gms | German Medical Science

The primary Ewing-Sarcoma is a highly malignant bone tumour of neuroectodermal origin. It usually becomes apparent in the second and third decade affecting the diaphysis of long tubular bones. We present the interesting case of a primary Ewing-Sarcoma of the petrous bone in a five month old nursling, which initially became symptomatic with a complete peripheral facial palsy.

When the infant presented to our department, we could also establish the diagnosis of an ipsilateral surdity by brainstem evoked response audiometry (BERA). Exploring the case history, there had already been a period of temporary facial weakness at the age of 2 months. A CT scan of the skull showed a destructively growing neoformation, 4.5 cm in diameter, having its origin in the left petrous bone. A biopsy of the tumour was taken by stereotaxic surgery and histopathological investigations estabished the diagnosis of a primary Ewing Sarcoma. Staging-examinations revealed no indication for metastatic spread.

For a period of 10 months the child underwent chemotherapy and radiation of the skull with a cumulative radiation dose of 45 Gray. The progress of disease was supervised by radiological control-investigations for a period of 25 months and showed ossification of the tumour matrix and regression of tumour size. Until now the development of the child is in accordance with the age.

Ewing Sarcoma primarily arising in the skull is extremely uncommon.

Surveying the international literature, case reports of Ewing sarcoma involving the petrous bone are very rare. Interestingly, in all described cases exceptionally young children were affected. To the best of our knowledge we present here for the first time a case of primary Ewing Sarcoma of the petrous bone, which became symptomatic with a peripheral facial palsy and ipsilateral surdity.