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Medulloblastoma

Brain Tumor Medulloblastoma

Medulloblastoma is a poorly differentiated malignant brain tumor that originates in the cerebellum or posterior fossa. It occurs most frequently in children. Patients with medulloblastoma most commonly have symptoms related to increased intracranial pressure.


Presentation

The common symptoms of this disease include signs of enhanced pressure on the brain, like nausea, headache, drowsiness, vomiting, coordination problems, behavioral changes and changes in the appetite. Headache usually will be present in the morning and will improve as the day passes on. Headaches will become aggressive and persistent, if the tumor is not treated in time [7].

Fatigue
  • Toxicities related to drug administration included acute bradycardia with somnolence, headache, fatigue, and CSF pleocytosis consistent with chemical meningitis and dystonic reaction.[ncbi.nlm.nih.gov]
  • Individuals with a medulloblastoma may present with: • Headaches which are often worse in the morning and get better during the day; • Nausea or vomiting in the morning; • Problems with motor skills, such as clumsiness or poor handwriting; • Tiredness/Fatigue[secure.ssa.gov]
  • Early stages of medulloblastoma show signs of nonspecific headache, slight imbalance, change in the personality and fatigue. Many studies were conducted to understand the biology of medulloblastoma.[symptoma.com]
  • Some of the general symptoms of medulloblastoma include: Balance problems or clumsiness Changes in thinking ability Dizziness Double vision or other eye problems Tiredness and lack of energy (fatigue) Headaches Nausea and vomiting Hearing loss Irritability[cedars-sinai.org]
  • […] include the following: Headaches Nausea Vomiting Clumsiness or similar balance problems Vision problems Symptoms will depend on the exact location of the tumor and which nerves are affected, but most patients experience headaches, nausea, vomiting, and fatigue[neurosurgery.ucsf.edu]
Nausea
  • Medulloblastoma occurs in the 4th ventricle and usually presents with symptoms of increased intracranial pressure (headaches, nausea, vomiting) and signs of obstructive hydrocephalus.[ncbi.nlm.nih.gov]
  • We present an unusual medulloblastoma in a 3.9-year-old boy who had a 2-week history of nausea and vertigo. MRI revealed a 5 5.5 5 cm sized tumor located in the fourth ventricle and spinal leptomeningeal dissemination.[ncbi.nlm.nih.gov]
  • A 12-year-old girl presented with dizziness and nausea. Brain magnetic resonance (MR) images revealed an approximately 4.2-cm enhanced mass on the cerebellar vermis associated with mild perilesional edema and increased cerebral blood volume.[ncbi.nlm.nih.gov]
  • He presented with sudden-onset nausea and vomiting due to intratumoral hemorrhage. The medulloblastoma was thought to be in an early developmental stage because the tumor volume was extremely small.[ncbi.nlm.nih.gov]
  • METHODS: Eligibility criteria for this biologic study included age 4-21 years, patients with CM undergoing craniectomy (as non-malignant surgical controls) and receiving dexamethasone for prevention of post-operative nausea, and those with newly diagnosed[ncbi.nlm.nih.gov]
Diplopia
  • Headaches which are often worse in the morning and get better during the day; • Nausea or vomiting in the morning; • Problems with motor skills, such as clumsiness or poor handwriting; • Tiredness/Fatigue; • Tilting the head to one side; • Double vision (diplopia[secure.ssa.gov]
  • Soon after, the child will develop a stumbling gait, truncal ataxia, frequent falls, diplopia, papilledema, and sixth cranial nerve palsy.[en.wikipedia.org]
  • As an example, elevated intracranial pressure can induce dysfunction of the abducens nerves, causing diplopia especially with lateral gaze.[pedclerk.bsd.uchicago.edu]
  • Such symptoms may include double vision (diplopia), rapid, jerky movements of the eyes (nystagmus), facial weakness, ringing in the ears (tinnitus), hearing loss and a stiff neck.[rarediseases.org]
Papilledema
  • A 3-year-old boy presented with macrocephaly, visual field constriction, and papilledema. Neuroimagings showed enlargement of the ventricles without any mass lesions.[ncbi.nlm.nih.gov]
  • […] neuroectodermal tumor Neurology A malignant brain tumor that comprises 1 4 of intracranial tumors in children and adolescents; it is most common at the midline of the cerebellum, and on the roof of 4 th ventricle Clinical Presents with N&V, headache, ataxia, papilledema[medical-dictionary.thefreedictionary.com]
  • Papilledema can cause reduced clarity of vision.[rarediseases.org]
  • Soon after, the child will develop a stumbling gait, truncal ataxia, frequent falls, diplopia, papilledema, and sixth cranial nerve palsy.[en.wikipedia.org]
Morning Headache
  • Symptoms that parents might notice include: nighttime or early-morning headaches nausea vomiting trouble walking dizziness double vision clumsiness What Causes Medulloblastoma? Doctors don't know what causes medulloblastoma.[kidshealth.org]
  • As a result, these tumors often result in a condition called hydrocephalus, in which the flow of CSF is disrupted and pressure inside the skull increases, resulting in symptoms, including: Irritability Nausea and vomiting Morning headache or headache[columbianeurosurgery.org]
  • The child typically becomes listless, with repeated episodes of vomiting, and a morning headache, which may lead to a misdiagnosis of gastrointestinal disease or migraine.[en.wikipedia.org]
  • headaches, nausea, vomiting, and altered mental status prolonged elevation of intracranial pressure which can lead to papilledema and complete or partial loss of vision. infants may present with irritability, anorexia, and developmental delay. cerebellar[pedclerk.bsd.uchicago.edu]
Altered Mental Status
  • Common presenting symptoms include headache, seizures, and altered mental status. Whereas magnetic resonance imaging helps define the anatomic extent of tumor, biopsy is often required to confirm the diagnosis.[ncbi.nlm.nih.gov]
  • mental status prolonged elevation of intracranial pressure which can lead to papilledema and complete or partial loss of vision. infants may present with irritability, anorexia, and developmental delay. cerebellar dysfunction tumors in the midline may[pedclerk.bsd.uchicago.edu]
  • Clinical Features • Symptoms of increased intracranial pressure • Nocturnal or morning headaches • Nausea • Vomiting • Altered mental status • Tumors in the midline • Gait ataxia • Truncal instability • Tumors in the lateral cerebellar hemispheres • Limb[slideshare.net]
Vertigo
  • After 19 months of treatment with temozolomide (TMZ) and radiotherapy, he presented with vertigo and headache. A homogeneously enhanced mass had developed in the left cerebellar hemisphere.[ncbi.nlm.nih.gov]
  • We present an unusual medulloblastoma in a 3.9-year-old boy who had a 2-week history of nausea and vertigo. MRI revealed a 5 5.5 5 cm sized tumor located in the fourth ventricle and spinal leptomeningeal dissemination.[ncbi.nlm.nih.gov]
  • Additional features that may be observed include lethargy, motor or cranial nerve impairment, gaze palsy, visual impairment due to hydrocephalia, vertigo/hearing loss, behavioral changes/irritability, and extracranial pain (e.g. back pain in those with[orpha.net]
Cerebellar Sign
  • Clinical Presentation Because of its posterior fossa location, medulloblastoma often produces hydrocephalus and symptoms of increased ICP, as well as cerebellar signs (eg, truncal and limb ataxia, nystagmus).[ncbi.nlm.nih.gov]

Workup

The laboratory evaluation of medulloblastoma is done by complete blood count, electrolytes presence, and renal and liver function tests. Viral titers and baseline thyroid function studies are also suggested. Computerized tomography scans have a 95 percent sensitivity for detecting a brain tumor. The anatomic origin and the extent of the tumor are better exposed by magnetic resonance imaging (MRI) technique with gadolinium for the spine and head. This technique can also be performed to detect any residual tumor after the surgery. Lumbar puncture, bone marrow aspiration and biopsy are necessary to identify the late stages of the disease. Tumor subtypes may be detected by histological findings [8].

Treatment

Treatment for this disease involves surgical removal of the tumor followed by radiation and chemotherapy. The extent of response to the treatment by the patient depends on the age of the patient at the time of diagnosis, tumor size and extent, tumor mass that can be safely removed and the metastatic disease level [9]. Postoperative magnetic resonance imaging is performed within 48 hours. This technique has to be repeated 2 weeks after surgery. It is advised to wait for 10 to 14 days after surgery to start with radiation therapy for sufficient wound healing. Clinicians perceive that they can depend on radiation more than chemotherapy in adults, although acute side effects are seen in adults than in children [10].

Prognosis

The prognosis of this disease reported by the ‘Central Brain Tumor Registry of the United States’ was that nearly 50 to 60 percent of the adults with medulloblastoma were found alive five years after the disease was diagnosed and 44 percent were found alive 10 years after diagnosis. These figures have no relation with the differences of outcomes between the low risk and high risk groups, differences in patient characteristics and their response to the treatment. Current treatment strategies can be adopted to save 70 to 80 percent of children from the risk of medulloblastoma and see them alive after 5 years from diagnosis. Children with high-risk of this disease can be given effective therapy and the disease can be controlled in 60 to 65 percent of the patients [5] [6].

Etiology

The etiology of this disease is not yet identified. Medulloblastoma has however been associated with certain conditions like neurofibromatosis, Turcot syndrome, Gorlin syndrome (nevoid basal cell carcinoma syndrome) and blue rubber-bleb nevus syndrome [2].

Epidemiology

Medulloblastoma accounts for 17.2 percent of brain tumors in children and 0.7 percent of all malignant tumors in adults. Incidence of this disease is approximately 2 cases per 100,000 people, and nearly 350 cases are reported in the United States every year. The incidence of this tumor decreases with increase in age and it peaks at the age of 3 to 5 years. It is estimated that 29 percent of cases occur in people aged 20+ and the tumor rarely occurs in people aged 50+. It is found that 80 percent are diagnosed in the first 15 years of life. The data from surveillance, epidemiology and end results program has revealed that children aged below 14 years in the United States have an incidence rate of 5.7 whites and 5 blacks in a million population [3].

Sex distribution
Age distribution

Pathophysiology

The medulloblastoma is molecularly different from embryonic tumors. It originates from primitive cells of the cerebellum. There are genetic abnormalities observed in medulloblastoma, like loss of 17p and duplication of 17q. It is also thought that abnormality in p53 gene, which is a part of chromosome 17, might lead to the development of medulloblastoma, as p53 is a tumor suppressor. Some cases have shown a better outcome due to increased expression of neurotrophin-3 receptor. Increased expression of beta catenin gene was also associated with better survival. Similarly, increased expression of ERBB2, PDGFRA, MYCC, MYCN genes was found to be associated with poor survival and greater likelihood of metastasis.

Based on the histology, medulloblastoma may be classified in five different types, the desmoplastic nodular medulloblastoma, undifferentiated medulloblastoma, anaplastic medulloblastoma, medulloblastoma with glial differentiation and medulloblastoma with neuroblastic differentiation [4].

Prevention

There are no pronounced preventive measures for medulloblastoma as the risk factors are mainly genetic diseases, like neurofibromatosis, Turcot syndrome, blue rubber-bleb nevus syndrome and Gorlin syndrome (nevoid basal cell carcinoma syndrome).

Summary

Medulloblastoma is a malignant brain tumor initiated in the granular cells of cerebellum of children and has a tendency to spread over the entire central nervous system. This disease is currently classified as a primitive neuroectodermal tumor (PNET). The actual cause of medulloblastoma is not known. Early stages of medulloblastoma show signs of nonspecific headache, slight imbalance, change in the personality and fatigue. Many studies were conducted to understand the biology of medulloblastoma. Genes and chromosomes were mutated to understand their role in the disease development. There are a few rare genetic syndromes, which are thought to be increasing the risk of tumor development. As the disease stage progresses, enhanced intracranial pressure leads to severe headache [1]. 

Patient Information

  • Definition: Medulloblastoma is a tumor which develops in the cerebellum of the brain. It occurs mostly in children and young adults. This disease accounts for less than 2 percent of all the brain tumors and 18 percent of all the brain tumors in children. 
  • Cause: No exact cause of medulloblastoma was identified. Mutations were made in the genes and chromosomes to know the role of these genes in tumor development. A few genetic syndromes were found to be associated with the risk of development of the tumor. 
  • Symptoms: The usual symptoms of medulloblastoma include nausea and vomiting, headache, vision changes, poor balance, sleepiness, loss of coordination, confusion, slurred speech and dizziness.
  • Diagnosis: Diagnosis is done first at physical level. Computerized tomography scan and magnetic resonance imaging are the techniques used to study the brain tumor. Later, histological examination is done to confirm the diagnosis of the tumor.
  • Treatment: Treatment for medulloblastoma includes surgical removal of the tumor tissue to the maximum extent. The residual tumor is removed with radiation therapy followed by chemotherapy.

References

Article

  1. Tarbell NJ, Loeffler JS, Silver B, et al. The change in patterns of relapse in medulloblastoma. Cancer 1991; 68:1600.
  2. Narayana A, Jeswani S, Paulino AC. The cranial-spinal junction in medulloblastoma: does it matter? Int J Radiat Oncol Biol Phys 1999; 44:81.
  3. Dufour C, Beaugrand A, Pizer B, Micheli J, Aubelle MS, Fourcade A, et al. Metastatic Medulloblastoma in Childhood: Chang's Classification Revisited. Int J Surg Oncol. 2012;2012:245385. 
  4. Raimondi AJ, Tomita T. Medulloblastoma in childhood. Acta Neurochir (Wien) 1979; 50:127.
  5. Albright AL, Wisoff JH, Zeltzer PM, Boyett JM, Rorke LB, Stanley P. Effects of medulloblastoma resections on outcome in children: a report from the Children's Cancer Group. Neurosurgery. Feb 1996;38(2):265-71.
  6. Chan AW, Tarbell NJ, Black PM, et al. Adult medulloblastoma: prognostic factors and patterns of relapse. Neurosurgery 2000; 47:623.
  7. Park TS, Hoffman HJ, Hendrick EB, et al. Medulloblastoma: clinical presentation and management. Experience at the hospital for sick children, toronto, 1950-1980. J Neurosurg 1983; 58:543.
  8. Perreault S, Lober RM, Carret AS, et al. Surveillance imaging in children with malignant CNS tumors: low yield of spine MRI. J Neurooncol 2014; 116:617.
  9. Huang E, Teh BS, Strother DR, et al. Intensity-modulated radiation therapy for pediatric medulloblastoma: early report on the reduction of ototoxicity. Int J Radiat Oncol Biol Phys 2002; 52:599.
  10. Rutkowski S, Bode U, Deinlein F, et al. Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 2005; 352:978.

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Last updated: 2019-07-11 21:54