Primary extraskeletal myxoid chondrosarcoma of pulmonary arteries: a rare mimic of acute pulmonary thromboembolism

Interactive CardioVascular and Thoracic Surgery, Mar 2015

Primary extraskeletal myxoid chondrosarcoma of the pulmonary arteries is a very rare entity. Multimodality imaging reports on this entity are few. Myxoid chondrosarcoma is characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas, and are most commonly found in the lower extremities, limb girdles, distal extremities and trunk. We report an unusual case of a 31-year old man with histopathologically proven extraskeletal myxoid chondrosarcoma of the pulmonary arteries mimicking acute pulmonary thromboembolism.

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Primary extraskeletal myxoid chondrosarcoma of pulmonary arteries: a rare mimic of acute pulmonary thromboembolism

Interactive CardioVascular and Thoracic Surgery Primary extraskeletal myxoid chondrosarcoma of pulmonary arteries: a rare mimic of acute pulmonary thromboembolism Karthik Gadabanahalli 0 Vinay V. Belaval 0 Venkatraman Bhat 0 Imran M. Gorur 0 0 Department of Radiology and Pathology, Narayana Health, Narayana Multispecialty Hospital and Mazumdar Shaw Cancer Center , Bangalore , India Primary extraskeletal myxoid chondrosarcoma of the pulmonary arteries is a very rare entity. Multimodality imaging reports on this entity are few. Myxoid chondrosarcoma is characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas, and are most commonly found in the lower extremities, limb girdles, distal extremities and trunk. We report an unusual case of a 31-year old man with histopathologically proven extraskeletal myxoid chondrosarcoma of the pulmonary arteries mimicking acute pulmonary thromboembolism. The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. Chondrosarcoma; Pulmonary arteries; Pulmonary thromboembolism emission tomography; Multi-detector computed tomography; Positron - A 31-year old male presented to the emergency care unit with a 3-month history of gradually progressive exertional dyspnoea, with symptoms worsening for the last 10 days. The patient had an episode of syncope and two episodes of haemoptysis. He was initially admitted to an outside hospital with breathlessness, and was diagnosed to have acute pulmonary thromboembolism (PE) by computed tomography pulmonary angiography (CTPA). He was thrombolysed with IV tenecteplase, but showed no improvement. Hence, he was referred to our hospital for further management. In view of the persistent breathlessness on admission, CTPA was repeated at our hospital and revealed acute PE with hypodense filling defects with expansion of the main pulmonary artery (MPA) extending into both pulmonary arteries, i.e. segmental and subsegmental branches. A saddle-shaped, irregular filling defect was seen at the MPA bifurcation (Fig. 1). There were few enlarged bronchial collaterals. No significant adenopathy was seen. There were no features of extrapulmonary extension of the lesion. The patient was put on anticoagulants and antiplatelet agents with supportive oxygen therapy. He remained symptomatic. In view of the enlarged pulmonary artery branches and absence of webs, a decision was taken to investigate with positron emission tomography (PET)CT to rule out a neoplasm. Whole-body 18 F-FDG-PETCT performed 2 days later revealed a mildly hypermetabolic saddle thrombus in the MPA with extension into the segmental arteries, suggestive of a neoplastic aetiology. No other hypermetabolic lesion was found in the rest of the body. The possibility of pulmonary angiosarcoma was raised. The patient was maintained on supportive oxygen therapy and medications. He underwent preoperative catheter angiography a week after admission, which did not reveal neovascularity or any additional features. The patient then underwent surgical exploration with total circulatory arrest and cardiopulmonary bypass. Hypothermia was K. Gadabanahalli et al. / Interactive CardioVascular and Thoracic Surgery Figure 2: Histopathology microphotographs show cellular growth pattern forming cellular lobules of myxoid and chondroid matrix. (A) H&E stain: tumour cells with hyperchromatic nuclei located in lacunar spaces. (B) Photomicrograph showing tumour cells with diffuse positivity for S-100 by Immunohistochemistry-3,3-diaminobenzidine-horseradish peroxidase (IHC-DAB-HRP). induced with systemic cooling to 20C, with infusion of a cold blood cardioplegia solution at 4C through the aortic root. On surgical exposure, the MPA was found to be distended and tense. Tumour excision was performed through the MPA removing the tumour thrombi from the MPA and pulmonary artery branches up to the hilum. Tumour excision was uneventful, and near-total removal was achieved. After tumour removal, left and right pulmonary arteries were closed directly during warming. The postoperative period was uneventful. A 2-week postoperative echo showed no residual mass in the MPA and its branches. The patient was discharged on anticoagulants. The histopathological and immunohistochemical analyses of the surgical specimen showed features of extraskeletal myxoid chondrosarcoma (EMC; Fig. 2A and B). Repeat CTPA performed after 4 weeks showed a reduction in vascularized intraluminal filling defects. The patient underwent three cycles of chemotherapy with gemcitabine and docetaxel under the supervision of an oncologist. Patient had deterioration of symptoms over the next 2 months. Repeat CTPA showed a significant increase in tumour thrombus and pericardial fluid. He expired about 9 months after presentation of the initial symptoms. Our case describes CT and PET a (...truncated)


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Karthik Gadabanahalli, Vinay V. Belaval, Venkatraman Bhat, Imran M. Gorur. Primary extraskeletal myxoid chondrosarcoma of pulmonary arteries: a rare mimic of acute pulmonary thromboembolism, Interactive CardioVascular and Thoracic Surgery, 2015, pp. 565-566, 20/4, DOI: 10.1093/icvts/ivu444