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原发性纵隔脂肪肉瘤:24例临床病理分析

Primary mediastinal liposarcoma: clinicopathologic analysis of 24 cases.

作者信息

Hahn Hejin P, Fletcher Christopher D M

机构信息

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.

出版信息

Am J Surg Pathol. 2007 Dec;31(12):1868-74. doi: 10.1097/PAS.0b013e318093f925.

DOI:10.1097/PAS.0b013e318093f925
PMID:18043041
Abstract

Liposarcomas are rare in the mediastinum. Here, we report the clinicopathologic features of 24 cases of mediastinal liposarcoma. Patients included 13 males and 11 females, with an age range of 3 to 72 years (median 58). Nine tumors were located in the anterior mediastinum, 7 in the posterior mediastinum, 1 in the superior mediastinum, and the precise location was not specified in 7 cases. Of the anterior mediastinal tumors, 3 appeared to arise from the thymus. Tumors were well-circumscribed, multinodular masses and ranged in size from 2.2 to 61 cm in greatest dimension (median 16 cm). Histologic examination revealed that most of the cases were well-differentiated liposarcomas (10), followed by dedifferentiated liposarcomas (8), pleomorphic liposarcomas (4), and myxoid liposarcomas (2). Of the pleomorphic liposarcomas, 2 had areas that resembled myxofibrosarcoma with atypical hyperchromatic spindle cells in a myxoid stroma, but the focal presence of lipoblasts confirmed the diagnoses. Clinical follow-up was obtained in 15 cases (range 1 to 59 mo; median 26). Complete surgical excision was attempted in 13 patients; however, local recurrence was common (5 cases), including 1 patient whose tumor recurred twice. Eleven patients were alive with no evidence of disease at last follow-up (5 well-differentiated, 5 dedifferentiated, and 1 myxoid liposarcoma). Two patients developed distant metastases (dedifferentiated and pleomorphic liposarcoma). One patient was alive with disease (pleomorphic liposarcoma), and 2 died of disease (pleomorphic and dedifferentiated liposarcoma). Overall, mediastinal liposarcomas appear to be similar, in clinicopathologic terms, to liposarcomas arising in the retroperitoneum.

摘要

脂肪肉瘤在纵隔中较为罕见。在此,我们报告24例纵隔脂肪肉瘤的临床病理特征。患者包括13名男性和11名女性,年龄范围为3至72岁(中位数为58岁)。9个肿瘤位于前纵隔,7个位于后纵隔,1个位于上纵隔,7例未明确具体位置。在前纵隔肿瘤中,3个似乎起源于胸腺。肿瘤边界清晰,为多结节状肿块,最大直径为2.2至61厘米(中位数为16厘米)。组织学检查显示,大多数病例为高分化脂肪肉瘤(10例),其次是去分化脂肪肉瘤(8例)、多形性脂肪肉瘤(4例)和黏液样脂肪肉瘤(2例)。在多形性脂肪肉瘤中,2例有类似黏液纤维肉瘤的区域,在黏液样基质中有非典型深染梭形细胞,但脂肪母细胞的局灶性存在证实了诊断。15例患者获得了临床随访(范围为1至59个月;中位数为26个月)。13例患者尝试进行了完整的手术切除;然而,局部复发很常见(5例),包括1例肿瘤复发两次的患者。11例患者在最后一次随访时无疾病证据存活(5例高分化、5例去分化和1例黏液样脂肪肉瘤)。2例患者发生远处转移(去分化和多形性脂肪肉瘤)。1例患者带瘤存活(多形性脂肪肉瘤),2例患者死于疾病(多形性和去分化脂肪肉瘤)。总体而言,纵隔脂肪肉瘤在临床病理方面似乎与腹膜后脂肪肉瘤相似。

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