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Journal of the American Academy of Dermatology
Volume 71, n° 1
pages 44-48 (juillet 2014)
Doi : 10.1016/j.jaad.2014.01.913
accepted : 16 January 2014
Original Articles

Glomus tumors in individuals with neurofibromatosis type 1

Monique G. Kumar, MPhil, MD a, Ryan J. Emnett, MS b, Susan J. Bayliss, MD a, David H. Gutmann, MD, PhD b,
a Division of Dermatology, Department of Internal Medicine, Washington University School of Medicine, Saint Louis, Missouri 
b Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri 

Reprint requests: David H. Gutmann, MD, PhD, Department of Neurology, Washington University School of Medicine, Box 8111, 660 S Euclid Ave, St. Louis, MO 63110.

Glomus tumors have recently been reported in individuals with the neurofibromatosis type 1 (NF1) cancer disposition syndrome. We compare the clinical and molecular features of these painful hamartomas in a series of sporadic and NF1-associated cases.


We sought to evaluate the association of NF1 with glomus tumors and to compare NF1-associated glomus tumors with sporadic glomus tumors.


We conducted a retrospective cohort study of all individuals with a histopathologic diagnosis of glomus tumor at a large tertiary care center from January 1998 to January 2013. Charts were reviewed for a coexisting diagnosis of NF1.


A total of 42 glomus tumors were identified in 34 individuals. Twelve (28.6%) were found in 6 patients with NF1. In 28 individuals with 30 sporadic tumors, there was no coexisting medical condition. Although multifocal tumors (16.7%) and tumor recurrence (33.3%) were more common in association with NF1, these trends did not reach statistical significance. NF1-associated glomus tumors exhibited no neurofibromin immunoreactivity, whereas their sporadic counterparts retained neurofibromin expression.


The retrospective design resulted in incomplete data capture.


Detection of glomus tumors should raise suspicion for a concurrent diagnosis of NF1.

The full text of this article is available in PDF format.

Key words : glomus tumor, neurofibromatosis, neurofibromatosis type 1, neurofibromin, tumor disposition syndrome

 Dr Bayliss is currently conducting a study with Scioderm for epidermolysis bullosa.
 Conflicts of interest: None declared.

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