Cases reported "Vulvar Diseases"

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1/32. 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.
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ranking = 1
keywords = carcinoma
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2/32. 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.
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ranking = 0.55555555555556
keywords = carcinoma
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3/32. Malignant potential of gigantic condylomatous lesions of the vulva.

    A diagnostic and therapeutic approach in the identification of malignant lesions and the types of HPV in 11 patients with gigantic condylomatous vulvar protuberances is presented. Different histological types of squamous cell vulvar carcinoma have been found in 8 (72.7%) cases: condylomatous (4), verrucous (3) and basaloid type of the carcinoma (1). HPV type 16, confirmed in 5 cases, was most often present (4 condylomatous and 1 basaloid carcinoma types). Other types of HPV such as HPV-6 was detected in 3 cases of verrucous, type 11 in 2 cases of verrucous and condylomatous carcinoma and type 18 in 1 case of condylomatous carcinoma. Radical vulvectomy followed by bilateral inguinofemoral lymphadenectomy was performed in 4 patients with condylomatous carcinoma, hemivulvectomy in basaloid carcinoma whereas wide local excision was performed in the verrucous type of carcinoma. No patient died except 1 with condylomatous carcinoma in whom 6 positive lymph nodes were detected after the primary operation. The patient died 4 years later following 3 excisions of local recurrences.
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ranking = 1.0404393594143
keywords = carcinoma, squamous cell
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4/32. Squamous cell carcinoma arising in Hailey-Hailey disease of the vulva.

    A 61-year-old woman, who was known to have Hailey-Hailey disease, presented with increasing vulval soreness. biopsy showed vulval intraepithelial neoplasia (VIN) 3 and subsequent histology from a vulvectomy specimen showed extensive VIN with early invasive squamous cell carcinoma. This may be another example of chronic inflammation of the vulval area leading to the development of squamous cell carcinoma. However, in this case, chronic human papillomavirus may also have played a part, leading to VIN and reactivation of the Hailey-Hailey disease. We can find no previous reports of squamous cell carcinoma developing in the setting of Hailey-Hailey disease.
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ranking = 0.89909585602063
keywords = carcinoma, squamous cell
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5/32. hidradenitis suppurativa polyposa.

    A case of severe chronic hidradenitis suppurativa of the perineum complicated by disfiguring fibrous, polypoid lesions is presented. The patient, a 41-year-old woman, had a long history of axillary hidradenitis which subsequently involved the perineum. Draining sinuses, scars and large pendulous masses of the vulva developed over 10 years. Cutaneous scars, ridges, papules and large fibrous polyps were present. Deep clefts, sinuses, dense fibrous scars and foci of chronic inflammation were seen. Rarely, large fibrous polyps may develop in chronic hidradenitis suppurativa and may be due to chronic local lymphedema. Careful pathologic examination is necessary to exclude squamous cell carcinoma.
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ranking = 0.15155047052539
keywords = carcinoma, squamous cell
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6/32. Clear cell adenocarcinoma of the vulva arising in endometriosis. A case report.

    A clear cell carcinoma, originating from a focus of endometriosis in the vulva, in a 52-year-old woman, operated on eight years before for ovarian endometriosis is reported. Malignant transformation of extraovarian endometriosis is rare. To date only three cases that originated in the vulva have been reported.
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ranking = 0.55555555555556
keywords = carcinoma
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7/32. An uncommon cause of a destructive vulval lesion.

    We present a case of a 39-year-old woman with a vulval lesion, which macroscopically looked consistent with a fungating squamous cell carcinoma of the vulva. However, further investigations demonstrated a less common cause for this presentation.
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ranking = 0.15155047052539
keywords = carcinoma, squamous cell
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8/32. Pseudoepitheliomatous hyperplasia in lichen sclerosus of the vulva.

    Small tentacles or separated nests of squamous cells in the dermis are not uncommonly seen in long-standing vulvar lichen sclerosus (LS) associated with epidermal thickening. We recently encountered a case where separated nests of well-differentiated squamous cells in the dermis were difficult to distinguish from squamous cell carcinoma (SCC). Further biopsies showed similar nests originating from every hair follicle. We postulated a diagnosis of multifocal pseudoepitheliomatous hyperplasia (PEH) to explain this phenomenon. Because we could find no reference to PEH in the setting of LS, we reviewed the biopsies of 92 women with extragenital and vulvar LS with and without carcinoma to determine its frequency and histological appearance. The study population, which excluded the index case, comprised 10 women with extra-anogenital LS, 58 with vulvar LS without carcinoma, and 24 with vulvar LS with carcinoma. The presence of PEH, epidermal thickness, predominant dermal collagen change, degree of inflammation, and presence of fibrin and red blood cells were recorded. The presence or absence of lichen simplex chronicus (LSC), squamous cell hyperplasia (SCH), and differentiated vulvar intraepithelial neoplasia (VIN) were recorded. PEH was identified only in vulvar LS, where it was seen in 7/58 (12.1%) women without carcinoma, 1/24 (8.3%) with carcinoma, and 0/10 (0%) with extra-anogenital LS. Two forms of PEH were seen: predominantly epidermal 7/8 (87.5%) and predominantly follicular 1/8 (12.5%). PEH was associated with increased epidermal thickness, less dermal edema, more dermal inflammation, fresh fibrin, and red blood cell extravasation. In all cases, there was associated LSC, but there was no SCH or differentiated VIN. In conclusion, PEH may explain many of the cases of dermal tentacles and separated squamous nests in vulvar LS with LSC. The association with fresh fibrin and red blood cells suggests that PEH might be a reaction to tissue damage. PEH is distinguished from SCC by its lack of atypia, confinement to the abnormal collagen, and limited growth. The pathologist must be careful about making a diagnosis of PEH in LS with epidermal thickening, looking carefully for basal atypia and other features of differentiated VIN in the overlying epidermis or dermal proliferation. We do not know whether PEH occurs in differentiated VIN and, if it does, how it could be distinguished from SCC.
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ranking = 0.8284241043238
keywords = carcinoma, squamous cell
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9/32. Recurrent verruciform xanthoma of the vulva.

    Verruciform xanthoma is a rare, benign, mucocutaneous, nondestructive lesion characterized by proliferation of non-Langerhans lipid-rich histiocytes. We describe the clinical and pathologic findings in a 30-year-old female with recurrent verruciform xanthoma of the vulva 8 years after initial therapy. The differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma.
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ranking = 0.11111111111111
keywords = carcinoma
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10/32. Transitional cell carcinoma presenting as clitoral priapism.

    BACKGROUND: Clitoral priapism is an uncommon cause of clitoromegaly. It should be suspected in the absence of hirsuitism and the presence of clitoral engorgement, pain, and local irritation. CASE: A 48-year-old female had a straight catheterization of her bladder for a history of frequent urinary tract infections. She was noted to have a clitoral size of 5 x 2.5 cm along with the classic findings of priapism. She had an 8 x 10 cm pelvic mass that was biopsied and revealed transitional cell carcinoma with papillary squamous component. CONCLUSION: Clitoral priapism presents with clitoral engorgement in the absence of sexual stimulation. The most common etiologies include medications, pelvic tumors, blood dyscrasias, or retroperitoneal fibrosis. A thorough investigation is warranted to identify potential pelvic venous or lymphatic obstruction.
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ranking = 0.55555555555556
keywords = carcinoma
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