Diffuse vascular hamartoma with malignant behavior

Abstract

Pulmonary hamartomas are benign lesions, usually asymptomatic and incidentally discovered on a routine chest radiograph; occasionally, however, this benign lesion may cause life threatening symptoms due to it's location and diffuse vascular involvement. We report the case of a 27 year-old male, non-smoker, who presented with dyspnea, cough, hemoptysis and weight loss. He was found to have a mass in the right hilar region which also involved the right main bronchus, pulmonary artery and esophagus. Surgical biopsy of the lesion led to the diagnosis of diffuse vascular hamartoma. Although it was a benign lesion, due to the size and location, surgical removal was not possible and patient died 10 years after being diagnosed with the condition.

Keywords: Hamartoma; Malignant behavior; Vascular hamartoma.

Fig. 1

a,b,c: Chest CT with mediastinal mass on right hilar region with right main bronchus caliber reduction, d: Right main bronchus caliber reduction and right pleural thickening.

Fig. 2

Blunting of the Carina with extrinsic compression.

Fig. 3

Pulmonary parenchyma with large vessels with thickened heterogenic walls and irregular form related to the hamartomatous nature of the disease. (a): Pulmonary parenchyma with large vessels. (b): Large vessels with thickened wall.