilic infiltrate with rare giant cells. Vulvar manifestations were consistent with metastatic Crohn's disease or with a reactive neutrophilic dermatitis. thalidomide reduces the activity of tumor necrosis factor (TNF) alpha by accelerating the degradation of its mRNA. Nowadays, it has become a promising drug in a broad variety of disorders in which TNF-alpha seems to play a pivotal role, including inflammatory bowel diseases. This is the first case report presenting the efficacy of thalidomide in the treatment of vulvar involvement in Crohn's disease.
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3/9. A case of condyloma acuminata with giant and multiple lesions on the vulva and breast: successfully treated with surgical operations.

    We report a case of a 24-year-old female patient with a history of giant and multiple lesions of condylomata acuminata on the vulva and breasts which were treated by surgical excision.
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4/9. A case of genital folliculosebaceous cystic hamartoma with an unique aggregated manifestation.

    Folliculosebaceous cystic hamartoma (FCH) is a recently recognized cutaneous hamartoma composed of follicular, sebaceous and mesenchymal components, and usually occurring on the head and neck. We describe herein a case of FCH with an unique aggregated manifestation in a genital lesion. The patient was a 40-year-old woman with a genital lesion composed of a pedunculated nodule, a dome-shaped nodule and a subcutaneous nodule, measuring 5 cm in the greatest dimension. The largest, pedunculated nodule was histologically composed of an infundibulo-cystic structure with follicular, sebaceous and mesenchymal elements accompanied by cystic structures of various sizes lined by stratified squamous epithelium and follicular germinative cells suggesting follicular cysts. The dome-shaped nodule consisted of anastomosing strands of epithelial cells with follicular components. The subcutaneous nodule had two components, an infundibulo-cystic structure and a cyst lined by squamous epithelium. In our case, the unusual clinical feature of large and multiple nodules was due to the presence of several prominent hamartomatous cystic structures with FCH. This is the third case of giant FCH. The clinical presentation and location of giant FCH is unusual.
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5/9. Giant lichenification of the vulva with marked ulcerations. A case report.

    A case of vulvar giant lichenification of Pautrier showed uncommon ulcerations in the center of hyperplastic verrucoid plaques. The diagnosis was made from the history, clinical findings and characteristic features on the skin biopsy. Other causes of vulvar ulcers, such as venereal disease, chronic bullous and autoimmune diseases, and neoplastic conditions were excluded. We treated the patient with systemic antihistamines, topical antiseptics and corticosteroids, and sublesional injections of triamcinolone. The itch-scratch cycle was interrupted by the patient's wearing cotton gloves at night. Complete healing of the ulcers and an improvement in the pruritus was achieved within 14 weeks.
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6/9. Nodular fasciitis (pseudosarcomatous fasciitis) of the vulva.

    Nodular fasciitis (pseudosarcomatous fasciitis) is a benign nonneoplastic connective tissue proliferation of unknown pathogenesis, which most commonly involves the upper limb subcutaneous fascia. Nodular fasciitis of the vulva is an entity rarely encountered by gynecologists or pathologists, but has considerable potential for misdiagnosis as sarcoma and for inappropriate treatment. The patient, a 32-year-old black female, presented with a 3-cm right labial mass, which was nodular, rubbery, and yellowish-white on section. Microscopically, there was an admixture of large mesenchymal cells, small blood vessels, and lymphocytes in a sparsely collagenous matrix. Other features included extravasated erythrocytes, intercellular clefts, and pseudocysts. Although mitoses were common (8 per 10 high power fields), there were no atypical mitoses and no bizarre tumor giant cells. The large mesenchymal cells were identified by electron microscopy as myofibroblasts, cells which abound in a variety of other reactive and non-neoplastic conditions. It is important not to misdiagnose nodular fasciitis as a vulvar sarcoma, because nodular fasciitis requires only simple excision and does not recur or metastasize.
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7/9. Recurrent mucinous adenocarcinoma of the ovary presenting as an inguino-labial hernia.

    We report a case of a 65-year-old woman who nine years previously had undergone total abdominal hysterectomy and bilateral salpingoophorectomy for a large ovarian cyst. During surgery the cyst had ruptured and some mucinous material had been spilled intraabdominally. Histopathological studies demonstrated the cyst to be a mucinous adenocarcinoma of low malignant potential. appendectomy had also been performed due to an enlarged appendix, which proved to be a mucocoele. The patient had been lost to subsequent follow-up. Her current presenting symptom was a giant inguino-labial hernia of 25 cm diameter with two small skin perforations leaking a gelatinous discharge. Subsequent laparotomy and inguinal exploration have disclosed herniated small intestine with an attached metastatic multicystic mucinous adenocarcinoma. This case represents a case of borderline mucinous adeno-carcinoma-pseudomyxoma peritonei recurring in a unique pattern as a huge inguino-labial hernia, and serves to emphasize the possible consequences of spillage of ovarian cyst contents during surgery.
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8/9. Nodular fasciitis of the vulva. Report of a case with immunohistochemical study.

    Nodular fasciitis (NF) is a tumor-like condition composed of fibroblasts and myofibroblasts, usually located in the subcutaneous tissue of the extremities. Histologically, it can mimic a myogenic sarcoma because of its hypercellularity, brisk mitotic rate and the presence of actin. NF of the vulva is rare with only eleven cases reported in the English literature. A 40 year-old woman developed a nodule in labium majus that was surgically excised. Microscopical examination revealed a typical example of NF with occasional multinucleated giant cells. The immunohistochemical study showed positivity for vimentin and muscle-specific actin in the spindle cells. The multinucleated cells stained with CD68. MIB-1 was positive in less than 5% of the cells which indicated a low proliferative activity. The patient has been followed for 5 years and no relapses occurred. The rarity of this lesion in the vulva and the potential difficulties for its diagnosis led us to report the clinical and immunohistochemical study of this case.
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9/9. Vulvar amyloidosis mimicking giant condylomata acuminata in a patient with multiple myeloma.

    We report a case of unusual cutaneous amyloidosis involving the vulva in a patient with multiple myeloma. Genital examination revealed a dense agglomeration of verrucous papules and pedunculated condyloma-like tumors. The correct diagnosis was established by immunohistochemical examinations that visualized large amounts of lambda light chains, whereas no reaction was detected for kappa light chains or human papilloma virus. In this way, the differential diagnosis of condylomata acuminata could be ruled out. Condyloma-like lesions have been described in patients suffering from multiple myeloma, but the present case is unusual because of the extensive involvement. Vulvar amyloidosis should be added to the list of possible presentations of myeloma-associated systemic amyloidoses.
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