Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report

Journal of Neuro-oncology
Thomas KrampulzMaximilian J A Puchner

Abstract

In adults, supratentorial primitive neuroectodermal tumor (sPNET) is a very rare undifferentiated embryoblastic neoplasm. Prognosis is worse in comparison to infratentorial medulloblastoma. Older age appears to be prognostically favorable. At present, 5-year survival rates remain below 50% in all age groups. Survival longer than 15 years in an adult has only been reported once so far. In 1987, a 33-year-old-male patient presented with seizures following a six-month's history of dizziness. CT- and MRI-scans revealed a right occipital tumor with moderate contrast enhancement. The tumor was completely removed. The original histological diagnosis was that of an undifferentiated sarcoma, malignant hemangioendothelioma, grade III. The patient was treated by CyVADIC chemotherapy and conventional radiation therapy (60 Gy). Admission for another reason in 2003 led to a re-evaluation of the original diagnosis. Microscopy revealed a malignant, highly cellular, poorly differentiated tumor with a desmoplastic component. Up to 20% of tumor nuclei were labeled for Ki-67. Almost all cells were stained for neuron specific enolase and NGF-Rp75, with neuronal and glial markers being present to a variable extent. According to these findings, the d...Continue Reading

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Citations

Apr 1, 2008·Journal of Medical Imaging and Radiation Oncology·R I Smee, J R Williams
Oct 26, 2012·Journal of Medical Case Reports·Shokry LawandyJaved Siddiqi
May 31, 2013·Journal of Neuro-oncology·Rikesh GandhiCory Adamson
Jan 22, 2021·Journal of Neurological Surgery. Part A, Central European Neurosurgery·Martina PiloniPietro Mortini

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