Neuromuscular Choristoma of the Brachial Plexus Presenting as a Supraclavicular Mass in a Pediatric Patient: A Case Report.

CASE: This case demonstrates a 2-year-old boy with a rare benign supraclavicular mass diagnosed as neuromuscular choristoma through open biopsy. Postoperatively, he underwent semiannual surveillance with ultrasound without development of neurological complaints, limb deformity, or recurrence at 2-year follow-up. CONCLUSION: Neuromuscular choristoma involving the brachial plexus is a rare tumor that should be in the differential diagnosis of pediatric peripheral nerve-based tumors. The intimate association with neural elements limits complete resection. Therefore, open biopsy with partial resection is recommended. While postoperative fibromatosis may occur, open biopsy remains the gold standard for definitive diagnosis. Ultrasound can be used to monitor recurrence.

Rapidly Growing Leiomyoma Mimicking Schwannoma of the Saphenous Nerve in the Lower Extremity: An Unusual Case Report.

Leiomyomas and schwannomas are both types of rare benign soft tissue tumours. Leiomyomas are more commonly found in the lower limbs than in the upper extremities, while schwannomas are rare peripheral nerve sheath tumours that can occur in different anatomical regions. However, they rarely occur in the saphenous nerve. This case study presents a 41-year-old female patient with a solitary mass lesion located deep in the soft tissue of the anteromedial lower extremity. The physical examination revealed a palpable, elastic-hard, mobile and non-tender mass. Magnetic resonance imaging (MRI) showed an oval-shaped subcutaneous mass on contrast-enhanced T1-weighted sections. The initial MRI images suggested a schwannoma, but the tumour was later confirmed to be a leiomyoma after total enucleation. An immunohistochemical study was performed for differential diagnosis. Solitary mass lesions in the lower extremities can be mistaken for various types of tumours and misdiagnosed and require histopathological examination and good radiological imaging for differential diagnosis. Complete surgical excision is usually a safe and effective treatment for leiomyomas.

[Schwannoma of Intercostal Nerve Resulting in Significant Pain Relief Following Excision:Report of a Case].

An 80-year-old male underwent chest computed tomography (CT) due to complaints of right-sided chest pain. A chest wall tumor was identified in the right eighth intercostal space, corresponding to the location of his pain. The patient was subsequently referred to our department for further evaluation and treatment. Utilizing single-port thoracoscopic surgery, the tumor was successfully excised. Intraoperatively, the tumor was found beneath the parietal pleura, was contiguous with to the intercostal nerve. Histopathological analysis confirmed the diagnosis of schwannoma originating from the intercostal nerve. The right-sided chest pain was reduced after operation significantly. No recurrence or relapse of symptoms was observed during follow-up. Although schwannoma of the chest wall in asymptomatic in many cases, in this case, localized pain corresponding to the tumor site was evident. This emphasizes the importance of considering schwannoma in the differential diagnosis of chest pain.

The neuroma map: A systematic review of the anatomic distribution, etiologies, and surgical treatment of painful traumatic neuromas.

BACKGROUND: This study analyzed all reported cases of painful traumatic neuromas to better understand their anatomic distribution, etiologies, and surgical treatment. METHODS: PubMed, Embase, Cochrane, and Web of Science were searched in October 2023 for articles describing painful traumatic neuromas. RESULTS: In total, 414 articles reporting 5,562 neuromas were included and categorized into head/neck, trunk, upper extremity, lower extremity, and autonomic nerves. Distribution was as follows: Head/neck: 83 articles reported on 393 neuromas (93.2% iatrogenic) most frequently involving the lingual (44.4%), cervical plexus (15.0%), great auricular (8.6%), inferior/superior alveolar (8.3%), and occipital (7.2%) nerves. Trunk: 47 articles reported on 552 neuromas (92.9% iatrogenic) most commonly involving the intercostal (40.0%), ilioinguinal (18.2%) and genitofemoral (16.2%) nerves. Upper extremity: 160 articles reported on 2082 neuromas (42.2% after amputation) most frequently involving the digital (47.0%), superficial radial (18.3%), and median (7.0%) nerves. Lower extremity: 128 articles reported on 2,531 neuromas (53.0% after amputation) most commonly involving the sural (17.9%), superficial peroneal (17.3%), and saphenous (16.0%) nerves. Autonomic nerves: 17 articles reported on 53 neuromas (100% iatrogenic) most frequently involving the biliary tract (64.2%) and vagus nerve (18.9%). Compared with the extremities, neuromas in the head/neck and trunk had significantly longer symptom duration before surgical treatment and the nerve end was significantly less frequently reconstructed after neuroma excision. CONCLUSION: Painful neuromas are predominantly reported in the extremities yet may occur throughout the body primarily after iatrogenic injury. Knowledge of their anatomic distribution from head to toe will encourage awareness to avoid injury and expedite diagnosis to prevent treatment delay.

Giant Common Peroneal Nerve Schwannoma Mimicking Synovial Sarcoma: An Unusual Case Report.

Schwannoma, also known as neurilemmoma or Schwann cell tumor, is one of the most common neoplasms of the nerve sheath which usually appears at the head, neck, or upper extremity. Schwannoma occurrence in the lower extremity originating from the common peroneal nerve is rarely reported according to literary findings. We report a case of a 32-year-old man who presented with a 6-month history of a growing lump in the left knee. MRT revealed a well-defined 9.6 cm × 7.8 cm × 6.5 cm multilobular mass of heterogeneous consistency with areas of necroses with a likely diagnosis of synovial sarcoma. After surgery, a final histopathological assessment of the tumor demonstrated Antoni A and B patterns with nuclear palisading, hallmarks of a schwannoma. Postoperatively the patient suffered a neurological complication-impaired dorsiflexion of the left foot. The patient started immediate physiotherapy in the Department of Rehabilitation. Three weeks after the operation, gradual improvement in neurological function was observed. To date, complete tumor excision combined with microscopic analysis and immunohistochemical staining remains the gold standard in diagnosing and treating a peripheral nerve schwannoma. Moreover, the use of additional nerve monitoring tools during surgery could help to prevent complications.

Long-term Follow-up with MRI Scans After Enucleation of Peripheral Nerve Schwannomas: Results from a Single-center Case Series.

BACKGROUND: Enucleation is a surgical technique to resect peripheral nerve schwannomas. The procedure has a low risk for postoperative deficit, but a small chance for recurrence, because tumor cells may remain inside the pseudocapsule that is left after resection. Magnetic resonance imaging (MRI) scans are frequently performed after surgery to investigate potential residual tumor, but currently there is little information in the literature on the value of follow-up with MRI. MATERIAL AND METHODS: All patients who underwent enucleation of a peripheral nerve schwannoma between October 2013 and June 2022 were included. Postoperative MRI scans (gadolinium-enhanced) made at different time points after the surgery were re-examined for residual enhancement. Patients with residual enhancement were contacted to inform whether symptoms had recurred. RESULTS: A total of 75 schwannoma enucleations in 74 patients were included. The first postoperative MRI scan, performed 3 months after the surgery, showed no residual enhancement in 50 patients. In the remaining 24 patients, another MRI scan was made 1 year after the surgery, which still showed a possible remnant in 11 patients. On the third MRI scan, performed 2 years after enucleation, there were 7 suspected cases (9%). None of these patients had clinical symptoms at a mean postoperative follow-up of 5 years. CONCLUSIONS: Our data show that the value of postoperative MRI scans after enucleation of peripheral nerve schwannomas is limited, because residual enhancement in the beginning can be non-specific and the small percentage of patients, that persistently had a potential remnant, were all asymptomatic.

Tumors of the Brachial Plexus: A Critical Analysis Review.

¼ Tumors of the brachial plexus are uncommon and can present as a mass, with or without neurological symptoms. At times, asymptomatic tumors are also picked up incidentally when imaging is performed for other reasons.¼ Magnetic resonance imaging is the main imaging modality used to evaluate tumors of the brachial plexus. Other imaging modalities can be used as required.¼ Benign tumors that are asymptomatic should be observed. Excision can be considered for those that are found to be growing over time.¼ Biopsies of tumors of the brachial plexus are associated with the risk of nerve injury. Despite this, they should be performed for tumors that are suspected to be malignant before starting definitive treatment.¼ For malignant tumors, treatment decisions should be discussed at multidisciplinary tumor boards, and include both the oncology and peripheral nerve surgical team, musculoskeletal radiology, neuroradiology, and general radiology.

Forelimb lameness caused by malignant peripheral nerve sheath tumors of the median nerve in a dog: a case report.

An 8-year-old Portuguese Water Dog presented with a 5-month history of left forelimb lameness. There was palmar pain. Ultrasonography revealed enlargement of the left median nerve. Subsequent MRI also showed enlargement of the median nerve in the distal palmar to the mid-forearm region. Rapid intraoperative diagnosis suggested malignant peripheral nerve sheath tumors (MPNST) and a neurotomy was performed. The lameness had almost disappeared in 1 month after surgery. Recurrence occurred 26 months postoperatively and the forelimb was amputated. At 950 days after the neurotomy, radiography revealed lung metastasis, and the dog died 988 days after the neurotomy. Neurotomy for MPNST should be performed with caution until more information is available regarding methods for early detection, margin determination, and indication determination for neurotomy.

Management of an Early-Onset, Painful Tibial Nerve Neuroma Using an Autologous Nerve Graft.

This case report describes the treatment of a postoperative painful neuroma of the tibial nerve using an autologous nerve graft in a dog. The patient presented with sudden non-weight-bearing lameness 10 days after iatrogenic tibial nerve injury during preparation of a reverse saphenous conduit flap. The dog showed severe pain at the surgical site without nerve deficits. A magnetic resonance imaging examination revealed an enlarged tibial nerve at the injury site, consistent with a neuroma. Analgesics were administered over 11 days, but the patient remained in severe pain and non-weight-bearing. Therefore, surgical resection was recommended. The fusiform neuroma was resected microsurgically, and a saphenous nerve graft was transplanted using an epineural nerve repair technique. Histopathological examination was consistent with a neuroma. The dog showed immediate pain relief and weight-bearing the day after surgery with normal motor function. The dog made a full recovery by the last follow-up 6 mo after surgery. If patients develop pain and lameness following surgery or nerve injury, neuroma formation must be considered, even shortly after surgery. Microsurgical resection and autologous nerve transplantation using an epineural nerve repair technique is a viable method to treat painful neuromas and minimize the risk for recurrence in dogs.

Utility of Multimodal Intraoperative Neuromonitoring for Excision of Filum Terminale Ependymoma in Close Proximity to Conus.

Myxopapillary ependymomas (MPEs) are well-circumscribed tumors arising mainly from the caudal neuraxis, i.e., conus medullaris (CM) and filum terminale (FT), commonly seen in adults with median age at presentation of 39 years.1 Owing to its partially aggressive clinical behavior involving cerebrospinal fluid dissemination and local recurrence, MPE is classified as grade 2 in the fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System.2 Gross total resection without capsular violation is key, with subtotal resection being directly associated with local recurrence.3The FT has an intradural and extradural component. The intradural FT extends from the inferior tip of the CM to the coccyx.4 The intradural FT-CM junction is not demarcated, but rather a zone of transition, with neural tissue being incrementally replaced by fibrous tissue of filum, gradually converging to a pure non-neural FT.5 In intramedullary FT MPE in close proximity to the lower end of conus, achieving gross total resection presents a great challenge. Neuromonitoring is crucial to ensure preservation of vital CM functions. We present the case of a 33-year-old man with focal nocturnal back pain of 6 months' duration followed by bilateral lower limb deep boring pain. He had no neurological deficits. Preoperative magnetic resonance imaging revealed a T2 hyperintense, heterogeneous contrast-enhancing intradural extramedullary mass at L1. Video 1 highlights step-by-step en bloc excision of the FT MPE with technical nuances, including intraoperative neurophysiological monitoring. Triggered electromyography (EMG) was used to positively map the eloquent CM and identify the intradural FT-conus interface at the superior pole of the tumor, which was then carefully dissected under continuous bulbocavernosus reflex monitoring. Similarly, we confirmed non-neural intradural FT at the lower pole by negative mapping and resected the lesion en bloc with an adequate stump for clear margins. Free-run EMG monitored all the rootlets that adhered to or were in close proximity to the lesion, ensuring their integrity and an uneventful postoperative recovery. Figure 1 depicts the anatomical orientation of the lesion with surrounding neural structures. Histopathology confirmed MPE. En bloc resection with preservation of neurological function remains the mainstay of treatment for FT ependymoma. Understanding the transitional intradural FT-CM interface is essential, often precluding a clear filum stump superiorly while resecting MPE. Intraoperative neurophysiological monitoring is an indispensable adjunct to ensure safe en bloc resection. It is also theoretically possible to use tibial and pudendal sensory evoked potentials (SEPs) in this surgical procedure. However, the clinical utility of SEPs is limited in FT surgery compared with triggered EMG or transcranial motor evoked potentials because conventional SEPs from posterior tibial nerve of the lower extremity do not cover all the root levels at risk, and the change in SEPs cannot be immediately recognized (as SEPs are averaged responses, and there is always a time lag). We did not use pudendal SEPs in this study because SEPs may give information only on the sensory sacral pathway.6 Dermatomal SEPs may be helpful, but again, they provide only sensory information. Instead, we used triggered EMG for mapping the nerve roots and transcranial motor evoked potentials to monitor the motor tracts. Further, we used the bulbocavernosus reflex, an alternative and more precise technique to monitor both motor and sensory nervous pathways at the sacral root level. Moreover, SEPs are more difficult to monitor in very young children and are less relevant in guiding the surgical strategy. Thus, we used both mapping (triggered EMG) and monitoring (transcranial motor evoked potentials and bulbocavernosus reflex) techniques, which can preserve sensory and motor sacral roots in this surgical procedure.

Superficial branch of the radial nerve regularly contains fibers from the lateral antebrachial cutaneous nerve: A role in neuroma treatment.

BACKGROUND: Many surgical strategies aim to treat the symptomatic neuroma of the superficial branch of the radial nerve (SBRN). It is still difficult to treat despite many attempts to reveal a reason for surgical treatment failure. The lateral antebrachial cutaneous nerve (LACN) is known to overlap and communicate with SBRN. Our study aims to determine the frequency of spreading of LACN fibers into SBRN branches through a microscopic dissection to predict where and how often LACN fibers may be involved in SBRN neuroma. METHODS: Eighty-seven cadaveric forearms were thoroughly dissected. The path of LACN fibers through the SBRN branching was ascertained using microscopic dissection. Distances between the interstyloid line and entry of LACN fibers into the SBRN and emerging and bifurcation points of the SBRN were measured. RESULTS: The LACN fibers joined the SBRN at a mean distance of 1.7 ± 2.5 cm proximal to the interstyloid line. The SBRN contained fibers from the LACN in 62% of cases. Most commonly, there were LACN fibers within the SBRN's third branch (59%), but they were also observed within the first branch, the second branch, and their common trunk (21%, 9.2%, and 22%, respectively). The lowest rate of the LACN fibers was found within the SBRN trunk (6.9%). CONCLUSION: The SBRN contains LACN fibers in almost 2/3 of the cases, therefore, the denervation of both nerves might be required to treat the neuroma. However, the method must be considered based on the particular clinical situation.

Mismatch between Augmented Reality Navigation Images and Actual Location of a Cauda Equina Tumor:A Case Report.

BACKGROUND: Augmented reality navigation is the one of the navigation technologies that allows computer-generated virtual images to be projected onto a real-world environment. Augmented reality navigation can be used in spinal tumor surgery. However, it is unknown if there are any pitfalls when using this technique. CASE PRESENTATION: The patient in this report underwent complete resection of a cauda equina tumor at the L2-L3 level using microscope-based augmented reality navigation. Although the registration error of navigation was <1 mm, we found a discrepancy between the augmented reality navigation images and the actual location of the tumor, which we have called "navigation mismatch". This mismatch, which was caused by the mobility of the spinal tumor in the dura mater, seems to be one of the pitfalls of augmented reality navigation for spinal tumors. CONCLUSIONS: Combined use of intraoperative ultrasound and augmented reality navigation seems advisable in such cases. J. Med. Invest. 71 : 174-176, February, 2024.

Peripheral nerve tumors.

The chapter is focused on the neoplastic peripheral nerve lesions, which primarily involve "cranial and paraspinal nerves," as outlined in the CNS volume (WHO_Classification_of_Tumours_Editorial_Board, 2021). These include classic peripheral nerve sheath tumors such as schwannoma, neurofibroma, intraneural perineurioma, and malignant peripheral nerve sheath tumors, with their variants as well as new and more precisely defined entities, including hybrid nerve sheath tumors and malignant melanotic nerve sheath tumor (previously melanotic schwannoma).

Primary intradural Extraosseous Ewing's sarcoma of the cauda equina: A case report and literature review.

Intradural Extraosseous Ewing sarcoma (IEES) is an infrequent occurrence. We report a case of a 66-year-old male who presented with a 2-month history of low back pain and bilateral S1 sciatica, with acute sphincter dysfunction. Imaging studies revealed an intradural extramedullary lesion in the cauda equina spanning from level L4 to S1. The patient underwent partial removal of the intradural lesion. Histopathological examination showed the presence of small round cells, which were consistent with Ewing Sarcoma. The patient was then treated with targeted radiation therapy and chemotherapy. The rarity of IEES in this specific location underscores the significance of evaluating and managing patients with intradural spinal tumors with careful consideration of this diagnosis. To further investigate this condition, we conducted a thorough review of the literature on IEES involving the lumbar spine and cauda equina. Our analysis revealed that patients with this condition frequently exhibit rapidly progressive neurological symptoms likely attributed to hemorrhagic transformation. This characteristic may serve as a distinguishing factor from other lesion types, particularly benign ones. Our study provides a comprehensive summary that can offer direction for clinical management in comparable uncommon and novel cases.

Pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion: a case report.

Pediatric fibromyxoid soft tissue tumors may be associated with gene fusions such as YHWAZ::PLAG1, with only three reported cases in the literature. We present the fourth case, a 13-year-old male with a pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion. This is also the first case to be reported in an adolescent, in the brachial plexus, and in the Philippines. The patient presented with a 10-year history of a slowly growing left supraclavicular mass and a 1-year history of intermittent dysesthesia in the left upper extremity. Neurologic examination was unremarkable. Imaging revealed a large left supraclavicular lesion with intrathoracic extension. Surgical excision was performed, and histopathology revealed a fibromyxoid tumor with YWHAZ::PLAG1 gene fusion. Although previous examples of this gene fusion pointed toward lipoblastoma as their primary pathology, our tumor does not completely fulfill the current diagnostic criteria for a lipoblastoma and may represent an intermediate form of the disease. Our case is unique not only because it is the first reported adolescent patient harboring such a lesion but also because of the relatively lengthy natural history exhibited by the tumor prior to its resection. This provided us with valuable information about its behavior, which suggests a more indolent growth pattern. This case also highlights the clinical importance of molecular testing of tumors, where recognition of disease entities can assist clinicians in deciding and advocating for the proper management.

An algorithmic approach to the management of peripheral nerve tumours.

This article reviews the pathology and management of peripheral nerve tumours, including a framework for investigation and decision-making. Most tumours are benign, including schwannomas and neurofibromas, but malignant peripheral nerve sheath tumours can occur. The risk of malignant change is remote for schwannomas but higher for neurofibromas, particularly in neurofibromatosis type 1. Magnetic resonance imaging is useful for defining the relationship of a swelling with adjacent nerves but is not definitive for tissue diagnosis. Increasing size, pain and neurological deficit suggest malignant change and TruCut needle biopsy is indicated, although there is a risk of sampling error. Excision biopsy preserving nerve function may be carried out for benign tumours to relieve symptoms. Malignant tumours require a multidisciplinary approach. Complete surgical excision with clear margins is the only curative treatment and may be supplemented with radiotherapy and chemotherapy. However, prognosis remains poor, particularly for patients with neurofibromatosis.

[Interdisciplinary Treatment Of Tumorous And Tumour-Like Lesions Of Peripheral Nerves]. / Interdisziplinäre Behandlung von Raumforderungen in Assoziation zu peripheren Nerven: Tumore und tumorähnliche Läsionen.

Tumorous or tumour-like lesions of peripheral nerves are generally rare, heterogeneous and challenging to diagnose and treat. They may become apparent by a palpable swelling (lump) near nerves, sensory and/or motor deficits, pain to touch or neuropathic pain. In 91% of cases, tumours are benign. The differentiation of entities and their characteristics as well as a function-preserving resection strategy are highly relevant. Misdiagnosis and inadequate treatment can lead to severe deficits and pain syndromes. Benign tumours include schwannomas and neurofibromas, which can occur sporadically but can also be associated with neurogenetic tumour disposition syndromes if they occur more frequently. Rarer benign nerve tumours include perineuriomas, lipomas, aggressive fibrosis (desmoid tumours), paragangliomas and haemangiomas. Ganglion cysts are described as tumour-like lesions. The association of nerve tumours with neurogenetic syndromes and the correct classification of potentially malignant lesions such as MPNST (malignant peripheral nerve sheath tumour) or intermediate stages such as ANNUBPs (atypical neurofibromatous neoplasms with unknown biological potential) pose particular challenges. Interdisciplinarity is highly relevant for clinical treatment and a correct diagnosis. The aim of our work is to provide an overview of the relevant entities, diagnostic evaluation and contemporary treatment strategies based on the current data situation and taking into account the recently published interdisciplinary AWMF S2k guideline "Diagnosis and Treatment of Peripheral Nerve Tumours".