[A case of Von Recklinghausen's disease associated with a hemothorax due to a rapidly growing malignant schwannoma].
A 17-year-old man with Von Recklinghausen's disease was admitted to our hospital because of progressive dyspnea on exertion. A chest X-ray film showed a massive left pleural effusion. Thoracentesis revealed dark blood. Hemothorax was diagnosed, and digital subtraction angiography (DSA) was done. DSA revealed a large mass that was supplied with blood via the inferior phrenic artery, but revealed no active bleeding. An intercostal tube was inserted and 1700 ml of dark blood was drained. A chest X-ray film taken after drainage revealed a giant mass showing an extra-pleural sign in the left lung field. A thoracotomy was done to determine the source of bleeding. A giant tumor with hypervascular tissue was seen, and was diagnosed as a malignant schwannoma. No spontaneous hemorrhage from the artery was seen. The source of the hemothorax was believed to be hemorrhage from the tumor vessels. The tumor was treated conservatively, but it continued to grow rapidly and the patient died of respiratory failure. Five other patients have been reported to have hemothorax associated with Von Recklinghausen's disease. The sources of the hemothorax were reported to be hemorrhage from tumor vessels, or spontaneous rupture of the subclavian artery, an intercostal artery, or an intercostal vein.
Fuyuno
G
G
Department of Medicine, Tokyo Saiseikai Central Hospital, Japan.
Kobayashi
R
R
Iga
R
R
Horio
H
H
Nomori
H
H
Kodera
K
K
Morinaga
S
S
D000293:Adolescent; D017809:Fatal Outcome; D006491:Hemothorax / Q000209:etiology*; D006801:Humans; D008297:Male; D009442:Neurilemmoma / Q000098:blood supply / Q000150:complications*; D009456:Neurofibromatosis 1 / Q000150:complications*; D012878:Skin Neoplasms / Q000098:blood supply / Q000150:complications*