P3.01-025 Primary Pulmonary Sarcomas: An Entity Lost in Misdiagnosis: Topic: Morphology

Abstract

Primary pulmonary sarcomas are very rare with an incidence rate of <0.5% of all lung malignancies. Their low incidence has impeded comprehensive evaluation of their association with smoking, definitive diagnostic and treatment-regimes. They are often misdiagnosed, both on radiology as well as on fine-needle-aspirate/small-biopsies. We present a series of primary pulmonary sarcomas diagnosed over the last two and a half years. All cases of primary pulmonary sarcomas (2014-2016) were retrieved and reviewed. A total of 21 sarcomas were identified. The most common was synovial sarcoma. Four exceptionally rare cases included pulmonary-artery intimal sarcoma, primary pulmonary myxoid sarcoma, malignant peripheral nerve-sheath tumor and follicular dendritic-cell sarcoma. The clinical and pathology details of which are provided in table1. The patients were distributed over a wide-age range (range: 9-65 years, median: 34 years) with a male-preponderance (M:F=2.2:1). Radiological features were non-specific except in case1 (table1). Histopathology revealed spindle-cell tumor in all cases (figure1) and an extensive immunohistochemical-panel and cytogenetic testing was required to clinch the diagnosis.View Large Image Figure ViewerDownload Hi-res image Download (PPT) This is a series of primary thoracic sarcomas with a highlight on four extremely rare cases which bring to light their unique clinical, radiological, histopathological and immunohistochemical findings. Awareness of such entities is essential for proper diagnosis, appropriate molecular-testing and treatment.