Abstract

Removal of benign peripheral nerve sheath tumors (bPNST) represents a surgical challenge. The morphological relation of bPNST and healthy nerve fascicles are of utmost importance for achieving both removal of the entire tumor and preservation of functional integrity of the peripheral nerve. Thus, we intraoperatively assessed the morphological patterns between bPNST and nerve fascicles using photo documentation obtained between January 2009 and September 2021. In 31 patients (20 women and 11 men) with a mean age of 48 ± 18 years a total of 34 bPNST were removed. Four constant morphological patterns between bPNST relatively to nerve fascicles were detected: (1) bPNST is located peripherally (n = 16), (2) it splits the nerve into two main fascicles (n = 5), (3) it totally splits up the nerve out of the nerve’s center (n = 8) und (4) it encloses the nerve and its fascicles (n = 5) without any detectable boundary layer. Histology revealed 28 schwannomas, five neurofibromas, and one perineurioma. The proposed classification reflects the increasing complexity of tumor removal with a higher type number. This might be beneficial for preoperative diagnostics, i.e., high-resolution ultrasound or MRI-tractography, as well as for planning the bPNST’s surgical resection and the possible need for nerve reconstruction.

Highlights

  • Benign peripheral nerve sheath tumors constitute 10–12% of all benign softtissue neoplasms [1,2]

  • Neurofibromas, schwannomas and perineuriomas represent the common, major categories of benign peripheral nerve sheath tumors (bPNST), which were historically defined according to the cell types involved [3]: while the last two entities consist of a uniform cell population, i.e., Schwann cells and perineurial cells, respectively, neurofibromas are composed of different cell types including fibroblasts, Schwann cells, perineurial cells and scattered axons [3,4]

  • Three cases had to be excluded because histological work-up did not confirm the tumor to be a bPNST, but rather showed an angioleiomyoma, a leiomyosarcoma, and a malignant neurofibroma, respectively

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Summary

Introduction

Benign peripheral nerve sheath tumors (bPNST) constitute 10–12% of all benign softtissue neoplasms [1,2]. Neurofibromas, schwannomas and perineuriomas represent the common, major categories of bPNST, which were historically defined according to the cell types involved [3]: while the last two entities consist of a uniform cell population, i.e., Schwann cells and perineurial cells, respectively, neurofibromas are composed of different cell types including fibroblasts, Schwann cells, perineurial cells and scattered axons [3,4]. Subtypes of the well-known schwannoma, neurofibroma, and perineurioma could be detected [3], and hybrid nerve sheath tumors were defined as an independent category in the World. BPNST occur either solitarily or due to neurofibromatosis as underlying disease, which can be genetically further divided into Neurofibromatosis Type 1, 2, and Schwannomatosis [7]

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