Abstract
Neurofibromatosis type I is a common tumor predisposing disease
in humans. Surgical therapy can be applied only in selected
patients with resectable masses. Hence, development of new
therapies for this disease is urgent. We used human neurofibroma
implants in mice with severe combined immunodeficiency (SCID) as
a model to test the toxicity and potential efficacy of
pirfenidone, a new therapeutic agent. Two hundred twelve human
neurofibromas were transplanted into various locations in 59
experimental animals, and 30 mice with implants received oral
pirfenidone for up to six weeks. Survival of neurofibromas in
animals treated with pirfenidone was lower than in the control
group