Clinicopathological characteristics of secondary trigeminal neuralgia due to cerebellopontine angle tumors. / æ¡¥å°è„‘è§’è‚¿ç˜¤ç»§å‘æ€§ä¸‰å‰ç¥žç»ç—›çš„ä¸´åºŠç— ç†ç‰¹å¾.

OBJECTIVES: Cerebellopontine angle (CPA) tumors are a common cause of secondary trigeminal neuralgia (TN), characterized by their concealed location, slow progression, and difficulty in early detection. This study aims to explore the clinicopathological characteristics of patients with secondary TN due to CPA tumors to enhance understanding and management of secondary TN. METHODS: A retrospective analysis was conducted on clinical data and pathological results of 116 patients with CPA tumor-related TN treated at Xiangya Hospital of Central South University from January 1, 2017 to December 31, 2022. The study analyzed the relationship of tumor pathological types with clinical manifestations, tumor location, surgical methods, and treatment outcomes. RESULTS: Among the cases, 95.7% (111/116) were benign tumors, 3.4% (4/116) were malignant tumors, and 0.9% (1/116) were borderline tumors. Benign tumors were predominantly acoustic neuromas, meningiomas, and schwannomas. Among the patients, 46.6% (54/116) presented with isolated TN, while 53.4% (62/116) exhibited other associated symptoms depending on factors such as tumor growth location and rate. The complete resection rate in this group was over 90%, with 41.4% (48/116) of patients undergoing concurrent microvascular decompression after tumor resection, predominantly for schwannomas. The overall effective rate of surgical treatment reached 93.9%, with schwannomas showing higher efficacy rates compared with acoustic neuromas and meningiomas (P<0.05). The recurrence rate of acoustic neuromas was significantly higher than that of meningiomas and schwannomas (P<0.05). CONCLUSIONS: CPA tumors are a major cause of secondary TN, predominantly benign, with occasional underdiagnosed malignant tumors. Early diagnosis and treatment significantly impact prognosis. Different tumor types vary in clinical symptoms, surgical approaches, and treatment efficacy. Surgical strategies should balance tumor resection extent and neural function preservation, with microvascular decompression as necessary.

[To the origins of surgery for acoustic neuromas]. / K istokam khirurgii nevrinom slukhovogo nerva.

Acoustic neuroma is one of the most common tumors of the posterior cranial fossa. Its removal is always a challenge for the neurosurgeon and the patient. The history of surgery for acoustic neuromas is inextricably linked with the history of neurosurgery in general. The modern surgical community must know history and be able to use it. Only then will the development of surgery lead to the preservation of the quality of life of patients. In the history of surgery for acoustic neuromas, the stages of its development are clearly visible from the description of the clinical picture through the study of the anatomy of the cerebellopontine angle to modern microsurgical removal.

Fully neuroendoscopic resection of cerebellopontine angle tumors through a retrosigmoid approach: a retrospective single-center study.

The objective of this study is to preliminarily investigate the surgical safety, efficacy, techniques, and clinical value of fully neuroendoscopic surgery for the resection of cerebellopontine angle (CPA) tumors via a retrosigmoid approach. The clinical data of 47 cerebellopontine angle area (CPA) tumors that were treated by full neuroendoscopic surgery from June 2014 to June 2023 were retrospectively analyzed. The efficacy and advantages of the surgical techniques were evaluated based on indicators such as duration of the surgery, neuroendoscopic techniques, intraoperative integrity of nerves and blood vessels, extent of tumor resection, outcomes or postoperative symptoms, and incidence of complications. The 47 cases of cerebellopontine angle tumors include 34 cases of epidermoid cysts, 7 cases of vestibular schwannomas, and 6 cases of meningiomas. All patients underwent fully neuroendoscopic surgery. Twenty tumors were removed using the one-surgeon two-hands technique, and 27 tumors were removed using the two-surgeons four-hands technique. The anatomical integrity of the affected cranial nerves was preserved in all 47 cases. None of the patients suffered a postoperative hemorrhage, cerebrospinal fluid leak, and aseptic or septic meningitis, or died. The rate of total tumor resection was 72.3% (34/47), and the symptom improvement rate was 89.4% (42/47). All patients were followed up for 2 to 12 months, and none died nor showed any signs of tumor recurrence. By analyzing 47 fully neuroendoscopic resections of CPA tumors using the posterior sigmoid sinus approach in our center, we believe that such method allows complete, safe, and effective resection of CPA tumors and is thereby worthy of clinical promotion.

POLR2A Mutation is a Poor Prognostic Marker of Cerebellopontine Angle Meningioma.

BACKGROUND AND OBJECTIVES: Recent molecular analyses have shown that the driver genetic mutations of meningiomas were associated with the anatomic location. Among these, POLR2A mutation is common among lesions in the skull base, mainly in the cerebellopontine angle (CPA). The objective of this study was to investigate the efficacy of POLR2A mutation as a prognostic marker for CPA meningiomas. METHODS: We retrospectively analyzed the clinical data of 70 patients who had World Health Organization grade I CPA meningiomas. Somatic DNA was analyzed by Sanger sequencing and microsatellite array to examine for NF2 , AKT1 , KLF4 , SMO , and POLR2A mutations and 22q loss. Genetic and clinical parameters were analyzed to identify the factors related with tumor recurrence. RESULTS: We detected clearly the clinical features of the CPA cases with POLR2A mutation. Compared with cases without POLR2A mutation, cases with POLR2A mutation had more meningothelial type ( P = 6.9 × 10 -4 ), and higher rate of recurrence ( P = .04). We found that the poor prognostic factors associated with the recurrence of CPA meningiomas were POLR2A mutation ( P = .03, hazard ratio [HR] 9.38, 95% CI 1.26-70.0) and subtotal resection (STR) ( P = 5.1 × 10 -4 , HR 63.1, 95% CI 6.09-655.0). In addition, in the group that underwent STR, POLR2A mutation was a poor prognostic factor associated with tumor recurrence ( P = .03, HR 11.1, 95% CI 1.19-103.7). CONCLUSION: POLR2A mutation and STR were the poor prognostic markers associated with the recurrence of CPA meningioma. For CPA meningioma cases that underwent STR, only POLR2A mutation was a poor prognostic factor. Detecting POLR2A mutation may be a cost-effective, easy, and useful marker for prognostication.

Unilateral Multifocal Inner Ear and Internal Auditory Canal or Cerebellopontine Angle Cochleovestibular Schwannomas-Genetic Analysis and Management by Surgical Resection and Cochlear Implantation.

OBJECTIVE: To describe the genetic characteristics and the management of two very rare cases of unilateral multifocal inner ear and internal auditory canal or cerebellopontine angle cochleovestibular schwannomas not being associated to full neurofibromatosis type 2-related schwannomatosis. PATIENTS: In a 29-year-old man and a 55-year-old woman with single-sided deafness multifocal unilateral cochleovestibular schwannomas were surgically resected, and hearing was rehabilitated with a cochlear implant (CI). Unaffected tissue was analyzed using next generation sequencing of the NF2 gene. Tumor tissue was analyzed using a 340-parallel sequencing gene panel. MAIN OUTCOME MEASURES: Mutations in the NF2 gene, word recognition score for monosyllables at 65 dB SPL (WRS 65 ) with CI. RESULTS: No disease-causing mutation was detected in the examined sequences in blood leucokytes. All tumor samples revealed, among others, somatic pathogenic NF2 mutations. While the anatomically separate tumors in case 1 were likely molecular identical, the tumors in case 2 showed different genetic patterns. WRS 65 was 55% at 6 years of follow-up and 60% at 4.5 years of follow-up, respectively. CONCLUSIONS: The occurrence of multifocal unilateral cochleovestibular schwannomas without pathogenic variants in NF2 in non-affected blood leucocytes can be associated with mosaic NF2 -related schwannomatosis (case 1), or with likely sporadic mutations (case 2) and may be overlooked due to their extreme rarity. Although challenging, successful hearing rehabilitation could be achieved through surgical resection of the tumors and cochlear implantation.

Malignant peripheral nerve sheath tumour of the cerebellopontine angle in a cat.

Malignant peripheral nerve sheath tumour is an uncommon tumour in cats. The current case report aims to present the diagnosis, treatment and histopathology of a malignant peripheral nerve sheath tumour that developed in the cerebellopontine angle region of a cat. A 4-year-old 4.2-kg female calico cat was brought to the animal hospital with the complaints of loss of balance, leaning against the wall and behavioural changes. During the neurological examination, the patient was observed to have a right-sided head tilt accompanied by ipsilateral ventral strabismus. On magnetic resonance imaging scan, a mass was identified at the right cerebellopontine angle. The surgical removal of the mass was carried out using a caudo-tentorial approach. The histopathological analysis revealed the presence of a malignant peripheral nerve sheath tumour. This case report contributes to the existing knowledge in the field presenting the characteristics of a malignant peripheral nerve tumour that was not associated with any nerve in the cerebellopontine angle in a cat.

Cerebellopontine angle tumor presenting as acute audiovestibular syndrome.

BACKGROUND: Acute audiovestibular deficits may be a harbinger of vestibular schwannoma (VS). OBJECTIVE: To investigate clinical and laboratory features of 25 consecutive patients with VS presenting with acute audiovestibular deficits. METHODS: A symptomatic combination of acute audiovestibular deficits was investigated. Audiometric and vestibular function tests, and internal auditory canal magnetic resonance imaging (IAC MRI) results were evaluated. RESULTS: Varying combinations of symptoms may develop in VS patients with acute audiovestibular deficits, of whom sudden hearing loss (HL) without acute vertigo or acute facial nerve palsy (FNP) was most common. The most common audiometric configuration was high-tone hearing loss, and no patient showed low-tone hearing loss. IAC MRI demonstrated that the tumor had an intracanalicular portion and attachment to the bony IAC wall in all patients and widened the IAC wall in some patients. CONCLUSION: Different symptomatic combinations of acute audiovestibular deficits may develop in patients with VS. Awareness about the possibility of VS as a cause of sudden HL, acute vertigo, and acute FNP, as well as subsequent IAC MRI scanning is vital to earlier diagnosis of VS in these patients.

Linfoma primario del sistema nervioso central aparentando lesión del ángulo pontocerebeloso / Primary central nervous system lymphoma mimicking cerebellopontine angle tumour

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Síndrome constitucional como presentación de meningioma pontocerebeloso / Constitutional syndrome as a presentation of a cerebellopontine meningioma

Los meningiomas son tumoraciones eminentemente benignas procedentes de las meninges, correspondiendo al 15-25% de las tumoraciones intracraneales en el adulto, cuya expresividad clínica viene representada por la compresión de estructuras vecinas quedando representada predominantemente por cefalea, alteraciones del comportamiento, y déficit neurológicos. Presentamos un caso donde el cuadro constitucional constituye la primera y principal manifestación de un meningioma intracraneal pontocerebeloso (AU)

Meningiomas are basically benign tumours arising in the meninges and account for 15-25% of intracranial tumours in adults. It is clinically signs are due to compression of the neighbouring structures, with the main symptoms being migraine, behavioural changes, and neurological deficits. We present a case where constitutional syndrome was the first and principal manifestation of an intracranial cerebellopontine meningioma (AU)

Condroma intracraneal que involucra al ángulo pontocerebeloso: un reto diagnóstico / Intracranial chondroma involving the cerebellopontine angle: a diagnostic challenge

El condroma intracraneal es un tumor cartilaginoso benigno inusual con una incidencia inferior al 1% de todos los tumores primarios intracraneales. El origen de los condromas intracraneales no se ha establecido definitivamente. Rara vez se detectan en el ángulo pontocerebeloso. Describimos un nuevo caso de condroma del ángulo pontocerebeloso que clínica y radiológicamente se diagnosticó como schwannoma. Aunque el estudio histopatológico sigue siendo el «gold standard» para el diagnóstico definitivo, es importante que neurólogos, radiólogos y neurocirujanos conozcan esta entidad para realizar el diagnóstico diferencial y tratamiento apropiados (AU)

Intracranial chondroma is a rare, benign, cartilaginous tumour accounting for less than 1% of all primary intracranial tumours and with an uncertain origin. Very few cases have been reported involving the cerebellopontine angle. We describe a new case of cerebellopontine angle chondroma which was clinically and radiologically diagnosed as schwannoma. Although histopathological examination is the gold standard for definitive diagnosis, it is important that neurologists, radiologists and neurosurgeons are aware of this entity for correct differential diagnosis and treatment (AU)

Cisto epidermoide da fossa posterior: relato de caso / Epidermoid cyst of the posterior fossa: a case report

Epidermoid cysts of the central nervous system are uncommon conditions, which are frequently located in the cerebellopontine angle and around the pons. They are covered with keratinized squamous epithelium and keratin lamella, which give its contents a soft, white-pearly appearance. Epidermoid cysts are mostly originated from malformations, presumably associated with surface elements of the nervous system ectoderm during the closure of the neural groove or formation of secondary cerebral vesicles. The authors describe a case of epidermoid cyst in the posterior fossa causing hydrocephalus and review morphologic and diagnostic criteria of this lesion.

Os cistos epidermoides do sistema nervoso central (SNC) são condições incomuns, estando localizados mais frequentemente no ângulo pontocerebelar e ao redor da ponte. Eles são revestidos por epitélio escamoso queratinizado e lamelas de queratina, tornando seu conteúdo branco-perolado e pastoso. Os cistos epidermoides são, na maioria das vezes, originados de malformações, possivelmente associados ao entremeio de elementos superficiais do ectoderma do SNC durante o fechamento da placa neural, ou formação das vesículas cerebrais secundárias. Os autores descrevem um caso de cisto epidermoide da fossa posterior determinando hidrocefalia e revisam critérios morfológicos e diagnósticos dessa lesão.

Neuralgia del trigémino provocada por un tumor del ángulo ponto-cerebelosocontralateral. Caso clínico / Trigeminal neuralgia caused by contra lateral cerebellopontine angle tumor. A case report

La gran mayoría de neuralgias del trigémino sonatribuidas a la compresión por estructuras vasculares,de la zona de entrada del nervio a nivel de la protuberancia.En torno al 1% de los casos de neuralgia deltrigémino están asociados a tumores del ángulo pontocerebelosoipsilaterales. Las neuralgias del trigéminoprovocadas por tumores contralaterales de la fosaposterior son muy raras. Los autores presentan el casoclínico de un paciente de 37 años, con una neuralgiadel trigémino derecha y un voluminoso neurinoma delacústico izquierdo. El dolor remitió completamente despuésde la escisión del tumor (AU)

In most cases, trigeminal neuralgia is due to compressionof the trigeminal nerve in the zone of entranceat the pons by vascular structures. About 1% of cases,have trigeminal neuralgia associated with the presenceof ipsilateral lesion of cerebellar-pontine angle. Rarely,trigeminal neuralgia may be due to contralateral posteriorfossa tumors. We present a case of a 37-year- oldpatient who presented with right trigeminal neuralgiaand harboured a left acoustic neuroma of significantdimension. Facial pain completely disappeared aftertumor removal (AU)

Eficacia del oxígeno hiperbárico en el tratamiento de la radionecrosis y el edemacerebral sintomáticos tras radiocirugía con acelerador lineal / Usefulness of hyperbaric oxygen in the treatment of radionecrosis and symptomatic brain edema after LINAC radiosurgery

La radio necrosis y el edema cerebral son complicaciones asociadas a la radiocirugía. Presentamos los casos de tres pacientes tratadas con radiocirugía mediante acelerador lineal, de un meningioma de polo frontal izquierdo, peñasco y para sagital parietooccipital, respectivamente, que desarrollaron, entre dos y ocho meses más tarde, lesiones de tipo radio necrótico con extenso edema peritumoral que resultaron resistentes al tratamiento con esteroides y que se han resuelto con la administración de 40 sesiones de oxígeno hiperbárico. Son pocos los casos publicados hasta ahora en la literatura con tan excelentes resultados, por lo que consideramos un hecho a tener muy en cuenta ante las posibles complicaciones de este tipo que puedan presentarse en el transcurso de la práctica radioquirúrgica antes de recurrir a la cirugía (AU)

Radionecrosis with brain edema is a complication of radiosurgery. Three female patients harbouring a frontal pole, petrous and parasagital parietoocipital meningiomas respectively who had been treated with LINAC radiosurgery are presented. Those patients developed, between two and eight months later, a severe symptomatic radionecrosis with a huge brain edema resistant to the usual steroid therapy. Only after 40 sessions of hyperbaric oxygen, a good remission of the lesions was obtained. There are few cases reported in the literature with such a good outcome. Consequentely, this therapy must be taken into account to treat this type of radiosurgical complication before considering surgery (AU)

Cerebral salt wasting syndrome: postoperative complication in tumours of the cerebellopontine angle

Cerebral salt wasting (CSW) is a rare complication in posterior fossa tumour surgery. We present two patients with cerebellopontine angle (CPA) tumours who developed cerebral salt wasting postoperatively. Both patients deteriorated in spite of intensive fluid and salt replacement. On CT scan the patients presented mild to moderate ventricular dilation, which was treated with an external ventricular drainage. After the resolution of hydrocephalus, fluid balance rapidly returned to normal in both patients and the clinical status improved. Identification and treatment of secondary obstructive hydrocephalus may contribute to the management of CSW associated to posterior fossa tumour surgery (AU)

El síndrome pierde sal cerebral (CSW, en sus siglas en inglés) es una complicación rara en la cirugía de los tumores de la fosa posterior. Presentamos a 2 pacientes con tumores del ángulo pontocerebeloso que desarrollaron un CSW posquirúrgico. Ambos pacientes tuvieron un empeoramiento pese a la fluidoterapia y la reposición de sal intensivas. La tomografía computarizada (TC) mostraba una dilatación ventricular leve a moderada que fue tratada mediante un drenaje ventricular externo. Tras la resolución de la hidrocefalia el balance hidroelectrolí-tico se normalizó rápidamente en ambos pacientes y su situación clínica mejoró. La identificación y el tratamiento precoz de la hidrocefalia obstructiva pueden contribuir al tratamiento del síndrome pierde sal asociado a la cirugía de tumores de la fosa posterior (AU)

Associação entre tamanho e potencial proliferativo em neurinomas do acústico / Size and proliferative index correlation in acoustic neuromas

Schwanomas do acústico são os tumores mais freqüentes localizados no ângulo pontocerebelar. Os mecanismos moleculares que levam a sua geração e crescimento ainda não são bem conhecidos. Várias características clínicas, radiológicas e imuno-histoquímicas já foram estudadas e correlacionadas ao crescimento tumoral. Estudamos e correlacionamos aspectos clínicos e imuno-histoquímicos (MIB-1) de 11 schwanomas do acústico operados no Hospital São Paulo/UNIFESP. O tamanho dos tumores correlacionou-se com o índice proliferativo (Ki-67), não havendo correlação com significância estatística entre a idade dos pacientes, duração dos sintomas e índice proliferativo.

Rabdomioma fetal intracraneal de fosa posterior / Intercraneal fetal type rhabdomyoma of the posterior fossa

Los rabdomiomas se clasifican en cardíacos y extracardíacos, e histológicamente en maduros y fetales. Los rabdomiomas intracraneales reportados son infratentoriales y de tipo adulto. Caso clínico. Mujer de 28 años con 2 meses de evolución con vértigo y pérdida progresiva de la audición. El estudio de resonancia magnética mostró un tumor bien delimitado, de 6×3cm en el ángulo pontocerebeloso derecho, hiperintenso en T2 y que reforzaba con gadolinio. Comprimía la porción anterior del hemisferio cerebeloso derecho y desplazaba considerablemente el tallo cerebral de derecha a izquierda. Se realizó craniectomía retrosigmoidea derecha con resección del 95% de la lesión. Conclusión. Los rabdomiomas son neoplasias intracraneales infrecuentes. Presentamos el primer caso del tipo fetal en el ángulo pontocerebeloso(AU)

Rhabdomyomas (RM) are benign, well circumscribed tumors consisting of striated muscle cells and are classified as cardiac or extracardiac, according to their location and histologically into adult or fetal types. Intracranial RM are extremely rare and are usually adult type tumors. Clinical history. A 28 year old woman presented with a two month history of dizziness and progressive unilateral hearing loss. MRI showed a well-circumscribed, 6×3cm tumor in the right cerebello-pontine angle, which was T2 hyperintense with gadolinium reinforcement. The tumor compressed the anterior portion of the right cerebellar hemisphere and caused a notable displacement of the brain stem from right to left. A right retrosigmoid craniectomy was performed and 95% of the tumor was resected. Conclusion. A case of extracardiac (intracranial) fetal type rhabdomyoma is reported for the first time in the cerebello-pontine angle of a female patient(AU)

Meningioma del conducto auditivo interno: una rara entidad / Meningioma of the internal auditory canal: A rare entity

Presentamos el caso de un varón de 61 años que en la preparación para un implante coclear en el oído izquierdo se observa en la resonancia magnética una lesión en el conducto auditivo interno (CAI) derecho compatible con un schwannoma vestibular (SV). Se decide realizar implante coclear en el oído izquierdo y a los 3 meses se extirpa la tumoración del CAI derecho con el resultado anatomopatológico de meningioma (AU)

We present the case of a 61-year-old man in whom the magnetic resonance imaging performed prior to left cochlear implant surgery revealed a lesion in the right internal auditory canal resembling a vestibular Schwannoma. Left cochlear implant surgery was performed and three months later, the right internal auditory canal lesion was removed. The final histopathological analysis revealed it to be a meningioma (AU)

Papiloma de plexos coroideos extraventricular / Extraventricular choroid plexus papilloma

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Comentario al trabajo Melanocitoma meníngeo del ángulo pontocerebeloso, ¿Un tumor benigno?de González-Tortosa y cols / No disponible

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