Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature

Abstract

Introduction. Primitive neuroectodermal tumors (PNETs) are malign neoplasms of the central nervous system which mainly locate in cerebellum (medulloblastoma). Primary intraspinal PNETs are rare. Within this group, we have found ten cases of purely intramedullary PNETs (IPNETs). In this report, we describe a new IPNET case and review the literature about these infrequent intramedullary tumors.Case report. A 17 month-old boy showed progressive decrease of motion in his lower extremities. Spine magnetic resonance imaging revealed an intramedullary expansive lesion from T3 to T10. A near-total removal was performed. The pathological diagnosis was PNET. Subsequent chemotherapy was recommended. Six months after operation, holocord progression has occurred.Conclusion. IPNETs are uncommon tumors affecting children and young adults. They are characterized by recurrence, progression or intracranial dissemination. Outcome is dismal: most patients die within two years in spite of surgical resection followed by radiotherapy and chemotherapy.KEYWORDS: Primitive neuroectodermal tumor. Spinal cord. Primary intramedullary. Tumor neuroectodérmico primitivo espinal exclusivamente intramedular: caso clínico y revisión de la literatura Resumen Introducción. Los tumores neuroectodérmicos primitivos (PNETs) son neoplasias malignas del sistema nervioso central que principalmente se localizan a nivel del cerebelo (denominadas en este caso meduloblastomas). Son muy poco frecuentes los PNETs primarios intraespinales. Dentro de este grupo, solamente hemos encontrado diez casos de PNETs exclusivamente intramedulares. En este artículo, describimos un nuevo caso de IPNET y procedemos a revisar la literatura existente sobre este tipo de tumores.Caso clínico. Se trata de un niño de 17 meses de edad que ha desarrollado una paraparesia progresiva. La resonancia magnética espinal muestra una lesión expansiva intramedular que se extiende desde T3 hasta T10. Se procedió a una extirpación macroscópica casi completa. El estudio anatomopatológico reveló un PNET. Se recomendó quimioterapia. Seis meses después de la cirugía, ha habido progresión a lo largo de la médula.Conclusión. Los PNETs exclusivamente intramedulares son raras neoplasias que afectan a niños y adultos jóvenes. Se caracterizan por la recurrencia, progresión o diseminación intracraneal. Son procesos de muy mal pronóstico, ya que los pacientes mueren en los primeros dos años a pesar de la resección quirúrgica y posterior radioterapia y quimioterapia.PALABRAS CLAVE: Tumor neuroectodérmico primitivo. Médula espinal. Primario intramedular

Metastatic thoracic and lumbar intramedullary and extramedullary Ewing’s sarcoma: a rare case report and literature review

“…The identified cases showed that intramedullary Ewing sarcoma was located mainly in the thoracic and lumbar regions, although cervical and sacral locations were also reported. 4–36 The current case had a thoracic intramedullary lesion and a lumbar extramedullary lesion, both causing neural compression and deficits.…”

“…2 The prognosis and survival rate for patients with spinal cord Ewing sarcoma is not well known because of the rarity of the condition; however, based on previous cases (Table 1), the survival rate was 45% to 50% in primary cases and 30% to 35% in recurrent and metastatic cases. 4–36 In surviving cases, rehabilitation and physical therapy should be considered part of the treatment program to improve the patient’s neurological status. In the current case, the patient improved markedly after 4 months of physical therapy and rehabilitation.…”

“…Although metastasis of Ewing sarcoma to the central nervous system (CNS) as well primary CNS disease have been reported previously, involvement of the spinal cord is extremely rare and requires specific management and considerations. 3 To date, only 36 cases of intramedullary Ewing sarcoma have been reported (summarized in Table 1), 4–36 among which there were 31 primary cases 4–11,13,15–17,19–32,34,36 and 5 recurrent and metastatic cases. 12,14,18,33,…”

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor

“…For example, a series of 28 patients with peripheral PNET in their limbs showed a 1-year and 2-year survival rate of 100% and 64%, respectively 17 . In contrast, purely intramedullary PNET has dismal outcome: most patients die within two years in spite of surgical excision followed by radiotherapy and chemotherapy 18 . Whether extraosseous tumor origin may be a favorable prognosis factor is still unclear 19,20 .…”

A Primary Primitive Neuroectodermal Tumor of the Central Nervous System in a 51-year-old Woman: a Case Report and Literature Review

“…These are small round cell tumors of neuroectodermal origin with high malignant potential (3). PNETs of the central nervous system (CNS) are uncommon malignant neoplasms of the cerebral hemispheres and suprasellar region and include supratentorial, infratentorial, brain stem, and spinal cord tumors (4). PNET usually occurs in children and only sporadically in adults (older than 20 years) (1) accounting for approximately 2.5% of brain tumors in children and 0.46% in adults (2).…”