Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report

Abstract

Study design: A case report of primary malignant peripheral nerve sheath tumor (MPNST) of the cauda equina in a child is presented, and the literature is reviewed. Objective: To discuss the problems involved in the treatment of primary intradural MPNSTs. Setting: A department of orthopaedic surgery in Japan. Methods: A 4-year-old boy complained of low-back pain radiating to the left calf. MRI revealed an intradural tumor at L3-L5 level. Following laminectomy of L3, L4 and L5, the tumor was removed en bloc. Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST. Results: Although adjuvant chemotherapy was administered local recurrence and cerebral and spinal metastases of the tumor were found 6 months after the operation. Following additional incomplete removal of the recurrent tumor, radiation therapy was administered. Although recurrent and metastatic tumors disappeared or diminished in size by radiation, tumors increased in size thereafter, despite additional adjuvant chemotherapy. At 21 months after the first operation, he died of pneumonia. Conclusions: Reported clinical outcomes for patients with primary intradural MPNST are very poor. Although no gold standard for the treatment of tumors has been established yet, surgical removal of tumors combined with postoperative high-dose radiation may be recommended.

Recurrent malignant peripheral nerve sheath tumour in a patient with neurofibromatosis Type 1: A case report

“…11 A primary cauda equina intradural MPNST in a 4-year-old patient without NF-1 had cerebrospinal metastases 6 months after tumour excision with adjuvant therapy. 12 Despite poor efficacy of radiotherapy and chemotherapy in most patients with MPNST, a good therapeutic response was reported in a 25-year-old male NF-1 patient with recurrent retroperitoneal MPNST to cisplatin and etoposide. 13 Gallo et al reported the successful use of ifosfamide, vincristine, and doxorubicin-combined chemotherapy (the IVA-2 protocol) in these patients.…”

Metastatic malignant peripheral nerve sheath tumor of the cauda equina

“…[5][6][7][8] (Supplementary Tables 1 and 2). However, these case reports describe patients with primary MPNST of the cauda equina, whereas our patient had metastatic MPNST to the cauda equina.…”

Malignant primary nerve sheath tumor of the cauda equina in a patient without Von Recklinghausen disease

“…MPNST has the highest recurrence rate of any sarcomas, and adequate initial treatment gives the best chance of survival. [10] This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.…”

“…However, only five case of MPNST of cauda equina had been reported in literature [7][8][9][10][11] all over the world and only one case in India. [7] We are reporting the sixth case in the literature from our center.…”