CONTEXT:

Primary skull lesions are rare in the pediatric population. The differential diagnosis of these lesions is broad and includes both congenital and acquired lesions. Previous studies of skull lesions in the pediatric population suggest that dermoid/epidermoid tumors are the most common childhood skull tumors.

OBJECTIVE:

To review the clinicopathologic features of primary skull lesions identified within the pediatric population of an academic tertiary medical center.

DESIGN:

A retrospective review of surgical pathology reports during a 25-year period identified 19 primary skull lesions occurring in the pediatric population. Lesions were excluded if they were of known soft tissue or intracranial origin with secondary calvarial involvement, or if they represented a metastasis from a known primary malignancy.

RESULTS:

Nineteen primary skull lesions were identified in 11 male (58%) and 8 female (42%) patients, with a median age at diagnosis of 9.5 years. These lesions were usually benign and most commonly presented as a painless mass (n = 8). The lesions were located in the occipital bone (n = 7), frontal bone (n = 5), parietal bone (n = 2), and temporal bone (n = 1). Diagnoses included epidermoid/dermoid cyst (n = 8), Langerhans cell histiocytosis (n = 6), intraosseous hemangioma (n = 2), osteoblastoma (n = 1), infantile myofibroma (n = 1), and fibroma (n = 1). Intracranial extension was identified in 1 case, and recurrence was found in only 2 cases.

CONCLUSIONS:

Epidermoid/dermoid cysts and Langerhans cell histiocytosis are the most commonly encountered skull lesions in the pediatric population at our institution. Intracranial extension of these lesions is rare, and recurrence is uncommon following complete surgical resection.