Pulmonary artery sarcoma masquerading as pulmonary embolism: an under-recognised entity

Abstract

Pulmonary artery sarcoma is a rare disease with only a handful of cases reported. It is histologically classified as leiomyosarcoma, spindle cell sarcoma, fibrous histiocytoma or undifferentiated sarcoma. The disease is mostly misdiagnosed as pulmonary thromboembolism and carries a grim prognosis with an average survival of only a few months. Misdiagnosis often results in patients being treated inappropriately and diagnosed in later stages of the disease. This delay in diagnosis can be associated with significant mortality in the setting of an already poor prognosis. Early aggressive surgery targeting complete surgical resection is the standard treatment. Chemotherapy and radiation therapy have been tried with variable outcomes. Given the aggressive nature of pulmonary artery sarcoma, regular post-surgery follow-up is indicated.

Primary Pulmonary Artery Leiomyosarcoma with Pulmonary Metastasis Depicted on Enhanced Computer Tomography: A Case Description and an Analysis of the Literature

“…Pulmonary embolism is the most common misdiagnosis; patients may undergo a period of conventional treatment such as anticoagulation and, when no effects are observed with such an intervention, other diseases begin to be considered, thereby missing the opportunity for early treatment [1]. PAL does not have specific imaging signs, but enhanced CT examinations play an important role in determining the nature of the lesion and dynamic observation [23]. On enhanced CT images, chronic pulmonary thromboembolism usually shows abrupt vascular narrowing and cut-offs instead of a continuous soft tissue filling of the pulmonary arteries as the neoplasm [24].…”

“…On enhanced CT images, chronic pulmonary thromboembolism usually shows abrupt vascular narrowing and cut-offs instead of a continuous soft tissue filling of the pulmonary arteries as the neoplasm [24]. The enhancement of a tumor versus the nonenhancement of an embolus as well as the distension of a vascular lumen by a tumor and the extravascular invasion into the adjacent structure are also important clues to distinguish between tumors and a pulmonary embolism [23]. Increased uptake in the area of the tumor on fluorine-18-2-fluoro2-deoxy-D-glucose positron emission tomography (FDG-PET) can also be helpful for differentiating a neoplasm from an organizing embolus [25].…”