Intracranial Myxoid Mesenchymal Tumour with EWSR1-ATF1 Fusion Sans Myxoid Stroma - Report of A Newer Entity with Brief Review of Literature.

Intracranial myxoid mesenchymal tumors (IMMTs) are a relatively new group of tumors, first described in 2017. We report this rare variant in a 27-year-old female which was initially suspected to be a high-grade glial neoplasm. Next-generation sequencing confirmed the presence of fusion between the FET and cAMP response element-binding (CREB) family of genes. This fusion is diagnostic of IMMT, with only 19 such cases reported so far. The authors would like to highlight the need for genomic sequencing for the diagnosis of this tumor, its propensity to recur locally, and its relatively better prognosis as compared to high-grade gliomas.

Hemangiopericytoma: long-term outcome revisited. Clinical article.

OBJECT: Hemangiopericytomas are rare tumors that behave aggressively with a high rate of local recurrence and distant metastases. With the aim of determining the outcome and response to various treatment modalities, a series of 39 patients who underwent microsurgical resection for primary meningeal hemangiopericytoma over a 24-year period is presented. METHODS: Patients with hemangiopericytoma were identified from histopathology records and their medical records were analyzed retrospectively by 2 independent reviewers to collect data on surgical treatment, adjuvant therapy, postoperative course, local or distant recurrence, and follow-up. RESULTS: Of the 39 patients, 19 were male and 20 were female. Mean patient age was 44.1 years. Thirty-four tumors were intracranial and 5 were spinal. The mean follow-up period was 123 months. Twenty-eight patients developed local recurrence. The recurrence rate at 1, 5, and 15 years was 3.5%, 46%, and 92%, respectively. Extraneural metastasis occurred in 8 patients (26%) at an average of 123 months after initial surgery. Recurrences and metastases were treated by surgical excision, external beam radiation therapy (EBRT), chemotherapy, and/or stereotactic radiosurgery. Adjuvant EBRT following initial surgery was found to extend the disease-free interval from 154 months to 254 months, although it did not prevent the development of metastasis. In those patients with EBRT and complete resection, the mean recurrence-free interval was found to be 126.3 months longer (p = 0.04) and overall survival 126 months longer than without EBRT. Furthermore, adjusting for resection, patients undergoing EBRT had 0.33 times increased risk of recurrence compared with those who did not (p = 0.03). A majority of patients remained able to live independently despite revision surgery for recurrence. CONCLUSIONS: The mean follow-up of this patient series represents the longest follow-up duration published to date and demonstrates extended survival in a significant number of patients with hemangiopericytoma. Gross-total resection followed by adjuvant EBRT provides patients with the highest probability of an increased recurrence-free interval and overall survival. Prolonged survival justifies long-term follow-up and aggressive treatment of initial, recurrent, and metastatic disease.