Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literatureReport as inadecuate

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BMC Research Notes

, 9:438

Clinical pathology


BackgroundPeripheral primitive neuroectodermal tumors are extremely rare tumors in the spine; only 18 cases of extra-dural peripheral primitive neuroectodermal tumor cervical region have been reported. The aim of this report is to highlight the challenges in diagnosis and management of this condition.

Case presentationWe present a case of 5-year-old Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1–C6 level. Multiple biopsy specimens were obtained. Histological examination revealed a highly cellular neoplasm composed of diffuse sheets of tumor cells having monomorphic, round to oval, finely vesicular nuclei. Immunohistochemical findings confirmed the diagnosis of intraspinal peripheral primitive neuroectodermal tumor .

ConclusionAfter this illustrative case, we review the literature on clinicopathological and therapeutic aspects. In practice, it is important to consider the diagnosis of peripheral primitive neuroectodermal tumor in children and adolescents with an apparent soft-tissue mass located in the spine.

KeywordsPNET Spine Extra-dural Tumors Childhood AbbreviationsPNETperipheral primitive neuroectodermal tumors

CT scancomputerized tomography scan

Author: Mouna Khmou - Abderrahmane Malihy - Najat Lamalmi - Lamia Rouas - Zaitouna Alhamany


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