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J Crit Spine Cases 2012 Nov; 5(2): 73-76
Cervical Primary Peripheral Primitive Neuroectodermal Tumor (pPNET) Mimicking Schwannoma and Meningioma
Yoon-Sik Oh, MD, Jun Seok Bae, MD and Jee-Soo Jang, MD, PhD
Department of Neurosurgery, Seoul Wooridul Spine Hospital, Seoul, Korea
Schwannomas and meningiomas are common intradural extramedullary tumors of the cervical spine. Cervical primary peripheral primitive neuroectodermal tumor is an uncommon malignancy that is increasingly being reported in literature. We report a very unusual cervical primitive neuroectodermal tumor that presented as an intradural extramedullary tumor mimicking a schwannoma or meningioma. A 25-year-old man presented with a 2-month history of progressive weakness in his upper extremities, difficulty in walking, and cervical pain radiating through both arms. Magnetic resonance images revealed a homogeneously contrast-enhanced eccentric mass, expanding into both foramens at the C4-7
level. The patient underwent surgery after a preliminary diagnosis of a schwannoma or meningioma. At surgery, a grayish tumor of soft consistency was found in the intradural extramedullary space, and the tumor margin appeared to be relatively well demarcated with severe adhesion. Spinal cord edema was also noted. The tumor appeared to originate from the right C5 nerve root and extended to the bilateral foramen at the C4-5 and C5-6 levels. Using an operating microscope, subtotal resection of the mass was achieved. Multiple biopsy specimens were obtained, and histological examination revealed a peripheral
primitive neuroectodermal tumour. A positron emission tomography scan at another institute did not reveal uptake at any site. These findings confirmed the diagnosis of a primary cervical intradural extramedullary primitive neuroectodermal tumor. To our knowledge, there is only 1 previous report of a peripheral primitive neuroectodermal tumour, which presented as an intradural extramedullary tumor of the cervical spine. In the present case, the preoperative diagnosis was confused with schwannoma or meningioma. Due to its rare prevalence, the radiologic findings of the present case failed to deliver a diagnosis. Peripheral primitive neuroectodermal tumour presenting as a cervical intradural extramedullary
tumor is rare, but needs be considered, since early recognition can improve prognosis.
CervicalㆍIDEMㆍPrimitive neuroectodermal tumor
J Crit Spine Cases 2012 Nov; 5(2): 73-76

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