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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.
Abstract
Background

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.

Objective

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

Methods

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.

Results

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.

Limitations

The retrospective design resulted in incomplete data capture.

Conclusions

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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