Abstract

BackgroundNeurofibromas are benign tumors of the peripheral nerves and hallmark of neurofibromatosis type 1 (NF1), a tumor suppressor gene syndrome. Neurofibromas mostly start developing at puberty and can increase in size and number during pregnancy. Expression of progesterone receptors has been found in 75% of the tumors. Many female NF1 patients are thus concerned about the possibility that hormonal contraceptives may stimulate the growth of their neurofibromas.MethodsA survey was carried out on 59 female NF1 patients who are practicing or have practiced hormonal contraception to examine the effect of the various contraceptives on the growth of neurofibromas.ResultsMajority (53 out of 58) of patients who received oral estrogen-progestogen or pure progestogen preparations reported no associated tumor growth. In contrast, significant tumor growth was reported by two patients who received depot contraceptive containing high dose of synthetic progesterone.ConclusionsOral contraceptives do not seem to stimulate the growth of neurofibromas in NF1 patients. High doses of progesterone might stimulate the growth of neurofibromas and deserve more caution.

Highlights

  • Neurofibromas are benign tumors of the peripheral nerves and hallmark of neurofibromatosis type 1 (NF1), a tumor suppressor gene syndrome

  • Physicians involved in the care of NF1 patients are concerned by the dilemma if hormonal contraceptives containing estrogen and progestogen could stimulate the onset of new or the growth of the yet present neurofibromas

  • The authors inferred an important role of progesterone in neurofibroma growth and suggested that antiprogestins may be useful in the treatment of this tumor

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Summary

Introduction

Neurofibromas are benign tumors of the peripheral nerves and hallmark of neurofibromatosis type 1 (NF1), a tumor suppressor gene syndrome. Neurofibromas mostly start developing at puberty and can increase in size and number during pregnancy. Many female NF1 patients are concerned about the possibility that hormonal contraceptives may stimulate the growth of their neurofibromas. Multiple neurofibromas are the most significant hallmark of NF1. These benign tumors of peripheral nerves mostly start developing at puberty and can increase in size and number during pregnancy [1]. Physicians involved in the care of NF1 patients are concerned by the dilemma if hormonal contraceptives containing estrogen and progestogen could stimulate the onset of new or the growth of the yet present neurofibromas. The authors inferred an important role of progesterone in neurofibroma growth and suggested that antiprogestins may be useful in the treatment of this tumor

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