Cluster of Neurofibromatosis Cases in Remote Area of Northern Karnataka: A Case Series

Tumours of the nerve system and skin are hallmarks of neurofibromatosis, a neurocutaneous condition. One class of inherited illnesses known as phakomatoses includes neurofibromatosis type 1 (NF1) or von Recklinghausen disease. It may show up anywhere in the body, including the skin, eyes, bones, and organs. We report a visually recorded seven cases of NF1 from same area. Every single patient had some kind of skin symptom. We describe the presence of several isolated neurofibromas, café au lait macules, axillary and inguinal freckling, neurological or cognitive dysfunction, and Lisch nodules in 100% of the eyes as ocular symptoms. Also, a rare occurrence of plexiform neuroma seen. Rare and familial presentation and similar cases in the same region, these findings collectively underscore the complex nature of the patient’s medical presentation, necessitating a multidisciplinary approach to management and care.

Best evidence summary of nutrition management in patients of acoustic neuroma during the perioperative period / 中国实用护理杂志

Objective:To summarize and evaluate the best evidence of perioperative nutrition management for patients with acoustic neuroma, and to provide evidence-based basis for clinical nurses.Methods:BMJ best clinical practice, UpToDate, Cochrane Library, Guidelines International Network, Scottish Intercollegiate Guidelines Network, the official website of Ontario Registered Nurses Association, JBI Australian evidence-based health care center, PubMed, Embase, CINAHL, Yimaitong, Chinese biomedical literature database, CNKI, Wanfang database and other domestic and foreign databases related to clinical practice guidelines, expert consensus, evidence summary, systematic evaluation, sysmtem analysis, clinical randomized scientific control were seached. The retrieval time was limited from June 1st, 2013 to June 1st, 2023. Evaluated the quality of the included literatures, and conduct evidence extraction, grade evaluation and summary analysis.Results:A total of 17 literatures were included, including 5 guidelines, 6 expert consensus, 2 best practices and evidence summary, 2 system evaluation and 2 randomized scientific control. A total 27 of best evidence was formed, covering five aspects: nutrition management team construction, screening and evaluation of nutrition and dysphagia, timing and implementation of nutrition support, specific measures of nutrition management, continuous nutrition management after discharge.Conclusions:This study summarized the best evidence of perioperative nutritional management of acoustic neuroma, and provides evidence-based basis for clinical nurses. However, its recommendations are not specialized, some of them are controversial, and come from many countries. In clinical practice, we should make a comprehensive analysis in combination with the actual clinical situation and other relevant factors, and make some choices, so as to promote the improvement of clinical nursing quality.

Cochlear radiation dose and hearing loss in patients with vestibular schwannoma undergoing radiosurgery: systematic review

Abstract Objectives: To determine the cut-off point of the cochlear radiation dose as a risk factor for hearing loss in patients with vestibular schwannoma treated with radiosurgery. Methods: A systematic review of the literature was performed without language or publication year restrictions in the MEDLINE/PubMed, EMBASE, Web of Science, LILACS/VHL and Cochrane Library databases. Studies that met the following criteria were included: 1) population: adults of both sexes who underwent radiosurgery for vestibular schwannoma treatment; 2) exposure: cochlear radiation; 3) outcome: hearing loss; 4) type of study: cohort. Two independent reviewers conducted the entire review process. The registration number in PROSPERO was CRD42020206128. Results: From the 333 articles identified in the searches, seven were included after applying the eligibility criteria. There was no standardization as to how to measure exposure or outcome in the included studies, and most studies did not present sufficient data to enable meta-analysis. Conclusion: It was not possible to determine a cut-off point for high cochlear dose that could be considered a risk factor for hearing loss.

Reconstrucción de neuroma de nervio radial superficial con injerto autólogo de nervio sural: presentación de dos casos / Reconstruction of painful neuroma of superficial branch of radial nerve after first dorsal compartment release: presentation of two cases

Resumen: Introducción: las lesiones iatrogénicas de nervio son complicaciones devastadoras de cualquier procedimiento quirúrgico. Ocasionan pérdida motora, dolor y formación de neuromas. En el abordaje para la liberación del primer compartimiento extensor de la muñeca, la rama superficial del nervio radial debe identificarse y protegerse previo a la liberación tendinosa. La lesión de este nervio sensitivo puede ocasionar dolor postoperatorio clínicamente significativo. La reconstrucción nerviosa con nervio autólogo ha demostrado en diversos escenarios buenos resultados para mejorar el dolor y recuperar la conducción nerviosa. Presentación de los casos: se presentan dos casos de mujeres adultas (F/47 y F/51) con antecedente de liberación de primer compartimiento dorsal de muñeca en otro centro hospitalario. Desarrollaron posteriormente dolor incapacitante y alodinia en sitio quirúrgico, así como limitación funcional. Fueron evaluadas y diagnosticadas como neuroma de rama superficial del nervio radial. No hubo mejoría con terapia física, por lo que se realizó reconstrucción nerviosa con injerto autólogo de nervio sural. Ambas pacientes tuvieron alivio del dolor de EVA 9-10 hasta EVA 1-2. A los cuatro meses de seguimiento, las dos mujeres recuperaron parcialmente la sensibilidad distal al sitio del neuroma, sin recurrencia del dolor presentado. Conclusiones: los neuromas son complicaciones devastadoras que ocurren con lesiones inadvertidas de nervios motores y sensitivos. La reconstrucción con nervio autólogo es una excelente opción para reconstrucción de pequeños tramos de nervio periférico.

Abstract: Introduction: nerve lesions are potentially catastrophic injuries. They can cause motor loss, severe pain and neuroma formation. The superficial branch of the radial nerve is at risk during first dorsal compartment release, its injury can cause neuroma formation. Autologous nerve reconstruction is the gold standard for treatment of small nerve gaps. Cases presentation: we present two cases of adult women (F/47 y F/51) with a prior history of first dorsal compartment release in another institution. Both patients developed debilitating neuropathic pain, as well as allodynia in the surgical site. They were diagnosed with superficial radial nerve neuroma. Oral medication and physical therapy was attempted without success. Surgical exploration and autologous nerve reconstruction was performed. Both patients had excellent relief of pain from visual analogue scale (VAS 9-10 to VAS 1-2). Postoperatively, both patients recovered partial sensitivity to pain in the zones distal to the repair. Conclusions: neuromas are feared complications that occur with unrecognized nerve lesions during surgery, they are difficult to treat and require multidisciplinary management. These two cases demonstrate that autologous nerve reconstruction is an excellent option for recovering function in small gaps of nerve tissue.

Neoplasia endocrina múltiple tipo 2B: un enfoque de diagnóstico genotipo-fenotipo y abordaje terapéutico / Multiple endocrine neoplasia type 2B: A genotype-phenotype diagnostic and therapeutic approach

Resumen La neoplasia endocrina múltiple tipo 2B (MEN 2B) es un síndrome genético poco común, con una prevalencia estimada de 0,9 a 1,6 por millón de individuos y una incidencia de 1,4 a 2,6 por millón de nacidos vivos por año. Es causada por una mutación en el protooncogén RET y se caracteriza por la presencia de carcinoma medular de tiroides (CMT) en el 100 % de los casos, feocromocitoma hasta en el 50 % y características extraendocrinas como un fenotipo marfanoide, alteraciones musculoesqueléticas y dentales, neuromas mucosos, alacrimia y ganglioneuromatosis intestinal difusa con síntomas de dismotilidad esofágica y gastrointestinal. Este síndrome se asocia con una calidad de vida deteriorada y una supervivencia deficiente, debidas principalmente a la agresividad del CMT, enfermedad que usualmente es de aparición temprana o metastásica. El diagnóstico generalmente se realiza de forma tardía, lo cual retrasa el enfoque integral, que incluye tipificación genética, tratamiento quirúrgico temprano del CMT y terapias específicas como inhibidores de tirosina quinasa (ITK) en caso de CMT avanzado. La cirugía es el tratamiento principal y la única posibilidad de cura, aunque el advenimiento de las terapias dirigidas parece estar mejorando la supervivencia libre de progresión en los casos avanzados.

Abstract Multiple endocrine neoplasia type 2B (MEN 2B) is a rare genetic syndrome with an estimated prevalence of 0.9 to 1.6 per million individuals and an annual incidence of 1.4 to 2.6 per million live births. It is caused by a mutation in the RET proto-oncogene and is characterized by the presence of medullary thyroid carcinoma (MTC) in 100% of cases, pheochromocytoma in up to 50%, and extraendocrine features such as a marfanoid phenotype, musculoskeletal and dental abnormalities, mucosal neuromas, alacrimia, and diffuse intestinal ganglioneuromatosis with symptoms of esophageal and gastrointestinal dysmotility. This syndrome is associated with a diminished quality of life and poor survival, primarily due to the aggressiveness of MTC, which often presents at an early or metastatic stage. Diagnosis is often delayed, resulting in a delayed comprehensive approach that includes genetic profiling, early surgical management of MTC, and specific therapies such as tyrosine kinase inhibitors (TKIs) for advanced MTC. Surgery remains the mainstay and the only potential cure, although the advent of targeted therapies appears to improve progression-free survival in advanced cases

Perfusion-weighted imaging in vestibular schwannoma: the influence that cystic status and tumor size have on perfusion profiles / Perfusão por ressonância magnética em schwannoma vestibular: influência do tamanho e estádio cístico no perfil da perfusão

Abstract Objective: The perfusion profile of vestibular schwannomas (VSs) and the factors that influence it have yet to be determined. Materials and Methods: Twenty patients with sporadic VS were analyzed by calculating parameters related to the extravascular extracellular space (EES)—the volume transfer constant between a vessel and the EES (Ktrans); the EES volume per unit of tissue volume (Ve); and the rate transfer constant between EES and blood plasma (Kep)—as well as the relative cerebral blood volume (rCBV), and by correlating those parameters with the size of the tumor and its structure (solid, cystic, or heterogeneous). Results: Although Ktrans, Ve, and Kep were measurable in all tumors, rCBV was measurable only in large tumors. We detected a positive correlation between Ktrans and rCBV (r = 0.62, p = 0.031), a negative correlation between Ve and Kep (r = -0.51, p = 0.021), and a positive correlation between Ktrans and Ve only in solid VSs (r = 0.64, p = 0.048). Comparing the means for small and large VSs, we found that the former showed lower Ktrans (0.13 vs. 0.029, p < 0.001), higher Kep (0.68 vs. 0.46, p = 0.037), and lower Ve (0.45 vs. 0.83, p < 0.001). The mean Ktrans was lower in the cystic portions of cystic VSs than in their solid portions (0.14 vs. 0.32, p < 0.001), as was the mean Ve (0.37 vs. 0.78, p < 0.001). There were positive correlations between the solid and cystic portions for Ktrans (r = 0.71, p = 0.048) and Kep (r = 0.74, p = 0.037). Conclusion: In VS, tumor size appears to be consistently associated with perfusion values. In cystic VS, the cystic portions seem to have lower Ktrans and Ve than do the solid portions.

Resumo Objetivo: O perfil de perfusão do schwannoma vestibular (SV) não tem sido estudado, nem os fatores que o influenciam. Materiais e Métodos: Vinte pacientes com SV esporádico foram analisados usando Ktrans, Ve, Kep e rCBV e correlacionados com tamanho e estádio cístico. Resultados: Ktrans, Ve e Kep foram medidos em todos os casos. rCBV só foi possível em tumores grandes. Ktrans e rCBV estavam correlacionados positivamente (r = 0,62, p = 0, 0 31 ) . Ve e Kep estavam negativamente correlacionados (r = -0,51, p = 0,021). Ktrans estava correlacionado positivamente com Ve em SVs sólidos (r = 0,64, p = 0,048). Em SVs pequenos, Ktrans foi menor (0,13 vs 0,029, p < 0,001), Kep foi maior (0,68 vs 0,46, p = 0,037) e Ve foi menor (0,45 vs 0,83, p < 0,001) que nos SVs grandes. Ktrans e Ve foram menores dentro dos cistos que nas porções solidas dos SVs císticos (0,14 vs 0,32, p < 0,001; 0,37 vs 0,78, p < 0.001, respectivamente). Foi encontrada correlação positiva em Ktrans (r = 0,71, p = 0,048) e Kep (r = 0,74, p = 0,037) entre as áreas sólidas e císticas. Conclusão: Nos SVs, o tamanho está consistentemente associado com os valores da perfusão. Nos SVs císticos, as porções císticas parecem ter valores menores de Ktrans e Ve do que nas porções sólidas.

Ultrasound in cervical traumatic neuromas after neck dissection in thyroid carcinoma patients: descriptive analysis and diagnostic accuracy

ABSTRACT Objective: Cervical traumatic neuromas (CTNs) may appear after lateral neck dissection for metastatic thyroid carcinoma. If they are misdiagnosed as metastatic lymph nodes (LNs) in follow-up neck ultrasound (US), unnecessary and uncomfortable fine-needle aspiration biopsy are indicated. The present study aimed to describe US features of CTNs and to assess the US performance in distinguishing CTNs from abnormal LNs. Subjects and methods: Retrospective evaluation of neck US images of 206 consecutive patients who had lateral neck dissection as a part of thyroid cancer treatment to assess CTN's US features. Diagnostic accuracy study to evaluate US performance in distinguishing CTNs from abnormal LNs was performed. Results: Eight-six lateral neck nodules were selected for analysis: 38 CTNs and 48 abnormal LNs. CTNs with diagnostic cytology were predominantly hypoechogenic (100% vs. 45%; P = 0.008) and had shorter diameters than inconclusive cytology CTNs: short axis (0.39 cm vs. 0.50 cm; P = 0.03) and long axis (1.64 cm vs. 2.35 cm; P = 0.021). The US features with the best accuracy to distinguish CTNs from abnormal LNs were continuity with a nervous structure, hypoechogenic internal lines, short/long axis ratio ≤ 0.42, absent Doppler vascularization, fusiform morphology, and short axis ≤ 0.48 cm. Conclusion: US is a very useful method for assessing CTNs, with good performance in distinguishing CTNs from abnormal LNs.

Brazilian Society of Otology task force - Vestibular Schwannoma - evaluation and treatment

Abstract Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Unusual Anterior Neck Swelling: Cervical Spinal Cord Schwannoma / Incomum inchaço anterior do pescoço: Schwannoma da medula espinhal cervical

Schwannomas are typically solitary, well-encapsulated, benign tumors running along or attached to a nerve. An intradural-extramedullary cervical spinal schwannoma, which first manifests as a swelling in the anterior neck, has not been reported to the best of our knowledge. We present the case of a 69-year-old patient complaining of a gradually worsening painful left cervical swelling for over 8 years. First, a posterior spinal midline approach was performed for the resection of the tumor and the tumor portion in the vertebral canal was totally removed. The second operation, the anterolateral approach, was planned to be executed in a second surgical session. The patient was discharged from the hospital without neurological deficits. Histopathological diagnosis was a schwannoma. The first aim of surgery is to treat neurological deficits in patients with cervical intraspinal schwannomas with/without extension into the extravertebral paravertebral neck regions. The surgical strategy combines the posterior midline and the anterolateral cervical approaches in the same session or at different times.

Schwannomas são tipicamente tumores solitários, bem encapsulados e benignos que correm ao longo ou aderem a um nervo. Até onde sabemos, um schwannoma espinhal cervical intradural-extramedular que se manifesta pela primeira vez como um inchaço na região anterior do pescoço não foi relatado. Apresentamos o caso de um paciente de 69 anos com queixa de inchaço cervical esquerdo doloroso de piora gradual há mais de 8 anos. Primeiramente, uma abordagem da linha média da coluna vertebral posterior foi realizada para a ressecção do tumor, e a porção do tumor no canal vertebral foi totalmente removida. A segunda operação, via anterolateral, foi planejada para ser realizada em uma segunda sessão cirúrgica. O paciente recebeu alta hospitalar sem déficits neurológicos. O diagnóstico histopatológico foi um schwannoma. O primeiro objetivo da cirurgia é tratar déficits neurológicos em pacientes com schwannomas intraespinhais cervicais com/sem extensão para as regiões extravertebrais do pescoço paravertebral. A estratégia cirúrgica combina a abordagem da linha média posterior e a abordagem anterolateral cervical na mesma sessão ou em momentos diferentes.

Clinicopathologic features and surgical efficacy of retroperitoneal ganglioneuroma / 中华普通外科杂志

Objective:To explore the clinical diagnosis and treatment methods and curative effect of retroperitoneal ganglioneuromaMethods:The clinical data of 32 cases of retroperitoneal ganglioneuroma admitted to Peking University International Hospital from Apr 2015 to May 2022 were retrospectively analyzed, and their clinical characteristics, surgical efficacy and prognosis were discussed.Results:Of the 32 patients with retroperitoneal ganglioneuroma, 17 had no obvious clinical symptoms, 7 complained abdominal distension and pain, 6 had lower back pain, and 2 had abdominal mass. Tumors were located near the adrenal and renal regions in 18 cases, on both sides of the spine below the kidneys in 11 cases, and in the pelvis in 3 cases. tumors were single in 28 cases, multiple in 4 cases.Tumors were surrounded by major blood vessels in 12 cases. R 0 or R 1 resection was carried out in 27 cases, and palliative R 2 resection in 5 cases, combined organ resection in 6 cases, and piecemed resection in 8 cases. The maximum tumor diameter was (13.2±4.9)cm, the intraoperative blood loss was 500 (50-6 000 ml), and 6 cases suffered from major postoperative complications. Between patients with tumors encircling and encroaching major blood vessels or not, there were significant differences in age, intraoperative blood loss, R 2 resection rate, and pieceneal resection rate between the two groups ( t=2.44, P=0.021; Z=2.37, P=0.018; χ2=4.57, P=0.033; χ2=11.38, P=0.001). There was no recurrence in patients with R 0 or R 1 resection. Conclusions:The prognosis of complete resection of retroperitoneal ganglioneuroma is good .Major blood vessels encroachment of the tumor often leads to incomplente (R 2) resection.

Multimodal image three-dimensional reconstruction combined with facial nerve tracking in acoustic neuroma surgery / 中华神经医学杂志

Objective:To investigate the application value of multimodal image three-dimensional reconstruction combined with facial nerve tracking in acoustic neuroma surgery.Methods:Forty-five patients with single acoustic neuroma accepted primary surgical treatment at Department of Neurosurgery, Yibin Hospital, West China Hospital, Sichuan University from November 2018 to June 2022 were chosen. The head, skull, brain tissues, arteriovenous system, tumor and facial nerves were reconstructed preoperatively by multimodal three-dimensional image reconstruction combined with facial nerve tracking. Preoperative planning was carried out according to the relationship between tumor and surrounding structures. The accuracy of facial nerve tracking was evaluated by intraoperative microscopic observation and nerve electrophysiological monitoring. House-Brackmann (H-B) criteria was used to evaluate facial nerve functions 2 weeks after surgery. Three months after surgery, all patients underwent enhanced MRI scanning, and combined with intraoperative findings, the degrees of acoustic neuroma resection were determined.Results:The facial nerves and their relations with acoustic neuroma were successfully tracked in all 45 patients; as confirmed by intraoperative microscopy and nerve electrophysiological monitoring, the results of facial nerves and their relations with acoustic neuroma were consistent in 42 patients and inconsistent in 3 patients. Using intraoperative nerve electrophysiological monitoring as gold standard, the accuracy of multimodal image three-dimensional reconstruction of the facial nerves was 93.3%. Preoperative facial nerve tracking results were highly consistent with intraoperative nerve electrophysiological monitoring results (Kappa=0.903, P<0.001). The facial nerves were intraoperatively preserved in 91.1% patients (41/45), and the facial nerve function was good in 86.7% patients (39/45) at 2 weeks after surgery. The total/subtotal resection rate of acoustic neuroma was 88.9% (40/45). No death, cerebrospinal fluid leakage, hematoma, or cerebral infarction were noted during the perioperative period. Conclusion:Multimodal image three-dimensional reconstruction combined with facial nerve tracking can help to protect blood vessels and nerves, improve total resection rate and facial nerve function retention rate in surgery of acoustic neuroma.

Mucosal Schwann cell hamartoma of the gallbladder

Mucosal Schwann cell hamartoma (MSCH) is a rare benign neurogenic tumor characterized by pure S100p positive spindle cell proliferation. Most cases occur in the distal colon. Involvement of the gall bladder is exceedingly rare. There have been no reports of recurrence or a syndromic association with MSCH. Herein, we describe a case of MSCH of the gallbladder in a 55-year-old female patient with prior history of gastrointestinal neurofibromas who presented with abdominal pain. MR imaging revealed choledocholithiasis, gallbladder thickening, and marked biliary and pancreatic ductal dilation. The patient subsequently underwent cholecystectomy with choledochoduodenostomy. Histologic evaluation of the gallbladder showed diffuse expansion of the mucosa with S100p positive cells with spindly nuclei and indistinct cytoplasmic borders and diagnosis of MSCH of the gallbladder was rendered.

Application of Mixed Reality Technology in Surgical Resection of Vestibular Schwannoma and Doctor-patient Communication / 肿瘤防治研究

Objective To investigate application value and significance of mixed reality technology in surgical treatment and doctor-patient communication for vestibular schwannoma. Methods We selected randomly 13 vestibular schwannoma patients treated with surgical treatment. After the three-dimensional models were constructed, preoperative surgical planning and doctor-patient communication were performed with mixed reality technology. Craniotomy through retrosigmoid sinus approach, tumor resection and facial nerve protection were achieved intraoperatively with the assistance of mixed reality technology. Questionnaires were collected and facial nerve function of 13 patients was recorded one week after operation. Results Holographic model images of 13 cases were showed successfully using mixed reality technology. The locations of preoperative facial nerves reconstructed were completely consistent with actual locations in 10 cases (84.6%). After preoperative anatomic analysis, it was decided to remove partly the posterior wall of the internal auditory canal in 11 cases. The result of doctor-patient communication questionnaire showed that 13 patients and their family all had a thorough understanding of the condition, operative plan and risks, and expressed satisfaction with the preoperative conversation. With the assistance of mixed reality technology, the tumors were resected totally without injury of vein sinus in 13 cases. The facial nerve function was gradeⅠin 3 cases, gradeⅡin 6 cases, grade Ⅲ in 3 case and grade Ⅳ in 1 case based on House-Brackmann grading one week after surgery. Conclusion Mixed reality technology is quite helpful in individual surgical planning and preoperative doctor-patient communication. It helps reduce the side injuries of surgery and protect the function of facial nerve as a surgical assistant tool intraoperatively.

Solitary circumscribed neuroma of the conjunctiva: Differential diagnosis from neurofibroma is a must? / Neuroma circunscrito solitário da conjuntiva: diagnóstico diferencial de neurofibroma obrigatório?

ABSTRACT A 42-year-old male presented with a 4-week history of a mass in the right inferior palpebral conjunctiva close to the punctum. An excisional biopsy of the lesion and histopathological examination revealed that the mass was composed of Schwann cells with thin conical nuclei, fine chromatin, and unnoticeable nucleoli. Immunohistochemically, the spindle cells were diffusely and strongly positive for S100 protein. Neurofilament immunostaining was also positive, which highlighted axons. In light of these findings, the tumor was diagnosed as solitary circumscribed neuroma. A comprehensive evaluation for multiple endocrine neoplasia type 2b was performed. However, no multiple endocrine neoplasia type 2b stigmata and no family history were detected. The diagnosis was therefore finalized as solitary circumscribed neuroma, which is considered as a rare condition. The differential diagnosis is based on the histopathological examination and immunohistochemical evaluation. As the tumor can be related with multiple endocrine neoplasia type 2b, it is essential to systematically investigate for multiple endocrine neoplasia type 2b in such cases.

RESUMO Um homem de 42 anos apresentou uma massa na conjuntiva palpebral inferior direita, próxima ao punctum, com evolução de 4 semanas. Uma biópsia excisional da lesão e o subsequente exame anatomopatológico revelaram que a massa era composta de células de Schwann com núcleos cônicos, croma­tina fina e nucléolos não visíveis. Ao exame imuno-histoquímico, as células fusiformes mostraram-se difusa e fortemente positivas para a proteína S100. A imunocoloração também foi positiva para neurofilamentos e evidenciou os axônios. Considerando esses achados, o tumor foi diagnosticado como um neuroma circunscrito solitário. Procedeu-se uma investigação completa para neoplasia endócrina múltipla tipo 2b, entretanto, estigmas característicos e história familiar não foram detectados. Assim, o diagnóstico foi firmado como neuroma circunscrito solitário, condição rara cujo diagnóstico diferencial baseia-se no exame anatomopatológico e na avaliação imuno-histoquímica. Já que esse tumor pode estar relacionado à neoplasia endócrina múltipla tipo 2b, torna-se essencial, nesses casos, a investigação da neoplasia de forma sistemática.

Establishment and evaluation of traumatic neuroma model / 中国组织工程研究

BACKGROUND: Traumatic neuroma is a common complication of peripheral nerve injury, and basic research on peripheral nerve repair and regeneration through a traumatic neuroma model is rarely reported. OBJECTIVE: To establish and identify a traumatic neuroma model. METHODS: The study was approved by the Laboratory Animal Ethical Committee of Chinese PLA General Hospital. Fifteen healthy Sprague-Dawley rats aged 8 weeks old were selected to expose the sciatic nerve under microscope, and the sciatic nerve was cut 1 cm away from the posterior gluteal muscle branch using microscissors. The proximal nerve was sutured to the surrounding muscle with 11-0 microlines, and the distal nerve was left open and reflexed. After 3 weeks, ultrasonic and histological examinations were used to verify the formation of neuroma. RESULTS AND CONCLUSION: (1) Ultrasound: After 3 weeks of modeling, the proximal nerve showed obvious spindle enlargement, which was a hypoechoic parenchymal nodule without echo. (2) Gross observation: The proximal nerves were enlarged and slightly stiffened, with fibrous connective tissue hyperplasia and adhesion to the surrounding tissues. (3) Hematoxylin-eosin staining indicated hyperplasia of fibrous tissue and disorderly growth of nerve fibers in the tumor. Immunofluorescence staining (S100, NF200) revealed irregular hyperplasia of Schwann cells and nerve axons in the tumor. (4) These results suggest that the model of traumatic neuroma is successfully constructed.

Recent advances in membranous structures of acoustic neuromas and their role in surgical treatment of acoustic neuromas / 中华神经医学杂志

At present, most scholars have found that majority of acoustic neuromas are subarachnoid tumors, and extra-arachnoid tumors are rare. However, with the further study of the membranous structures of the acoustic neuromas, it has been found that the membrane structures of the surface of the acoustic neuromas may be composed of arachnoid membrane, vestibular nerve membrane, degraded vestibular nerve fibers, and dural fibrous tissues. Combined with the characteristics of membrane structure of the acoustic neuromas, it is very important to adopt appropriate surgical strategies to perform micromanipulation on tumor resection and nerve function preservation. This article reviews the characteristics of the membrane structures of the acoustic neuromas and their surgical treatment, and proposes the role of the membrane structure in surgery.

Ictericia obstructiva por neuroma de amputación de vía biliar: Reporte de caso / Obstructive ictericia by neuroma of amputation of via biliar: Case report

Los neuromas del conducto biliar se desarrollan a partir de las fibras nerviosas simpáticas y parasimpáticas que envuelven la pared de la vía biliar. Mujer de 44 años de edad con antecedente de colecistectomía convencional seis meses previos al ingreso acude a emergencia por ictericia obstructiva de 15 días de evolución. En los estudios de imagen impresiona la presencia de una masa a nivel de las vías biliares considerándose el diagnostico de una neoplasia maligna. Por los antecedentes, ausencia de marcadores tumorales se decide realizar una biopsia percutánea sin resultados concluyente, realizándose posteriormente una intervención quirúrgica con estudio anatomo-patológico compatible con neuroma de amputación de vía biliar. El neuroma de amputación a nivel de la vía biliar es un tumor infrecuente. Puede manifestarse clínicamente como una ictericia obstructiva y suele simular a un tumor maligno de las vías biliares. El manejo quirúrgico es el tratamiento definitivo.

Neuromas of the bile duct develop from the sympathetic and parasympathetic nerve fibers that surround the wall of the bile duct. A 44-year-old woman with a history of conventional cholecystectomy six months prior to hospital admission attended emergency due to obstructive jaundice that lasted 15 days. In the imaging studies, the presence of a mass at the level of the bile ducts is considered, considering the diagnosis of a malignant neoplasm. Due to the antecedents, the absence of tumor markers, it was decided to perform a percutaneous biopsy without conclusive results, performing later a surgical intervention with anatomopathological study compatible with neuroma of biliary tract amputation. The amputation neuroma at the level of the bile duct is an infrequent tumor. It can manifest clinically as obstructive jaundice and usually simulates a malignant tumor of the bile ducts. Surgical management is the definitive treatment.

Different clinical presentation of intralabyrinthine schwannomas - a systematic review / Diferentes apresentações clínicas do schwannoma intralabiríntico --- uma revisão sistemática

Abstract Introduction: Intralabyrinthine schwannoma is a rare, benign tumor that affects the most terminal portions of the vestibular and cochlear nerves. This tumor can be classified into 10 subtypes, according to its inner ear location. Objective: To carry out a comprehensive review of the most frequent auditory manifestations secondary to the intralabyrinthine schwannoma, describing the possible underlying pathophysiological mechanisms. Methods: Systematic review of the literature until October 2017 using the PubMed, Web of Science and Scopus databases. The inclusion criteria were clinical manifestations of the intralabyrinthine schwannoma. Three researchers independently assessed the articles and extracted relevant information. The description of a case of an intravestibular subtype intralabyrinthine schwannoma with multiple forms of clinical presentations was used as an example. Results: Twenty-seven studies met our inclusion criteria. The most common intralabyrinthine schwannoma subtype was the intracochlear, followed by the intravestibular type. All the cases demonstrated hearing loss, usually progressive hearing loss. Conclusion: The diagnosis of intralabyrinthine schwannomas is based on high-resolution magnetic resonance imaging and should be included in the differential diagnosis of patients with vestibulocochlear complaints. Although there are approximately 600 cases in the literature, we still lack a detailed description of the clinical evolution of the patients, correlating it with MRI findings of temporal bones and tumor subtype.

Resumo Introdução: Schwannoma intralabiríntico é um tumor benigno, raro, que afeta as porções mais terminais dos nervos vestibular e coclear. Este tumor pode ser classificado, de acordo com sua localização na orelha interna, em 10 subtipos. Objetivo: Realizar uma revisão abrangente das manifestações auditivas mais frequentes secundárias ao schwannoma intralabiríntico e descrever os possíveis mecanismos fisiopatológicos subjacentes. Método: Revisão sistemática da literatura até outubro de 2017 nas bases de dados PubMed, Web of Science e Scopus. O critério de inclusão foi manifestações clínicas do schwannoma intralabiríntico. Três pesquisadores avaliaram de forma independente os artigos e extraíram informações relevantes. Exemplificamos com a descrição de um caso de schwannoma intralabiríntico subtipo intravestibular com múltiplas formas de apresentações clínicas. Resultados: Vinte sete estudos contemplaram nossos critérios de inclusão. O subtipo do schwannoma intralabiríntico mais comum encontrado foi o intracoclear, seguido pelo intravestibular. Todos os casos apresentaram alteração auditiva, normalmente perda auditiva progressiva. Conclusão: O diagnóstico de schwannomas intralabirínticos baseia-se em exames de ressonância magnética de alta resolução e deve ser incluído no diagnóstico diferencial de pacientes com queixas vestibulococleares. Apesar de termos aproximadamente 600 casos na literatura, ainda nos falta descrição detalhada da evolução clínica dos pacientes em correlação com achados na ressonância magnética de ossos temporais e o subtipo tumoral.

Anthropometry Of Internal Acoustic Meatus In Dry Adult Human Skull Using Casting Method

Background: The internal acoustic canal (IAC), is a bony canal within the petrous portion of the temporal bonethat transmits the VII and VIII cranial nerves and labyrinth vessels. Quantitative and morphometric assessmentof the internal auditory canal (IAC) are essential to establish the anatomical bases for microsurgery of thecerebellopontine angle and acoustic neuroma, which may produce bone changes and is an important intracranialpathology.Materials and Methods: The present study was conducted on 40 adult temporal bone of adult skulls.The impressionof IAM/IAC was taken using impression material Poly Vinyl Silioxane, then the measurement were taken on drycast using digital vernier calliper. The obatined data was tabulated and analysed statistically.Results: The mean height and width of right and left side of IAM at porous end, middle portion and fundus endwas 3.56 & 3.86mm & 3.48 & 4.13 mm, 3.52 & 3.37mm & 3.44 & 3.47mm, 3.26mm & 3.08mm, 3.36 & 3.19mmrespectively. The Mean superior length & inferior length on right side was 9.89 & 8.43mm & 9.94 & 8.59 mm onleft side respectively. On comparing the parameters between right and left sides by using unpaired t test, it wasfound to be statistically insignificant.Conclusion: The present study presents a baseline data on morphometric measurements of IAM of dry adultsskulls among south Indian population, also confirms that there is a differences in dimensions of IAM amongdifferent races and regions and thus emphasises need to have knowledge of normal data of different population.

Clinical efficacy of ultrasound-guided interventional therapy for Morton neuroma / 中国医学影像技术

Objective: To investigate the clinical efficacy of ultrasound-guided interventional therapy for Morton neuroma. Methods All 36 patients with Morton neuroma were randomly divided into observation group (n=19) and control group (n=17). The patients of observation group were given ultrasound-guided interventional therapy, while the patients of control group were treated with traditional occlusive therapy. Vascular and tendon injuries during therapy, infection and other complications during 8 weeks after treatment were observed. Numerical rating scale (NRS) were assessed before treatment and 4 weeks and 8 weeks after treatment, and the maximum diameter of tumor before treatment and 8 weeks after treatment were compared. Results: No complications such as vascular and tendon injuries were observed in 2 groups during treatment. Six patients in control group had local infection during 8 weeks after treatment, which were relieved after symptomatic treatment. In observation group, NRS scores decreased obviously in 4 weeks and 8 weeks after treatment compared with before treatment (both P0.05), as well as between 8 weeks and 4 weeks after treatment (P>0.05). There was no significant difference of NRS scores before treatment and 4 weeks after treatment in both 2 groups (both P>0.05), while NRS score of observation group decreased compared with control group 8 weeks after treatment (P0.05). Conclusion: Ultrasound-guided intervention is efficacy and safety in treatment of Morton neuroma.