Neurofibromas growth

Key points
- 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).
- 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.
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.
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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).
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Rosa Sabatini and Giuseppe Loverro
Dept. Obstetrics and Gynecology,
General Hospital Policlinico-University of Bari, Italy
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REFERENCES
- La Vecchiam, C., Negri, E., Franceschi, S., Parazzini, F. (1993). Long-term impact of reproductive factors on cancer risk. Int J Cancer, Jan 21, 53(2), 215-9.
- Medard, M.L., Ostrowska, L. (2007).Combined oral contraception and the risk of reproductive organs cancer in women .Ginekol Pol, Aug,78(8), 637-41.
- Casey, P.M., Cerhan, J.R., Pruthi, S. (2008). Oral contraceptive use and risk of breast cancer. Mayo Clin Proc,Jan,83),86-90
- Deligeoroglou, E., Michailidis, E., Creatsas, G. (2003).Oral contraceptives and reproductive system cancer. Ann N Y Acad Sci,Nov,997,199-208.
- White, E., Malone, K.E., Weiss, N.S., Daling, J.R. (1994). Breast cancer among young US women in relation to oral contraceptive use. J. Nati Cancer Inst, 86(7), 505-14
- Brohet, R.M., Goldgar, D.E., Easton, D.F., Antoniou, A.C., Andrieu, N., Chang-Claude, J., Peock, S., Eeles, R.A., Cook, M., Chu, C., Nogue`s, C., Lasset, C., Berthet, P., Meijers-Heijboer, H., Gerdes, A.M., Olsson, H., Caldes, T., van Leeuwen, F.E., Rookus, M.A. (2007). Oral contraceptives and breast cancer risk in the international BRCA1/2 carrier cohort study: a report from EMBRACE, GENEPSO, GEO-HEBON, and the IBCCS Collaborating Group.J Clin Oncol, Sep 1,25(25), 3831-6.
Written and revised by
Gerald Hurowitz, M.D.
Our doctors write and review articles for accuracy and keep them up to date.
health.am provides general information, not medical advice. Always consult a qualified health professional about your own health.
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