Carcinosarcoma Ex Eccrine Spiradenoma of the Vulva: Report of the First Case
Department of Pathology, Fujian Tumor Hospital, Fuzhou, China.International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists (Impact Factor: 1.67). 04/2011; 30(3):301-5. DOI: 10.1097/PGP.0b013e3182055a3d
Carcinosarcoma is exceedingly rare in the vulva. We describe a case of carcinosarcoma in a 67-year-old female patient who presented with recent enlargement and pain of a vulval nodule noted for 15 years. The excised tumor showed intermixed carcinomatous (adenocarcinoma and anaplastic carcinoma) and sarcomatous elements (osteosarcoma, chondrosarcoma, and leiomyosarcoma), which focally merged with several lobules of typical eccrine spiradenoma. The inguinal lymph nodes showed metastasis of the carcinomatous component only. This case represents the first reported case of vulval carcinosarcoma of the skin adnexal origin, and has to be distinguished from sarcomatoid carcinoma of epidermal origin because of a probable more aggressive behavior.
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ABSTRACT: Malignant spiradenoma/cylindroma of the vulva is an extremely rare adnexal tumor. We report the clinicopathological features of a 58-year-old woman who presented with malignant spiradenoma/cylindroma originating in the vulva and metastasized to the inguinal lymph nodes. Surgical excision with adequate margins and lymph node dissection was performed. Sections from the case were stained with Periodic Acid Schiff stain before and after diastase. Immunohistochemical study of the case using antibodies to carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), cytokeratin5/6 (CK 5/6), p63, cytokeratin7 (CK 7), smooth muscle actin (SMA), and S100 were performed. Microscopic examination revealed that spiradenoma nodules were positive to EMA and CEA. However, the cylindroma lobules showed strong immunoreactivity to p63 and CK5/6, whereas both tumor components were negative to S100, SMA, and CK7. Malignant spiradenoma/cylindroma is a rare tumor with controversial histogenesis that should be considered in the differential diagnosis of primary adnexal carcinoma and secondary metastatic tumors in the vulva. Further studies on a wider cohort should be encouraged.
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ABSTRACT: Context.-Because the skin and modified mucosal surfaces of the vulvar region contain dense apocrine glands and anogenital mammary-like glands, in addition to eccrine glands and folliculosebaceous units, benign as well as malignant lesions derived from these adnexal structures are, not surprisingly, found in the vulva. However, their incidence occurring in the vulva has not been reported, to our knowledge. Objective.-To determine the incidence of various vulvar adnexal lesions. Design.-We performed a retrospective review (1978-2010) of the cases at our institution. Results.-A total of 189 vulvar adnexal lesions were identified. Most of these lesions were benign (133 of 189; 70%), with hidradenoma papilliferum being the most common, followed by syringoma and various types of cysts. Rare cases of tubular adenoma, poroma, spiradenoma, hidradenoma, cylindroma, sebaceoma, and trichoepithelioma were identified. Malignant adnexal neoplasms comprised the remaining 30% (56 of 189) of the cases. Extramammary Paget disease was the most common (49 of 56), and 29% (14 of 49) demonstrated an invasive component. Rare cases of basal cell carcinoma, sebaceous carcinoma, apocrine carcinoma, adenoid cystic carcinoma, and spiradenocarcinoma were identified. Conclusions.-In this retrospective review, we identified several benign entities that have not been previously reported on the vulva, namely pilomatricoma, poroma, spiradenoma, and sebaceoma. Hidradenoma papilliferum and extramammary Paget disease were the most common benign and malignant adnexal neoplasms, respectively. The spectrum of various vulvar adnexal lesions appears to reflect the frequency of the underlying glandular elements.
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