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.

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.

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.

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.

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.

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.

Multistep translation and cultural adaptation of the Penn Acoustic Neuroma Quality of Life scale in Chinese / 中国实用护理杂志

Objective@#To translate and culturally adapt the Penn Acoustic Neuroma Quality of Life (PANQOL) scale into Chinese, and introduce the first specific evaluation tool for the quality of life of acoustic neuroma patients.@*Methods@#Based on strict scale introduction requirements (scale preparation, translation, cultural adaptation and performance evaluation), elaborate on the multistep translation model (forward translation, synthesis, back translation, back translation review, comprehensive coordination) and cultural adaptation process (expert committee, preliminary experiment) to the preliminary experiments PANQOL scale in Chinese version. A preliminary survey and cognitive interview were conducted on 30 patients with acoustic neuroma, and the scale was further revised to establish the Chinese version of PANQOL scale.@*Results@#The translation validity index of each item (I-TVI) in the Chinese version of the PANQOL scale was 96%-100%, and the translation validity index at the scale level (S-TVI) was 100%, reaching the translation standard. The PANQOL scale of 30 patients in the preliminary experiment was generally divided into 73.75 ± 12.12.@*Conclusions@#The multistep translation and cultural adaptation of PANQOL scale is a complex and time consuming process. Strict implementation of this process can ensure the quality of the scale introduction. The performance of the Chinese version of PANQOL scale needs to be studied and evaluated by expanding the sample size latterly.

Multistep translation and cultural adaptation of the Penn Acoustic Neuroma Quality of Life scale in Chinese / 中国实用护理杂志

Objective To translate and culturally adapt the Penn Acoustic Neuroma Quality of Life (PANQOL) scale into Chinese, and introduce the first specific evaluation tool for the quality of life of acoustic neuroma patients. Methods Based on strict scale introduction requirements (scale preparation, translation, cultural adaptation and performance evaluation), elaborate on the multistep translation model (forward translation, synthesis, back translation, back translation review, comprehensive coordination) and cultural adaptation process (expert committee, preliminary experiment) to the preliminary experiments PANQOL scale in Chinese version. A preliminary survey and cognitive interview were conducted on 30 patients with acoustic neuroma, and the scale was further revised to establish the Chinese version of PANQOL scale. Results The translation validity index of each item (I-TVI) in the Chinese version of the PANQOL scale was 96%-100% , and the translation validity index at the scale level (S-TVI) was 100%, reaching the translation standard. The PANQOL scale of 30 patients in the preliminary experiment was generally divided into 73.75 ± 12.12. Conclusions The multistep translation and cultural adaptation of PANQOL scale is a complex and time consuming process. Strict implementation of this process can ensure the quality of the scale introduction. The performance of the Chinese version of PANQOL scale needs to be studied and evaluated by expanding the sample size latterly.

Ulcerated bleeding palisaded encapsulated neuroma of the tongue

@#Introduction: Palisaded encapsulated neuroma (PEN) is a benign lesion of Schwann cells and its occurrence in the oral mucosa especially the tongue is very rare. Case Report: This article describes a case of a 41-year-old male, a chronic smoker with an actively bleeding, ulcerated, solitary, firm lesion on the lateral border of the tongue which had bled thrice before. A differential diagnosis of pyogenic granuloma, haemangioma, fibroma, nerve sheath tumour, salivary gland tumour and malignancy was made and surgically excised. Histopathology of the excised specimen revealed a well-circumscribed lesion with spindle-shaped cells arranged in interlacing fascicles and with the help of immunohistochemical markers confirmed it to be a PEN. Discussion: To our knowledge, this is the first description of an ulcerated PEN presented with an active bleed.

Palisaded encapsulated neuroma clinically mimicking a mucocele: report of a case / Neuroma encapsulado em paliçada mimetizando mucocele: relato de caso

ABSTRACT We report the case of a 21-year-old female patient with the chief complaint of a small tumor on the lower left lip. Intraoral examination revealed a two-month evolution nodular lesion on the left labial mucosa, of resilient consistency and asymptomatic. A history of prior trauma was reported by the patient. A provisional diagnosis of mucocele was established, and an excisional biopsy was performed. The diagnosis was palisaded encapsulated neuroma (PEN). As PEN is considered underdiagnosed by many authors, a discussion on the morphologic diagnostic criteria of this lesion will be provided.

RESUMO Relatamos o caso de uma paciente de 21 anos de idade, com queixa principal de um pequeno nódulo no lábio inferior esquerdo. O exame intraoral revelou lesão nodular na mucosa labial esquerda de consistência resiliente, assintomática, com evolução de dois meses. Histórico de trauma prévio foi relatado pela paciente. Diagnóstico provisório de mucocele foi estabelecido; realizou-se biópsia excisional. O diagnóstico foi neuroma encapsulado em paliçada (NEP). Como o NEP é considerado subdiagnosticado por muitos autores, será fornecida uma discussão sobre os critérios morfológicos de diagnóstico dessa lesão.

Intratumoral Bleeding in a Vestibular Schwannoma

Vestibular schwannomas (VSs) account for 70% of all tumors of the cerebellopontine angle (CPA). Their clinical presentation is often insidious, with progressive hearing loss and involvement of other cranial nerves. Spontaneous hemorrhage in those tumors is very unusual, and generally presents with acute clinical features such as nausea, vomiting, headache and altered consciousness, usually with marked dysfunction of the cranial nerve involved, and with new deficits of neighboring cranial nerves. Asymptomatic patients are extremely rare. We present a case report of an incidental VS with asymptomatic bleeding, which evolved to death after surgery.

Schwannomas vestibulares (SVs) são responsáveis por cerca de 70% de todos os tumores do ângulo pontocerebelar. Sua apresentação costuma ser insidiosa, com perda auditiva progressiva e envolvimento de outros nervos cranianos. Hemorragia espontânea nesses tumores é incomum, e geralmente apresenta-se agudamente, com náusea, vômitos, cefaleia e alterações de consciência, normalmente com disfunção importante dos nervos cranianos envolvidos e com novos déficits dos nervos próximos. Pacientes assintomáticos são extremamente raros. Apresentamos um relato de caso de um SV incidental com sangramento assintomático que evoluiu para o óbito após cirurgia.

Multiple Endocrine Neoplasia Type 2b Syndrome: A Rare Case Presentation

Multiple endocrine neoplasia Type 2b is a rare syndrome caused by mutations in RET proto oncogene. It is a rare entity, which is found 1 in 30,000 people. We report a case of 35–year–old male patient who presented with 1 episode of syncope; had bumpy lips and mucosal neuromas over the tongue which on detailed biochemical and radiological investigations turned out to be MEN Type 2B syndrome

Relationship of Vertigo and Postural Instability in Patients With Vestibular Schwannoma

OBJECTIVES: Growth of vestibular schwannomas (VS) causes progressive vestibular symptoms and postural instability. Since the tumor grows slowly, compensation of decaying vestibular input may decrease subjective symptoms of dizziness. This study aims to estimate the relationship of subjective vestibular symptoms and objective postural instability in patients with VS. METHODS: A retrospective review of 18 patients newly diagnosed with VS and with subjective vertigo symptoms was performed. The results of vestibular function tests including the sensory organization test (SOT) using computerized dynamic posturography, caloric test, and self-report measures of subjective dizziness handicap (Dizziness Handicap Inventory) and visual analogue scale were compared according to the onset of vertigo symptoms. RESULTS: In VS patients, SOT showed decreased equilibrium score for all vestibular function related conditions, condition (C) 5 and 6, and composite (COMP) score. COMP scores were not correlated with visual analogue scale or Dizziness Handicap Inventory scores. Acute onset group included six patients and insidious onset group, 12 patients. Equilibrium scores for C5 and C6, and COMP scores were lower for insidious onset group, but the difference was not statistically significant. CONCLUSION: Our findings confirmed postural instability is prevalent in VS patients. SOT parameters did not differ significantly between acute onset and insidious onset groups, but increased tumor size and canal weakness were noted in the insidious onset group. Clinicians should consider that postural instability is likely present even in patients who do not complain of acute vertigo, and appropriate counseling should be discussed with the patients.

Morbidity Rate of the Retrosigmoid versus Translabyrinthine Approach for Vestibular Schwannoma Resection

BACKGROUND AND OBJECTIVES: Controversy related to the choice of surgical approach for vestibular schwannoma (VS) resection remains. Whether the retrosigmoid (RS) or translabyrinthine (TL) approach should be performed is a matter of debate. In the context of a lack of clear evidence favoring one approach, we conducted a retrospective study to compare the morbidity rate of both surgical approaches. SUBJECTS AND METHODS: 168 patients underwent surgical treatment (2007-2013) for VS at our tertiary care center. There were no exclusion criteria. Patients were separated into two groups according to the surgical approach: TL group and RS group. Signs and symptoms including ataxia, headache, tinnitus, vertigo and cranial nerve injuries were recorded pre- and postoperatively. Surgical complications were analyzed. Perioperative facial nerve function was measured according to House-Brackmann grading system. RESULTS: Tumor resection was similar in both groups. Facial paresis was significantly greater in RS group patients preoperatively, in the immediate postoperative period and at one year follow-up (p < 0.05). A constant difference was found between both groups at all three periods (p=0.016). The evolution of proportion was not found to be different between both groups (p=0.942), revealing a similar rate of surgically related facial paresis. Higher rate of ataxic gait (p=0.019), tinnitus (p=0.039) and cranial nerve injuries (p=0.016) was found in RS group patients. The incidence of headache, vertigo, vascular complications, cerebrospinal fluid leak and meningitis was similar in both groups. No reported mortality in this series. CONCLUSIONS: Both approaches seem similar in terms of resection efficacy. However, according to our analysis, the TL approach is less morbid. Thus, for VS in which hearing preservation is not considered, TL approach is preferable.