When a pulmonary embolism is not a pulmonary embolism: a rare case of primary pulmonary leiomyosarcoma

Abstract

Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (PE). A 72-year-old Caucasian female was initially diagnosed with ‘saddle pulmonary embolism’ based on computerized tomographic angiography of the chest 2 months prior to admission and placed on anticoagulation. Dyspnea escalated, and serial computed tomography scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches. An echocardiogram on admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. Respiratory distress and multiorgan failure developed and, unfortunately, the patient expired. Autopsy showed a lobulated, yellow mass throughout the main pulmonary arteries measuring 13 cm in diameter. The mass extended into the parenchyma of the right upper lobe. On microscopy, the mass was consistent with a high-grade primary pulmonary artery leiomyosarcoma. Median survival of patients with primary pulmonary artery leiomyosarcoma without surgery is one and a half months, and mortality is usually due to right-sided heart failure. Pulmonary artery leiomyosarcoma is a rare but highly lethal disease commonly mistaken for PE. Thus, we recommend clinicians to suspect this malignancy when anticoagulation fails to relieve initial symptoms. In conclusion, early detection and suspicion of pulmonary artery leiomyosarcoma should be considered in patients refractory to anticoagulation, prompting initiation of early intervention.

Primary Pulmonary Leiomyosarcoma in a Forty-Year-Old Woman

“…PPLs are extremely rare lesions that typically show an aggressive and often lethal clinical course [ 1 , 2 , 3 , 4 , 5 , 6 ]. In a large population-based study, examining 231 patients with PPL, Qin et al [ 1 ] found the median age at presentation was 65.8 years, with a patient survival time of 14.0 months, with 1, 3, and 5-year survival rates of 52.7%, 29.0%, and 22.2%, respectively.…”

“…PPLs present with symptoms similar to other pulmonary neoplasms, including cough, hemoptysis, dyspnea, asthenia, chest and back pain, and weight loss [ 1 , 3 ]. Due to their rarity, PPLs have been misdiagnosed as pulmonary emboli, as other pulmonary neoplasms, or as cardiac neoplasms [ 1 , 3 , 4 , 5 ]. Pulmonary artery leiomyosarcomas have also been misdiagnosed as chronic thromboembolic disease, due presenting with dyspnea, chest pain, and right-sided heart failure [ 4 ].…”

“…Due to their rarity, PPLs have been misdiagnosed as pulmonary emboli, as other pulmonary neoplasms, or as cardiac neoplasms [ 1 , 3 , 4 , 5 ]. Pulmonary artery leiomyosarcomas have also been misdiagnosed as chronic thromboembolic disease, due presenting with dyspnea, chest pain, and right-sided heart failure [ 4 ]. In some cases, the diagnosis of sarcoma is considered either too late, or not at all [ 5 ].…”

“…Additionally, the early detection, diagnosis, and complete surgical resection of PPLs contribute significantly to increased patient survival times [ 6 ]. For this reason, clinicians should be aware of these rare lesions, especially when the symptoms of thromboembolic disease fail to resolve with anticoagulation therapy [ 4 ].…”

JCHIMP at 5 years: growth and development

“…The case reports are CHF associated with protease inhibitors ( 9 ); Kaposi’s sarcoma of the heart ( 10 ); 2 Takotsubo reports ( 11 , 12 ); GI bleeding secondary to aortic cavitary fistula ( 13 ) in endocarditis with complete heart block; pulmonic valve endocarditis presenting as neck pain ( 14 ); tricuspid valve endocarditis with Mobitz II heart block ( 15 ); primary pulmonary leiomyosarcoma disguised as a pulmonary embolus ( 16 ); aspergillus endophthalmitis ( 17 ); cutaneous tuberculosis ( 18 ); and, finally, a report increasing our awareness of complications of acetaminophen overdose ( 19 ), and a review on GI bleeding and fistulas ( 20 ).…”