Neuroblastic primitive neuroectodermal tumor of the spinal cord: case report / Tumor neuroblástico primitivo neuroectodermal da medula espinal: relatório do caso
DOI:
https://doi.org/10.34119/bjhrv4n6-194Keywords:
PNET, Ewing Sarcoma, advanced neuroblastic tumors, spinal cord.Abstract
Primitive neuroectodermal tumors (PNETs) are high-grade mesenchymal malignancies that usually occur in children and adolescents. 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.
In this report we present a patient of 29 years-old, female, with sudden low back pain that evolved with paraparesis and loss of sensitivity up to umbilical scar level. After the MRI has demonstrated an intramedullary solid lesion, a biopsy was performed and revealed a small cell neoplasia, suggesting PNET. Even with the clinical measures for spinal cord compression syndrome, the patient presented worsening of the paresthesia, evolving with sphincter alterations. She was submitted to surgical treatment achieving complete resection of the tumor, and remained with paresthesia and a slight alteration in the coordination of the lower limbs. A new MRI was performed on April 28th 2017, and confirmed disease progression. A new attempt was made to surgically decompress the tumor, without success. The patient was referred to a regional oncology hospital, when new images showed absence of metastatic disease but identified intramedullary nodules, intrathecal nodules in the subarachnoid space, leptomeningeal in the thoracic spinal cord and surgical cavity in the posterior spinal cord new nodules. The patient started palliative chemotherapy with VAC protocol, with manageable toxicities, achieving a reduction of the tumor, followed by local treatment Radiotherapy. She remained with good local control until 12 weeks post-radiotherapy, when developed rapid clinical deterioration by disease progression, evolving to death due to intracranial hypertension.
There are still few cases of PNET reported in the world, and therefore studies with the best treatment approach are not yet well stablished. At present, the treatment of this condition is similar to other high-grade sarcomas, and therapies standardization are urgently needed for more effective treatments and survival outcomes.
References
Cabral GA, Nunes CF, Melo JO, Jr, Guimarães RD, Gonçalves MB, Rodrigues RS, et al. Peripheral primitive neuroectodermal tumor of the cervical spine. Surg Neurol Int. 2012;3:91.
Engelhard HH, Villano JL, Porter KR, et al. Clinical presentation, histology, and treatment in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina. J Neurosurg Spine 2010;13:67–77.
Frank Chen, MDa, Shyh-Shin Chiou, MD, PhDb, Sheng-Fung Lin, MD, PhDc, et al. Recurrent spinal primitive neuroectodermal tumor with brain and bone metástases. Medicine (2017) 96:46(e8658).
Gaspar, N., Hawkins, D. S., Dirksen, U., Lewis, I. J., Ferari, S., Le Deley, M. C., Oberlin, O. Ewing Sarcoma: Current Menagement and Future Approaches Through Collaboration. Journal of Clinical Oncology 2015;33(27).
Kampman WA, Kros JM, De Jong TH, Lequin MH (2006) Primitive neuroectodermal tumors (PNETs) located in the spinal canal: the relevance of classification as central or peripheral PNET: case report of a primary spinal PNET occurrence with a critical literature review. J Neurooncol 77:65–72.
Khmou M, Malihy A, Lamalmi N, et al. Peripheral primitive neuroectodermal tumors of the spine: a case report and review of the literature. BMC Res Notes 2016;9:438.
Kim DW, Chang HJ, Jeong JY, et al. Ewing’s sarcoma/primitive neuroectodermal tumor (ES/PNET) of the small bowel: a rare cause of intestinal obstruction. Int J Colorectal Dis 2007;22:1137–8.
Kleihues P, Burger PC, Scheithauer BW (1993) The new WHO classification of brain tumors. Brain Pathol 3:255–268.
Rorke LB, Hart MN, McLendon RE (2000) Supratentorial primitive neuroectodermal tumor (PNET). In: Kleihues P, Cavennee WK (eds) Pathology and genetics of tumors of the central nervous system. IARC Press, Lyon, pp 141–144.
Sharma P, Das KK, Mehrotra A, et al. Cervicomedullary intramedullary peripheral primitive neuroectodermal tumor with intratumoral bleed: report of one case and review of literature. J Craniovertebr Junction Spine 2016;7:111–4.
Shimada H, Brodeur GM (1998) Tumors of peripheral neuroblastomas and ganglion cells. In: Bigner DD et al (eds) Russel and Rubinstein’s pathology of tumors of the nervous system, 6th edn. Arnold, London, pp 493–533.
Song DE, Choi G, Jun SY, et al. Primitive neuroectodermal tumor of the gallbladder. Arch Pathol Lab Med 2004;128:571–3.
Tong X, Deng X, Yang T, et al. Clinical presentation and long-term outcome of primary spinal peripheral primitive neuroectodermal tumors. Neurooncol 2015;124:455–63.
Yan Y, Xu T, Chen J, et al. Intraspinal Ewing’s sarcoma/primitive neuroectodermal tumors. J Clin Neurosci 2011;18:601–6.
