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睾丸旁脂肪肉瘤:一项临床病理研究。

Paratesticular liposarcoma: a clinicopathologic study.

作者信息

Montgomery Elizabeth, Fisher Cyril

机构信息

Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland USA.

出版信息

Am J Surg Pathol. 2003 Jan;27(1):40-7. doi: 10.1097/00000478-200301000-00005.

Abstract

Paratesticular liposarcomas are rare and typically reported as isolated cases or as components of larger studies of liposarcomas. We studied a series of these tumors. All cases of paratesticular liposarcomas were retrieved from the archives of the Royal Marsden Hospital and the Johns Hopkins Hospital. Slides were reviewed and clinical information obtained. There were 30 paratesticular liposarcomas from men aged 41-87 years (mean 63 years; median 65 years) that involved the spermatic cord (23, 76%), testicular tunics (6, 20%), and epididymis (1, 4%). Tumors ranged from 3 to 30 cm (mean 11.7 cm; median 10 cm). Nineteen were well-differentiated liposarcomas (WDLs) and 10 were dedifferentiated liposarcomas (DDLs, five with high-grade and five with low-grade dedifferentiation). One was a myxoid/round cell liposarcoma with 70% round cell areas. All patients were treated by radical orchiectomy. One patient with WDL received radiation after his second recurrence and the myxoid/round cell liposarcoma received radiation and chemotherapy. Follow-up information was available for 16 of the patients, including 10 WDLs (range 24-216 months, mean 106 months), 5 DDLs (14-30 months, median 24 months), and for the myxoid/round cell liposarcoma (14 months) (range for all cases 14 months to 22 years; mean 87 months, median 36 months). Six of the WDLs (60%) recurred at 2, 4, 6, 10, 18, and 21 years (median 8 years). The lesion that recurred at 18 years (case no. 6) displayed foci of high-grade dedifferentiation in the recurrence, although the patient was disease free at 19 years. One patient with WDL had two recurrences at 4 and 7 years, and another had six recurrences over a 17-year period. Only one example of DDL recurred, at 30 months; another patient, who refused therapy for 15 years, had a primary tumor 30 cm in diameter, displayed pulmonary metastases 1 month after excision, and died after 14 months. The patient with MRCL had abdominal metastases after 1 year and was alive at 14 months. In summary, paratesticular WDL had a prolonged course with recurrences in more than half the cases, sometimes late. There were no metastases and the overall prognosis was good. One DDL recurred and only one of five (20%) developed metastases, but the mean follow-up for DDL was only 24 months.

摘要

睾丸旁脂肪肉瘤罕见,通常作为孤立病例报道,或作为脂肪肉瘤大型研究的组成部分。我们研究了一系列此类肿瘤。所有睾丸旁脂肪肉瘤病例均取自皇家马斯登医院和约翰霍普金斯医院的档案。对切片进行了复查并获取了临床信息。共有30例睾丸旁脂肪肉瘤,患者年龄在41至87岁之间(平均63岁;中位数65岁),肿瘤累及精索(23例,76%)、睾丸被膜(6例,20%)和附睾(1例,4%)。肿瘤大小从3厘米至30厘米不等(平均11.7厘米;中位数10厘米)。19例为高分化脂肪肉瘤(WDL),10例为去分化脂肪肉瘤(DDL,5例为高级别去分化,5例为低级别去分化)。1例为黏液样/圆形细胞脂肪肉瘤,圆形细胞区域占70%。所有患者均接受了根治性睾丸切除术。1例WDL患者在第二次复发后接受了放疗,黏液样/圆形细胞脂肪肉瘤患者接受了放疗和化疗。16例患者有随访信息,包括10例WDL(随访时间24至216个月,平均106个月)、5例DDL(14至30个月,中位数24个月)以及黏液样/圆形细胞脂肪肉瘤患者(14个月)(所有病例随访时间范围为14个月至22年;平均87个月,中位数36个月)。6例WDL(60%)在2年、4年、6年、10年、18年和21年复发(中位数8年)。18年复发的病变(病例6)在复发时显示高级别去分化灶,不过该患者在19年时无疾病。1例WDL患者在4年和7年复发两次,另1例在17年期间复发6次。只有1例DDL复发,时间为30个月;另1例患者拒绝治疗15年,其原发肿瘤直径30厘米,切除后1个月出现肺转移,14个月后死亡。黏液样/圆形细胞脂肪肉瘤患者1年后出现腹部转移,14个月时仍存活。总之,睾丸旁WDL病程较长,半数以上病例会复发,有时复发较晚。无转移情况,总体预后良好。1例DDL复发,5例中只有1例(20%)发生转移,但DDL的平均随访时间仅24个月。

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