Stone, Jonathan J.

Background: Interfascicular resection is a surgical technique used to safely treat benign peripheral nerve sheath tumors through careful dissection of functional neural elements off the tumor surface 1,2 . Description: Proper operative technique is essential to improving symptoms, preserving neurologic function, and minimizing the chance for recurrence. Accurate tumor localization, ideal patient positioning, and placement of a longitudinal incision permit adequate exposure. Prior to tumor resection, normal nerve should be identified proximally and distally and controlled with vessel loops. This allows functional fascicles streaming around the tumor in the pseudocapsule to be visualized during resection. A fascicle-free window is identified on the tumor surface through visual inspection and intraoperative neurophysiology monitoring if desired. The pseudocapsule layers are divided with a sharp instrument until a smooth and shiny true capsule layer is found. This plane should have minimal resistance and is developed circumferentially until the tumor can be enucleated in toto. At the poles of the tumor, a single nonfunctional nerve fascicle that courses into the tumor is typically found. If there is >1 fascicle running into the tumor, further pseudocapsule layers should be undermined to sweep fascicles off the true capsule surface. The entering-exiting fascicle can be tested for function and is cut sharply. The specimen should be sent to pathology for permanent sectioning. The sides of the pseudocapsule are spread in opposite directions to evaluate for residual tumor, and any remaining tumor is removed if it can be done safely. Meticulous hemostasis is achieved, and the surgical site is closed in anatomical layers. Alternatives: Pain is the most common presenting symptom, and neuroleptic medications should be used in escalating dosage prior to surgical intervention. Nonoperative medical therapy does not typically result in symptom freedom, and patients often opt for resection. For tumors that are suspected of being malignant, an image-guided percutaneous or open biopsy and staging (positron emission tomography and/or computed tomography scans of the chest, abdomen, and pelvis) are recommended prior to treatment planning. For symptomatic benign extremity lesions, surgical resection is the treatment of choice, and adjuvant therapies like radiation and/or chemotherapy are not recommended. For malignant lesions, more aggressive surgery (wide resection or amputation) and preoperative, intraoperative, or postoperative radiation with or without chemotherapy are often utilized. Rationale: The treatment approach depends on a variety of presenting features such as onset, progression, symptom severity, tumor size, location, imaging features, presence of a syndrome, and patient age. There is little benefit from the resection of an incidentally found, small, nongrowing lesion. The most common reasons for removal of extremity lesions are a painful mass and/or radiating “nerve” pain. There is a high likelihood of relieving the symptoms and minimizing the risk of recurrence, and a relatively low risk of causing neurologic injury. The procedure provides a definitive diagnosis. For patients with severe pain, progressive weakness, rapid tumor growth, or concerning imaging characteristics, biopsy should be considered to determine malignant potential.

Abstract BACKGROUND Intraneural ganglion cysts are joint-connected, with the primary pathology residing in the associated joint. For peroneal intraneural ganglion cysts, the surgical strategy can include resection of the synovial surface of the superior tibiofibular joint (STFJ). However, the rate of instability postoperatively is unclear. OBJECTIVE To evaluate the rate of STFJ instability, following surgery for peroneal intraneural ganglion cysts. The second goal of the study was to assess the relationship between volume of resection of the STFJ and risk of extraneural recurrence. METHODS We performed a retrospective analysis of a cohort of patients with peroneal intraneural ganglion cysts. We analyzed clinical factors, including recurrence, and assessed the rate of postoperative STFJ instability. We created 3-dimensional models of the STFJ pre- and postoperatively to compare the volume of resection in recurrent cases and nonrecurrent cases using a case-control design. RESULTS The total cohort consisted of 65 subjects. No patient had evidence of radiological or clinical instability of the STFJ postoperatively. Extraneural radiological recurrence occurred in 6 (9%) patients. No intraneural recurrences were observed. The average volume of resection for patients with recurrence was 1349 mm 3 (SD = 1027 mm 3 ) vs 3018 mm 3 (SD = 1433 mm 3 ) in controls that did not have a recurrence ( P = .018). CONCLUSION This study supports performing an aggressive STFJ resection to minimize the risk of extraneural recurrence. Superior tibiofibular joint resection is not associated with postoperative joint instability. A smaller volume resection is correlated with recurrence risk.

Abstract BACKGROUND Intraneural dissection is a useful technique for achieving gross total resection and preserving functional fascicles for peripheral nerve tumors. Finding the correct tissue plane is the critical step for safe successful enucleation. The authors hypothesized that the yellow color of benign nerve tumors can be used to identify surgical planes. OBJECTIVE To describe a technique to find the correct intraneural dissection plane based on a quantified yellow appearance during resection of benign peripheral nerve sheath tumors. METHODS Intraoperative photographs were reviewed to determine the percentage of yellow that tumors appeared at different phases of surgery. A technique was developed to quantitatively measure the amount of yellow using Photoshop. Previously published journal articles containing color images of peripheral nerve tumor resections were also analyzed for yellow tumor color. RESULTS There were 24 patients with suitable images to permit measurement of color for 3 steps of the procedure. The average percentages of yellow for tumor exposure, tumor resection, and removed specimen were 36.5%, 59.1%, and 80.4%, respectively ( P < .001). Three publications were found that contained high-quality images of at least 2 phases of the surgery with average yellow content of 47.4% and 84% ( P < .01). CONCLUSION The simple observation that a benign nerve sheath tumor is yellow in color can be used to guide surgical resection and achieve excellent outcomes. Intraneural dissection through the pseudocapsule should be pursued layer by layer until a yellow true capsule is found. By sparing functional nervous tissue within the pseudocapsule, this technique may lead to improved neurological outcomes.

OBJECTIVEDesmoid-type fibromatosis (DTF) presents a therapeutic dilemma. While lacking metastatic potential, it is a locally aggressive tumor with a strong propensity for occurrence near nerve(s) and recurrence following resection. In this study, the authors introduce the association of an occult neuromuscular choristoma (NMC) identified in patients with DTF.METHODSAfter experiencing a case of DTF found to have an occult NMC, the authors performed a retrospective database review of all other cases of biopsy-proven DTF involving the extremities or limb girdles in patients with available MRI data. Two musculoskeletal radiologists with expertise in peripheral nerve imaging reviewed the MRI studies of the eligible cases for evidence of previously unrecognized NMC.RESULTSThe initial case of a patient with an occult sciatic NMC is described. The database review yielded 40 patients with DTF—18 (45%) in the upper limb and 22 (55%) in the lower limb. Two cases (5%) had MRI findings of NMC associated with the DTF, one in the proximal sciatic nerve and the other in the proximal tibial and sural nerves.CONCLUSIONSThe coexistence of NMC may be under-recognized in a subset of patients with extremity DTF. This finding poses implications for DTF treatment and the likelihood of recurrence after resection or biopsy. Further study may reveal crucial links between the pathogenesis of NMC and DTF and offer novel therapeutic strategies.

Abstract Schwannomas are benign peripheral nerve sheath tumors that are typically round, smooth, and straightforward to resect. Occasionally they are more complicated to resect because of their size, location, degree of bony erosion, and vascular or neural compression. These complex lesions frequently require multidisciplinary surgical teams in order to decrease surgical morbidity. Historically, surgical planning consisted of imaging review and verbal communication among team members. 3-dimensional (3D) printing offers a new method for preoperative planning and is becoming more popular in Neurosurgery. This video demonstrates the use of 3D printing for complex paraspinal tumors in the cervical, thoracic, and sacral regions. The Institutional Review Board Office for Human Research Protection does not require approval for single participant case studies, or a case series with multiple participants. The subjects involved in this study were not identifiable and the general surgical consent form included permission for intraoperative photos.

Abstract Neuromuscular choristoma (NMC) is a rare congenital nerve lesion that is classified by the presence of heterotopic muscle fibers intercalated within nerve fascicles. 1 The presence of NMC typically causes progressive neuropathy and bony/soft tissue undergrowth, often leading to a shortened atrophic limb. 2,3 The typical appearance of NMC on magnetic resonance imaging is an enlarged fusiform nerve that exhibits minimal gadolinium enhancement. 4 While there is increasing documentation of pathognomonic clinical and radiographic features for various diffuse fusiform peripheral nerve lesions, nerve biopsy is still occasionally required for some patients given the rarity of these diseases and progressive neuropathic features. The intraoperative appearance of NMC and its unique electrical stimulation properties are quite interesting. Intrafascicular replacement of nerve by skeletal muscle gives the nerve a red beefy color and causes the fascicles to contract with electrical stimulation. 5 In this video, we demonstrate the intraoperative appearance and contractile properties of NMC in a 60-yr-old woman with progressive sciatic neuropathy. Histologic and immunohistochemical studies identified a gradient of intralesional mature skeletal muscle within the NMC, with substantially fewer desmin-positive skeletal muscle fibers in the distal aspect. A gradient of contractility with intraoperative stimulation seemed to correlate with this pathology. These findings suggest that the lesional extent of NMC may not be clinically or grossly evident, reflecting a varying distribution of skeletal muscle within the affected nerve. The Mayo Clinic Institutional Review Board Office for Human Research Protection does not require approval for single participant case studies. The subject involved in this study was not identifiable and the general surgical consent form included permission for intraoperative photos and video.

Inflammatory etiologies are becoming increasingly recognized as explanations of some neuropathies, especially those occurring in the perioperative period. Although “brachial neuritis” is known to affect extraplexal nerves, accessory nerve palsy following median sternotomy has been attributed to stretch on the nerve. To better elucidate stretch as a potential cause, a cadaveric study was performed. Two patients who developed accessory nerve palsy following median sternotomy are presented to illustrate features consistent with the diagnosis of a perioperative inflammatory neuropathy. Five adult unembalmed cadavers underwent exposure of the bilateral accessory nerves in the posterior cervical triangle. A median sternotomy was performed and self‐retaining retractors positioned. With the head in neutral, left rotation and right rotation, retractors were opened as during surgery while observing and recording any accessory nerve movements. The self‐retaining sternal retractors were fully opened to a mean inter‐blade distance of 13 cm. Regardless of head position, from the initial retractor click to maximal opening there was no gross movement of the accessory nerve on the left or right sides. Opening self‐retaining sternal retractors does not appear to stretch the accessory nerve in the posterior cervical triangle. Based on our clinical experience and cadaveric results, we believe that inflammatory conditions, (i.e., idiopathic brachial plexitis) can involve the accessory nerve, and might be triggered by surgical procedures. Clin. Anat. 31:417–421, 2018. © 2017 Wiley Periodicals, Inc.