Vnitr Lek 2010, 56(8):810-823

Successful treatment of angiomatosis with thalidomide and interferon α. A description of five cases and overview of treatment of angiomatosis and proliferating hemangiomas

Z. Adam^1,*, L. Pour¹, M. Krejčí¹, E. Pourová², O. Synek², L. Zahradová¹, M. Navrátil¹, M. Mechl³, T. Nebeský³, J. Neubauer³, J. Feit⁴, J. Vokurková⁵, Z. Král¹, O. Bednařík¹, P. Šlampa⁶, H. Doležalová⁶, R. Hájek¹, J. Mayer¹

¹ Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.

² Interní oddělení nemocnice Vyškov, přednosta prim. MUDr. Oldřich Synek

³ Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil A. Válek, CSc.

⁴ Ústav patologie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Josef Feit, CSc.

⁵ Klinika popálenin a rekonstrukční chirurgie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Pavel Brychta, CSc.

⁶ Radioterapeutická klinika Lékařské fakulty MU a Masarykova onkologického ústavu Brno, přednosta prof. MUDr. Pavel Šlampa, CSc.

Our paper describes 5 patients with a vascular malformation - angiomatosis. In the first patient, a young man, angiomatosis affected the stomach, intestine, the area of mesenterium and retroperitoneum as well as mediastinum. Angiomatous mass had invaded pelvic bones and vertebrae. Treatment was initiated with interferon α in a maximum tolerated dose of 3 million units 3 times a week. Because of low efficacy of interferon α, thalidomide was added at a dose of 100 mg per day. Bone pain disappeared following a few applications of zoledronate administered in regular monthly intervals. After 3 years of concomitant administration of interferon α and thalidomide, we changed the regimen due to adverse effects and are administering thalidomide and interferon alternatively in 4-monthly intervals. Treatment has resulted in 50% reduction, according to imaging, of angiomatous mass, reduced intensity of disseminated intravascular coagulation and disappearance of clinical signs. The second was a case of multiple angiomatosis affecting the intestine only (multiple intestinal angiodysplasias) where we used thalidomide monotherapy. This treatment reduced blood losses and haemoglobin concentrations rose to normal levels. This male patient had consumed 120 transfusion units per year before the initiation of thalidomide. The third case was a slowly progressing vascular malformation of the face. This vascular malformation troubled its sufferer by spontaneous shortening that could not be resolved surgically because of its fragility. Two years of combined treatment with interferon α 6 million unites 3 times a week and thalidomide 100 mg daily led to a reduction and flattening of the malformation, paling of its colour and ceasing of spontaneous bleeding. This development enabled minor surgery - partial excision of this large vascular malformation. Histology examination confirmed that there was no evidence of new capillary formation. Histological examination thus confirmed efficacy of the treatment. The fourth case involved a patient with large vascular malformations affecting supraclavicular region of the neck and nape in whom radiotherapy was applied (54 Gy) leading to a reduction of the malformation mass by a at least 50%. The fifth is a case of an extensive periorbital lymphangioma that diminished following treatment with interferon α. These cases illustrate the benefits of combined treatment including thalidomide and interferon α in patients with multiple angiomatosis or large proliferating hemangioma (vascular malformation). If combined treatment with thalidomide and interferon α is not possible, it is beneficial to use thalidomide monotherapy. Radiotherapy is another alternative, although it is necessary to apply doses exceeding 50 Gy which may not be always possible.

Keywords: vascular malformation; angiomatosis; hemangiomatosis; hemangioma; angiodysplasia; lymphangioma; thalidomide; lenalidomide; interferon α; zoledronate; bevacizumab; antiangiogenic treatment; radiotherapy; monoclonal gammopathy

Received: November 20, 2009; Accepted: January 4, 2010; Published: August 1, 2010  Show citation