Pineal region glioblastoma: report of two long term survivors

Abstract

BACKGROUND

Pineal region glioblastoma (GBM) is an rare entity and is considered to have a poor prognosis similar to cerebral GBM. The current literature describes approximately 28 cases of pineal region GBM reported worldwide with no case series of more than 3 patients. Even with radical treatment the median survival is reported as 6 months (range 6–24 months) with occasional reports describing survival in the range of 24–38 months.

METHODOLOGY

Retrospective audit of all cases of GBM treated in our unit between 2008 and 2018. Data were collected from pathology reports, case notes, clinic letters and MDT outcomes.

RESULTS

We identified only two cases of pineal region GBM in male patients, aged 21 years and 50 years at the time of diagnosis (in 2013 and 2011 respectively), who are alive at 53 and 75 months since diagnosis. They are still under follow-up, maintaining a good performance status with no radiological evidence of tumour recurrence till date. Both patients had a biopsy, CSF diversion procedure, radical radiotherapy with concomitant temozolomide chemotherapy and 6 cycles of adjuvant temozolomide.

CONCLUSION

Although the overall survival for GBM following only a biopsy and chemoradiotherapy is considered poor, our experience of these tumours in the pineal region show a longer survival with similar treatment, when compared to GBM in cortical location. A larger study including detailed molecular genetic analysis may help in better understanding of the tumour behaviour and whether the location in the pineal region may be of prognostic value. Due to the rarity of GBM in the pineal region, this may only be possible if multiple centres collaborate in such studies.