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Mediastinal angiomyolipomas in a male patient affected by tuberous sclerosis

¹ Institute of Pathology, University Hospital Heidelberg, and Depts of ² Radiology, ³ Thoracic Surgery, and ⁴ Pneumonology and Critical Care Medicine, Thoraxklinik Heidelberg, University of Heidelberg, Heidelberg, Germany.

CORRESPONDENCE: P. A. Schnabel, Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, D-69120 Heidelberg, Germany. Fax: 49 62213961672. E-mail: philipp.schnabel{at}med.uni-heidelberg.de

Keywords: Angiomyolipoma, chylothorax, mediastinum, pleural effusion, tuberous sclerosis complex

Received: February 21, 2007
Accepted July 27, 2007

Classical angiomyolipomas are benign tumours composed of various tissues, including components of fat, abnormal blood vessels and smooth muscle cells. They are often found in association with tuberous sclerosis complex (TSC).

The present study reports a male patient affected by TSC with intermittent, massive chylous pleural effusions, who developed recurrent mediastinal angiomyolipomas. The tumours were characterised via histological and immunohistochemical methods.

Although angiomyolipomas frequently occur in the kidneys of TSC patients, this case is the first report of mediastinal angiomyolipomas associated with TSC.

Besides lymphangioleiomyomatosis, this differential diagnosis has to be taken into account in the case of chylous pleural effusions and mediastinal masses in tuberous sclerosis complex patients.

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