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儿童混合型纵隔脂肪肉瘤:1例具有不寻常组织学特征的病例报告

Mediastinal liposarcoma of mixed type in childhood: a report of a case with unusual histologic features.

作者信息

Anand Rajan K D, Subbarao Kiran Chikkanahalli, Agarwala Sandeep, Gupta Siddhartha Datta

机构信息

Wilmer Eye Institute at the Johns Hopkins Hospital, Baltimore, MD 21287-9248, USA.

出版信息

Indian J Pathol Microbiol. 2010 Jul-Sep;53(3):525-8. doi: 10.4103/0377-4929.68297.

Abstract

We hereby report the occurrence of mediastinal liposarcoma in a 11-year-old female child. Dyspnea and wheezing of long-standing duration were the presenting complaints and a preoperative biopsy failed to yield the diagnosis. Histologic examination revealed heterogeneous areas with well-differentiated liposarcoma-like areas, areas resembling myxoid liposarcoma, and areas of dedifferentiation. Osteosarcomatous foci and whorled areas reminiscent of meningioma were identified in the dedifferentiated areas. Liposarcomas in the mediastinum are extremely rare tumors of childhood and the present case showed unusual histologic features. Complete surgical excision with clear surgical margins remains the primary modality of treatment, although chemotherapy and radiotherapy have been tried.

摘要

我们在此报告一名11岁女童发生纵隔脂肪肉瘤的病例。长期存在的呼吸困难和喘息是主要症状,术前活检未能明确诊断。组织学检查显示存在异质性区域,包括高分化脂肪肉瘤样区域、类似黏液样脂肪肉瘤的区域以及去分化区域。在去分化区域发现了骨肉瘤病灶和类似脑膜瘤的漩涡状区域。纵隔脂肪肉瘤是儿童期极为罕见的肿瘤,本病例具有不寻常的组织学特征。尽管已尝试化疗和放疗,但完整切除且手术切缘清晰仍是主要的治疗方式。

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