Table 1 Clinical, pathological, treatment, and outcome information for the five new IMT cases
From: Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor
Age (years) | Sex | Presentation | Tumor location | Imaging | Initial surgical management | Histology | Gene fusion | Outcome | |
|---|---|---|---|---|---|---|---|---|---|
Case 1 | 26 | F | Generalized tonic–clonic seizure | Anterior Falx | Avidly enhancing lobulated 1.7 × 1.3 × 1.1 cm mass with surrounding vasogenic edema | GTR | Hypercellular areas composed of spindled cells in whorls. Hypocellular areas composed of spindled or epithelioid cells in small nests or cords in a myxoid background. A dense lymphocytic infiltrate at the periphery as well as within the tumor | EWSR1::CREM | Recurrence free at 12 months |
Case 2 | 27 | F | Headache and blurry vision; papilledema | Atrium of right lateral ventricle | Homogenously enhancing 2.3 × 3.0 × 3.6 cm mass with obstructive hydrocephalus | GTR | Densely packed groups of cells in a collagenous stroma, or singly distributed cells in a myxoid background. Abundant lymphocytes, plasma cells, and scattered histiocytes | EWSR1::ATF1 | Recurrence free at 6 months |
Case 3 | 32 | M | Headache | Inferior right frontal lobe near midline | Heterogeneously enhancing 3.5 cm mass | GTR | Predominantly epithelioid cells with pleomorphic, large, hyperchromatic nuclei, intranuclear inclusions and abundant cytoplasm. Some cells are spindled. Mixed inflammatory cells, plasma cells, multinuclear giant cells, and histiocytes | EWSR1::CREM | Recurrence free at 1 month |
Case 4 | 17 | F | Headache and neck pain; papilledema | Left frontal, superior paramedian, involving middle and posterior frontal lobe | Solid and cystic mass with prominent adjacent edema and midline shift | GTR | Hypercellular, tightly packed sheets of small round tumor cells with scant cytoplasm. Scattered mitoses and apoptotic debris. No lymphocytic infiltrate | EWSR1::CREM | Recurrence at 3 months treated with proton beam radiotherapy; later developed bilateral pulmonary metastases and treated with chemotherapy; alive with extensive disease at 27 months |
Case 5 | 13 | F | Intermittent blurry vision; papilledema | Suprasellar | Heterogeneously enhancing solid and cystic mass measuring 2 cm with compression of the optic chiasm | Needle biopsy | Scattered spindled cells in a myxoid stroma. No mitotic activity | EWSR1::ATF1 | Developed profound cerebral edema at 8 months; recurrence free at 2 years |