CIC-rearranged sarcoma, a rare, undifferentiated small round cell tumor characterized by CIC gene fusions, arises in deep soft tissues of extremities and trunk, whereas an intracranial origin is rare. We report an 11-year-old girl with dural CIC-rearranged sarcoma presenting with intracranial hemorrhage at both initial diagnosis and recurrence. She presented with a sudden-onset headache and motor aphasia. Computed tomography revealed a left frontal convexity lesion with extensive acute subdural hematoma. Due to rapidly deteriorating consciousness, emergent hematoma evacuation and tumor resection were performed. Intraoperatively, the tumor was attached to the dura, suggesting a dural origin. Histopathologic examination demonstrated densely packed, atypical, small round cells within a stromal background; tumor cells were also identified in the dura mater. Comprehensive genomic profiling identified a CIC::DUX4 fusion, leading to the definitive diagnosis of CIC-rearranged sarcoma. She received adjuvant chemotherapy and radiotherapy, without recurrence evidence at 9 months postoperatively, but developed recurrent hemorrhagic lesions in the middle cranial fossa and along the tentorial edge 2 months later. Emergent surgery revealed two lesions with dural attachment; pathology confirmed recurrence. Aggressive growth and marked hemorrhagic tendency observed in this first reported case of dural CIC-rearranged sarcoma highlight the importance of vigilant follow-up and timely clinical intervention.
Luteinizing hormone-releasing hormone (LHRH) agonists are widely used for androgen-deprivation therapy in prostate cancer. Recent reports have raised concern that LHRH may influence the biological behavior of LHRH receptor-expressing intracranial tumors, including meningiomas; however, clinical evidence remains limited. We describe two cases of rapidly progressive meningioma temporally associated with the initiation of leuprorelin acetate. Case 1 was a 67-year-old man with a left cerebellopontine angle meningioma that had demonstrated gradual enlargement over 26 months. Following the initiation of leuprorelin acetate, the tumor exhibited accelerated growth, necessitating surgical resection. Pathology confirmed a fibrous meningioma with LHRH and its receptor positive. Case 2 was a 72-year-old man previously treated with subtotal resection and radiotherapy for an atypical meningioma. After 6 years of radiographic stability, the tumor rapidly regrew soon after leuprorelin acetate initiation and required re-resection. Both the primary and recurrent tumors were LHRH- and LHRH receptor-positive. Freshly resected tumor tissue cultured ex vivo demonstrated a dose-dependent increase in cell viability following 7-day exposure to leuprorelin acetate. These findings suggest that LHRH agonists may be associated with increased cell viability in a subset of receptor-positive meningiomas. Caution is warranted when initiating LHRH agonist therapy in patients with known or suspected meningiomas.
TERT promoter (TERTp) mutations are among the most frequent molecular alterations in isocitrate dehydrogenase (IDH) wild-type glioblastoma (GBM), yet the molecular profiles and prognostic implications associated with TERTp status remain incompletely characterized. This study aimed to delineate differences in molecular characteristics and prognostic factors according to TERTp status in histologically defined, IDH wild-type GBM. Clinical and molecular characteristics and survival outcomes were retrospectively assessed according to TERTp status. Among 112 patients, 70 (62.5
Central nervous system germ cell tumors (CNS GCTs) are exceptionally rare in patients aged 60 years or older. We report a 61-year-old man with progressive weight loss and endocrine dysfunction over 20 months, in whom magnetic resonance imaging revealed enhancing lesions including the suprasellar and sellar regions, optic tracts, ventricular walls, corpus callosum, and dorsal medulla. Biopsy findings supported a diagnosis of germinoma in the sampled tissue, and DNA methylation profiling demonstrated a molecular profile consistent with conventional germinoma. The patient underwent whole ventricular irradiation and carboplatin-etoposide chemotherapy. Febrile neutropenia during the first cycle required subsequent etoposide dose reductions, but he achieved marked radiographic regression. A descriptive review identified five cases, including the present case, for which sufficient individual clinicopathological information was available. This case highlights that CNS GCTs in older adults may radiologically mimic primary CNS lymphoma and that integrated pathological assessment, including morphology and immunohistochemistry, is essential for diagnosis. In this diagnostically challenging presentation, DNA methylation profiling provided complementary molecular support for the histopathological diagnosis of conventional germinoma.
