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Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report.
Spinal Cord. 2004 Mar; 42(3):199-203.SC

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.

Authors+Show Affiliations

Department of Orthopaedic Surgery, Kagoshima Graduate School of Medical and Dental Sciences, Kagoshima, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

15001982

Citation

Yone, K, et al. "Primary Malignant Peripheral Nerve Sheath Tumor of the Cauda Equina in a Child Case Report." Spinal Cord, vol. 42, no. 3, 2004, pp. 199-203.
Yone K, Ijiri K, Hayashi K, et al. Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report. Spinal Cord. 2004;42(3):199-203.
Yone, K., Ijiri, K., Hayashi, K., Yokouchi, M., Takenouchi, T., Manago, K., Nerome, Y., Ijichi, O., Ikarimoto, N., & Komiya, S. (2004). Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report. Spinal Cord, 42(3), 199-203.
Yone K, et al. Primary Malignant Peripheral Nerve Sheath Tumor of the Cauda Equina in a Child Case Report. Spinal Cord. 2004;42(3):199-203. PubMed PMID: 15001982.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report. AU - Yone,K, AU - Ijiri,K, AU - Hayashi,K, AU - Yokouchi,M, AU - Takenouchi,T, AU - Manago,K, AU - Nerome,Y, AU - Ijichi,O, AU - Ikarimoto,N, AU - Komiya,S, PY - 2004/3/6/pubmed PY - 2004/4/16/medline PY - 2004/3/6/entrez SP - 199 EP - 203 JF - Spinal cord JO - Spinal Cord VL - 42 IS - 3 N2 - 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. SN - 1362-4393 UR - https://www.unboundmedicine.com/medline/citation/15001982/Primary_malignant_peripheral_nerve_sheath_tumor_of_the_cauda_equina_in_a_child_case_report_ L2 - http://dx.doi.org/10.1038/sj.sc.3101567 DB - PRIME DP - Unbound Medicine ER -