Are we over treating Pineal Parenchymal tumour with intermediate differentiation? Assessing the role of localised radiation therapy and literature review

摘要

Pineal Parenchymal tumour with intermediate differentiation (PPTID) is a rare disorder, first classified by World Health Organisation in 2000. There are very few published data available and optimal management is yet to be determined. Management has varied from surgery alone to craniospinal radiotherapy with or without chemotherapy. We present our experience of PPTID treated with radiotherapy alone. We conducted a retrospective review of patients who were diagnosed with PPTID and treated with radiation therapy at our institute from 2010 onwards. Between January 2010 to January 2013, 5 patients of PPTID were treated at our institute. Median age is 44 (range 24–62). All patients had preoperative MRI scan of brain and spine. Imaging did not identify any spinal dissemination. None of the patients underwent a gross total resection, due to the tumour location and technical difficulties. All patients were treated with external beam radiation therapy to primary lesion only with a dose of 54 Gy in 30 fractions after surgery. 4 patients had good partial response and the remaining 1 has stable disease. After 21.4 months of median follow up no disease recurrence was reported. So far there is no evidence of cerebral white matter abnormalities on MRI scan or neurocognitive disorders. Our experience indicated that localised radiation therapy could be an effective treatment strategy for PPTID, considering the long natural course of the disease and the late adverse effects of intensive treatment.