[Interferon alpha-2a in the treatment of diffuse thoracic angiomatosis in adulthood--a ase report].
A diffuse angiomatosis affecting thoracal organs is a rare, frequently fatal disorder occurring mainly in childhood. The definite diagnosis is usually made by autopsy. A specific treatment does not exist. In the case report presented here we describe the course of an 37-year old patient presenting initially with dyspnea and hemoptysis. Radiological investigations showed a mass lesion in the anterior superior mediastinum, bihilar masses and a periesophageal mass with soft tissue density and a pericardial effusion. Bilateral interstitial infiltrations were also present. CT-guided mediastinal biopsies and bronchial mucosal biopsies were nonspecific. Open-lung biopsy revealed the diagnosis of an extensive diffuse angiomatosis affecting mediastinum, pericardium and pleura. During the 18-month monotherapy with interferon-alpha 2a the patient showed significant improvements of clinical as well as laboratory and radiological parameters. The presented case confirms the experience of several authors, that the diagnosis of a diffuse thoracal authors can only be made by open lung biopsy or autopsy. The presented case also underlines the possibility of a palliative therapy of aggressive angiomatous diseases with interferon-alpha 2a.
Medizinische Universitätsklinik V, Pathologisches Institut der Universität des Saarlandes.
A
A
Eichler
D
D
Ukena
H
H
Wilkens
K
K
Reitnauer
M
M
Girmann
K
K
Remberger
B
B
Kramann
G W
GW
Sybrecht
D000328:Adult; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D000798:Angiomatosis / Q000175:diagnosis / Q000188:drug therapy*; D004417:Dyspnea; D006469:Hemoptysis; D006801:Humans; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008297:Male; D011994:Recombinant Proteins; D013896:Thoracic Diseases / Q000175:diagnosis / Q000188:drug therapy*
D020533:Angiogenesis Inhibitors; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins