Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literature.
BACKGROUND
Peripheral 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.


METHODS
We 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.


CONCLUSIONS
After 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.
Department of Pathology, Children's Hospital, Ibn Sina University Hospital, Ibn Sina bd Ibn Rochd, Souissi, 10100, Rabat, Morocco. mouna.khmou@yahoo.fr.|Faculty of Medicine and Pharmacy Rabat, University Mohammed V, Rabat, Morocco. mouna.khmou@yahoo.fr.
Mouna
M
Khmou
Department of Pathology, Children's Hospital, Ibn Sina University Hospital, Ibn Sina bd Ibn Rochd, Souissi, 10100, Rabat, Morocco.|Faculty of Medicine and Pharmacy Rabat, University Mohammed V, Rabat, Morocco.
Abderrahmane
A
Malihy
Department of Pathology, Children's Hospital, Ibn Sina University Hospital, Ibn Sina bd Ibn Rochd, Souissi, 10100, Rabat, Morocco.|Faculty of Medicine and Pharmacy Rabat, University Mohammed V, Rabat, Morocco.
Najat
N
Lamalmi
Department of Pathology, Children's Hospital, Ibn Sina University Hospital, Ibn Sina bd Ibn Rochd, Souissi, 10100, Rabat, Morocco.|Faculty of Medicine and Pharmacy Rabat, University Mohammed V, Rabat, Morocco.
Lamia
L
Rouas
Department of Pathology, Children's Hospital, Ibn Sina University Hospital, Ibn Sina bd Ibn Rochd, Souissi, 10100, Rabat, Morocco.|Faculty of Medicine and Pharmacy Rabat, University Mohammed V, Rabat, Morocco.
Zaitouna
Z
Alhamany
D000071443:12E7 Antigen / Q000378:metabolism; D002648:Child; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000473:pathology*
D000071443:12E7 Antigen
Childhood; Extra-dural; PNET; Spine; Tumors