1/73. 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 = adenocarcinoma (Clic here for more details about this article) |
2/73. 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 = 0.5keywords = adenocarcinoma (Clic here for more details about this article) |
3/73. 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 = 7keywords = adenocarcinoma (Clic here for more details about this article) |
4/73. Cloacogenic adenocarcinoma of the vulva.Primary adenocarcinoma of the vulva is rare, and cloagocenic adenocarcinoma of the vulva is extremely rare. Here we report a vulvar tumor characterized by columnar cells with prominent brush border and the presence of goblet cells and endocrine cells, presenting the tubulovillous pattern and mucin histochemistry of enteric adenocarcinoma. Electron microscopy verified a colon-like pattern. We suggest that cloacogenic carcinoma of the vulva arises from embryonic tissue, normally present in the vulvar introital area. In our case, a wide local excision was sufficient for radical cure.- - - - - - - - - - ranking = 3.5keywords = adenocarcinoma (Clic here for more details about this article) |
5/73. 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 = 0.5keywords = adenocarcinoma (Clic here for more details about this article) |
6/73. Extension of extramammary Paget disease of the vulva to the cervix.Extramammary Paget disease of the vulva was found in association with vulval adenocarcinoma in an elderly woman who also had a uterine prolapse. The characteristic histological appearances of extramammary Paget disease were masked by striking reactive changes in the squamous epithelium. Primary excision of both the intraepithelial and invasive disease appeared complete. However, a subsequent hysterectomy with repair of the prolapse revealed extramammary Paget disease in the upper vaginal mucosa and cervix, a finding which is very rarely described. Pathogenesis and diagnosis of extramammary Paget disease is discussed, with differential diagnosis and reference to immunohistochemical methods.- - - - - - - - - - ranking = 0.5keywords = adenocarcinoma (Clic here for more details about this article) |
7/73. Metachronous carcinoma of the vulva and fallopian tube.BACKGROUND: Metachronous carcinoma of the vulva and fallopian tube is an unusual co-occurrence of gynecological malignancies. A report of such a case that developed and recurred over a 7-year period is presented. CASE: A 53-year-old G3P3 female presented with a verrucous carcinoma of the vulva and a serous papillary adenocarcinoma of the left fallopian tube metachronously. To investigate a possible association between the co-occurrence of the rare neoplasms and factors associated with multiple gynecological malignancies, we analyzed the status of human papillomavirus infection and dna mismatch repair deficiency as indicated by microsatellite instability. All samples analyzed were negative for these factors. CONCLUSION: The present results support the possibility that metachronous carcinomas of the vulva and fallopian tube involve unknown etiological factors or arise independently.- - - - - - - - - - ranking = 0.5keywords = adenocarcinoma (Clic here for more details about this article) |
8/73. 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 = 0.5keywords = adenocarcinoma (Clic here for more details about this article) |
9/73. 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.- - - - - - - - - - ranking = 2keywords = adenocarcinoma (Clic here for more details about this article) |
10/73. radiotherapy with concurrent chemotherapy for vulvar adenocarcinoma associated with extramammary Paget's disease.BACKGROUND: There have been no reports of the efficacy of primary radiotherapy with concurrent chemotherapy for vulvar adenocarcinoma associated with extramammary Paget's disease. In this report we discuss the differential diagnosis and efficacy of treatment for this rare tumor. CASE: The patient was diagnosed as having vulvar carcinoma stage IVb. A dose of 40 Gy in 20 fractions was administered to the vulva, including the tumor mass, and the inguinal-femoral lymph nodes with concurrent systemic chemotherapy. Outside the radiated field, bone scintigraphy showed an increase in abnormal uptake images. However, in the radiated field, the tumors disappeared macroscopically, and bone scintigraphy also showed a disappearance of the abnormal uptake images. CONCLUSION: Within the radiated field, the efficacy was brought about by either radiotherapy only or by radiotherapy together with the sensitizing effects of chemotherapy. As for the chemotherapy itself, it was not effective in this case.- - - - - - - - - - ranking = 2.5keywords = adenocarcinoma (Clic here for more details about this article) |
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