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Is There Any Prognostic Relationship Between Malignant Peripheral Nerve Sheath Tumors And NF Type 1?
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Original Article
VOLUME: 53 ISSUE: 1
P: 46-56
2020

Is There Any Prognostic Relationship Between Malignant Peripheral Nerve Sheath Tumors And NF Type 1?

Acta Haematol Oncol Turc 2020;53(1):46-56
DOI: 10.5505/aot.2020.44154
Coskun Ulucakoy 1
Aliekber Yapar 1
Recep Öztürk 1
Güray Toğral 1
Bedii Şafak Güngör 1
1. Ankara Oncology Training and Research Hospital
2.
No information available.
No information available
Received Date: 2019-06-27T13:43:05
Accepted Date: 2020-04-28T11:10:21
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Abstract

INTRODUCTION

In this study, we aimed to research the clinical features of malignant peripheral nerve sheath tumors (MPNST) which are rare soft tissue sarcomas and their prognostic relationship with Neurofibromatosis type 1 (NF1).

METHODS

The clinical features, treatment modalities, clinical outcomes and treatment failures of 42 (26 males, 16 females) patients operated for MPNST between January 2002 and December 2015 were evaluated retrospectively. 16 patients (38%) (10 males, 6 females, mean age 40 ± 14) were associated with NF1. 26 patients (62%) (16 males, 10 females, mean age 38 ± 15) were treated and followed up with sporadic MPNST diagnosis. The mean follow-up period was 30 months (± 12) in the NF1-associated group and 42 months (± 15) in the sporadic MPNST group. The most common site was extremity (85%) and mean tumor size was 11.2 cm (range between 2 and 21 cms). All patients underwent wide resection after diagnosis.

RESULTS

There was no significant difference between the two groups in terms of tumor size, tumor location, lymph node involvement and distant metastasis. In all patients, macroscopic surgery margin was negative, while 5 patients (31%) in the NF1-associated group and 9 patients (34%) in the sporadic group the margin was reported as positive (R1). Recurrence was seen in 20 of 42 patients (47%). Recurrence was seen in 13 patients (81%) in the NF1-associated group and 7 patients (26%) in the sporadic group. Mortality rate increased by 11.4 times in patients with positive microscopic surgical margin.

DISCUSSION AND CONCLUSION

The prognosis of malignant peripheral nerve sheath tumors developing on NF1 background is worse than sporadic type. In addition, tumor size, tumor depth and positive surgical margin are indicators of poor prognosis. The most important factor affecting survival is the presence of NF1 and the positive surgical margin.