Page 94 - ARNM-2-4

Advances in Radiotherapy &

Nuclear Medicine

CASE REPORT
Chemotherapy for a malignant peripheral nerve

sheath tumor: A case report

Phuong Cam Pham 1,2,3 , Hien Quang Le 1,3 , Duong Binh Nguyen 3 ,
1,3
Hoang The Tran 3 , Khoa Trong Mai 1,2 , and Thai Van Pham *
1 The Nuclear Medicine and Oncology Center, Bach Mai Hospital, Hanoi, Vietnam
2 Department of Oncology and Nuclear Medicine, University of Medicine and Pharmacy, Vietnam
National University, Hanoi, Vietnam
3 Department of Nuclear Medicine, Hanoi Medical University, Hanoi, Vietnam

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon and aggressive
soft-tissue sarcomas. They are difficult to manage due to their aggressiveness and
limitations in early diagnosis and management. Complete surgical resection with or
without adjuvant radiotherapy is the most effective treatment for patients with primary
disease, whereas in those with metastatic disease, cytotoxic chemotherapy is beneficial.
We herein present the case of a woman who complained of severe lower back pain.
Computed tomography and magnetic resonance imaging revealed multiple bone and
lung lesions. The patient underwent a bone lesion biopsy and was diagnosed with an
advanced MPNST, which was appropriately treated with systemic chemotherapy.

*Corresponding author: Keywords: Malignant peripheral nerve sheath tumor; Treatment; Chemotherapy;
Thai Pham Van
(phamvanthai@hmu.edu.vn) Metastasis; Anthracycline; Enhancement; Rare cancer; Rare disease
Citation: Pham PC, Le HQ,
Nguyen DB, Tran HT, Mai KT, Pham
TV. Chemotherapy for a malignant
peripheral nerve sheath tumor: A 1. Introduction
case report. Adv Radiother Nucl
Med. 2024;2(4):3462. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive tumors that
doi: 10.36922/arnm.3462 account for 5 – 10% of all soft-tissue sarcomas, and 50 – 60% of MPNST cases are associated
1
1
Received: April 21, 2024 with neurofibromatosis Type 1, whereas others are radiation-induced or sporadic types.
High-resolution positron emission tomography scans are used for diagnosing MPNSTs,
Accepted: October 28, 2024
and surgical excision is their definitive treatment. Distant metastases, usually to the
Published Online: December 10, lung, liver, or bones, are found in 40 – 70% of patients. Localized disease is treated with
2
2024 radical surgery accompanied by adjuvant chemotherapy or radiotherapy. Post-operative
Copyright: © 2024 Author(s). radiation therapy influences disease-free survival rates. Chemotherapy is administered
This is an Open-Access article to patients with systemic disease. In general, MPNST has a poor prognosis and high
distributed under the terms of the
Creative Commons Attribution mortality rates. The median survival depends largely on the subtypes of tumor and
License, permitting distribution, variations in the molecular biology profiles. In Vietnam, currently, no official data on
and reproduction in any medium, the morbidity and mortality due to this malignant disease have been reported. Here, we
provided the original work is
properly cited. report a clinical case of metastasis MPNST treated by chemotherapy.
Publisher’s Note: AccScience 2. Case presentation
Publishing remains neutral with
regard to jurisdictional claims in A 43-year-old woman was admitted to our hospital because of severe lower back pain
published maps and institutional
affiliations that lasted for 3 weeks and severe lower limb numbness and weakness. She had no

Volume 2 Issue 4 (2024) 1 doi: 10.36922/arnm.3462