Nasal fetal rhabdomyoma: A rare element to consider in the differential diagnosis of frontonasal masses

Fetal rhabdomyoma is a benign neoplasm of rhabdomyoblasts that is found in both children and adults. Although there is often a predilection for the head and neck region, these tumors have been identified in several anatomic sites. A primary frontonasal presentation, however, has not been described yet. We report the first case of a fetal rhabdomyoma arising in the frontonasal region of a 3-year-old girl. This rare form of presentation is very interesting because of the need for a differential diagnosis with other entities. The clinician must keep in mind other possibilities typical of frontonasal region in childhood like nasal dermal sinus, anterior cephalocele and nasal glioma. For the pathologist, the most important reason to recognize and diagnose fetal rhabdomyoma in its various forms and locations is to avoid confusion with his malignant counterpart, the rhabdomyosarcoma.