Anaplastic Pleomorphic Xanthoastrocytoma With Long-term Survival: An Autopsy Case Report

Background: The survival rate of anaplastic pleomorphic xanthoastrocytoma (APXA) is not well established due to the limited number of cases, but a few reports have suggested that the 5-year overall survival rate for APXA patients isaround 50%. We herein report an autopsy case of an APXA patient who survived 30 years after her rst surgery.Case presentation: A 37-year-old woman initially underwent total resection of a right temporal lobe tumor. The primary diagnosis at that time was grade II-III astrocytoma. Postoperative radio-chemotherapy was completed and follow-up was stopped after 22 years of no recurrence. However, 30 years after the initial treatment, the patient returned presenting with deterioration in consciousness. Magnetic resonance imaging revealed multiple heterogeneously enhanced tumors. A diagnosis of APXA was reached after a reevaluation of the initial resected specimens. She died four months after the recurrence. An autopsy revealed that the recurrence was glioblastoma multiforme.Conclusion: To our knowledge, this patient is the longest APXA survival case. Autopsy specimens did not indicate local recurrences, which suggested primary lesion control could be associated with survival.

RARE-13. INFLAMMATORY MYOFIBROBLASTIC TUMOR MIMICKING DESMOPLASTIC INFANTILE GANGLIOGLIOMA (DIG) OF THE TEMPORAL LOBE

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm composed of fascicles of myofibroblastic spindle cells in a background of prominent inflammatory infiltrate. It is categorized as ‘intermediate, rarely metastasizing’ in the World Health Organization classification of tumors of soft tissue and bone. We present a novel case of concurrent brain and lung tumor with diagnosis of TFG-ROS1-rearranged IMT in a 14 year old female patient, in which targeted next-generation sequencing became a powerful tool for detection of genomic alterations in both lung and brain tumors. At age 9, the patient’s lung mass was incidentally found and investigated for various infectious diseases with negative result. At age 14, she presented with seizure and was noted to have a stable size lung mass and a left temporal lobe tumor. The left temporal lobe tumor showed a desmoplastic spindle cell neoplasm involving the meninges and cerebral cortex and Desmoplastic Infantile Ganglioglioma (DIG) was considered one of differentials. Subsequently, her right lung mass was resected and showed a similar spindle cell neoplasm with a background of dense fibrosis and chronic inflammation, consistent with Inflammatory Myofibroblastic Tumor. Molecular microdissection revealed that both tumors shares TFG-ROS1 fusion which is associated with (t(3;6) (q12;q22)), thus it is strongly suggestive that two tumors arose from the same origin. No predisposition syndrome was identified.

Neurocognitive Changes Associated With Surgical Resection of Left and Right Temporal Lobe Glioma

BACKGROUND: Little is known regarding the neurocognitive impact of temporal lobe tumor resection. OBJECTIVE: To clarify subacute surgery-related changes in neurocognitive functioning (NCF) in patients with left (LTL) and right (RTL) temporal lobe glioma. METHODS: Patients with glioma in the LTL (n = 45) or RTL (n = 19) completed comprehensive pre- and postsurgical neuropsychological assessments. NCF was analyzed with 2-way mixed design repeated-measures analysis of variance, with hemisphere (LTL or RTL) as an independent between-subjects factor and pre- and postoperative NCF as a within-subjects factor. RESULTS: About 60% of patients with LTL glioma and 40% with RTL lesions exhibited significant worsening on at least 1 NCF test. Domains most commonly impacted included verbal memory and executive functioning. Patients with LTL tumor showed greater decline than patients with RTL tumor on verbal memory and confrontation naming tests. Nonetheless, over one-third of patients with RTL lesions also showed verbal memory decline. CONCLUSION: In patients with temporal lobe glioma, NCF decline in the subacute postoperative period is common. As expected, patients with LTL tumor show more frequent and severe decline than patients with RTL tumor, particularly on verbally mediated measures. However, a considerable proportion of patients with RTL tumor also exhibit decline across various domains, even those typically associated with left hemisphere structures, such as verbal memory. While patients with RTL lesions may show even greater decline in visuospatial memory, this domain was not assessed. Nonetheless, neuropsychological assessment can identify acquired deficits and help facilitate early intervention in patients with temporal lobe glioma.