Pediatric spinal pilomyxoid astrocytoma

Abstract

<jats:p>Pediatric spinal pilomyxoid astrocytoma (PMA) is an extremely rare tumor that merits recognition as a specific, unique entity. The authors present the case of an intramedullary PMA in the thoracic spinal cord of an 11-year-old boy who presented with back pain, scoliosis, and multiple lung nodules. The patient underwent T5–11 laminoplasty and near-total resection of the spinal tumor. The final pathological diagnosis was WHO Grade II PMA. The patient did well for 14 months until the tumor progressed both clinically and radiographically. A literature review focusing on the clinical characteristics, histology, and treatment of PMAs provides a better understanding of these rare lesions. Because of the small number of cases optimal treatment guidelines have not been established, but gross-total resection and adjuvant chemotherapy with alkylating agents appear to confer a better long-term prognosis. Pediatric patients with PMAs can remain recurrence free at least 5 years after surgery, although these tumors may disseminate or dedifferentiate into more malignant gliomas. Recognition of intramedullary PMA as a unique entity in children is vital to the development of specific surgical and adjuvant treatment regimens.</jats:p>

Single-Agent Carboplatin for a Rare Case of Pilomyxoid Astrocytoma of the Spinal Cord in an Adult with Neurofibromatosis Type 1

“…These cases varied widely according to age, spinal cord location and outcome [3, 4, 5, 6, 7, 8, 9, 10]. In these cases, various treatment regimens were administered and often involved combinations of surgery, radiotherapy, vincristine and/or platinum-based chemotherapy with mixed success rates.…”

A rare case of multicystic disseminated astrocytoma with pilomyxoid characteristics in a 4-year-old child

“…The radiological features for diagnosing PMA has gradually emerged, with most imaging supporting the appearance of a predominantly solid, homogenously enhancing tumour commonly located in the hypothalamic-chiasmatic region with minimal peritumoural oedema and no hydrocephalus or CSF dissemination (2)(3)(4)(5)(6). Other anatomical locations for PMA have also been reported including the cerebellum and spinal cord (9)(10)(11)(12)(13)(14)(15)(16)(17).…”

Thoracolumbar pilomyxoid astrocytoma concomitant with spinal scoliosis: A case report and literature review

“…Intramedullary pilomyxoid astrocytoma (PMA) is a variant of pilocytic astrocytoma (PA). [2,5,6] That is categorized as the WHO Grade 2. [6] However, PMA is more aggressive than PA and is usually located within the spinal cord of younger patients.…”

“…[9] The treatment options for PMA include surgical resection which is often limited to debulking (e.g., extent restricted to minimize neurological deficit) followed by radiotherapy with or without chemotherapy. [5,10] Here, we are presenting a 9-year-old with a thoracic PMA and scoliosis who successfully underwent laminectomy with partial tumor debulking/resection plus duraplasty and exhibited an adequate 2-year postoperative neurological outcome.…”