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

Abstract

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.

Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases

“…An induction chemotherapy before tumor excision tends to reduce the tumor size, and also to control systemic disease. [ 2 ] But in intrathecal PNET, the blood–brain barrier prevents chemotherapeutic agents from gaining access to the tumor, making it difficult to treat. [ 9 ] In our case, no chemotherapy was given after the first operation under the consideration that chemotherapy with multiregimen might also have numerous side effects in children.…”

“…[ 1 ] Although PNET is the second most common malignant neoplasm in childhood, the spinal cord, as primary site for PNET, is relatively rare in all age groups. [ 2 ] A large series of 430 patients showed that PNETs represent less than 1% of primary spinal tumors, [ 3 ] and according to the data from Taiwan Health Promotion Administration, there were only 2 patients with newly diagnosed PNET of nerve system in 2013. With reviewing publications, most reports on PNET focused on supratentorial PNET, whereas PNET affecting spinal location is extremely rare.…”

“…Due to the low incidence of these tumors, there are currently no standard clinical guidelines outlining their management. [ 2 ] A reviewed article concluded that recommended treatment for spinal PNET should include decompressing neural elements to prevent further neurological decline, obtaining an adequate tissue sample for pathological examination, and resecting as much tumor as can be safely removed. Evidence of benefit from adjuvant therapy in treating spinal PNETs is not well-established at present.…”

Lumbosacral epidural primitive neuroectodermal tumor (pnet); case report and literature review

“…Spinal PNET is a rare condition, but the incidence is increasing because of improved diagnostic modalities (13). Several cases of spinal PNET have been reported in the literature, mostly in children (4,6,7,15). However, epidural PNET is an extremely rare presentation with less than 15 cases reported in the literature (1-3,5,8,10-13).…”

“…PNETs of the central nervous system (CNS) have been reported in several locations including the cerebrum, cerebellum, brainstem, pineal gland, spinal cord and peripheral nerves (9,14,16). PNET of the spinal column is a rare condition and most of the reported cases are intramedullary (4,6,7,15,17). Only few cases of isolated epidural PNET have been reported in the literature, most of them in the children (2,3,10,11).…”