Primitive Neuroectodermal Tumor Of The Spine In A 64-year-old Male Successfully Treated With Surgery, Craniospinal Radiotherapy And Concurrent Chemotherapy: A Case Report | 109321
Journal of Neurological Disorders
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Introduction: Primitive Neuroectodermal Tumors (PNETs) are a heterogeneous group of malignant neoplasms that occur
mostly in childhood and early adulthood. Primary spinal PNETs represent a small percentage of these tumors. In this study,
we describe the case of a 64-years-old male with primary spinal PNET who was successfully treated with surgery, craniospinal
radiotherapy and concurrent chemotherapy.
Case Presentation: This is the case of a 64-year-old male who presented with a two month history of bilateral lower extremity
weakness and numbness associated with urinary and bowel incontinence. Work-up was done and plain Magnetic Resonance
Imaging (MRI) of the spine revealed a heterogeneously enhancing intradural lesion with extradural component at the right
T9/T10 level causing mild to moderate cord compression. The patient underwent laminectomy and gross total resection of the
tumor. Histology and immunohistochemistry were consistent with a primitive neuroectodermal tumor of the spine. The tumor
recurred three months after the surgery and the patient was then referred for radiation therapy with concurrent chemotherapy.
Repeat spinal MRIs at four and ten months after treatment showed no tumor recurrence.
Conclusion: Primary spinal PNET is rarely found in adults, especially in the elderly. Due to its rare incidence in this age group,
the management of these tumors was largely based on studies done on that of the pediatric population and on Central Nervous
System (CNS) PNETs. Despite the tumor being radiosensitive and chemo sensitive, the prognosis remains poor and there are
no current recommendations or guidelines regarding its management and surveillance.