Department of Diagnostic Pathology and Cytology, Osaka International Cancer Institute, Osaka, Japan.
Department of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1, Tsukiji, Chuo-ku, Tokyo 104-0045, Japan; Rare Cancer Center, National Cancer Center, Tokyo, Japan.
Surg Pathol Clin. 2024 Jun;17(2):243-255. doi: 10.1016/j.path.2023.11.008. Epub 2023 Dec 15.
Primary sarcoma of the lung and mediastinum is rare. The diagnosis requires careful exclusion of sarcomatoid carcinoma, sarcomatoid mesothelioma, and metastases from extra-thoracic sites. This review summarizes the key morphologic, immunohistochemical, and molecular characteristics of sarcomas that are encountered in the lung and mediastinum. The tumor types discussed are synovial sarcoma, well-differentiated/dedifferentiated liposarcoma, myxoid pleomorphic liposarcoma, intimal sarcoma of the pulmonary artery, inflammatory myofibroblastic tumor, epithelioid hemangioendothelioma, primary pulmonary myxoid sarcoma, malignant peripheral nerve sheath tumor, Ewing sarcoma, and CIC-rearranged sarcoma. Relevant differential diagnoses are also addressed.
原发性肺及纵隔肉瘤罕见。诊断需要仔细排除肉瘤样癌、肉瘤样间皮瘤和来自胸外部位的转移瘤。本文综述了肺及纵隔中常见肉瘤的主要形态学、免疫组化和分子特征。讨论的肿瘤类型包括滑膜肉瘤、高分化/去分化脂肪肉瘤、黏液样多形性脂肪肉瘤、肺动脉内膜肉瘤、炎性肌纤维母细胞瘤、上皮样血管内皮细胞瘤、原发性肺黏液样肉瘤、恶性外周神经鞘瘤、尤文肉瘤和 CIC 重排肉瘤。还讨论了相关鉴别诊断。