1/146. 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.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/146. 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.- - - - - - - - - - ranking = 25.093035841554keywords = sweat gland, sweat, gland (Clic here for more details about this article) |
3/146. Extramammary Paget's disease of the vulva: report of five cases and review of the literature.Five patients with vulvar extramammary Paget's disease (EMPD) are reported. They accounted for 4.4% of all vulvar malignancies diagnosed in the south of israel between 1961-1997. Mean age was 64.4 years and the predominant symptom was vulvar pruritus. Three patients had intra-epidermal lesions (managed by wide local excision or, at most, simple vulvectomy), one had a minimally invasive lesion (managed by simple vulvectomy) and one had EMPD with an underlying apocrine gland adenocarcinoma (managed by radical vulvectomy and bilateral groin dissection followed by pelvic radiotherapy). EMPD recurred in two patients: 1) local recurrence after simple hemivulvectomy for an intra-epidermal lesion was successfully treated by wide local excision; 2) widespread recurrence at distant skin sites after simple vulvectomy for a minimally invasive lesion was unsuccessfully treated with systemic chemotherapy. It is concluded that vulvar EMPD is an uncommon neoplasm that primarily affects postmenopausal women. Its histogenesis is uncertain and most commonly it is an intra-epidermal lesion. The high rate of recurrent disease remains a challenge for optimal management.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
4/146. Malignant myoepithelioma of the vulva resembling a rhabdoid tumour.AIMS: We report an example of malignant myoepithelioma of the vulva, which has not been hitherto described. We discuss the differential diagnosis and briefly review the literature. methods AND RESULTS: The lesion was found in an 81-year-old woman as an indolent 40 mm tumour. The neoplastic cells showed a myoid, spindled, epithelioid and plasmacytoid phenotype. Hyalinization of extracellular material and myxoid changes were present. There was a partly solid and microcystic pattern and a tight cohesiveness of cells was lacking. The circumscribed multinodular tumour somewhat resembled an extrarenal rhabdoid tumour, having large tumour cells with prominent nucleoli and large amounts of acidophilic cytoplasm. Immunohistochemically, the tumour cells were immunoreactive for cytokeratin, vimentin, muscle-specific actin, alpha-smooth muscle actin, and S100 protein, but not for desmin, epithelial membrane antigen, factor viii-related antigen, CD34 and CD31. CONCLUSIONS: The histological and cytomorphological appearance of the tumour well as the immunohistochemical findings suggest the diagnosis of malignant myoepithelioma, possibly derived from minor vestibulary glands or ectopic breast tissue. Differential diagnoses are, in particular, extrarenal rhabdoid tumour and 'proximal type' epithelioid sarcoma. Differentiation is important, because the tumours show a different behaviour and prognosis.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
5/146. Paget's disease of the vulva associated with local adenocarcinoma and previous breast adenocarcinoma: report of two cases.We report two women in whom vulval Paget's disease occurred in association with local adenocarcinoma and previous breast adenocarcinoma. The first patient presented at the age of 83 years with moist erythematous changes over the perineum and an indurated area near the anus. biopsy of the indurated area showed Paget's cells throughout the epidermis and, below, adenocarcinoma infiltrating the dermis. Ten years previously, she had undergone a left mastectomy for infiltrating ductal carcinoma of the breast. The second patient was diagnosed as having Paget's disease at the age of 74 years. A vulval biopsy showed Paget's cells in the epidermis but, in addition, there were changes suggestive of adenocarcinoma of the sweat glands. Her symptoms of vulval itching had started at the age of 45 years and had led to a simple vulvectomy at the age of 57 years. Retrospective review of this vulvectomy specimen showed Paget's disease. She had also previously been treated for infiltrating ductal adenocarcinoma of the breast and adenocarcinoma of the rectum. The management of Paget's disease is difficult because of its high recurrence rate and, as illustrated by our two cases, treatment is difficult if the patients are elderly and in poor general health.- - - - - - - - - - ranking = 25.093035841554keywords = sweat gland, sweat, gland (Clic here for more details about this article) |
6/146. Adenoid cystic carcinoma of Bartholin's gland: what is the optimal approach?AIMS AND methods: We report the case of a locally advanced adenoid cystic carcinoma of Bartholin's gland which required posterior pelvic exenteration, radical vulvectomy and ablation of the ischiorectal fossa in order to obtain tumour clearance with negative margins. Including this case, only three pelvectomies have been performed for this disease. This is the first reported case in which a controlateral unsuspected intravulvar metastasis was found on histology. RESULTS AND CONCLUSIONS: No consensus exists on the adequate surgical management of this particular disease. Nevertheless, a review of the literature and this reported case suggest that radical vulvectomy with negative margins should be preferred to wide local excision as the primary surgical procedure. It also suggests that inguinofemoral lymph-node dissection should only be performed when suspect lymph nodes are found at clinical examination.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
7/146. Recurrent squamous cell carcinoma of the Bartholin's duct treated with en bloc resection.Bartholin's gland carcinomas are a rare entity. A case of a recurrent Bartholin's gland carcinoma is described. These neoplasms have a myriad of treatment options for primary therapy but there is a paucity of information regarding treatment for a lethal recurrence. The patient's primary therapy consisted of an initial wide local excision followed by radiation therapy with chemosensitization. She was disease-free for 2 years before her recurrence. A novel treatment approach incorporating a mulitdisciplinary en bloc radical surgery is described. The patient is alive and well without evidence of disease at 22 months.- - - - - - - - - - ranking = 2keywords = gland (Clic here for more details about this article) |
8/146. Vulval squamous cell carcinoma arising in chronic hidradenitis suppurativa.hidradenitis suppurativa is a chronic inflammatory disease of the sweat glands and hair follicles which is rarely associated with squamous cell carcinoma (SCC). A case of vulval SCC complicating hidradenitis suppurativa is presented. In addition to being the first case to report the association of vulval SCC and hidradenitis suppurativa in the English language literature, it also illustrates the difficulty inherent in distinguishing between invasive SCC and pseudoepitheliomatous hyperplasia on histological examination. The success of wide local excision of the vulval lesion and primary closure without recourse to skin grafts, rotational flaps, or healing by secondary intention is demonstrated.- - - - - - - - - - ranking = 25.093035841554keywords = sweat gland, sweat, gland (Clic here for more details about this article) |
9/146. Multimodality therapy for carcinoma of the Bartholin gland.OBJECTIVE: The aim of this study was to report the value of chemoradiation in the management of cancers of the Bartholin gland. methods: Primary treatment consisting of 45-46 Gy teletherapy radiation to the vulva, pelvis, and groins in combination with 50 mg/m(2) of cisplatin and 1000 mg/m(2)/day of 5-fluorouracil for 5 days during the first and fifth weeks of irradiation was delivered, followed by interstitial implant or excision. RESULTS: Two patients were free of disease at 30 and 59 months following therapy. Both patients required myocutaneous flap closure, one after excision of tumor after radiation and one after radionecrosis at the implant site. CONCLUSIONS: Primary chemoradiation may allow continence-sparing therapy for women with primary carcinoma of the Bartholin gland.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
10/146. Sebaceous carcinoma of the vulva.Extraocular sebaceous carcinoma is an uncommon neoplasm usually localized on the head and neck. sebaceous glands are abundant on the vulva, but vulvar sebaceous carcinoma is an uncommon neoplasm. To our knowledge, there are only five previously reported cases of sebaceous carcinoma on this location. We report an additional case of vulvar sebaceous carcinoma associated with bowen's disease in the overlying epidermis. The patient also had bowenoid papulosis involving the skin of labia majora. We analyzed by immunohistochemistry, Southern blot hybridization, and polymerase chain reaction (PCR) techniques for the presence of dna of human papilloma viruses (HPVs) in the specimen of sebaceous carcinoma and in lesions of bowenoid papulosis. immunohistochemistry, Southern blot hybridization, and PCR studies in specimens of bowenoid papulosis lesions and sebaceous carcinoma did not detect dna of HPVs. A significant increase in intranuclear p53 staining was demonstrated in several areas of neoplastic aggregations of sebaceous carcinoma.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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