Neurofibromas are benign tumors of the peripheral nerve sheath, which may occur sporadically and, in association with the common familial cancer syndrome, neurofibromatosis type 1 (NF1). NF1 is a hereditary disease caused by mutations of the NF1 gene at 17q11.2. Loss of the NF1 gene product in Schwann cells leads to the development of benign nerve sheath tumors ⁽¹¹⁷⁾.

There are intriguing links between the growth of neurofibromas and levels of circulating hormones: in fact, dermal neurofibromas usually arise during puberty,  increase in number and size during   pregnancy,  and   shrink   after   giving   birth. 

The   majority   (75%)  of neurofibromas express progesterone receptor (PR), whereas only a minority (5%) of neurofibromas express estrogen receptor (ER).

It has been suggested that hormones may influence the neurofibromas of patients with NF1 and may increase potential for malignant transformation of plexiform tumors.  Within neurofibromas, PR is expressed by non-neoplastic tumor-associated cells and not by neoplastic Schwann cells. 

We hypothesize that progesterone may play an important role in neurofibroma growth and suggest that antiprogestins may be useful in the treatment of this tumor. ^{(118,119,120)}.

—
Rosa Sabatini and Giuseppe Loverro
Dept. Obstetrics and Gynecology,
General Hospital Policlinico-University of Bari, Italy
—

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