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

Abstract

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.

Pineal Parenchymal Tumor with Intermediate Differentiation Treated with Radio-Chemotherapy: First Case Report in Our Institution

“…The study of 30 patients of pineal tumors demonstrated a link between radiation dose and survival time. 7 In this patient, as the first case report of PPTID in our hospital, the total tumor removal was hard to achieve due to the tumor location and invasion toward the surrounding areas. This patient will be monitor with a brain and spine MRI scan for residual lesions and recurrence which will be presented at a multidisciplinary conference.…”

“…Prognostic factors were grading of tumor, neuraxis spread, and extent of resection. 7,11 Watanabe et al, 9 reported on patients with PPTID with cerebrospinal dissemination who received 36Gy of CSI and adjuvant chemotherapy in the management of CSF dissemination. The total radiation dose >50Gy and chemotherapy may be an effective treatment for PPTID with dissemination.…”

“…Localized radiation therapy could be an effective treatment strategy for PPTID. 6,7 All findings were presented at the multidisciplinary meeting and it was agreed that the patient would be treated first with radiotherapy to include all the thecal sac (craniospinal irradiation, CSI) with or without adjuvant chemotherapy. The 3600cCGy in 20 fractions of CSI were treated with 3D/IMRT technique with minimally interrupted neutropenia.…”

Therapeutic strategy for pineal parenchymal tumor of intermediate differentiation (PPTID): case report of PPTID with malignant transformation to pineocytoma with leptomeningeal dissemination 6 years after surgery

“…Also, craniospinal irradiation would be a logically acceptable approach for patients with leptomeningeal dissemination. 7 Schild et al reported on 30 patients with pineal tumors and found a relationship between the radiation dose and survival time in patients with PPT. 25 They reported that patients receiving a higher dose (> 50 Gy) of radiation had a significantly improved 3-year survival rate compared with those receiving a lower dose (94% vs 56%, respectively).…”

“…Also, craniospinal irradiation would be a useful treatment for PC patients with leptomeningeal dissemination. 7 Gomez et al reported a patient in whom PC was diagnosed by biopsy and who was treated with local irradiation. Ten years after treatment, the patient showed craniospinal CSF dissemination, and histological examination of the recurrent tumor revealed a pathologically malignant change (MIB-1 index 10%-15%), and a diagnosis of PPTID was established.…”