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Adult Hemispheric Cerebellar Medulloblastoma Felipe De Oliveira, José Alberto Landeiro, Igor De Castro1

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Adult Hemispheric Cerebellar Medulloblastoma Felipe De Oliveira, José Alberto Landeiro, Igor De Castro1 OPEN ACCESS Editor: Dennis Malkasian, M.D., SNI: Neuroanatomy and Neurophysiology For entire Editorial Board visit : University of California at http://www.surgicalneurologyint.com Los Angeles, Los Angeles, CA, USA Case Report Adult hemispheric cerebellar medulloblastoma Felipe de Oliveira, José Alberto Landeiro, Igor de Castro1 Department of Neurosurgery, Antônio Pedro Hospital Federal Fluminense University, Rio de Janeiro, Brazil, 1Hot Springs Neurosurgical Clinic P.A, Hot Springs, Arkansas, USA E‑mail: *Felipe de Oliveira ‑ [email protected]; José Alberto Landeiro ‑ [email protected]; Igor de Castro ‑ [email protected] *Corresponding author Received: 26 September 17 Accepted: 05 December 17 Published: 14 February 18 Abstract Background: Medulloblastoma is an embryonal neoplasm and accounts for 1% of all adult intracranial tumors. It is associated with many familiar cancer syndromes, but there is no known cause for medulloblastoma. Many studies have documented differences between childhood and adult medulloblastomas in terms of location, proliferation, and apoptotic indices. There are four histological groups – classic and the variant forms (desmoplastic/nodular, anaplasic, and large cell). There are four major subgroups according to molecular configuration: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4 with differences between them according to prognostic outcomes. Case Description: We present the case of a 19‑year‑old female who complained of headache and vomiting. On neurological exam, she was awake, conscious, and had mild truncal ataxia, dysmetria, and intentional tremor. Brain magnetic resonance imaging (MRI) showed an intra‑axial left hemisphere cerebellar lesion causing midline shift tonsilar herniation. She was submitted for posterior fossa craniotomy and microsurgical resection of cerebellar tumor and then to 18 Gy Access this article online adjuvant radiotherapy to the tumor bed and 23 Gy to the neuroaxis. Website: Conclusion: This article briefly discusses the newest points in classification, www.surgicalneurologyint.com diagnosis, and treatment of medulloblastoma. This case illustrates the diagnostic DOI: 10.4103/sni.sni_341_17 workup and treatment of a rare tumor in adults showing the importance of Quick Response Code: molecular and histological studies for the treatment and counseling of the patient. Medulloblastoma has different prognosis depending on the histological and molecular feature. Accessing these different features is essential to better plan the treatment as well as inform the patient regarding the disease and its prognosis. Key Words: Brain tumor, medulloblastoma, molucular profile INTRODUCTION This is an open access article distributed under the terms of the Creative Commons Medulloblastoma (MB) is an embryonal tumor. Most Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, cases in this age group (adults) occur between the third tweak, and build upon the work non-commercially, as long as the author is credited and and the fourth decade with a higher incidence in males. the new creations are licensed under the identical terms. MB is associated with many familiar cancer syndromes, For reprints contact: [email protected] but there is no known cause for MB. Prenatal exposure How to cite this article: de Oliveira F, Landeiro JA, de Castro I. Adult hemispheric to dietary N‑nitroso compounds increases the risk. John cerebellar medulloblastoma. Surg Neurol Int 2018;9:34. Cunningham polyomavirus (JC Virus) T‑antigen was http://surgicalneurologyint.com/Adult-hemispheric-cerebellar-medulloblastoma/ © 2018 Surgical Neurology International | Published by Wolters Kluwer - Medknow Surgical Neurology International 2018, 9:34 http://www.surgicalneurologyint.com/content/9/1/34 identified in MB cells, suggesting a role of this viral evoked potentials) and motor evoked potentials. There infection in tumor physiopathology. was no change during the surgery. In the pediatric group, MB is the most common A linear incision was made over the left side between the malignant brain tumor, but accounts for less than 1% mastoid and inion. Skin and muscle were dissected all of all adult intracranial tumors,[11,5] with an annual the way to the bone. A burr hole was perforated at the incidence of approximately only 0.5 per million transverse sinus and another one lower down. The dura individuals. Many studies have documented the mater was easily dissected. Using craniotome, the two differences between childhood and adult MBs in burr holes were connected and the bone flap was elevated. terms of location, histological subtypes, as well as Dura was opened and elevated cranially, exposing the the proliferation and apoptotic indices. There are cerebellum. Using a superior approach to the cerebellum, four histological groups – classic and the variant the lesion was observed and resection was initiated forms (desmoplastic/nodular, anaplasic, and large cell), with cavitron ultrasonic surgical aspirator (CUSA). The with desmoplastic/nodular being the most frequent tumor was completely removed in a piece‑meal fashion. variant. Transcriptional profiling studies have shown Hemostasis was made using bipolar coagulation and that MB is not a single disease. There are four major injection of thrombin on the tumor bed. Dura mater subgroups according to molecular configuration: was closed and a 2 × 2 piece of DuraGen® was placed wingless (WNT), sonic hedgehog (SHH), group 3, and over the dura. The bone flap was reapproximated and group 4. These subgroups carry different prognostic the fascia, muscle, and skin were closed. The patient outcomes and should be noted when planning the was extubated and transferred to the intensive care treatment strategy. The studies related to tumor unit (ICU) in a stable condition. biological and clinical features in adults are limited due to the low incidence of the disease in this age group. One month after the surgery, the patient was subjected to This article aims to briefly review the available literature 18 Gy adjuvant radiotherapy to the tumor bed and 23 Gy and report a case of MB in a 19‑year‑old woman. to the neuroaxis. No chemotherapy was administered. Pathology and molecular biology CASE REPORT Histopathologic examination showed highly cellular neoplasm composed of large cells with hyperchromatic We present the case of a 19‑year‑old female who nuclei, scant cytoplasm, nuclear molding, and moderate complained of headache and vomiting. On neurological pleomorphism, consistent with undifferentiated examination, she was awake, conscious, and had mild medulloblastoma [Figure 3]. truncal ataxia, dysmetria, and intentional tremor. On immunohistochemistry, the tumor was SHH subtype. Brain magnetic resonance imaging (MRI) showed an No MYCN amplification or TP53 mutation were noted intra‑axial left hemisphere cerebellar lesion. One small during molecular examinations. area was visible within the mass. Severe vasogenic edema was noticed at the cerebellum causing mass effect, midline shift, and mild tonsilar herniation [Figure 1]. DISCUSSION The patient underwent a posterior fossa craniotomy and Tumor biology microsurgical resection of cerebellar tumor [Figure 2]. MB is a friable mass that arises most frequently in the Patient was in the sitting position during surgery, and cerebellar vermis, usually from the inferior medullary neuromonitoring was performed during the procedure. velum, but its presence in cerebellar hemispheres is not Neuromonitoring consisted of SSEPs (somatosensory rare, especially in adults. Aqueduct of Sylvius and cisterna Figure 1: Axial contrast-enhanced T1 image showing the tumor’s dimensions (right). Sagital contrast-enhanced T1 image showing Figure 2: Post- operative MRI showing complete ressection of the the relation between the mass and the cerebellar tentorium (left) tumor Surgical Neurology International 2018, 9:34 http://www.surgicalneurologyint.com/content/9/1/34 Figure 3: Micropcopy showing highly cellular neoplasm composed of large cells with hyperchromatic nuclei, scant cytoplasm, nuclear molding and moderate pleomorphism, consistent with undifferentiated medulloblastoma magna may be involved as the tumor grows. MB has a high propensity to metastasize commonly to the subarachnoid Figure 4: Scheme represents sinalization cascade for genesis of space along the brain and spinal cord. Metastatic spread MB. Mutations in the genes that code for SHH signaling pathway [4] receptors or downstream inhibitors may lead to hyperstimulation has higher incidence in children than adults. Outside of the pathway, resulting in accumulation of oncogenic transcription central nervous system, metastases are rare, commonly factor GLI 1/2, activation of N-Myc and cellular deregulated involving the bone marrow, lungs, and liver. Due to the proliferation. Mutations in genes that codify b-Catenin, results undifferentiated appearance of MB, there is speculation in accumulation of cytosolic b-catenin, activation of C-Myc and oncogenesis that the tumor arises from multipotent stem cells in the subependymal–subventricular region. tumors are located laterally in the cerebellar hemispheres, There are four histological groups – classic and the variant suggesting an alternative pathogenetic pathway with a forms (desmoplastic/nodular, anaplasic, and large cell). different cell of origin.[12,13] Among MB variants, the most common is desmoplastic, During embryonic growth, the cerebellum develops at the especially in adults. It carries better
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