Elsevier

The Journal of Hand Surgery

Volume 38, Issue 8, August 2013, Pages 1571-1574
The Journal of Hand Surgery

Scientific article
The Association Between Glomus Tumors and Neurofibromatosis

https://doi.org/10.1016/j.jhsa.2013.05.025Get rights and content

Purpose

To determine whether an epidemiologic association exists between glomus tumors and neurofibromatosis.

Methods

Using a pathology database, we established a study cohort consisting of all patients who had undergone excision of a glomus tumor of the hand between 1995 and 2010. We created a control cohort by randomly selecting 200 patients who had undergone excision of a ganglion cyst over the same period. We reviewed medical records for each cohort to identify patients with a diagnosis of neurofibromatosis. We calculated the odds ratio was calculated and performed Fisher's exact test to determine the significance of the association.

Results

We identified 21 patients with glomus tumors of the hand. Six of these patients carried the diagnosis of neurofibromatosis (29%). In contrast, no patients in the control group carried the diagnosis of neurofibromatosis. The odds ratio for a diagnosis of neurofibromatosis in association with a glomus tumor compared with controls was 168:1.

Conclusions

This study provides evidence of a strong epidemiologic association between glomus tumors and neurofibromatosis. Glomus tumor should be included in the differential diagnosis in neurofibromatosis patients who present with a painful lesion of the hand or finger.

Type of study/level of evidence

Diagnostic III.

Section snippets

Materials and Methods

After we obtained approval from our institutional review board, we used the institution's pathology database to create a case cohort consisting of all patients who had undergone excision of a glomus tumor of the hand or wrist between 1995 and 2010. We created a control cohort of referent patients by randomly selecting 200 patients who had undergone excision of a hand or wrist ganglion over the same period.

In both cohorts, we reviewed existing patient records including inpatient notes,

Results

A total of 21 patients who underwent excision of a glomus tumor of the hand or wrist from 1995 through 2010 were identified and were included in the case cohort. A review of their existing records showed that the glomus tumors were diagnosed preoperatively by history and physical examination in all but 1 patient and confirmed postoperatively by pathology. In 1 patient, a glomus tumor was not suspected preoperatively, but was identified postoperatively by pathology. Chart review revealed that 6

Discussion

Although glomus tumors have not historically been associated with other disease processes, a number of case reports in the literature suggest an association with neurofibromatosis. Neurofibromatosis is an autosomal dominant disease affecting neural crest cells and has been classified into 2 types (Table 3). Neurofibromatosis type 1 is the most common form and affects approximately 1 in 3,500 people.23 It has multiple clinical findings, but its most notable feature is the presence of cutaneous

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