Treatment of Pineal Region Rosette-Forming Glioneuronal Tumors (RGNT)

Autor: Anna Michel, Thiemo Florin Dinger, Ramazan Jabbarli, Philipp Dammann, Anne-Kathrin Uerschels, Marvin Darkwah Oppong, Neriman Özkan, Andreas Junker, Ulrich Sure, Karsten Henning Wrede
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: Cancers, Vol 14, Iss 19, p 4634 (2022)
Druh dokumentu: article
ISSN: 2072-6694
DOI: 10.3390/cancers14194634
Popis: Background: Rosette-forming glioneuronal tumor (RGNT) is an extremely rare entity described for the first time in the WHO classification of tumors of the central nervous system in 2007. Predominantly, single case reports of RGNT in the pineal region have been published, and specific therapy concepts are pending. Methods: The study group comprised all patients with the RGNT (CNS WHO grade 1) in the pineal region that underwent microsurgical tumor removal in our center (August 2018–June 2021). Surgical strategy, histological findings, and clinical outcome are presented, and the results are evaluated and compared to published case reports. Results: Four male patients aged under 50 years (range between 20 and 48 years) and one female patient, 51 years old, were included in this study. Chronic headaches and generalized epileptic seizures were the main symptoms. Supra-cerebellar infratentorial gross total tumor resection (GTR) was performed in two cases, two patients underwent subtotal tumor resection, and an endoscopic biopsy was performed in case five. Conclusion: In cases where surgical resection seems feasible with a reasonable surgical risk, we advocate GTR. Regular and long-term MRI follow-up is essential to detect a slow tumor progression. The role of additional chemotherapy or radiotherapy remains unclear.
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