Ultrasound-guided nerve block prior to biopsy of suspected neurogenic tumors: safety and feasibility in a pilot study.

OBJECTIVE: The aim of this study is to investigate the safety and feasibility of ultrasound-guided nerve block prior to biopsy of potentially neurogenic tumors. MATERIALS AND METHODS: A retrospective review of the medical record from June 2017 to June 2022 identified ultrasound-guided biopsies of potentially neurogenic tumors that were performed with a pre-procedural nerve block. Patient demographics, biopsy site, number of passes, needle gauge, use of sedation, pathology results, and procedural complications were recorded and summarized. RESULTS: The structured search found 16 patients that underwent biopsies of 18 potentially neurogenic tumors with the use of a pre-procedural nerve block at a variety of upper and lower extremity locations. Average patient age was 52 (range 18-78) and 9 patients (56%) were female. Of the 16 patients, 10 were performed without intravenous sedation. Three patients were unable to tolerate biopsy until a nerve block was used. All biopsies yielded a diagnostic sample with 13 of the tumors neurogenic in origin. One patient reported mild postprocedural pain which resolved with conservative treatment; no other complications were reported. CONCLUSION: Nerve block prior to ultrasound-guided biopsy of potentially neurogenic tumors is a safe and feasible technique. Further study is needed to determine the extent to which nerve block can decrease intra-procedural pain and reduce or eliminate the need for sedation during biopsy.

Tracheal schwannoma mimicking asthma.

Schwannoma in primary neurogenic tumors of the trachea is an extremely rare disease. A 21-year-old male patient with stridor was followed up with a diagnosis of asthma for two years. While no lesion was observed in the chest X-ray, tracheal schwannoma was diagnosed in the case who underwent tracheotomy due to the lesion obstructing the trachea almost completely on chest CT. The clinical findings of the patient improved ultimately in the postoperative period. We aimed to discuss the approach and follow-up of the disease with our case, although it is rare that schwannoma might be in the differential diagnosis of masses with upper airway obstruction.

Pancreatic/peripancreatic neurogenic tumor; little known masses not to be missed.

BACKGROUND: Retroperitoneal neurogenic tumors are extremely rare pathological entities; therefore, few clinical features and natural courses, especially originating from the pancreatic/peripancreatic regions, have been reported. This study aimed to investigate the clinicopathological features of pancreatic and peripancreatic neurogenic tumors and assess the diagnostic value of computed tomography (CT) and endoscopic ultrasound-guided fine needle biopsy (EUS-FNB). METHODS: Between 2006 and 2018, patients who were diagnosed with neurogenic tumors were included. In total, 90 histologically confirmed cases of neurogenic tumors located in the pancreatic/peripancreatic regions were selected for analysis. RESULTS: The mean age was 49.2 ± 13.1 years. There were no differences in sex distribution of the tumors. Schwannomas (44.4%) and paragangliomas (41.1%) were the most common neurogenic tumors. The sensitivity of CT was 62.2% in 90 cases. EUS-FNB was performed in 30 cases and the sensitivity of it was 83.3%. The diagnosis of neurogenic tumors with EUS-FNB or CT was not significantly associated with tumor location and size. Surgical resection was performed in 78 cases. Of the 12 patients who did not undergo surgery, 10 cases were followed-up without any increase in tumor size. CONCLUSIONS: Through the present study, we verified radiological, pathological, and clinical aspects of the pancreatic/peripancreatic neurogenic tumors which little known before, therefore, this study can serve as the basis for research to present an optimal diagnosis and treatment of neurogenic tumors. In addition, EUS-FNB is useful in the diagnosis of pancreatic/peripancreatic neurogenic tumors with relatively high sensitivity and can help establish therapeutic plans before the surgery.

Multiple non-polypoid mucosal Schwann cell hamartomas presenting as edematous and submucosal tumor-like lesions: a case report.

BACKGROUND: Mucosal Schwann cell hamartomas are rare neurogenic tumors which present most commonly in the distal colon. They are usually discovered as small, single polyps in asymptomatic patients. CASE PRESENTATION: An asymptomatic 64-year-old man was referred to us after a 12 mm subepithelial lesion was discovered incidentally on screening colonoscopy. Follow-up colonoscopy conducted 2 months later revealed that the tumor had disappeared, leaving multiple edematous, submucosal tumor-like elevations presenting as skip lesions throughout the sigmoid colon. Lesions had elongated pits on magnifying endoscopy and were limited to the first layer on endoscopic ultrasound. Biopsies revealed unclearly delineated foci of spindle-shaped cells limited to the lamina propria. On immunohistochemistry, all lesions were positive for S-100 protein and negative for CD34, CD56, and neurofilament protein. The patient was diagnosed with multiple mucosal Schwann cell hamartomas of the sigmoid colon. He remains asymptomatic after 18 months of follow-up, but the lesions have also remained unchanged. CONCLUSION: We report a case of multiple non-polypoid mucosal Schwann cell hamartomas. Endoscopic findings may assist in the differential diagnosis, particularly when presenting as non-polypoid, submucosal tumor-like lesions.

[Surgical treatment of retroperitoneal neurogenic tumors in children]. / Khirurgicheskoe lechenie detei s neirogennymi opukholyami zabryushinnogo prostranstva.

OBJECTIVE: To evaluate feasibility and advantages of laparoscopic access in children with retroperitoneal neurogenic tumors. MATERIAL AND METHODS: A comparative analysis of postoperative results was performed in 2 groups of patients with retroperitoneal neurogenic tumors without IDRF risk factors. Different surgical approaches were applied. The main group (laparoscopic access) consisted of 18 patients. The control group included 22 patients after laparotomy. We compared the following variables: surgery time, blood loss, postoperative need for analgesics, intraoperative and postoperative complications, timing of postoperative enteral feeding, postoperative hospital-stay and regional recurrence rate. RESULTS: Duration of laparoscopic and open surgery was similar (80.5 vs. 86.5 min, p>0.05). Intraoperative blood loss was significantly lower in the laparoscopy group (10 vs. 17 ml/kg, p<0.05). Two patients required conversion of laparoscopic approach. In laparotomy group, massive intraoperative bleeding occurred in 1 patient. Early postoperative period was more favorable in the main group compared to the control group. There were no local and metastatic recurrences in delayed postoperative period after laparoscopic surgery. One (4.5%) patient had metastatic recurrence after laparotomy. There were no regional recurrences in this group. CONCLUSION: Laparoscopic resection of neurogenic retroperitoneal tumors in children is feasible if great vessels are not involved in neoplastic process. Open surgery is preferable in patients with invasion of great vessels.

Evaluation of clinical application of multi-slice computerized tomography in primary retroperitoneal tumors.

BACKGROUND: To accurately identify primary retroperitoneal tumors by multi-slice spiral CT (MSCT) for better treatment. MATERIALS AND METHODS: The common types of 380 cases of primary retroperitoneal tumor, lesion sites, and MSCT features were compared with pathological results. Fisher's or chi-square test approaches have been applied in this study. RESULTS: Multi-slice computerized tomography multi-directional reconstruction has a high accuracy for primary retroperitoneal tumors (95.7%). Seventy-three liposarcoma cases were located in peri-renal space, accounting for 76.8% (13/95) of the tumors in this region. Meanwhile, 65 cases of neurogenic tumors were located in the paravertebral column, accounting for 90.3% (65/72) of the tumors in this region. MSCT examination could effectively distinguish benign from malignant of primary retroperitoneal tumor (sensitivity = 87.2%, specificity = 82.7%, accuracy rate = 84.5%). Malignant tumors showed more irregularity shape than benign tumor (χ2  = 20.468, P < .001). 82.7% (191/231) of the malignant tumors showed adhesion or even invasion of surrounding tissues and organs (χ2  = 23.262, P < .001). Fat density of the lipoma is uniform, and lesion is not enhanced. Liposarcoma can be seen in varying proportions of fat and soft tissue density. CONCLUSION: The accuracy of MSCT scan for retroperitoneal tumors is high. Meanwhile, the coincidence rate of qualitative diagnosis before operation and/or before biopsy is also high.

Primary mediastinal melanotic schwannian tumors: A clinicopathological and immunohistochemical study of 5 cases.

Mediastinal neurogenic tumors are unusual and more so is the presence of melanotic neurogenic tumors. We present five cases of mediastinal melanotic neurogenic tumors. The patients are five men between the ages of 34 and 43 years (average: 38.5 years). All patients presented with non-specific symptoms that included back pain and cough. Diagnostic imaging revealed the presence of a posterior mediastinal mass without connection to the spinal canal, and surgical resection was accomplished in all of the patients. Histologically, the five tumors showed a spindle epithelioid cellular proliferation, nuclear atypia, mitotic activity, and melanin deposition. Histochemical stain for Fontana Masson clearly demonstrated the presence of melanin pigment in all the cases, while S-100 protein was only focally positive in tumor cells. Other immunohistochemical stains including SOX-10, MITF, HMB-45, and Melan A were negative. Clinical follow-up showed that two patients died 22 and 30 months after initial diagnosis; one remains alive, 6 months after initial diagnosis; two patients were lost to follow up. Melanotic neurogenic tumors represent a diagnostic challenge for pigmented thoracic tumors and careful analysis of the morphology and immunohistochemistry is required to lead to proper diagnosis.

[Management of neurogenic tumors of the sacrum and sacral area]. / Taktika lecheniia neirogennykh opukholei kresttsa i kresttsovoi oblasti.

Tumors of the sacrum rarely occur in routine practice. Due to the absence of pathognomonic symptoms, the diagnosis often becomes apparent at a late disease stage. In this case, the treatment approach depends on the degree of tumor malignancy, growth pattern and location, and relationship between the tumor and the nervous structures, major vessels, and pelvic bones. OBJECTIVE: Analysis of the overall and relapse-free survival of patients with neurogenic tumors of the sacrum. MATERIAL AND METHODS: In this study, we analyzed the treatment outcomes in 27 patients with neurogenic sacral tumors who underwent surgery at the Burdenko Neurosurgical Institute. RESULTS: The median survival time of patients with neurogenic tumors was 72 months; the shortest survival time was observed in a group of neurogenic sarcomas, with the mean survival time being 30 months. Malignant tumors of the peripheral nerves recurred in 100% of cases. CONCLUSION: Surgical treatment of neurogenic tumors of the sacrum reduces pain, but does not lead to regression of the neurological symptoms caused by compression of the sacral plexus roots. Patients with malignant tumors of the sacrum should undergo partial or complete sacrectomy. In this case, the choice of treatment strategy should be based on assessment of the functional status, tumor histology, and somatic features of the patient.

Robotic paravertebral schwannoma resection at extreme locations of the thoracic cavity.

Solitary paravertebral schwannomas in the thoracic spine and lacking an intraspinal component are uncommon. These benign nerve sheath tumors are typically treated using complete resection with an excellent outcome. Resection of these tumors is achieved by an anterior approach via open thoracotomy or minimally invasive thoracoscopy, by a posterior approach via laminectomy, or by a combination of both approaches. These tumors most commonly occur in the midthoracic region, for which surgical removal is usually straightforward. The authors of this report describe 2 cases of paravertebral schwannoma at extreme locations of the posterior mediastinum, one at the superior sulcus and the other at the inferior sulcus of the thoracic cavity, for which the usual surgical approaches for safe resection can be challenging. The tumors were completely resected with robot-assisted thoracoscopic surgery. This report suggests that single-stage anterior surgery for this type of tumor in extreme locations is safe and effective with this novel minimally invasive technique.

Laparoscopic resection of paraaortic/paracaval neurogenic tumors: surgical outcomes and technical tips.

BACKGROUND: Due to variations in location and size, laparoscopic surgery for paraaortic or paracaval neurogenic tumors is challenging. We evaluated the surgical outcomes, as well as surgical tips and tricks. METHODS: Between 2000 and 2015, 25 procedures were performed in 24 patients. One patient underwent second surgery due to the recurrence of paraganglioma. Data were collected on the tumor diameter, tumor location, perioperative outcomes, pathology, and last-known disease status. Regarding the operative procedures, we reviewed the operative charts or videos to identify surgical tips and tricks. RESULTS: The median tumor diameter was 5.0 cm (range 1.5-10). The tumor location was suprahilar in 10, hilar in 6, and infrahilar in 9 cases. Regarding the approach, a transperitoneal approach was selected in 24 cases and retroperitoneal approach in 1. The median operative time and blood loss were 208 min (range 73-513) and 10 mL (range 0-1020), respectively. No patient required blood transfusion or conversion to open surgery. Pathological examination revealed paraganglioma in 12, ganglioneuroma in 7, and schwannoma in 6 cases. At the last follow-up, 23 patients were free of disease, while one patient developed metastatic multiple recurrence of paraganglioma 54 months after the second laparoscopic surgery. A review of the surgical records revealed several tips and tricks, including taping the vena cava/renal vein (n = 2) being helpful for detaching a retrocaval tumor from these great vessels, or rotating the kidney to provide a favorable operative view of tumors behind the renal hilum (n = 2). In recent cases, 3D-CT was helpful for preoperative planning. CONCLUSIONS: Laparoscopic resection of paraaortic or paracaval neurogenic tumors is feasible in experienced hands. Surgeons should be familiar with detaching maneuvers around great vessels and the mobilization of adjacent organs. Careful preoperative planning is mandatory.

Spine extra-osseous chordoma mimicking neurogenic tumors: report of three cases and review of the literatures.

BACKGROUND: For a long time, chordoma has been known as an osseous tumor mainly found at the clivus and sacrococcygeal region. However, spine extra-osseous chordoma (SEC) with a better prognosis than the classic type has been neglected. According to our literature review, only several case reports have been published in English literatures. Here in this article, three cases of SEC, plus a literature review, are presented. CASE PRESENTATION: Three cases of SEC were presented from our center. Surprisingly, neurologic tumors were considered as the first diagnosis. Thereafter, en bloc resection was performed in all the three cases. Especially, the dumbbell-shaped one in the cervical spine was removed by en bloc through the combined anterior and posterior approach for the first time. Follow-up within 12-58 months after surgeries proved no recurrence or metastasis. CONCLUSIONS: Spine extra-osseous chordoma, commonly located in the cervical and epidural region, is extremely rarely met. SEC is characterized with less aggressiveness, the lower rate of recurrence and metastasis, and better prognosis than those of the osseous origin. Though complete excision can be achieved generally, differential diagnosis of spine neurogenic tumors and the following en bloc resection should be made as carefully as possible.

MR imaging of cavernous sinus lesions: Pictorial review.

The main purpose of this pictorial review is to highlight the important MR imaging findings of various conditions involving the cavernous sinus in addition to brief description of normal anatomy. The pathological conditions that can involve the cavernous sinus can be categorized into infective, inflammatory, granulomatous, vascular and neoplastic causes. Imaging, especially with MRI, plays an important role not only in detection but also in definition of disease extent and in characterization of the pathology. Currently, high-resolution MR images clearly show various components of cavernous sinus which help in making a proper diagnosis and thus appropriate further management.

The supraclavicular approach in the management of cervicothoracic-junction benign neurogenic tumors: A real-world analysis.

Objectives: The study objectives were to evaluate the safety, feasibility, and risk of neurologic complications with the supraclavicular approach in the operative management of cervicothoracic-junction benign neurogenic tumors. Methods: Between January 2012 and April 2023, 115 patients who underwent surgical resection for cervicothoracic-junction benign neurogenic tumors were retrospectively enrolled. Patients were divided into 3 groups based on the surgical approach: supraclavicular alone (Supraclav-Alone), n = 16; Transthoracic-Alone (video-assisted thoracoscopic surgery/Open), n = 87; and supraclavicular combined with transthoracic (Supraclav + video-assisted thoracoscopic surgery/open), n = 12. Clinicopathologic variables and postoperative morbidity including neurologic complications were summarized among the groups. Logistic regression analysis was performed to identify predictors for long-term (>6 months) brachial plexus injuries. Results: The cohort comprised 28 patients (24.3%) who underwent surgical resection using a supraclavicular approach. The Supraclav-Alone group portended the most cephalad location of tumor, the smallest pathologic tumor size, the shortest operative time, the least blood loss, and the least postoperative pain. The incidence of surgical complications, phrenic nerve neuropraxia, recurrent laryngeal nerve neuropraxia, or Horner's syndrome was similar among the groups postoperatively. However, use of the supraclavicular-alone approach (adjusted odds ratio, 0.165; 95% CI, 0.017-0.775) was a predictor for long-term brachial plexus injury complications. Among patients who experienced brachial plexus injury complications, the proportion of patients achieving complete resolution was higher among those undergoing a supraclavicular approach group (Supraclav-Alone: 80.0% vs Supraclav + video-assisted thoracoscopic surgery/Open: 60.0% vs video-assisted thoracoscopic surgery/Open: 25.8%). Conclusions: The supraclavicular approach may be a safe and feasible strategy in the management of cervicothoracic-junction benign neurogenic tumors that does not increase surgical complications and minimizes the severity of brachial plexus injury.

Schwannoma diagnosed by endobronchial ultrasound-guided intranodal forceps biopsy using standard-sized biopsy forceps: A case report.

Schwannomas are classified as neurogenic tumors and are the most frequent nerve sheath tumors in the paravertebral mediastinum. Recently, the addition of endobronchial ultrasound-guided intranodal forceps biopsy (EBUS-IFB) using standard-sized biopsy forceps (SBFs) to endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for metastatic lymph nodes in lung cancer patients reportedly improved the quality and quantity of the obtained specimens without significant complications. However, reports on the usefulness of this technique for benign diseases remain scarce. Here we report a case of schwannoma in the middle mediastinum, which was diagnosed by EBUS-IFB using SBFs, despite inadequate specimens obtained via EBUS-TBNA. An 80-year-old woman presented with dyspnea and a 5-cm sized middle mediastinal tumor. EBUS-TBNA and EBUS-IFB using SBFs were performed for histological diagnosis. No complications were associated with the bronchoscopy procedure, and schwannoma was solely diagnosed using the EBUS-IFB specimens. EBUS-IFB using SBFs is potentially useful for diagnosing benign diseases, including schwannomas, which are often difficult to diagnose with EBUS-TBNA.

Outcomes of Head and Neck Neurogenic Tumors.

BACKGROUND: Lateral neck mass management frequently challenges surgeons. Nerve tissue neoplasms are an uncommon cause of such nodules. Neurogenic tumors form a tiny percentage of the head and neck neoplastic lesions. Considering the number of nerves in this area, it is surprising that such neoplasms are not more frequently seen. METHODS: A retrospective study was conducted on all patients who presented to the National Cancer Institute of Cairo, Egypt, with head and neck neurogenic neoplasms. RESULTS: During the last 10 years at the National Cancer Institute of Egypt (2006-2015), 40 cases of neurogenic tumors of the head and neck were treated at the head and neck unit. Patients' ages ranged from two to 78 years with a mean age of 34.7 years. Childhood neurogenic tumors accounted for nine cases (22.5%) only in this study. Male patients diagnosed with these tumors comprised 16 cases, while female patients comprised 24 cases, with a female-to-male ratio of 1.5:1. Patient presentation depends on the biological behavior of the tumor; for instance, some of them present by slowly growing painless well-circumscribed mobile swelling, and others present by rapidly growing swelling with neurological deficit. Clinical picture and imaging studies such as CT and MRI raise suspicion and may help delineate such tumors, but a definitive diagnosis is obtained by tissue biopsy. Surgery is the mainstay of treatment in most head and neck neurogenic tumors, whereas adjuvant therapy is of limited benefit in some types of neurogenic tumors. The five-year survival rate was 60% for the malignant group, while death was reported in six out of 15 cases (40%).  Conclusion: Most neurogenic head and neck tumors are benign. Accurate preoperative assessment and a high degree of suspicion are the initial steps in the management. Proper treatment involves complete surgical excision; however, debulking procedures have an important role. Malignant neurogenic tumors are aggressive and are treated with combined radical surgical resection and radiation. Chemotherapy is tried for locally advanced unresectable or metastatic disease.

An Unusual and Discrepant Presentation of a Skull Base Paraganglioma.

Paragangliomas are rare tumors of neuroendocrine origin. Within the head and neck, these tumors are slow-growing and locally destructive, with a small malignant potential. Vagal paragangliomas (VPs) originate from paraganglia around the vagus nerve, typically at the level of the skull base. Cranial nerve deficits are common at presentation, with the vagus nerve and hypoglossal nerves being most affected. Similarly, hypoglossal paragangliomas (HPs) originate from around the hypoglossal nerve but are extremely rare and less documented. We describe the case of a patient presenting with an isolated hypoglossal nerve palsy in the setting of a tumor that radiologically represents a VP. A descriptive literature review was conducted to highlight presentation, management, and outcomes related to this pathology. A 65-year-old male presented to the clinic with tongue fasciculations and several years of dysarthria. Physical examination showed intermittent right tongue fasciculations in addition to ipsilateral hemi-atrophy. A computed tomography scan with contrast revealed an enhancing skull base mass inferior to the right carotid space. Subsequently, magnetic resonance imaging with contrast further delineated its anatomic involvement and site of origin, allowing for the diagnosis of a VP. After further discussion with the patient about his clinical findings, the decision was made to proceed with observation and serial imaging. Skull base paragangliomas are a rare pathologic entity that may pose a challenging multidisciplinary approach to optimize management strategies. Treatment may vary on a case-by-case basis and is dependent on patient and tumor characteristics.

Anesthetic management of mini sternotomy and excision of mediastinal neurogenic tumor: Brain-Heart crosstalk.

Brachial plexus tumors are rare and pose challenges for neurosurgeons due to their anatomical complexity. Retrosternal extension of a tumor makes it more difficult for the surgeons as well as for the anesthesiologists to secure a definitive airway. A cardiopulmonary bypass would be lifesaving in the event of acute cardiorespiratory decompensation. Multidisciplinary collaboration and cooperation between the neurosurgeon, oncosurgeon, cardiothoracic surgeon, and anesthesiologist are imperative to ensure good patient outcomes. Meticulous preoperative evaluation and operative planning are essentially the key factors in anesthetic management. Here we report a successful management of a 49-year-old male patient presented with a large painless mass arising from his right supraclavicular region and compressing the roots of the brachial plexus, trachea, and esophagus and extending up to the apex of the lungs, posted for mini sternotomy and excision of the mass.

Pediatric laryngeal schwannoma: a case report.

Schwannomas are benign tumors originating from Schwann cells in the peripheral nervous system. They mostly occur in the head and neck region but are rare in the larynx, and present with various symptoms. Surgical removal is the recommended treatment. This study presents a 12-year-old female with sudden onset hemoptysis, snoring, difficulty breathing, dysphagia, and voice changes. On examination, she was conscious and had muffled voice while speaking, without signs of respiratory distress. Endoscopic laryngoscopy revealed a large laryngeal mass obstructing the vocal cords. Endoscopic excision and biopsy confirmed the presence of the laryngeal schwannoma. Postoperative recovery was uneventful, with normal vocal cord function and no recurrence at follow-up.

Radial nerve myofibroma: a rare benign tumor with perineural infiltration. Illustrative case.

BACKGROUND: Myofibromas are benign mesenchymal tumors, classically presenting in infants and young children in the head and neck region. Perineural involvement, especially in peripheral nerves within the upper extremity, is extremely rare in myofibromas. OBSERVATIONS: The authors present the case of a 16-year-old male with a 4-month history of an enlarging forearm mass and rapidly progressive dense motor weakness in wrist, finger, and thumb extension. Preoperative imaging and fine needle biopsy confirmed the diagnosis of a benign isolated myofibroma. Given the dense paralysis, operative management was indicated, and intraoperative exploration showed extensive involvement of tumor within the radial nerve. The infiltrated nerve segment was excised along with the tumor, and the resulting 5-cm nerve gap was reconstructed using autologous cabled grafts. LESSONS: Perineural pseudoinvasion can be an extremely rare and atypical feature of nonmalignancies, resulting in dense motor weakness. Extensive nerve involvement may still necessitate nerve resection and reconstruction, despite the benign etiology of the lesion.

Resection of a schwannoma originating from vagus nerve in the right superior mediastinum.

BACKGROUND: Schwannoma is the most common mediastinal neurogenic tumor, while schwannoma originating from mediastinal vagus nerve is rare. CASE PRESENTATION: This article reported one case of schwannoma originating from vagus nerve in the right superior mediastinum. The mediastinal schwannoma was completely resected through a right-sided video-assisted thoracoscopic thoracotomy. Histologic examination clarified the diagnosis as schwannoma. CONCLUSION: Chest CT scan and MRI can be used to determine the location of mediastinal schwannoma and its relationship with adjacent tissue. Histologic examination showing distinctive feature of Antoni A areas and Antoni B areas can help clarify the diagnosis. Complete surgical resection is the first-line treatment option for mediastinal schwannomas.