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

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 28 th 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.