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Fully Endoscopic Retrosigmoid Approach for Cerebellopontine Angle Tumors

Saied M, Najibullah M, Shabbir Z, Saleem A, Ali A, Azab WA

Adv Tech Stand Neurosurg, 2024;52:229-244.
PMID: 39017797 DOI: 10.1007/978-3-031-61925-0_16

BACKGROUND: Fully endoscopic or endoscope-controlled approaches are essentially keyhole approaches in which rigid endoscopes are the sole visualization tools used during the whole procedure. At the early attempts of endoscope-assisted cranial surgery, it was noted that rigid endoscopes enabled overcoming the problem of suboptimal visualization when small exposures are used. The technical specifications and design of the currently available rigid endoscopes are associated with a group of unique features that define the endoscopic view and lay the basis for its superiority over the microscopic view during brain surgery. Fully endoscopic retrosigmoid approach for cerebellopontine angle tumors is a minimally invasive approach that is not routinely practiced by neurosurgeons, with few series published so far. Unfamiliarity with the technique, steep learning curve, and concerns about inadequate exposure, neurovascular injury, and decreased visibility may explain this fact. In this chapter we elaborate on the surgical technique and nuances of the fully endoscopic retrosigmoid approach and present an overview of the published series.
METHODS: From a prospective database of endoscopic procedures maintained by the senior author, clinical data, imaging studies, operative charts, and videos of cases undergoing fully endoscopic retrosigmoid approach for cerebellopontine angle tumors were retrieved and analyzed. The pertinent literature was also reviewed.
RESULTS: The surgical technique of the fully endoscopic retrosigmoid approach was formulated.
CONCLUSION: The endoscopic technique has many advantages over the conventional procedures. In our hands, the technique has proven to be feasible, efficient, and minimally invasive with excellent results.

Matched MeSH terms: Cerebellar Neoplasms/pathology; Cerebellar Neoplasms/surgery
Cerebellar intra-axial dermoid cyst: a case of unusual location

Pant I, Joshi SC

Childs Nerv Syst, 2008 Jan;24(1):157-9.
PMID: 17657495

Intra-axial dermoid cysts are rare intracranial space occupying lesions, more so in the pediatric age group. Dermoid cysts account for about 0.2 to 1.8% of all intracranial tumors and are commonly located in the cisternal spaces, mainly in the cerebellopontine angle and parasellar cisterns. A purely intra-axial position as reported in this paper is quite exceptional.

Matched MeSH terms: Cerebellar Neoplasms/diagnosis*; Cerebellar Neoplasms/surgery
Fulltext Cerebellopontine angle medulloblastoma

Nyanaveelan M, Azmi A, Saffari M, Banu SK, Suryati MY, Jeyaledchumy M

Med J Malaysia, 2007 Jun;62(2):173-4.
PMID: 18705459 MyJurnal

An extremely rare case of a right cerebellopontine angle medulloblastoma in a five year old Malay girl which had eroded into the petrous bone and extended into the temporal fossa is reported. Combined subtemporal and retromastoid approach to achieve gross total surgical resection was achieved followed by radiotherapy and chemotherapy.

Matched MeSH terms: Cerebellar Neoplasms/diagnosis; Cerebellar Neoplasms/pathology; Cerebellar Neoplasms/therapy*
Fulltext Molecular subtyping of paediatric medulloblastoma by immunohistochemistry

Yin, Ping W., L., C-Khai, Hamidah, A., Nur Maya Sabrina, T., Muaatamarulain, M., Geok, Chin T.

Medicine & Health, 2020;15(2):124-139.
MyJurnal

Empat subkumpulan molekul teras medulloblastoma yang mempunyai profil transkripsi dan nilai prognostik yang berbeza diperkenalkan baru-baru ini. Kajian ini bertujuan untuk menentukan varian histologi dan subkumpulan molekul medulloblastoma melalui aplikasi imunohistokimia (YAP-1 dan beta catenin) sebagai penanda surrogate di populasi kami, di samping menghubungkan varian histologi dan subkumpulan molekul ini dengan parameter klinikopatologi. Kami telah melibatkan seramai tujuh belas pesakit medulloblastoma yang berusia empat bulan hingga 14.3 tahun dari tahun 2002 hingga 2017. Histologi klasik (76.5%) adalah histologi medulloblastoma yang paling umum, diikuti oleh varian sel besar/ anaplastik (LCA) (17.6%) dan desmoplastik/nodular (DN) (59%). Subkumpulan molekul yang paling kerap adalah tumor bukan SHH/WNT (64.7%), diikuti oleh tumor SHH (35.3%). Di antara tumor SHH, 66.7% adalah histologi klasik dan selebihnya 33.3% adalah varian LCA. Yang menariknya, satu kes yang menunjukkan histologi DN memaparkan imunonegativiti kepada YAP-1 dan beta catenin, dan tergolong kepada subkumpulan molekul bukan SHH/WNT. Majoriti (88.2%) medulloblastoma berada di lokasi pertengahan ventrikel keempat, termasuk varian DN. Dianggarkan tiga tahun kelangsungan hidup bebas penyakit (DFS) dan survival keseluruhan (OS) masing-masing adalah 60% dan 86.7%. Umur 5 cm, histologi LCA dan kumpulan berisiko tinggi adalah berkorelasi terbalik dengan DFS. Bayi

Matched MeSH terms: Cerebellar Neoplasms
Metastatic endodermal sinus tumor of cerebellum and brainstem

Sinniah D, Chee CP, Pathmanathan R, Nuruddin R

Med. Pediatr. Oncol., 1988;16(1):57-61.
PMID: 3340064
Matched MeSH terms: Cerebellar Neoplasms/therapy
Fulltext Acoustic neuroma with orofacial paresthesia: description of an atypical presentation

Lim CC, Misron K, Liew YT, Wong EHC

BMJ Case Rep, 2019 Nov 04;12(11).
PMID: 31690691 DOI: 10.1136/bcr-2019-232275

Acoustic neuroma (AN) usually manifests with asymmetric hearing loss, tinnitus, dizziness and sense of disequilibrium. About 10% of patients complain of atypical symptoms, which include facial numbness or pain and sudden onset of hearing loss. Patients with atypical symptoms also tend to have larger tumours due to the delay in investigation. We report a particularly interesting case of a patient presented to us with numbness over her right hemifacial region after a dental procedure without significant acoustic and vestibular symptoms. Physical examination and pure tone audiometry revealed no significant findings but further imaging revealed a cerebellopontine angle mass. The changing trends with easier access to further imaging indicate that the presentation of patients with AN are also changing. Atypical symptoms which are persistent should raise clinical suspicion of this pathology among clinicians.

Matched MeSH terms: Cerebellar Neoplasms/complications; Cerebellar Neoplasms/pathology*; Cerebellar Neoplasms/surgery
Recurrence of a nasopharyngeal carcinoma manifesting as a cerebellopontine angle mass

Kong MH, Jeevanan J, Jegan T

Ear Nose Throat J, 2013 Dec;92(12):E11-3.
PMID: 24366707

As many as 31% of patients with nasopharyngeal carcinoma present with intracranial extension. Despite this high percentage, extension to the cerebellopontine angle is rare. The mechanism of tumor spread to the cerebellopontine angle is not completely understood. The most likely mechanism is direct extension to the skull base with involvement of the petrous apex and further extension posteriorly via the medial tentorial edge. We report the case of a 46-year-old woman with nasopharyngeal carcinoma who had been treated initially with chemoradiation and subsequently with stereotactic radiosurgery for residual tumor. One year later, she presented with an intracranial recurrence of the nasopharyngeal carcinoma in the cerebellopontine angle; the recurrence mimicked a benign tumor on magnetic resonance imaging. The tumor was ultimately diagnosed as an undifferentiated carcinoma of nasopharyngeal origin. She was treated with palliative chemotherapy.

Matched MeSH terms: Cerebellar Neoplasms/drug therapy; Cerebellar Neoplasms/secondary*
Fulltext Correlation between MRI characteristics of medulloblastoma with histopathological subtypes and 2-year survival

Hussain IZ, Mohd Zaki F, Mukari SA, Md Pauzi SH, Loh CK, Alias H

Indian J Radiol Imaging, 2020 03 30;30(1):46-51.
PMID: 32476749 DOI: 10.4103/ijri.IJRI_209_19

Objectives: The objective of this study is to describe the imaging features of medulloblastoma (MB) and correlate the MR characteristics with the different histological subtype of MB with 2-year survival.
Materials and Methods: This is a retrospective descriptive study. A total of 29 patients diagnosed with MB from January 2005 to December 2015 were included in this study. The MRI brain and spine studies of these patients were retrieved and reviewed by a pediatric radiologist and a neuroradiologist independently, both blinded from the histological type of the MB. The HPE slides were also retrieved and reviewed by a pathologist.
Results: 80% of desmoplastic MB showed the presence of intracranial leptomeningeal seeding and 57.1% of anaplastic MB showed the presence of necrosis. The presence of intracranial leptomeningeal seeding (P = 0.002) and necrosis (P = 0.019) was predictive of the histological subtypes. There is a significant correlation between the enhancement pattern and the 2-year outcome (P = 0.03) with 6 out of 8 patients whose tumors showed minimal enhancement having disease progression within 2 years. A significant correlation was also seen between the presence of necrosis with a poorer outcome (P = 0.03) and between the HPE subtype and 2-year outcome (P = 0.03) with anaplastic MB having the poorest prognosis.
Conclusion: MR imaging features of intracranial leptomeningeal seeding and the presence of necrosis were correlated with a specific histologic subtype of MB. The enhancement pattern as well as necrosis correlated with 2-year poorer outcome of the disease.

Matched MeSH terms: Cerebellar Neoplasms
Spermatic cord metastasis from a medulloblastoma

Thambi dorai CR, Azmi A, Rahman AJ, Subathra S, Hayati AR, Zulfiqar A

Pediatr Surg Int, 2001 Nov;17(8):654-6.
PMID: 11727063

A 4-year-old boy presented with metastases in the spermatic cord and ribs 2 years after treatment for a primary medulloblastoma (MB). The testis was free of tumor. A MB presenting with a metastasis to the spermatic cord has not been reported to date. The role of a ventriculo-peritoneal shunt in promoting such spread should be considered in planning management.

Matched MeSH terms: Cerebellar Neoplasms/pathology*
Fulltext Brain metastasis of unknown origin

Chee CP

Singapore Med J, 1990 Feb;31(1):48-50.
PMID: 2333544

From 1973 to 1984, 119 patients presented to the Department of Neurosurgery, Royal Victoria Hospital, Belfast, with brain metastases, the primary sites of which remained unknown in 33 cases one month after discharge. About half of these cases were solitary and neurological lateralising signs were the commonest presentation. Of those cases in which surgery was performed, the majority remained improved one month after surgery. The one month mortality rate was only 3%. This study shows that with aggressive and appropriate treatment including surgical excision or decompression in solitary cases, an improved quality of life in the immediate postoperative period can be achieved in this particular group of brain metastases. A small number of patients remained alive and well after 6 months.

Matched MeSH terms: Cerebellar Neoplasms/secondary*
Fulltext Palliative care for a child: role of a primary care doctor

Tan CE, Md Radzniwan R, Khairani O, Ednin H

Malays Fam Physician, 2011;6(1):26-28.
PMID: 25606216 MyJurnal

A 7-year-old boy with a diagnosis of advanced medulloblastoma refractory to treatment was discharged from hospital for further palliative care at home. During this short and eventful period, the child developed spinal cord compression with progressive weakness of upper and lower limbs, neck pain, faecal and urinary incontinence. This case highlights the important roles of a primary care doctor in the provision of palliative care for a child in the community.

Matched MeSH terms: Cerebellar Neoplasms
Fulltext Medulloblastoblastoma with excessive nodularity: typical imaging appearance

Win Myint Tun, Norlisah Ramli, Mun, Kein Seong

Neurology Asia, 2017;22(2):173-176.
MyJurnal

Medulloblastoma is the most common form of childhood primary brain tumour arising from the
cerebellar vermis. It is classified as WHO grade IV embryonal tumours and currently at least four
histological variants have been established. Only few case reports been published on the imaging
features of the medulloblastoma with excessive nodularity variant. We report the MRI features of a rare
case of medulloblastoma with excessive nodularity in a child which is confirmed by histopathology.

Matched MeSH terms: Cerebellar Neoplasms
Hedgehog signalling molecule, SMO is a poor prognostic marker in bladder cancer

Mohd Ariffin K, Abd Ghani F, Hussin H, Md Said S, Yunus R, Veerakumarasivam A, et al.

Malays J Pathol, 2021 Apr;43(1):49-54.
PMID: 33903305

INTRODUCTION: Hedgehog (HH) pathway is an important signalling cascade for growth and patterning during embryonic development. Constitutive activation of Hedgehog pathway can be found in various types of malignancies including medulloblastoma, basal cell carcinoma, gastrointestinal, breast, pancreatic, prostate cancer and leukaemia. Little is known about the expression and role of Hedgehog signalling in bladder cancer.
MATERIALS AND METHODS: The purpose of this study was to investigate the immunohistochemical expression of SMO in 112 bladder cancer cases and determine their association with demographic and clinicopathological parameters. Bladder cancer tissues were obtained from the Hospital Kuala Lumpur.
RESULTS: SMO was expressed in the cytoplasm of all cases of bladder cancer. 6 cases (5.4%) showed low expression, while 106 cases (94.6%) showed high expression. Positive expression of SMO protein was correlated with a few variables which include grade and stage of tumour, lymph node metastasis and distant metastasis. SMO expression showed statistically significant association with higher grade (p=0.001) and higher stage (p=0.042) of bladder cancer. SMO expression also showed borderline association with lymph node metastasis (p=0.056).
CONCLUSION: These findings indicate that SMO expression may be a poor prognostic marker in bladder cancer.

Matched MeSH terms: Cerebellar Neoplasms
Fulltext Neuropsychological Consequences for Survivors of Childhood Brain Tumor in Malaysia

Alias H, Lau SCD, Schuitema I, de Sonneville LMJ

Front Psychol, 2018;9:703.
PMID: 29896137 DOI: 10.3389/fpsyg.2018.00703

Objective: This study aimed to evaluate neuropsychological consequences in survivors of childhood brain tumor. Method: A case-control study was conducted over a period of 4 months in a tertiary referral center in Kuala Lumpur, Malaysia. Fourteen survivors of childhood brain tumor aged 7-18 years, who were off-treatment for at least 1 year and were in remission, and 31 unrelated healthy controls were recruited. The median age at diagnosis was 8.20 years (range: 0.92-12.96 years). The diagnoses of brain tumors were medulloblastoma, germ cell tumor, pineocytoma, pilocystic astrocytoma, suprasellar germinoma, and ependymoma. Eleven survivors received central nervous system irradiation. Seven tasks were selected from the Amsterdam Neuropsychological Tasks program to evaluate alertness (processing speed), and major aspects of executive functioning, such as working memory capacity, inhibition, cognitive flexibility, and sustained attention. Speed, stability and accuracy of responses were the main outcome measures. Results: Survivors of childhood brain tumor showed statistically significant poorer performance on all tasks compared to healthy controls. Both processing speed and accuracy were impaired in the survivors, in particular under more complex task conditions. The survivors demonstrated deficits in alertness, sustained attention, working memory capacity, executive visuomotor control, and cognitive flexibility. Longer duration off treatment appeared to be correlated with poorer alertness, memory capacity, and inhibition. Conclusion: Survivors of childhood brain tumor in our center showed impaired neuropsychological functioning. Development of less toxic treatment protocols is important to prevent late effects of cognitive deficits in survivors of childhood brain tumor.

Matched MeSH terms: Cerebellar Neoplasms
Fulltext Patched-1 and smoothened, a hedgehog receptor and signal transducer are highly expressed in diffuse large B-cell lymphoma

Siti Nur Lina Azman, Huzlinda Hussin, Salmiah Md Said, Zanariah Alias, Maizaton Atmadini Abdullah

Malaysian Journal of Medicine and Health Sciences, 2017;13(2):1-6.
MyJurnal

Introduction: The Hedgehog (Hh) signalling pathway is a developmental signalling pathway involved in normal mammalian developmental and homeostasis of adult renewable tissues. In most adult tissues, this pathway remains silent and previous studies have shown that constitutive activation of Hedgehog signalling pathway leads to various types of malignancies including medulloblastomas, basal cell carcinoma, gastrointestinal, breast and prostate cancer. The purpose of this study was to investigate the immunohistochemical expression of Hedgehog pathway proteins in Diffuse Large B-cell Lymphoma and determine their association with overall survival (OS). Methods: Positive control using normal tonsils were included in each batch of immunohistochemical staining procedure. Results: PTCH1 proteins were highly expressed in DLBCL and showed strong staining intensity in 107 (100%) cases and SMO proteins were expressed in 105 (98.1%) cases. PTCH1 proteins were localised in the nucleus of tumour cells, whereas SMO proteins were mainly localised in the cytoplasm of tumour cells. Positive expression of PTCH1 and SMO proteins and overall survival of DLBCL patients were correlated with age, gender, race and tumour location. There was no significant correlation between the expression of these two proteins with any of the parameters. PTCH1 expression showed significant association with SMO expression (P=0.03). Conclusions: Our findings suggest that high expression of both PTCH1 and SMO may be important in the pathogenesis of DLBCL. However, additional mechanisms that may contribute to the activation of HH signalling in DLBCL needs to be further explored.

Matched MeSH terms: Cerebellar Neoplasms

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