Hemothorax presenting as a primitive thoracic paraganglioma. Case illustration.

This 59-year-old woman with recurrent back pain presented with respiratory distress after suffering an acute episode of sharp pain involving the left hemithorax. Chest radiography revealed a significant left pleural effusion. Thoracic computerized tomography (CT) and magnetic resonance (MR) imaging demonstrated an inferior lobe atelectasis of the left lung and a large extra- and intracanalicular tumor at the level of T10‐11, which eroded the intervertebral foramen (Fig. 1). The pleural effusion proved to be hemorrhagic during thoracentesis. Whole-body CT scanning showed no signs of abnormality. The patient underwent radical surgery via a left thoracotomy along the 10th rib and a costotransversectomy. The pleura and lung were covered by a film of old blood. The lesion had a well-defined cleavage plane and a rich vascular supply from both the intercostal arteries and aorta. Considerable intraoperative bleeding occurred during resection; this made the procedure demanding and required that the patient be given multiple blood transfusions. The histological diagnosis was paraganglioma (Fig. 2). Because the levels of catecholamines and their metabolites in plasma and urine were normal, the lesion was classified as a nonfunctioning paraganglioma. The postoperative course was uneventful. Four months after surgery, MR imaging confirmed complete removal of the tumor and disappearance of the pleural effusion. At the 14month follow-up examination there was no sign of disease recurrence, and whole-body CT scanning documented no other lesions. To the best of our knowledge, including our patient, only 14 cases of primitive thoracic paragangliomas have been reported. The tumor was extradural in 12 cases and intradural in two. In two patients local recurrence was demonstrated; cerebrospinal fluid (CSF) or distant metastases occurred in three patients. 1,2,4 These features are different from those of the more common paragangliomas of the lumbar spine and cauda equina, which are mostly intradural and are never associated with metastases outside the central nervous system; CSF dissemination was described in 1% of these cases.3 Acute onset of symptoms due to hemothorax has not been previously reported in lesions involving the thoracic spine. Hemorrhage secondary to the hypervascularized tumor may explain the pleural effusion in our patient, because large extraadrenal paragangliomas in other locations are known to become hemorrhagic and necrotic. 5