RT Journal Article
SR Electronic
T1 Mediastinal angiomyolipomas in a male patient affected by tuberous sclerosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 678
OP 680
DO 10.1183/09031936.00021207
VO 31
IS 3
A1 Warth, A.
A1 Herpel, E.
A1 Schmähl, A.
A1 Hoffmann, H.
A1 Herth, F. J. F.
A1 Schirmacher, P.
A1 Dienemann, H.
A1 Schnabel, P. A.
YR 2008
UL http://erj.ersjournals.com/content/31/3/678.abstract
AB 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.