Cases reported "Vulvar Neoplasms"

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1/851. Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study.

    We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type iv, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract. ( info)

2/851. lipoma of the preputium clitoridis in neonate: an exceptional abnormality different from ambiguous genitalia.

    A lipoma of the preputium clitoridis was found in a newborn girl. Ambiguous genitalia was first suspected. Surgery was successfully performed at 5 months. ( info)

3/851. Metastatic melanoma of the vulva identified by peritoneal fluid cytology.

    Malignant melanoma of the vulva is an uncommon disease, with a significant portion of cases demonstrating metastasis to inguinal lymph nodes with potential distal spread. Identification of such metastases often requires fine-needle aspiration or biopsy. The cytologic diagnosis of metastatic vulvar melanoma from peritoneal effusions has not been previously described. We present the case of a 54-yr-old woman who underwent en bloc radical vulvectomy with bilateral inguinal lymphadenectomy for melanoma of the right labium minora. No evidence of metastatic disease was identified, and all surgical margins were free of tumor. Despite chemotherapy, the patient returned approximately 2 yr later with abdominal pain and distention. Computed tomography revealed marked ascites and three hepatic lesions. Cytologic examination of the ascites revealed recurrent, metastatic melanoma. Although very rare, metastatic melanoma of the vulva may present as a malignant effusion. In such an event, the diagnosis may be rendered by exfoliative cytology. ( info)

4/851. Primary breast carcinoma of the vulva: a case report and literature review.

    BACKGROUND: In 1872, Hartung was the first to describe the case of a fully formed mammary gland arising in the left labium majora of a 30-year-old woman. Since Hartung's initial report, 38 additional cases of ectopic vulvar breast tissue have been described. This case report describes the rare occurrence of primary mammary adenocarcinoma arising within the vulva. CASE: A 64-year-old G4P4 white female presented with a 4-year history of a 2 x 1 cm firm, indurated, raised lesion of the left lateral mons. A wide local excision with ipsilateral inguinofemoral lymphadenectomy was performed. Given histological findings characteristic of both invasive ductal carcinoma and invasive lobular carcinoma, in conjunction with the presence of estrogen and progesterone receptors within the tumor, a diagnosis of infiltrating adenocarcinoma arising within ectopic breast tissue was made. CONCLUSIONS: Thirty-nine reported cases of ectopic breast tissue arising within the vulva have been reported in the world literature. Though the diagnosis of primary breast carcinoma arising within the vulva is based primarily upon histologic pattern, estrogen and progesterone receptor positivity provide supporting evidence. Given the rarity of this condition, guidelines for therapy are unavailable; we therefore suggest looking to the current management of breast cancer in order to establish a sensible approach. ( info)

5/851. Epithelioid sarcoma of the vulva.

    We report a case of a 23-year-old woman diagnosed as having an epithelioid sarcoma of the vulva. She was treated by a clitoris-sparing hemivulvectomy and lymph node sampling of the ipsilateral groin. Vulvar reconstruction was performed with a rectus abdominis myocutaneous flap. Four years after the operation there is no evidence of disease and the patient has a normal sex life. The English literature on this subject is reviewed with special attention to the biological behavior and therapeutic approach. ( info)

6/851. Laser ablation of recurrent Paget's disease of vulva and perineum.

    We describe the management by CO 2 laser of a case of recurrent extramammary Paget's disease of vulva and perineum, previously managed on three occasions with conventional surgical excision. Ablation of the whole lesion took less than 30 minutes, and the patient was able to go home on the third postoperative day. There were no significant complications. At follow-up after 12 months, multiple biopsies from the lasered area have shown no recurrence, and the symptomatic improvement has been satisfactory. ( info)

7/851. vulvodynia and vulvar vestibulitis: challenges in diagnosis and management.

    vulvodynia is a problem most family physicians can expect to encounter. It is a syndrome of unexplained vulvar pain, frequently accompanied by physical disabilities, limitation of daily activities, sexual dysfunction and psychologic distress. The patient's vulvar pain usually has an acute onset and, in most cases, becomes a chronic problem lasting months to years. The pain is often described as burning or stinging, or a feeling of rawness or irritation. vulvodynia may have multiple causes, with several subsets, including cyclic vulvovaginitis, vulvar vestibulitis syndrome, essential (dysesthetic) vulvodynia and vulvar dermatoses. Evaluation should include a thorough history and physical examination as well as cultures for bacteria and fungus, KOH microscopic examination and biopsy of any suspicious areas. Proper treatment mandates that the correct type of vulvodynia be identified. Depending on the specific diagnosis, treatment may include fluconazole, calcium citrate, tricyclic antidepressants, topical corticosteroids, physical therapy with biofeedback, surgery or laser therapy. Since vulvodynia is often a chronic condition, regular medical follow-up and referral to a support group are helpful for most patients. ( info)

8/851. Vulvar paraneoplastic amyloidosis with the appearance of a vulvar carcinoma.

    Nodular cutaneous amyloidosis of the vulva is a rare phenomenon. We describe a patient with localized nodular lesions on the vulva that mimicked kissing ulcers such as are seen with vulvar carcinoma. These lesions were a result of multiple myeloma with subsequent primary systemic amyloidosis. The patient died of cardiac and renal decompensation 2 months after diagnosis. ( info)

9/851. Epithelioid leiomyoma of the vulva.

    Smooth muscle tumors are uncommon lesions of the vulva and represent a variety of histologic types. When encountered, surgical treatment is guided by the malignant potential of the tumors. This article presents the case of a 45-year-old woman who underwent conservative excision of a 10-cm vulvar lesion consistent with benign epithelioid leiomyoma. This unusual case provides an opportunity to review the clinical and pathologic features of this uncommon variant of leiomyoma and to describe the recently suggested pathologic criteria for determining the malignant potential of smooth muscle tumors arising in the vulva. knowledge of these criteria can guide the clinician in selecting the appropriate management. ( info)

10/851. The 1396del A mutation and a missense mutation or a rare polymorphism of the WRN gene detected in a French Werner family with a severe phenotype and a case of an unusual vulvar cancer. Mutations in brief no. 136. Online.

    The Werner's syndrome (WS) is a rare recessive disease characterized by an early onset of geriatric disorders. The Werner's syndrome gene (WRN) recently cloned, encodes for an helicase and therefore plays a role in DNA metabolism and dna repair. Here, we report the study of a French family with two affected members and numerous cancers. Using the protein truncation test and sequencing, we identified a homozygous mutation in the WRN gene. This mutation generates a frame shift leading to a very short 391 amino acids truncated protein without the helicase motif. A particularly severe phenotype of the affected patient was associated with an unusual vulvar cancer traditionaly observed in elderly patients and therefore likely to be related to the Werner's syndrome. An additional substitution of G for A at nucleotidic position 1392 was also described. We suggest that a relation between genotype and phenotype could exist in the studied family. ( info)
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