FRI337 Progressive Suprasellar Chordoid Glioma Presenting With Panhypopituitarism, Diabetes Insipidus And Encephalopathy

Abstract

Abstract Disclosure: J.D. Gatillo: None. M.D. Maningat-Goco: None. F.K. Co: None. Chordoid glioma is a rare central nervous system neoplasm of uncertain origin, with most cases seen at anterior wall or roof of the third ventricle. It is described as a perplexing lesion as it tends to mimic benign lesions at the onset but eventually some develop with threatening complications. We present a case of a 39 year old Filipino, female, who initially presented with a 1- year history of memory lapses, behavioral changes, and blurring of vision. MRI showed 4.3 x 3 x 4.2 cm heterogeneously enhancing suprasellar mass and was initially worked-up as pituitary macroadenoma. The imaging also showed heterogeneously enhancing extra-axial lesion along the right anteromedial temporal convexity. She underwent pterional craniotomy and sub temporal craniectomy with excision biopsy of tumor. Histopathology confirmed the diagnosis of chordoid glioma WHO Grade 2. However, only a few days after the surgery, she began to experience emotional lability and more behavioral changes. She then received oral Temozolomide for 4 cycles and whole brain radiotherapy for 32 cycles. Approximately 10 months since diagnosis was made, patient eventually developed seizures and manifested progressive drowsiness and generalized weakness. Patient was diagnosed and clinically presented with Panhypopituitarism and Diabetes Insipidus. She was maintained on levothyroxine, prednisone and desmopressin. A repeat MRI was done which showed apparent progression in the size (4.6 x 3.7 x 4.1 cm) of the heterogeneously enhancing suprasellar mass with signs of tumor infiltration and extension in the left lateral ventricle. Further, patient was treated with Bevacizumab and Nimotuzumab. Repeat MRI showed regression in size of the known suprasellar mass and improvement of symptoms. Little is still known about chordoid gliomas especially in the Asian population - their initial presentation as well as the best diagnostic approach and treatment strategy for this elusive disease. There is no formally recognized treatment protocol for chordoid glioma, and postoperative mortality and morbidity is high. More case reports are needed in order to ascertain the best diagnostic and treatment strategy for patients with chordoid glioma and its endocrine complications. Presentation: Friday, June 16, 2023