Abstract

INTRODUCTION: Pineal parenchymal tumors of intermediate differentiation (PPTID) represent less than 0.04% of brain tumors. Due to the rarity of this disease, there is no consensus as to appropriate treatment of this tumor. We present a patient with PPTID and our novel approach thereafter. CASE: A 34 year-old male presented with headache, nausea and vomiting, and an MRI showing a 2-cm pineal mass. Biopsy showed PPTID. He underwent whole brain radiation therapy and chemotherapy with carboplatin, bleomycin and VP-16. He showed recurrence after 5-years, after which partial tumor resection was performed and he underwent further treatment with cisplatin/melphalan and radiation. Two years later, MRI showed hydrocephalus and diffuse leptomeningeal spread with several solid tumors, which was treated with a VP shunt. After in-vitro chemosensitivity analysis, the patient was started on temozolamide. He had resolution of symptoms and radiographic masses. Eight months later, patient presented with further symptoms and thrombocytopenia. MRI showed tumor progression and cerebellar hemorrhage. The family elected not to treat and care was withdrawn. METHODS/RESULT: Tumor cells collected from CSF were grown in culture and sensitivity to chemotherapeutic agents was determined. In-vitro chemosensitivity trials showed DM-CHOC-PEN, a novel agent that is currently in phase-2 clinical trials at our institution, and temozolamide each had excellent response with 75% tumor cell death. We elected to treat the patient with temozolamide. CONCLUSION: We took a practical and rational approach of applying several low morbidity treatments to treat this patient's disease. Based off of our chemosensitivity trials, we found excellent results with DM-CHOC-PEN as well as temozolamide. We elected to treat the patient with temozolamide. With this approach, we were able to control this patient's disease for up to 8-years. In cases of rare tumors, we suggest the use of similar rational approaches to individual patients until proven treatment regimens are available.

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