Heavily lipidized, calcified giant cell glioblastoma in an 8-year-old patient, associated with neurofibromatosis type 1 (NF1): report of a case with long-term survival.

摘要

Giant cell glioblastoma (GCG-BM) with predominance of bizarre, multinucleated giant cells is a rare subtype of glioblastoma, however, its clinical behavior and histological features are still not fully understood. We report an unusual case of a heavily lipidized form of giant cell glioma corresponding mostly to GCGBM in a young patient with neurofibromatosis 1 (NF1). Histologically, the tumor revealed numerous characteristic histopathological features of giant cell glioblastoma including cellular pleomorphism with numerous giant tumor cells, pseudopalisades around necrotic foci and mitotic activity, accompanied by additional unique morphological elements such as massive lipidization of the neoplastic cells, abundant microcalcifications and angiomatous pattern of vascularization. Such aberrant morphology might be associated with the unusually long survival period of 12 years without clinical evidence of tumor recurrence. The coexistence of intracerebral heavily lipidized, calcified giant cell glioblastoma with NF1 has not been previously reported in literature.