Extensive Fibrous Dysplasia and Sarcoma of the Frontal Skull Base and Orbit: Case Report

Introduction: Fibrous dysplasia is regarded as a benign disease with the option of surgical therapy. In rare cases a malignant transformation or a combination with an aneurysmal bone cyst can be observed. We present a case of extensive fibrous dysplasia of the frontal base combined with soft tissue tumor of sarcoma.

Clinical Presentation: A 52-year-old male patient presented with an extreme bilateral proptosis, more present in the right than in the left orbit. Also, the vision of the right eye decreased and diplopia appeared. The “bone-disease” and even the bony growth were observed clinically for about 50 (!) years without any other diagnostics. The patient had a normal social life and is working as a scientist in nature science. NMR and CT scans show a huge enlargement of the frontal bone in the area of the skullbase and of the surface of the frontal bone. Two soft tissue masses on the orbital roof of both sides are visible, first addressed as aneurysmal bone cyst.

Intervention: The tumor was approached by a bifrontal osteoclastic trephination and extended removal of soft and bony tissue tumor mass. The diagnosis was malignant sarcoma. Since then, three other operations have been performed for tumor removal, liquorrhea, and infection. Radiotherapy and chemotherapy have also been given, and the patient is now (2.5 years after diagnosis) in good clinical condition, with minor wound problems but no evident neurological deficit.

Conclusions: From our experience and the literature we recommend an early and if possible complete surgical resection of the “benign” lesion fibrous dysplasia, as well as NMR/CT scans once a year.