Artikel
Treatment of pineal region rosette-forming glioneuronal tumours (RGNT)
Behandlung Rosetten-bildender glioneuraler Tumore (RGNT) der Pinealisloge
Suche in Medline nach
Autoren
Veröffentlicht: | 4. Juni 2021 |
---|
Gliederung
Text
Objective: Rosette-forming glioneuronal tumour (RGNT) is an extremely rare entity described for the first time in the WHO classification in 2007. So far only single case reports of RGNT in the pineal region have been published and clear therapy concepts are pending.
Methods: The study group comprised all patients with the histopathological diagnosis of RGNT (WHO grade I) that underwent microsurgical tumour removal in the pineal region in our centre between 08/2018 and 11/2020. Surgical strategy, histological findings and clinical outcome are presented and the results are evaluated in comparison to published case reports.
Results: Four male patients with age under 50 years (range between 20 and 48 years) were included in this study. Two patients presented with chronic headache since several month and two patients presented with a generalised epileptic seizure. Cystic lesions adjacent to the pineal gland and the third ventricle were diagnosed in MRI and supra-cerebellar infratentorial gross total tumour resection was performed in three cases without permanent neurological deficits. One patient underwent subtotal tumour resection with deliberate tumour remnants bilaterally in the thalamus. This patient had no neurological deficit except a vertical gaze palsy which recovered three months after surgery. None of the patients underwent additional chemotherapy or radiotherapy.
Figure 1 [Fig. 1], Figure 2 [Fig. 2]
Conclusion: Resection of RGNT in the pineal region is feasible without permanent neurological deficits and should be the treatment of choice. No additional chemotherapy or radiotherapy is recommended if gross total resection can be achieved.