A PDF file should load here. If you do not see its contents
the file may be temporarily unavailable at the journal website
or you do not have a PDF plug-in installed and enabled in your browser.
Alternatively, you can download the file locally and open with any standalone PDF reader:
https://icvts.oxfordjournals.org/content/20/4/565.full.pdf
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)