Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases

Abstract

Rationale:Primary spinal primitive neuroectodermal tumor (PNET) is relatively rare in all age groups, and the prognosis in most cases of spinal PNETs appears to be poor, with a median patient survival of 1 to 2 years. We present a case with recurrent spinal PNET with brain and bone metastases that was successfully treated by multimodality treatment.Patient concerns:A 14-year-old teenage girl had suffered from progressive left upper back pain with bilateral lower legs weakness and numbness for 1 year. After treatment, left neck mass was noted 3 years later.Diagnoses:Initially, magnetic resonance imaging (MRI) showed neurogenic tumor involving intradural extramedullary space of T5-T10. Pathology report showed PNET (World Health Organization grade IV) featuring lobules of neoplastic cells with round regular nuclei, high nucleus-to-cytoplasm ratio, and fibrillary cytoplasm. At the time of tumor recurrence, chest MRI then showed recurrent tumor at T2-T3 level of the epidural space with right neural foramina invasion. Brain MRI showed extensive bilateral calvarial metastases and leptomeningeal metastases in the right frontoparietal regions. Bone scan showed multiple bone metastases.Interventions:T-spine tumor removal and adjuvant radiotherapy (RT) to T-spine tumor bed were performed in the initial treatment. After clinical tumor recurrence, tumor removal was done again. She then received chemotherapy followed by whole brain irradiation with hippocampal sparing with 35 gray in 20 fractions.Outcomes:After treatment, follow-up images showed that the disease was under control. There was no neurological sequela. She has survived more than 7 years from diagnosis and more than 4 years from recurrence to date.Lessons:Multimodality treatments including operation, RT, and chemotherapy should be considered in the initial treatment planning, and salvage chemotherapy was useful in this case.

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

“…According to our results, pPNETs can metastasize to distant sites like the brain, bone, lungs, lymph nodes, and liver, 14 which is consistent with reported cases. 15,16 As such, preoperative whole-body CT and/or MRI is highly recommended.…”

“…Chen et al 16 reported that a 14-year-old girl survived >7 years after being diagnosed with pPNET, with no recurrence in >4 years. The patient underwent craniospinal radiotherapy and chemotherapy after surgery.…”

Neuroblastic primitive neuroectodermal tumor of the spinal cord: case report / Tumor neuroblástico primitivo neuroectodermal da medula espinal: relatório do caso

“…Although PNET is the second most common malignant neoplasm in childhood, the spinal cord as the primary site is extremely rare in all age groups [1][2][3]. According to the study of Amirjanshidi et al, there aer only 29 cases reported in 22 studies in 2012.…”

“…Primary spinal PNETs are more prevalent in pediatric populations and young adults, with a median age at diagnosis around 24 years-old, and are more commonly seen in males than in females, with a prevalence of approximately 2:1 [3].…”

“…The prognosis in most cases of PNET of the spinal cord appears to be poor, with an average survival of 1 to 2 years. Approximately one third of the patients will present cerebrospinal dissemination [3].…”