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Published on: May 17, 2024
Perioperative Observations and Outcome in Surgical Treatment of Malignant Peripheral Nerve Sheath Tumors
Julian Zipfel1,2,3,4, Jonas Tellermann1,3, Kevin Paul Ferraris4
1Department of Neurosurgery, University Hospital Tübingen, BW 72076 Tübingen, Germany.
Background/Objectives:
This retrospective observational study aimed to investigate the perioperative outcome in Malignant Peripheral Nerve Sheath Tumors (MPNSTs) with and without relation to Neurofibromatosis Type 1 (NF1) and to detect possible influencing factors.
Methods:
Clinical reports, histopathological evaluations, imaging, and treatment characteristics were reviewed in 35 operated MPNSTs in 33 patients. Possible predictive valuables included disease type, preoperative tumor volume, SUV and MIB-1 proliferation index, resection margins, the presence of metastasis, and whether radio-/chemotherapy was received.
Results:
Patients with NF1 were younger (mean age: 29 ± 13, 8-54 years) than sporadic cases (mean age: 45 ± 13, 24-67 years) and exhibited significantly larger preoperative tumor volumes (mean 299 vs. 18 cm3, p = 0.048). Most tumors were located in the facial/cervical/neck area (34%, n = 12), followed by the trunk (31%, n = 11), lower extremity (17%, n = 6), upper extremity (14%, n = 5), and intraspinal area (3%, n = 1). NF1-associated MPNSTs appeared predominantly on the trunk (39%) and sporadically in the facial/cervical/neck area (50%). Complete resection was possible in 66% and an improvement in or stability of function was achieved in most cases (motor 69%, sensory 74%), as well as a decrease in pain intensity (63%). NF1-associated MPNSTs exhibited more severe pain scores (median VRS scale 2, p = 0.002) compared to sporadic tumors (median VRS scale 0.5). Sporadic MPNSTs located at the head/facial/brachial plexus and upper extremities exhibited better preoperative functions compared to those on the lower extremities. In 12 cases with available [18F]FDG PET, the mean preoperative SUV (9.8 ± 7.2) positively correlated with the mean maximum MIB-1 index (34 ± 26%, p = 0.005) and the mean preoperative tumor volume (474.7 ± 68.6 cm3, p = 0.047). The overall survival (OS) was significantly longer in tumors with higher resection extents (R0, p = 0.01) and without accompanying metastasis (p = 0.046), and tended to be longer, but not significantly so, in sporadic MPNSTs. In six and seven tumors, with R1/R2 resection margins and present metastasis, respectively, solid or combined neo-/adjuvant radio-/chemotherapy led to a significantly shorter OS (p = 0.014).
Conclusions:
NF1-associated MPNSTs have larger tumor volumes, higher SUVs and MIB-1 proliferation indices, and a shorter overall survival period. Nevertheless, surgery can improve symptoms, particularly medication-resistant pain, and should also be considered in advanced disease for symptom control/improvement.
Insights
Malignant Peripheral Nerve Sheath Tumors (MPNSTs) in patients with Neurofibromatosis Type 1 (NF1) are larger and have poorer survival. Surgery can still improve symptoms like pain, even in advanced cases.
Area of Science:
- Oncology
- Surgical Oncology
- Genetics
Background:
- Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare and aggressive cancers.
- Neurofibromatosis Type 1 (NF1) is a genetic disorder that increases the risk of MPNST development.
- Understanding the differences and outcomes between NF1-associated and sporadic MPNSTs is crucial for treatment.
Purpose of the Study:
- To compare perioperative outcomes of MPNSTs in patients with and without NF1.
- To identify factors influencing outcomes in MPNSTs.
- To evaluate the role of surgery in managing MPNSTs, including those associated with NF1.
Main Methods:
- Retrospective observational study of 35 operated MPNSTs in 33 patients.
- Review of clinical, histopathological, imaging, and treatment data.
- Analysis of predictive factors including tumor volume, SUV, MIB-1 index, resection margins, metastasis, and adjuvant therapy.
Main Results:
- NF1-associated MPNSTs were larger, occurred in younger patients, and showed higher SUV and MIB-1 indices.
- Complete resection was achieved in 66% of cases, with functional improvement in most patients.
- NF1-associated MPNSTs had more severe pain scores.
- Overall survival was longer with complete resection (R0) and without metastasis.
- Adjuvant radio-/chemotherapy was associated with shorter overall survival in cases with incomplete resection or metastasis.
Conclusions:
- NF1-associated MPNSTs present with larger tumor volumes, higher proliferation rates, and worse overall survival.
- Surgery offers significant benefits for symptom improvement, especially pain relief, even in advanced stages.
- Surgical intervention should be considered for symptom management in advanced MPNSTs, including those related to NF1.
