Primary Intrathoracic Sarcomas: A Review of Cross-sectional Imaging and Pathology

Abstract

The most common sarcomas in the thorax are metastasis from an extrathoracic primary malignancy. Primary intrathoracic sarcomas are rare albeit aggressive malignancies that are diagnosed on histopathology. Although a few imaging characteristics have been described that are common to sarcomas, it is still a diagnosis of exclusion as other tumors are much more common. Like elsewhere, primary thoracic sarcomas are also classified according to their histologic features. They are a rare group of tumors that can arise from the mediastinal structures, lung, pleura, or chest wall. On imaging, differentiating these from more common malignancies like lung cancer is difficult and often requires multimodality workup and tissue sampling. A few sarcomas are very specific to their locations, such as angiosarcoma in the right atrium, leiomyosarcoma in the pulmonary artery, where imaging has high accuracy for the diagnosis. Despite being nonspecific in a majority of cases, imaging plays a pivotal role in determining the organ of origin, tumor extent, invasion of adjacent structures, and thus help to assess the surgical resectability. Although sarcomas arising from chest wall are the most common primary sarcomas in the chest, they are excluded from this review to focus only on primary intrathoracic sarcomas. The article provides a comprehensive imaging and pathology review of the rare primary intrathoracic sarcomas, including but not limited to angiosarcoma, Kaposi sarcoma, fibrosarcoma, malignant transformation of fibrous tumor of pleura, sarcomatoid mesothelioma, leiomyosarcoma, and malignant small round blue cell tumors.Key points:Primary intrathoracic sarcomas are rare but clinically important.Imaging helps to determine local extent, invasion, metastases and appropriate site/mode of biopsy.Role of pathology is paramount in diagnosis and guiding treatment based on immunogenetic/molecular typing.