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女性生殖道肺泡软部肉瘤:9例报告并文献复习

Alveolar soft-part sarcoma of the female genital tract: a report of nine cases and review of the literature.

作者信息

Nielsen G P, Oliva E, Young R H, Rosenberg A E, Dickersin G R, Scully R E

机构信息

James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.

出版信息

Int J Gynecol Pathol. 1995 Oct;14(4):283-92. doi: 10.1097/00004347-199510000-00001.

Abstract

Nine alveolar soft-part sarcomas of the female genital tract (four previously reported) occurred in patients 14-38 (mean 29) years of age. The most common clinical presentation was abnormal uterine bleeding. Two tumors were located in the vagina, three in the cervix or lower uterine segment, three in the uterine corpus, and one in the broad ligament. The tumors were solid and well circumscribed, ranging in maximum dimension from 1 to 9.8 (average 3.6) cm and had the characteristic histologic features of alveolar soft-part sarcoma. Granules that were diastase resistant and positive by periodic acid-Schiff (PAS) were present in all the tumors, and PAS-positive, diastase-resistant crystals were present in eight of them. Follow-up information ranging from 9 months to 17 years (average 62 months) was available for all the patients. One patient died of metastatic disease 25 months after diagnosis; eight other patients had uneventful follow-up periods ranging from 9 months to 17 years (average 67 months). The differential diagnosis of these tumors is discussed and the literature reviewed.

摘要

9例女性生殖道肺泡软部肉瘤患者(4例曾有报道)年龄在14至38岁(平均29岁)之间。最常见的临床表现为子宫异常出血。2例肿瘤位于阴道,3例位于宫颈或子宫下段,3例位于子宫体,1例位于阔韧带。肿瘤质地坚实,边界清晰,最大直径为1至9.8厘米(平均3.6厘米),具有肺泡软部肉瘤的典型组织学特征。所有肿瘤中均存在对淀粉酶抵抗且过碘酸希夫染色(PAS)呈阳性的颗粒,其中8例存在PAS阳性、淀粉酶抵抗的晶体。所有患者均有9个月至17年(平均62个月)的随访信息。1例患者在诊断后25个月死于转移性疾病;其他8例患者随访期顺利,从9个月至17年(平均67个月)。本文讨论了这些肿瘤的鉴别诊断并回顾了相关文献。

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