Malignant ossifying fibromyxoid tumor of the brain treated with post-operative fractionated stereotactic radiation therapy: A case report and literature review

Abstract

Background: Ossifying fibromyxoid tumor (OFMT) is a rare musculoskeletal soft-tissue neoplasm of uncertain histogenesis most frequently occurring in the lower extremities. Conventionally, considered benign, these tumors are often managed by surgical resection followed by surveillance. However, malignant OFMTs with an increased propensity for local recurrence and distant metastasis have been recently identified, and the role of adjuvant therapy in these more aggressive cases is unclear. Case Description: We present, to the best of our knowledge, the first reported case of a primary, malignant, and intracranial OFMT. A 29-year-old female presented with recurrent headaches secondary to a large mass in her right frontal lobe. She underwent gross total resection of the brain mass with final pathology consistent with malignant OFMT demonstrating high-risk features including increased cellularity, grade, and mitotic activity. Due to these high-risk features, she received postoperative fractionated stereotactic radiation therapy (FSRT) to the resection cavity, and to the best of our knowledge, she represents the only known patient with OFMT to be treated with adjuvant FSRT. She tolerated the adjuvant treatment well with no acute or late toxicities and remains disease-free over 5 ½ years after resection. Conclusion: Adjuvant FSRT appears to be a safe and efficacious approach for managing this rare intracranial disease presentation. We review this patient’s clinical course in the context of the literature to demonstrate the difficulties associated with accurate diagnosis of this rare tumor and the controversial role of adjuvant therapy in preventing disease recurrence in this patient population.

Malignant Ossifying Fibromyxoid Tumor of the Chest Wall Metastasized to the Lung Even After Complete Resectioning of the Primary Tumor - A Case Report and a Comprehensive Review

“…OFMTs resemble other mesenchymal tumors microscopically; therefore, immunostaining for molecular markers is essential for diagnosis [ 13 ]. The peripheral rim of bone tissue and positive S-100 immunostaining are the best predictors of typical OFMTs.…”

“…There is limited literature regarding adjuvant radiation and chemotherapy in aggressive OFMTs. Our literature review found two OFMT cases where radiation therapy was used to treat the tumor and prevent local recurrence [ 13 , 15 ]. In these case reports, after using adjuvant radiation therapy, one patient was disease-free for more than 5 ½ years after resection, and the second was disease-free for more than 18 months on the routine follow-up.…”

Polymeric Metal Contrast Agents for T1-Weighted Magnetic Resonance Imaging of the Brain

“…T 1 MR image of a brain with a fibromyxoid tumor (a) without contrast (b) with contrast . Reproduced with permission from ref . Copyright 2021 Surgical Neurology International.…”

When molecular outsmarts morphology: Malignant ossifying fibromyxoid tumors masquerading as osteosarcomas, including a novel CREBZF::PHF1 fusion

“…Herein, we present the narrative of two clinicopathologically unconventional cases of malignant OFMT originally misclassified as highgrade osteosarcoma, including one of four reported cases primary to the brain which also harbored a heretofore undescribed CREBZF::PHF1 fusion. [1][2][3] Pitfalls inherent to the differential diagnosis are discussed.…”