1/39. Acquired vulvar lymphangioma mimicking genital warts. A case report and review of the literature.A 44-year-old female developed confluent, dusky red, pruritic labial papules clinically suspected to be genital warts. She had a long-standing history of Crohn's disease with vulvar fistulae. The papular eruption developed after several bouts of cellulitis in a region of vulvar lymphedema. Shave biopsy of a papule exhibited papillated epidermal hyperplasia overlying a dermis with a 'Swiss-cheese' appearance secondary to lymphedema and superficial ectatic thin-walled vascular spaces characteristic of lymphangiectasias. review of published cases reveals that acquired lymphangiomas often affect the vulva compared to other cutaneous sites and can be associated with surgery, radiation therapy, infection (e.g., erysipelas, tuberculosis), Crohn's disease, congenital dysplastic angiopathy and congenital lymphedema. Rather than translucent vesicles ('frog spawn') typical of extragenital cutaneous lymphangiomas, vulvar lymphangiomas often present as verrucous papules that can be mistaken for genital warts. In this case, we believe that the combination of vulvar Crohn's disease and recurrent cellulitis resulted in local lymphatic destruction, lymphedema and ultimately symptomatic lymphangiectasias that mimicked genital warts.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/39. 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 = 3keywords = dermis (Clic here for more details about this article) |
3/39. 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 = dermis (Clic here for more details about this article) |
4/39. Prognostic factors in Paget's disease of the vulva: a study of 21 cases.Twenty-one cases of vulvar Paget's disease were studied to assess possible prognostic indicators, including presence and depth of invasion, status of resection margins, tumor dna cell content, and immunoreactivity for p53 and estrogen receptor proteins. Immunostaining for cytokeratin 7 (CK7), cytokeratin 20 (CK20), and gross cystic disease fluid protein-15 (GCDFP) were also performed. patients were 45 to 82 years of age (mean, 66.9 years). Ten of 21 patients (47.6%) had invasive Paget's disease. Dermal invasion was < or = 1 mm in 7 of 10 cases and 2 mm, 3 mm, and 8 mm in the remaining three invasive tumors. Of the seven patients with minimally invasive Paget's disease (< or = 1 mm depth of invasion), five are alive with no evidence of disease, one died of an unrelated illness, and one is alive with biopsy-proven in situ Paget's disease, having refused operative treatment. Of the three patients with more than minimally invasive Paget's disease (> 1 mm), all had nodal metastases; one patient is alive with no evidence of disease, one died of undertermined causes, and one died of metastatic Paget's disease. The remaining 11 patients had Paget's disease confined to the epidermis and its adnexal structures. Seven of these patients were alive at last follow-up with no evidence of disease. Of the remaining four patients, one died of metastatic cervical cancer, one died of metastatic bladder cancer, one died of an unrelated illness, and one patient is alive with biopsy-proven in situ Paget's disease and awaiting operative treatment. Twenty of the 21 cases represented primary vulvar Paget's disease while one represented possible local spread from a cervical adenocarcinoma. The immunoprofiles were GCDFP /CK7 /CK20- in 14 cases, GCDFP /CK7 /CK20 in 4 cases, and GCDFP-/CK7 /CK20- in 2 cases. All tumors were estrogen receptor-negative. Immunostaining for p53 was positive in 16 tumors and negative in four tumors. Seven of 12 (58%) patients with positive margins experienced local recurrence of Paget's disease, while the disease recurred in 1 of 4 patients with negative margins. recurrence was observed in 3 of 5 patients with diploid tumors and in 4 of 10 patients with aneuploid tumors. Neither of these differences is statistically significant. This study supports the recognition of a category of minimally invasive vulvar Paget's disease that has a low risk of distant metastasis and death caused by disease. Status of surgical resection margins, tumor cell dna ploidy, estrogen receptor expression, and p53 immunoreactivity are not predictive of local recurrence.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/39. Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction.Two cases of extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor arising in unusual, superficial sites are reported. One tumor involved the vaginal wall of a 35-year-old woman, and the other neoplasm arose in the dermis of the vulva in a 28-year-old woman. The tumors showed characteristic microscopic features of Ewing's sarcoma/peripheral neuroectodermal tumor with nodular monotonous proliferations of undifferentiated, small, round, hyperchromatic cells with a low mitotic index. Rare rosette-like formations were apparent only in the vulvar neoplasm. The tumors displayed intense immunoreactivity in a membranous pattern for CD99, the cell surface glycoprotein encoded by the MIC2 gene. Genetically, the tumors expressed the EWS/FLI-1 chimeric transcript, derived from the t(11;22)(q24;q12) chromosomal translocation. Both patients had localized disease treated with wide local excision; one received postoperative chemotherapy, and the other received chemotherapy and radiotherapy. To date, 18 and 19 months after diagnosis, neither patient has had clinical evidence of local recurrence or metastasis. To our knowledge, these are the first reported cases of vaginal and vulvar Ewing's sarcoma/peripheral neuroectodermal tumor, confirmed with molecular genetic analysis, in the English literature.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
6/39. Two cases of vulval pigmented extramammary Paget's disease: histochemical and immunohistochemical studies.We describe two Japanese female patients with pigmented extramammary Paget's disease (EMPD); one patient had a dark brown plaque and the other had a reddish patch with a pigmented area, both affecting the vulval region. Histochemical and immunohistochemical examinations confirmed EMPD with melanocyte colonization; plump tumour cells with a large nucleus and pale cytoplasm that were positive for CAM 5.2 and CEA proliferated singly or in nests in the epidermis, and scattered among the tumour cells were many dendritic cells with a large amount of melanin that were positive for HMB-45 and S-100 protein. Fontana-Masson (FM) stain showed many positive cells with well-developed dendritic processes within and around tumour nests. Histochemical and immunohistochemical studies of non-pigmented EMPD cases on the same region showed that HMB-45 positive cells were sparse or not detected at all, and that also FM staining-positive cells were decreased or not detected, and their dendritic processes were poorly formed. The present study suggests that there might be heterogeneity in EMPD in terms of relationships between Paget's cells and melanocytes.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
7/39. Extramammary Paget disease with underlying hidradenoma papilliferum: guilt by association?Extramammary Paget disease (EMPD) is a heterogenous entity representing either an intraepidermal adenocarcinoma in situ with apocrine differentiation or an expression of underlying malignancy of the skin or of the intestinal or genitourinary tract. The coexistence of EMPD with a benign underlying hidradenoma papilliferumn (HP) is, however, exceptional. We present the case of a 79-year-old woman with diffuse and patchy gray-white lesions involving her left vulva as well as an underlying 0.7-cm asymptomatic firm nodule. Histologically, the epidermis and dermis showed features characteristic of EMPD and HP, respectively. Malignant transformation in HP giving rise to EMPD in the overlying epithelium has been reported. In our case, however, failure to demonstrate continuity between the two lesions together with the lack of cytologic atypia, mitoses, and necrosis in the HP lends additional support to the possibility that the HP is "innocent" and that its association with EMPD is thus coincidental. A common histogenetic derivation of these two lesions from the mammary-like glands or from related germinative cells in the epidermis is suggested to explain this rare presentation.- - - - - - - - - - ranking = 3keywords = dermis (Clic here for more details about this article) |
8/39. Primary, localized vulvar B-cell lymphoma expressing CD44 variant 6 but not cadherins. A case report.BACKGROUND: Primary extranodal lymphoma of the vulva is rare, with only 17 prior cases reported. Its immunohistochemical profile has not been characterized beyond B- or T-cell phenotype. CASE: A 64-year-old, white woman presented with a nontender enlargement of the right labium minus and labium majus. Bilateral vulvar punch biopsies revealed an infiltrate of neoplastic lymphocytes that filled the reticular dermis and extended down to the subcutaneous fat. lymphoma cells were positive for CD20 and expressed CD43 in an aberrant manner. The tumor was examined for adhesion protein expression. There was expression of CD44 standard and variant 6 but not of E-, N- or P-cadherin. No systemic spread of this rare lymphoma was evident after one year. CONCLUSION: Adhesion protein expression in primary vulvar lymphoma may have prognostic implications.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
9/39. Epithelioid blue naevus of the genital mucosa: report of four cases.Epithelioid blue naevi are an unusual cytological variant of blue naevus that have been recently described mostly in patients with the carney complex, although they may also occur in isolation. This variant of blue naevus is composed of melanin-laden polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive depth of dermal infiltration and, in contrast with the usual stromal changes in blue naevi, epithelioid blue naevi exhibit no dermal fibrosis. We describe four cases of epithelioid blue naevus located on the genital mucosa in four patients with no evidence of the carney complex. Three male patients showed an epithelioid blue naevus on the mucosa of the glans penis and a female patient had a lesion of the right labium minoris. Histopathologically, the lesions consisted of entirely intradermal melanocytic naevi composed mostly of heavily pigmented epithelioid melanocytes involving the dermis of the genital mucosa. Immunohistochemically, in all cases, epithelioid melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A and MiTF antibodies.- - - - - - - - - - ranking = 2keywords = dermis (Clic here for more details about this article) |
10/39. lymphangioma circumscriptum of the vulva mimicking genital wart: a case report and review of literature.lymphangioma circumscriptum (LC) is an uncommon dermatologic problem that rarely affects the vulva and it is considered to be a localised developmental defect of lymphatic tissue in the dermis. We report a case of vulval LC, clinically diagnosed as genital wart, in a 48-year-old woman without evidence of secondary lymphatic damage. The patient required extensive vulval surgery and there was no recurrence after 16 months.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
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