Case Presentations and Review of Plexiform Schwannoma in the Foot

Plexiform schwannoma is a soft tissue tumor that rarely presents in the lower extremity. We have reported on 2 cases of plexiform schwannomas that occurred in the foot and ankle. Owing to the relative obscurity of this condition in the lower extremity, we have also provided an overview of plexiform schwannomas and discussed the differences between this and other suspicious nodular soft tissue masses. The first patient was a 38-year-old female, who had a soft tissue mass located on the lateral aspect of her right foot that became symptomatic. The second patient was an 11-year-old male, who had a symptomatic soft tissue mass on the plantar aspect of his right foot. Both patients underwent surgical excision of the tumor.     

Unusually Large Pedal Schwannoma

We present a rare case of an unusually large schwannoma of the posterior tibial nerve that extended through the tarsal tunnel into the forefoot. Radiographic, computerized tomographic and magnetic resonance images supported the diagnosis, and the diagnosis was confirmed by pathological analysis. We found this schwannoma to be unique because of the combination of its location and extensive involvement of the foot.     

Posterior Tibial Nerve Lymphoma Presenting as Tarsal Tunnel Syndrome: A Case Report

We report a case of isolated posterior tibial B-cell lymphoma of the posterior tibial nerve presenting as tarsal tunnel syndrome. This diagnosis was considered because of the clinical presentation and electrophysiologic abnormalities. It was further confirmed by the magnetic resonance imaging findings of the ankle and tissue pathologic findings. Whole body positron emission tomography confirmed this to be a localized lymphoma involving the peripheral nerve. The patient underwent chemotherapy with complete tumor resolution. She had had no relapse after 8 months of follow-up. Isolated peripheral nerve lymphomas are very rare, and involvement of the posterior tibial nerve has not been previously reported. Furthermore, the present case report highlights the importance of the clinical examination in the diagnosis of tarsal tunnel syndrome before performing surgical decompression.     

Magnetic Resonance Imaging Findings in Patients with Tarsal Tunnel Syndrome

Tarsal tunnel syndrome (TTS) is a common entrapment syndrome whose diagnosis can be difficult. We compared preoperative magnetic resonance imaging (MRI) and operative findings in 23 consecutive TTS patients (28 sides) whose mean age was 74.5 years. The 1.5T MRI sequence was 3D T2* fat suppression. We compared the MRI findings with surgical records and intraoperative videos to evaluate them. MRI- and surgical findings revealed that a ganglion was involved on one side (3.6%), and the other 27 sides were diagnosed with idiopathic TTS. MRI visualized the nerve compression point on 23 sides (82.1%) but failed to reveal details required for surgical planning. During surgery of the other five sides (17.9%), three involved varices, and on one side each, there was connective tissue entrapment or nerve compression due to small vascular branch strangulation. MRI studies were useful for nerve compression due to a mass lesion or idiopathic factors. Although MRI revealed the compression site, it failed to identify the specific involvement of varices and small vessel branches and the presence of connective tissue entrapment.     

Giant Schwannoma of the First Metatarsal: A Rare Entity

Schwannomas of osseous origin are rare, and schwannomas of the short tubular bones are even rarer. These benign-looking tumors are difficult to diagnose using imaging alone. However, histopathologic evaluation of a biopsy specimen can establish the diagnosis by identifying Antoni type A and B zones. Curettage and bone grafting will probably be adequate for treatment because malignant changes are unlikely. Large lesions can require en bloc excision and reconstruction. We describe what appears to be only the second case of a schwannoma in the first metatarsal of the foot in a 48-year-old woman. The lesion was poorly contained, with obvious breaks in the cortical shell. The diagnosis was confirmed by pathologic analysis. The lesion was successfully treated with en bloc resection and reconstruction with a nonvascularized fibular graft.     

前跗管综合症的临床与解剖学研究

本文报告12例前跗管综合症,并对25只新鲜成人足的前跗管利用显微解剖方法进行了研究。腓深神经在前跗管内受压为本症发病的主要因素。本组病例发病原因为足背严重挫伤、束紧鞋带、前跗管内骨赘及腱鞘囊肿、空凹足等。除临床特征外,肌电图检查在鉴别诊断中颇有帮助。10例进行了手术,通过1.5年至4年随访观察效果良好。     

Tarsal tunnel syndrome caused by epineural ganglion of the posterior tibial nerve: report of 2 cases and review of the literature

Ganglia within the posterior tibial nerve is a rare condition. The authors report 2 cases of epineural ganglion of the posterior tibial nerve, causing tarsal tunnel syndrome. Both cases presented with numbness on the plantar surface of the foot. Magnetic resonance imaging showed the presence of the cyst within the tarsal tunnel. During surgery, these cysts were found within the epineurium of the posterior tibial nerve and were successfully removed without damage to nerve fibers. Both patients were free of symptoms after surgery. Ganglion cysts in the peripheral nerve are either intrafascicular or epineural. Intrafascicular ganglia present beneath the epineurium and involve the nerve fibers, whereas epineural ganglia are located in the epineurium and do not involve the nerve fibers. A review of the literature discusses these concepts. The authors suggest that epineural ganglion should be clinically distinctive from an intrafascicular ganglion because of the differences in surgical treatment, postoperative nerve function, and the recurrence rate.     

Cystic Adventitial Disease of the Tibial Vein Arising From the Subtalar Joint: A Case Report

Soft tissue ganglion cysts are a well-known cause of tibial nerve compression in the tarsal tunnel. We describe a patient who presented with tibial nerve symptoms and was found to have an adventitial cyst of the tibial vein arising from the subtalar joint, with the joint connection confirmed both on imaging and at surgery. Surgical decompression of the cyst with transection of the vascular pedicle arising from the subtalar joint improved her symptoms at 6 months, and postoperative magnetic resonance imaging showed resolution of the cyst. Cystic adventitial disease is a rare, poorly understood condition in which a cyst is identified in the adventitia of a vessel, usually an artery. Only 3 cases of adventitial cysts have been reported in the foot and ankle region, 2 in the lesser and 1 in the greater saphenous vein. None of the previous cases have been recognized to be joint connected. This case provides additional evidence for an articular origin for adventitial cysts and helps guide management strategies for these joint-connected cysts.     

Malignant Peripheral Nerve Sheath Tumor: An Unusual Cause of Intussusception

Malignant peripheral nerve sheath tumors (MPNST) are defined as any tumor arising from a peripheral nerve or showing nerve sheath differentiation. The majority of these tumors arise on the trunk, extremities, or head and neck region. The literature to date has fewer than 14 cases of MPNST arising in the gastrointestinal tract, and only two cases were ever reported in the small intestine, one of which was a recurrent disease. In this paper, we report the first US case of an MPNST arising in the small intestine and presenting as intussusception.     

Neurothekeoma of the median nerve: case report

We report the case of a large intraneural neurothekeoma of the median nerve at the wrist. Neurothekeomas are rare; they are small, superficial, and typically asymptomatic benign tumors of undetermined cellular origin. Complete excision is usually curative. This case is interesting owing to the tumor's large size and location within the median nerve, which made it highly symptomatic, mimicking carpal tunnel syndrome.     

Frequency of Diplopia after Intraoperative Nerve Disturbance in Trochlear Nerve Schwannoma: A Case Report and Systematic Review

Schwannomas of the trochlear nerve are relatively rare, and most patients present with preoperative diplopia because of trochlear nerve palsy. We describe the case of a 61-year-old male patient with a trochlear nerve schwannoma and no pre- and postoperative diplopia, despite his trochlear nerve being cut during the operation. We aimed to investigate the frequency of postoperative diplopia associated with intraoperative trochlear nerve disturbance by reviewing previous case reports, wherein postoperative diplopia did not occur after the trochlear nerve was cut intraoperatively. We recorded the frequency of diplopia because of intraoperative trochlear nerve disturbance, such as the trochlear nerve being cut, in cases without pre- and postoperative diplopia. We searched the PubMed, Medline, and Google Scholar databases for works published from 1976 to 2020 and followed the preferred reporting items for systematic reviews and meta-analyses guidelines. We reviewed 36 publications and found 92 cases of trochlear nerve schwannoma. Surgical resection was performed for 43 patients, of whom 40 were kept under observation and 9 were treated with radiation therapy. Of the 43 cases, 9 without preoperative diplopia underwent gross total resection. We analyzed ten cases (including ours) without preoperative diplopia to check for postoperative diplopia. In total, four cases, including ours, did not display postoperative diplopia despite the trochlear nerve being cut. This may be attributed to the preoperatively acquired motor and sensory fusion in the patient’s vision because of tumor progression. Our findings may benefit neurosurgeons who treat patients with schwannomas and help them predict patients’ outcomes.     

Surgical Management of Solitary Nerve Sheath Tumors of the Cervical Spine: A Retrospective Case Analysis Based on Tumor Location and Extension

Complete resection of spinal nerve sheath tumors (NSTs) does not always result in significant neurological deficit. The purpose of this retrospective case analysis was to discuss the optimal surgical strategy for spinal NST of the cervical spine. Twenty-four patients who underwent surgery for solitary cervical NST over the past decade were included in this retrospective study. Patients with neurofibromatosis or schwannomatosis were excluded. Seventeen of the 24 cases (70.8%) showed extradural dumbbell extension, most frequently at the C1 or C2 vertebral level. Neurological condition was assessed using the modified McCormick functional schema and sensory pain scale. Total removal of the tumor was achieved in 20 of 24 cases (83.3%). Staged surgery using combined anterior and posterior approaches was applied for 2 of 17 cases with extradural dumbbell extension. Tumor involvement with nerve root fibers critical for upper extremity function (C5–C8) was recognized in 6 of 24 cases (25.0%), with complete resection in all 6 cases. Final assessment of neurological function revealed satisfactory or acceptable recovery in all 6 patients. Spinal NSTs with extradural dumbbell extension are a common condition in the cervical spine. Complete removal of spinal NST of the cervical spine may carry a risk of permanent neurological deficit, but such sequelae appeared to be the exception in the present case analysis. A radical and safe surgical strategy, including staged surgery combining anterior and posterior approaches, should be tailored to the individual case.     

Median Nerve Compression in the Forearm: A Clinical Diagnosis

Background: Median nerve entrapment in the forearm (MNEF) without motor paralysis is a challenging diagnosis. This retrospective study evaluated the clinical presentation, diagnostic studies, and outcomes following surgical decompression of MNEF. Methods: The study reviewed 147 patient medical charts following MNEF surgical decompression. With exclusion of patients with combined nerve entrapments (radial and ulnar), polyneuropathy, neurotmetic nerve injury, or median nerve motor palsy, the study sample included 27 patients. Data collected include: clinical presentation and pain, strength, provocative testing, functional outcomes, and Disabilities of the Arm, Shoulder and Hand (DASH) scores. Results: The study included 27 patients (mean follow-up = 7 months), and 13 patients had previous carpal tunnel release (CTR). Clinical presentation included pain (n = 27) (forearm, n = 22; median nerve innervated digits, n = 21; and palm, n = 21) and positive clinical tests (forearm scratch collapse test, n = 27; pain with compression over the flexor digitorum superficialis arch/pronator, n = 24; Tinel sign, n = 11). Positive electrodiagnostic studies were found for MNEF (n = 2) and carpal tunnel syndrome (n = 11). Primary CTR was performed in 10 patients and revision CTR in 7 patients. Postoperatively, there were significant (P < .05) improvements in strength, pain, quality of life, and DASH scores. Conclusions: The MNEF without motor paralysis is a clinical diagnosis supported by pain drawings, pain quality, and provocative tests. Patients with persistent forearm pain and median nerve symptoms (especially after CTR) should be evaluated for MNEF. Surgical decompression provides satisfactory outcomes.     

Oculomotor Nerve Neurinoma: Report of two Cases

Summary  Being motor nerves neurinomas originating from ocular nerves are very rare, unless associated with neurofibromatosis. Authors describe two cases of oculomotor nerve i.e. third nerve, Schwann cell tumours. One of them presented as a cavernous sinus mass in a middle aged lady while the other was a middle aged man with a large cisterno-cavernous tumour. Surgical approach is discussed and the relevant literature reviewed.     

Clinical Relevance of Pathological Grades of Malignant Peripheral Nerve Sheath Tumor: A Multi-Institution TMTS Study of 56 Cases in Northern Japan

BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is a relatively rare soft tissue tumor, and its clinical relevance of pathological grades remains obscure. METHODS: Fifty-six cases of MPNST identified from the files of seven oncology centers of the Tohoku Musculoskeletal Tumor Society (TMTS) and National Cancer Center were analyzed for histologic grades, demographics, treatments, and prognostic factors. The average follow-up period was 41 months. RESULTS: Twenty-two men and 34 women with a mean age of 45 years were involved. Forty-four (78.6%) of 56 tumors were in the lower extremity or trunk. Fifty tumors (89%) were classified as high grade, and the remaining six as low grade. Twenty-one (39.6%) of 53 patients who underwent tumor excision developed local recurrences. An axial site and inadequate surgical margin were defined as risk factors for local recurrence. The overall survival rates of the 56 patients were 55.1% at 3 years and 43.3% at 5 years. Univariate analysis of the 56 patients revealed large-sized tumors, metastasis at presentation, and histologically high grade were significantly associated with poor prognosis. Multivariate analysis revealed a large tumor and metastasis at presentation to be independent prognostic factors. CONCLUSIONS: The current study involving 56 patients with MPNST showed the aggressive clinical behavior of the tumor. Large-sized tumors, metastasis at presentation, and high histological grade were related to poor prognosis on univariate analysis, but independency of histological grade was still obscure. In the treatment for a large and high-grade MPNST, an alternative strategy should be further considered.     

Melanotic Schwannoma of the Cervical Spine Progressing With Pulmonary Metastasis: Case Report

Melanotic schwannoma (MS) is an unusual variant of nerve sheath neoplasm. Only 10% of these tumors will undergo malignant degeneration, with exceedingly rare reported metastasis. We present a 32-year-old woman with a 6-month history of cervical pain and left arm progressive weakness. Neurological examination showed a left upper limb radicular pain, with pyramidal syndrome at C5 level. The magnetic resonance imaging (MRI) study highlighted an intradural extramedullary heterogeneous mass along the spinal cord at the C4–C5 level, slightly hyperintense with T₁ and hypointense with T2-weighted sequences, invading the left neural foramen. The patient underwent C3–C5 laminectomy with total resection of a black tumor. In the postoperative period, a patent deficit of shoulder abduction ensued related to the nervous section. Microscopically, compactly fascicles of spindle-shaped cells with pleomorphic and hypercromatic nuclei, dark brown intracellular pigments, as well as some mitotic figures were seen. Immunohistochemical stains for S-100, Human Melanoma Black-45 (HMB-45), and vimentin were positive, with Ki-67 labelling index (LI) of 15% compatible with MS. Six months after radiotherapy she presents local recurrence and lung metastatic dissemination of the MS. She underwent left pulmonary segmentectomy, followed by chemotherapy and radiosurgery. The patient developed a febrile neutropenia and worsening of general status, and died after 3 months due to respiratory complications. MS are rare tumors with potential for local recurrence and distal metastasis. Complete surgical resection remains as the treatment of choice, once the uncommon cases with malignant progression shows low response to chemo and radiotherapy.     

The Sensitivity and Specificity of Nerve Conduction Studies for Diagnosis of Carpal Tunnel Syndrome: A Systematic Review

Background: The utility of nerve conduction studies (NCS) for diagnosis of carpal tunnel syndrome (CTS) has continued to be a subject of debate. Proponents of NCS assume a high sensitivity and specificity; however, many are unaware of the actual literature on this topic and the cutoff values commonly used for diagnosis. The purpose of this systematic review of the literature is to report the sensitivity and specificity of NCS for diagnosis of CTS in various studies. Methods: A literature review of PubMed and EMBASE databases was performed for all articles on NCS for diagnosis of CTS. The outcome of interest was the sensitivity and/or specificity of the NCS distal motor latency (DML) or distal sensory latency (DSL) cutoff value used to diagnose CTS in each study. Results: A total of 3066 total articles were screened and 21 were included in the review after assessment by two independent reviewers. The mean cut-off value for DSL was 3.37 ms (range 2.8-4 ms) and the mean cutoff value for DML was 4.28 ms (range 3.8-4.6 ms). Weighted mean DSL sensitivity was 73.4% and weighted mean DSL specificity was 93.6%. Weighted mean DML sensitivity was 56.2% and weighted mean DML specificity was 95.8%. Conclusions: There is significant variation in the cutoff values used for both DSL and DML. The wide range of cut-off values makes it difficult to interpret the literature, and there is a lack of high-quality studies with control groups using a priori cut-off values for diagnosis.     

Tarsal Tunnel Decompression in Leg Lengthening and Deformity Correction of the Foot and Ankle

Decompression of the tarsal tunnel has been performed since the 1960s and has resulted in variable outcomes. The success rate of this procedure has ranged from 44% to 95%. We review the anatomy of the tarsal tunnel region and offer a detailed surgical approach for the decompression of its contents. We also discuss the role of this procedure in leg lengthening and deformity correction of the foot and ankle.