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Washington University Experience | NEOPLASMS (GLIAL) | Diffuse midline glioma, H3 K27-altered | 12A0 Case 12 History
Case 12 History ---- The patient is a 22-year-old male who initially presented with double vision in May 2019. He underwent a biopsy at UCSF on 6/2019 which revealed diffuse midline glioma, H3K27M+, p53+, IDH1-, ATRX-. He then returned to receive radiation therapy locally in Kansas. He was treated with temozolomide and Depakote concurrently, which he completed on 8/2019. His last dose of radiation was completed on 8/15/2019. He was enrolled in a study at Stanford. He underwent Flu/Cy conditioning 9/19/20-9/21/20 with CAR-T cell infusion on 9/23/20. He had two Ommaya reservoirs in his right frontal ventricle as well as his pontine cyst. He is received CAR T-cell infusions at Stanford, most recently on 06/15/2021 (his 5th), which he tolerated well. He was seen in clinic on 6/28/21 and appeared well. He returned to Kansas City for the holidays on 06/30. On 7/01 at 2:00 a.m. he woke up with vomiting, severe headache, and worsened neurological function including trouble swallowing, increased right hemiparesis, and lethargy. He was evaluated via FaceTime by his provider, who noted a new partial right 3rd nerve palsy. He was brought to an outside hospital ED where a neurosurgeon performed two 30 cc taps through the frontal reservoir. An opening pressure was not measured at that time. He received 10 mg of dexamethasone and was transferred to Saint Louis Children's for further care. He has had previous episodes of hydrocephalus related to CAR T-cell infusions which were managed with steroids and continuous drainage of CSF from his frontal Ommaya reservoir. Also of note, patient has a migraine history that is exacerbated by tumor burden. He has been on VPA previously and done well with this for acute migraines. Patient does not have a history of seizures but has a history of abnormal EEG secondary to CNS lesion and is currently on Keppra for prophylaxis. Brain MRI on 7/2 showed a T2 heterogenous, peripherally enhancing solid and cystic mass centered in the midbrain extending to the left superior cerebral peduncle measuring 3.7 x 3.4 x 4.8 cm (transaxial by craniocaudal), which was increased in size compared to the prior December. There was extensive susceptibility within the lesion, suggestive of chronic low-level bleeding. On 7/3, he was inpainhad with complaints of pain that required Dilaudid for relief. He also developed shallowed breathing and diminished arousal. Chest x-ray revealed a persistent left sided opacification with mediastinal shift suggesting consolidative lesion. Over a period of 20-30 minutes, he continued to exhibit shallow irregular breathing. Clinicians were concerned that his decline was reflective of CNS disease progression, not solely respiratory failure associated with left lung findings. Due to continued worsening of his symptoms and respiratory status,his goals of care were transitioned to comfort measures only. He passed away on 7/2021.
