RESUMEN
Subependymal giant cell astrocytoma (SEGA) is a low-grade periventricular tumor that is closely associated with tuberous sclerosis complex (TSC). SEGA typically arises during the first two decades of life and rarely arises after the age of 20-25 years. Nevertheless, it has also been reported that glioma histologically resembling SEGA, so-called SEGA-like astrocytoma, can arise in neurofibromatosis type 1 (NF1) patients, even in the elderly. Herein, we report a case of SEGA-like circumscribed astrocytoma arising in the lateral ventricle of a 75-year-old woman. Whole-exome sequencing revealed a somatic variant of NF1. Methylation array analysis led to a diagnosis of "methylation class glioblastoma, IDH-wildtype, mesenchymal-type (GBM, MES)" with a high calibrated score (0.99). EGFR amplification, CDKN2A/B homozygous deletion, chromosomal +7/-10 alterations, and TERT promoter mutation, typical molecular abnormalities usually found in GBM, were also observed. While most reported cases of SEGA-like astrocytoma have arisen in NF1 patients, the patient was neither TSC nor NF1. Near total removal was accomplished with endoscopic cylinder surgery. At the 36-month follow-up, there was no tumor recurrence without adjuvant therapies. This clinical behavior did not match GBM. SEGA-like astrocytoma of the elderly is rare, and this is the oldest case reported so far. In addition, high-grade molecular features found in circumscribed tumor remain unclear. Further investigations among larger series are needed for clarifying the underlying molecular mechanisms.
RESUMEN
Background: Although there have been some reports on endoscopic glioblastoma surgery, the indication has been limited to deep-seated lesions, and the difficulty of hemostasis has been a concern. In that light, we attempted to establish an endoscopic procedure for excision of glioblastoma which could be applied even to hypervascular or superficial lesions, in combination with pre-operative endovascular tumor embolization. Methods: Medical records of six consecutive glioblastoma patients who received exclusive endoscopic removal between September and November 2020 were analyzed. Preoperative tumor embolization was performed in cases with marked tumor stain and proper feeder arteries having an abnormal shape, for instance, tortuous or dilated, without passing through branches to the normal brain. Endoscopic tumor removal through a key-hole craniotomy was performed by using an inside-out excision for a deep-seated lesion, with the addition of an outside-in extirpation for a shallow portion when needed. Results: Endoscopic removal was successfully performed in all six cases. Before resection, endovascular tumor embolization was performed in four cases with no resulting complications, including ischemia or brain swelling. Gross total resection was achieved in three cases, and near total resection in the other three cases. Intraoperative blood loss exceeded 1,000 ml in only one case, whose tumor showed a prominent tumor stain but no proper feeder artery for embolization. In all patients, a smooth transition to adjuvant therapy was possible with no surgical site infection. Conclusion: Endoscopic removal for glioblastoma was considered to be a promising procedure with minimal invasiveness and a favorable impact on prognosis.
