Cerebellopontine Angle Medulloblastoma Research Articles

Unusual manifestation of cerebellopontine angle medulloblastoma with tinnitus and sensorineural hearing loss

Abstract BACKGROUND. Medulloblastoma is the most common central nervous system embryonal tumor in children. In adults, this tumor is extremely rare, accounting for nearly 1% of primary brain tumors. Raised intracranial pressure signs are common manifestations of posterior fossa tumors, but tinnitus and/or sensorineural hearing loss are very uncommon presenting symptoms. MATERIAL AND METHODS. Starting from a very rare case of a 39-year-old male with left tinnitus and progressive left sensorineural hearing loss as isolated symptoms of a medulloblastoma, we performed a literature survey using the PubMed, ProQuest, Web of Science, Science Direct, Wiley Online search engines for patients with medulloblastoma and tinnitus and/or sensorineural hearing loss. RESULTS. All patients found in the relevant literature with auditory dysfunctions presented sensorineural hearing loss. Other frequent manifestations were: ataxia, facial numbness, vertigo, headache, nystagmus. Two patients were found with tinnitus and sensorineural hearing loss as isolated symptoms of medulloblastoma, as in our case, and in two other cases the sensorineural hearing loss was the unique symptom. With refers to the onset of medulloblastoma, just 3 patients had the first symptoms sensorineural hearing loss and tinnitus. Concerning the tumor location, in patients manifested with isolated tinnitus and sensorineural hearing loss, like our patient, the tumor arised from the internal auditory meatus, extended to the cerebellopontine angle or involved the vestibulocochlear nerve. With regards to treatment, surgery in association with radiotherapy and chemotherapy was elected in most cases (38%). CONCLUSION. It is important to pay attention at patients with isolated auditory dysfunction that may mimic significant posterior fossa tumors, such as a medulloblastoma.

The Extra-axial Cerebellopontine Angle Medulloblastoma in an Adult Patient: A Case Report and Review of Literature

Background and Importance: Medulloblastoma (MB) is regarded as a scarce primary brain neoplasm in adulthood, originating from the fourth ventricle or the cerebellum, and cerebellopontine angle (CPA)-MB has been described less often in the literature. Few cases of CPA MB have been reported in the English-written literature, most of which are intra-axial, mainly in children; adults' extra-axial CPAMB is even scarcer. To the best of the authors’ knowledge, 12 cases of extra-axial CPA MBs have been reported in the English-written literature. Case Presentation: A 23-year-old man presented to our center complaining of a generalized pulsatile headache, imbalance, swallowing impairment, and right-sided hearing loss for the past 20 days. Computed tomography (CT) scan of the brain showed a hyper-dense extra-axial mass lesion (41*37mm) in the right CPA with a significant compression effect on the fourth ventricle, causing a three ventricular obstructive hydrocephalus. Magnetic resonance imaging (MRI) showed a well-defined heterogeneous extra-axial, lobulated, dural-based mass lesion in the right CPA, hypointense on the T1 sequence, and hyperintense on the T2 sequence compared with the adjacent parenchyma, which has a bright heterogeneous enhancement during gadolinium injection. A significant mass effect was observed on the adjacent parenchyma, brain stem, and fourth ventricle without evidence of parenchymal edema. The patient underwent emergent surgery the next day regarding the mass effect and hydrocephalus. On the postoperative examination in the intensive care unit, no new neurological deficit was detected, and the swallowing and gag reflex significantly improved. Conclusion: Though rare, clinical considerations, along with early supportive radiologic measures, should be considered in subjects with suspected MB. A total tumor excision approach followed by aggregative chemotherapy/radiotherapy is designed to hinder tumor relapse.

Microsurgical Resection of Cerebellopontine Angle Medulloblastoma, Case Report and Review of Literature

Cerebellopontine angle (CPA) medulloblastomas (MB) are rare lesions with few cases previously described in the literature. We report one more case of CPA MB. The patient was a (36)-year-old female with a mass developing in the CPA. The preoperative radiological diagnosis was (hemangioblastoma) in the case. The patient was operated in Tripoli university hospital September 2020 neurosurgical department. Operated through a (retrosigmoid) approach. The intraoperative findings revealed an extra-axial tumor and the histological diagnosis was classic type of MB in the case. We review the literature and discuss pathological, radiological features and clinical follow up of CPA MB, underlining the necessity to consider MB in the differential diagnosis of CPA lesions.

Extra-Axial Cerebello-Pontine Angle Medulloblastoma in an Infant: A Rare Case Report with Review of Literature.

Medulloblastoma is a fairly common neoplastic growth seen majorly in children, presenting as an intra-axial midline mass arising from the cerebellar vermis. However, its presentation as an extra-axial mass in the cerebellopontine angle (CPA) is extremely rare, such that, only 39 cases have been reported in the world literature till 2016. Only one case has ever been reported of an extra-axial CPA medulloblastoma in an infant; who was aged 1 year. We present a case report of an 8-month-old infant, with an extra-axial CPA medulloblastoma and discuss its management strategy.

The clinical treatment and outcome of cerebellopontine angle medulloblastoma: a retrospective study of 15 cases

Medulloblastoma (MB) is the most common malignant pediatric brain tumor arising in the cerebellum or the 4th ventricle. Cerebellopontine angle (CPA) MBs are extremely rare tumors, with few cases previously described. In this study, we sought to describe the clinical characteristics, molecular features and outcomes of CPA MB. We retrospectively reviewed a total of 968 patients who had a histopathological diagnosis of MB at the Beijing Neurosurgical Institute between 2002 and 2016. The demographic characteristics, clinical manifestations and radiological features were retrospectively analyzed. Molecular subgroup was evaluated by the expression profiling array or immunohistochemistry. Overall survival (OS) and progression-free survival (PFS) were calculated using Kaplan-Meier analysis. In this study, 15 patients (12 adults and 3 children) with a mean age at diagnosis of 25.1 years (range 4–45 years) were included. CPA MBs represented 1.5% of the total cases of MB (15/968). Two molecular subgroups were identified in CPA MBs: 5 WNT-MBs (33%) and 10 SHH-MBs (67%). CPA WNT-MBs had the extracerebellar growth with the involvement of brainstem (P = 0.002), whereas CPA SHH-MBs predominantly located within the cerebellar hemispheres (P = 0.004). The 5-year OS and PFS rates for CPA MB were 80.0% ± 10.3% and 66.7% ± 12.2%, respectively. Pediatric patients with CPA MBs had worse outcomes than adult patients (OS: P = 0.019, PFS: P = 0.078). In conclusion, CPA MB is extremely rare and consists of two subgroups. Adult patients with CPA MB had a good prognosis. Maximum safe surgical resection combined with adjuvant radiotherapy and chemotherapy can be an effective treatment strategy for this rare tumor.

Extra-axial Cerebellopontine Angle Adult Medulloblastoma: An Unusual Presentation at an Unusual Site

AbstractMedulloblastoma is a rare adult primary brain tumor and its presence in the cerebellopontine angle (CPA) constitutes an even rarer site for origin. There are only 40 reported cases of CPA medulloblastoma in the literature, with most being intra-axial and occurring in the pediatric population. Extra-axial CPA medulloblastoma in adults are extremely rare and only 10 adult cases have been reported in world literature.We report a rare case of primary extra-axial CPA medulloblastoma occurring in an adult. We review the literature for such an unusual presentation of medulloblastoma in adults and discuss its appropriate clinico-radiological features along with its possible pathogenesis and surgical management.With increasing reporting of such tumors, CPA region, medulloblastoma should be considered a differential diagnosis of intra-axial or extra-axial CPA tumor, as their further management and prognosis differ significantly. Adjuvant radiotherapy and combined chemotherapy regimen should be added to the postoperative management.

Medulloblastomas in cerebellopontine angle: Epidemiology, clinical manifestations, imaging features, molecular analysis and surgical outcome

Cerebellopontine angle (CPA) medulloblastoma is rare and short of system description. We attempted to clarify its epidemiology, clinical manifestations, imaging features, pathological and molecular types, and surgical outcomes. 8 patients from 7 to 52 years old were enrolled in this retrospective study, with mean age 21.6 ± 16.4 years. The most frequent symptoms were raised intracranial pressure (100%), followed by cerebellar signs (50%), decreased hearing (50%), facial paralysis (50%), abducent paralysis (50%), and facial paresthesia (37.5%). MRI demonstrated a solid CPA lesion with heterogeneously weak or significant enhancement after gadolinium administration, accompanied with peritumoral oedema (75%), cystic change (62.5%) and dural tail sign (50%), while CT showed petrous bone and internal auditory canal intact. All cases received tumor excision, with 6 (75%) cases undergoing gross total resection, and the remaining (25%) getting partial excision. Pathological examination confirmed 5 (62.5%) classic, 2 (25%) desmoplastic, and 1 (12.5%) anaplastic. Further molecular analysis identified 5 (62.5%) WNT and 3 (37.5%) SHH. Immediately after the primary surgery, 7 (87.5%) cases gained improvement of the symptoms and signs and 1 (12.5%) kept the preoperative status stable. Follow up was available ranged from 5 to 34 months, during that period 5 cases kept symptom free and 3 cases recurred/progressed. In conclusion, CPA medulloblastoma is rare and lacking of special clinical manifestations and radiological features, and should be considered in the differential diagnosis of CPA lesions. In this series the most frequent pathological and molecular type is classic and WNT. Microsurgery excision is effective to prevent progressive decline of neurological status.

Reappraisal of cerebellopontine angle medulloblastomas: Report of a fatal case and lessons learned

CPA Medulloblastomas are rare in adults. We describe a case of medulloblastoma in the right CPA in a middle aged male patient who presented with short history and was preoperatively diagnosed as vestibular schwannoma. Intra-operatively it turned out to be a highly vascular and malignant lesion. Histopathology demonstrated medulloblastoma with high ki67 index. Patient was re-explored for hematoma evacuation and remained vegetative postoperatively. This is a unique case considering the older age at presentation, short duration of symptoms with atypical presentation of only 7th, 8th and lower cranial nerve involvement. MRI showed a lobulated heterogeneous extra axial right CPA mass lesion extending in the right acoustic meatus and causing mass effect and effacement of the fourth ventricle with heterogeneous enhancement post-gadolinium, suggestive of vestibular schwannoma. The aggressive behaviour of this tumor with short history has to prompt one to keep less common lesions like medulloblastomas as differential diagnosis in the CPA region. Though medulloblastomas are rare lesions in the CPA they have to be considered in the differential diagnosis based on atypical clinical behaviour, a suspicion of such diagnosis preoperatively can alter the treatment strategy, focusing on tumor embolization, safe surgical excision and adjuvant chemoradiation, aiming towards a better quality of life, in the post-operative period.

Medulloblastoma of the Cerebellopontine Angle in a Child: A Case Report and Review of the Literature

Cerebellopontine angle medulloblastoma is a very uncommon presentation of medulloblastoma, especially in children. We report such a case in a 14-year-old boy. Clinical onset by acute hearing loss and MRI features were, furthermore, unusual findings. The tumour had an homogeneous signal on T1 and FLAIR sequences a high-signal intensity on diffusion-weighted images with slightly restricted ADC value (0.85 × 10³ mm²/s) and enhanced slightly and homogeneously after contrast. A subtotal removal of the tumour, followed by entire neuroaxis irradiation and chemotherapy were achieved. MRI data of the literature reported cases were reviewed. Features on conventional MRI sequences do not allow differentiating CPA medulloblastoma from other rare tumour of this location. We point out that ADC value and Spectroscopy might be useful differentiating CPA tumours, especially from ependymoma in children.

Extra axial adult cerebellopontine angle medulloblastoma: An extremely rare site of tumor with metastasis

Dear Sir, Medulloblastomas are rarely seen in the adult population, accounting to less than 1% of primary adult brain tumors.[6] It commonly arises from the cerebellar vermis.[5,6] There are only a few cases of cerebellopontine (CP) angle medulloblastomas. Most of them are intra-axial. Extra-axial site of this tumor is extremely rare.[2] Ours is the first case of metastasis from CP angle medulloblastoma. A 22-year-old male patient presented with headache, vomiting, ataxia, and left facial weakness of one-month duration. Vision was normal. Fundus examination showed papilloedema. He had left lower motor neuron facial paresis, left IX and X cranial nerve paresis, and left cerebellar signs. Computed tomography scan of the brain showed a left CP angle mixed-density nonenhancing lesion (5.4 × 2.8 cm) with broad-based tentorial attachment and displacement of the fourth ventricle causing obstructive hydrocephalus [Figure 1]. Findings were confirmed by magnetic resonance imaging (MRI) [Figure 2]. He had previously undergone right ventriculo peritoneal shunt for obstructive hydrocephalus elsewhere. He underwent left retromastoid craniectomy and gross total excision of the lesion. There was clear plane between the tumor and cerebellum, whereas it was adherent to tent laterally. Histopathology showed a highly cellular tumor composed of rosettes of small round cells, with high nucleus-cytoplasm ratio and increased mitotic figures—suggestive of classical medulloblastoma—WHO grade IV [Figure 3]. Postoperatively, he improved. He was advised craniospinal radiotherapy which he failed to receive and presented 15 months later with progressive quadriparesis and sensory impairment of five-month duration with bladder and bowel involvement. On examination, he had 3/5 motor power in the upper limbs and 2/5 in lower limbs and sensory impairment below D4 dermatome. All deep tendon reflexes were brisk with bilateral extensor plantar reflexes. All superficial reflexes were absent. MRI of the brain and spine showed recurrent left CP angle medulloblastoma and intramedullary lesion at cervical level and intradural extramedullary lesion at sacral level, suggestive of drop metastasis in the spine from CP angle medulloblastoma [Figure 4]. Figure 1 Contrast Computed tomography scan of the brain axial and coronal reconstruction showed a left CP angle mixed-density nonenhancing lesion with broad-based tentorial attachment Figure 2 MRI of the brain axial T1, T2 and coronal sections showed a left CP angle lesion with broad-based tentorial attachment Figure 3 Microphotograph of the histopathology slide. (H and E, ×20). showed a highly cellular tumor composed of rosettes of small round cells, with high nucleus-cytoplasm ratio —suggestive of medulloblastoma Figure 4 Saggital MRI of the cervical and sacral region showing drop metastasis (shown by arrows) Medulloblastoma usually occur in inferior medullary velum in the midline.[6] However, rarely they may occur laterally in the cerebellar hemisphere in adults.[3] Origin of medulloblastoma may be either from germinal cells or their remnants situated at the end of posterior medullary velum or from remnants of the external granular layer.[5,7] Their development in the CPA may be from the remnants of the external granular layer in the cerebellar hemisphere, including the flocculus which faces the CP angle.[5] In CP angle medulloblastomas, though fifth, sixth, and eighth cranial nerves are frequently involved,[4] these nerves were spared in this patient. CP angle medulloblastomas are rare with nearly 35 cases published in the literature,[2,5] of which only 9 are in adults.[2,5] The lack of association with any cerebellar tissue and extra-axial location in the region of CP angle is an extremely rare phenomenon.[2] Medulloblastomas are known to metastasize through CSF to the spinal canal, leptomeninges, and supratentorial regions. Metastasis in medulloblastomas vary between 38 and 60% in various series,[1,6] with the spinal canal being the commonest site with approximately 58%.[6] Kumar et al. have reported a case of vermian medulloblastoma with metastasis to the CP angle.[5] To the best of authors’ knowledge, spinal metastasis from CP angle medulloblastoma has not been reported till date. Extra-axial site of this tumor is extremely rare but must be considered in the differential diagnosis of extra-axial CP angle lesions. Any neurological deterioration seen in follow-up patient must be evaluated for metastasis.

Cerebellopointine angle medulloblastoma.

Cerebellopontine angle (CPA) medulloblastoma is a rare tumour. To date, only 19 cases have been reported over a period of 8 years, we have treated 14 cases of CPA medulloblastomas. This is first and the largest series of CPA medulloblastomas reported in the world literature.