Von Recklinghausen Disease Nipple-Areolar Neurofibromas
Vincent M. Riccardi, The Neurofibromatosis Institute
10 June 2010
Von Recklinghausen Disease Nipple-Areolar Neurofibromas
M.R. Bongiorno, et al., might want to reconsider several lines of text in their recent article about neurofibromas of the nipple and areola among women patients with von Recklinghausen disease (VRD), known to be due a mutation in the NF1 gene.1 I wish to comment with specific reference to the third complete paragraph on page 5. First, while technically the number of women studied by Bongiorno, et al., was 8 more than in the study by Riccardi and Eichner2, the order of magnitude is the same. Further, contradicting the paragraph’s final statement, substantial data were provided for the Riccardi-Eichner series of 123 women and 115 men. Second, and most important, nipple-areolar neurofibromas are not “rare,” as claimed by Bongiorno, et al. The Riccardi-Eichner 1986 data, corroborated by my own personal follow-up with hundreds of additional women with VRD, document that “nipple and/or areolar neurofibromas were present in … 83% of women at or above 18 years.” In my estimation, nipple and/or areolar neurofibromas are among the most common, most consistent findings in women with VRD. The point is that clinicians should not be surprised to find these lesions among women with VRD. Rather, clinicians should expect them when the woman is at or beyond the second decade. Their presence is unrelated to sexual activity, gravidity, parity or suckling, except that the lesions can compromise the latter. They have no relationship whatsoever to breast cancer.
Vincent M. Riccardi, MD The Neurofibromatosis Institute 5415 Briggs Avenue La Crescenta, CA USA riccardi@medconsumer.com
Reference List
(1) Bongiorno MR, Doukaki S, Arico M. Neurofibromatosis of the nipple-areolar area: a case series. J Med Case Reports 2010;4:22. (2) Riccardi VM, Eichner JE. Neurofibromatosis: Phenotype, Natural History, and Pathogenesis. 1 ed. Baltimore: Johns Hopkins University Press, 1986.
Journal of Medical Case Reports
Von Recklinghausen Disease Nipple-Areolar Neurofibromas
10 June 2010
Von Recklinghausen Disease Nipple-Areolar Neurofibromas
M.R. Bongiorno, et al., might want to reconsider several lines of text in their recent article about neurofibromas of the nipple and areola among women patients with von Recklinghausen disease (VRD), known to be due a mutation in the NF1 gene.1 I wish to comment with specific reference to the third complete paragraph on page 5. First, while technically the number of women studied by Bongiorno, et al., was 8 more than in the study by Riccardi and Eichner2, the order of magnitude is the same. Further, contradicting the paragraph’s final statement, substantial data were provided for the Riccardi-Eichner series of 123 women and 115 men. Second, and most important, nipple-areolar neurofibromas are not “rare,” as claimed by Bongiorno, et al. The Riccardi-Eichner 1986 data, corroborated by my own personal follow-up with hundreds of additional women with VRD, document that “nipple and/or areolar neurofibromas were present in … 83% of women at or above 18 years.” In my estimation, nipple and/or areolar neurofibromas are among the most common, most consistent findings in women with VRD. The point is that clinicians should not be surprised to find these lesions among women with VRD. Rather, clinicians should expect them when the woman is at or beyond the second decade. Their presence is unrelated to sexual activity, gravidity, parity or suckling, except that the lesions can compromise the latter. They have no relationship whatsoever to breast cancer.
Vincent M. Riccardi, MD
The Neurofibromatosis Institute
5415 Briggs Avenue
La Crescenta, CA USA
riccardi@medconsumer.com
Reference List
(1) Bongiorno MR, Doukaki S, Arico M. Neurofibromatosis of the nipple-areolar area: a case series. J Med Case Reports 2010;4:22.
(2) Riccardi VM, Eichner JE. Neurofibromatosis: Phenotype, Natural History, and Pathogenesis. 1 ed. Baltimore: Johns Hopkins University Press, 1986.
Competing interests
None