Background: Multiple glomus tumors are extremely rare and seldom demonstrate malignant change.
Objective: To describe an exceptionally rare case of familial multiple glomus tumors involving the glans penis.
Methods: Multiple glomus tumors in a father and son were studied by light and electron microscopy.
Results: The clinical presentation of the two patients was similar, and atypical glomus cells were identified in both son and father. The histology of the tumor cells suggested that they were malignant, yet the clinical course has been benign.
Conclusion: Despite their malignant histologic appearance, the behavior of familial glomus tumors is benign. A conservative approach to treatment appears warranted. The diagnosis and management of these lesions should be based on clinical behavior rather than histopathology.