Hahn Hejin P, Fletcher Christopher D M
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Am J Surg Pathol. 2007 Dec;31(12):1868-74. doi: 10.1097/PAS.0b013e318093f925.
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.
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。