Not a Meatball - Path PPT

Endoscopic transphenoidal biopsy; giant cells were present; RANK-L +. Extended endonasal approach to the clivus cleared the sphenoid sinus. Pathology revealed giant cells with a background of mononuclear stromal cells. Two months later she developed new ptosis OS consistent with a left cranial nerve III palsy. MRI scan showed recurrent mass in the sphenoid and she underwent repeat transphenoidal resection. Post operatively, she was started on infusions of denosumab.Six month follow up demonstrated an essentially normal exam. The first pathologic description of this condition was in 1954 by Jaffe. Giant cell lesions are more common in children or young adults and women. While they have been described in long bones, they are not infrequent in the mandible and maxilla, but only 2% involve the cranium. When the sphenoid is involved, patients present with diplopia(most commonly VI), change in vision, and headaches. CT scan usually reveals lytic changes. MRI scanning demonstrates low intensity on both T1 and T2, usually with Gadolinium enhancement. Radiographically it can be difficult to distinguish giant cell tumors from giant cell granulomas, emphasizing continued need for pathology. Pathologic evaluation reveals fewer nuclei in giant cell granulomas than giant cell tumors. Although giant cell granulomas may recur, they do not, like giant cell tumors, metastasize (1-6% usually to lungs). Definite treatment is unfortunately yet to be defined. Surgical decompression can be helpful. Radiation therapy has been employed. Calcitonin may also play a role inhibiting osteogenesis reducing abnormal bone turnover. Currettage alone is associated with a high rate of recurrence. Because of the bone involvement and the positive RANK-L markers monoclonal antibodies (denosumab) have been postulated to alter osteoclastogenesis.