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不寻常的腹膜后软组织肉瘤:巨大的伪装者。

Unusual Retroperitoneal Soft Tissue Sarcomas: Giant Masqueraders.

作者信息

Singh Guddi Rani, Kumari Mamta, Singh Ayushi, Atreya Kshiti, Sinha Neetu, Haldar Debaditya

机构信息

Pathology department, IGIMS Patna, India.

Radiology department, IGIMS Patna, India.

出版信息

Niger Med J. 2025 Jun 16;66(2):783-790. doi: 10.71480/nmj.v66i2.618. eCollection 2025 Mar-Apr.

Abstract

Soft tissue sarcomas (STS) in the retroperitoneum are rare and account for a small percentage of adult STS. The most common type is liposarcoma, while fibrosarcoma and extra skeletal Ewing sarcoma (EES) are rarer. Retroperitoneal fibrosarcoma's are extremely uncommon. Diagnosis requires a comprehensive evaluation including clinical, radiological, histopathological, and immunohistochemical examinations to determine the specific subtype of STS in the retroperitoneum. A 76-year-old male presented with a large retroperitoneal tumour. Surgery successfully removed the tumor which was diagnosed as dedifferentiated liposarcoma based on microscopic analysis and positive vimentin and MDM2 markers. We also report a case of 69-year-old male with a retroperitoneal mass that was ultimately diagnosed as adult fibrosarcoma after ruling out other possibilities. The third case was of 55-year-old female who presented with left lumbar pain, backache, and weight loss. Imaging revealed a retroperitoneal mass with hepatic metastasis. Histopathological examination and IHC analysis confirmed the diagnosis of extraskeletal Ewing sarcoma. The importance of accurate diagnosis through histopathological examination and immunohistochemical analysis is emphasized for effective management and prognosis.

摘要

腹膜后软组织肉瘤(STS)较为罕见,在成人STS中占比很小。最常见的类型是脂肪肉瘤,而纤维肉瘤和骨外尤因肉瘤(EES)则更为罕见。腹膜后纤维肉瘤极为少见。诊断需要进行全面评估,包括临床、放射学、组织病理学和免疫组织化学检查,以确定腹膜后STS的具体亚型。一名76岁男性患者出现一个巨大的腹膜后肿瘤。手术成功切除了肿瘤,根据显微镜分析以及波形蛋白和MDM2标记物呈阳性,该肿瘤被诊断为去分化脂肪肉瘤。我们还报告了一例69岁男性患者,其腹膜后肿块在排除其他可能性后最终被诊断为成人纤维肉瘤。第三例是一名55岁女性,她出现左腰痛、背痛和体重减轻。影像学检查发现一个伴有肝转移的腹膜后肿块。组织病理学检查和免疫组化分析证实诊断为骨外尤因肉瘤。强调通过组织病理学检查和免疫组化分析进行准确诊断对于有效管理和预后的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b75f/12280285/5128717257e3/nmj-66-783-f1.jpg

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