Medulloblastoma: Difference between revisions

Jump to navigation Jump to search
No edit summary
No edit summary
 
(25 intermediate revisions by 8 users not shown)
Line 1: Line 1:
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


{{Infobox_Disease |
{{Medulloblastoma}}
  Name          = {{PAGENAME}} |
{{CMG}} {{AE}}{{HL}}{{sali}}
  Image          = CompT.jpg|
  Caption        = CT scan, showing a tumorous mass in the [[posterior fossa]], giving rise to obstructive [[hydrocephalus]], in a six year old girl.|
  DiseasesDB    = 31105 |
  ICD10          = |
  ICD9          = |
  ICDO          = 9470/3 |
  OMIM          = 155255 |
  MedlinePlus    = |
  MeshID        = D008527 |
}}
{{SI}}
{{CMG}}


{{Editor Help}}
{{SK}} Medulloblastomas; Medulloblastomi; Infratentorial PNET; Infratentorial primitive neuroectodermal tumor


==Overview==
==[[Medulloblastoma overview|Overview]]==
'''Medulloblastoma''' is a highly [[malignant]] primary [[brain tumor]] that originates in the [[cerebellum]] or [[posterior cranial fossa|posterior fossa]].


Originally considered to be a [[glioma]], medulloblastoma is now known to be of the family of cranial [[primitive neuroectodermal tumor|primitive neuroectodermal tumors]] (PNET).<ref name="emed-medullo">George Jallo, MD, [http://emedicine.com/neuro/topic624.htm Medulloblastoma], eMedicine 2007.</ref>
==[[Medulloblastoma historical perspective|Historical Perspective]]==


Tumors that originate in the cerebellum are referred to as [[infratentorial_neoplasms|infratentorial]] because they occur below the [[tentorium_cerebelli|tentorium]], a thick membrane that separates the [[cerebral hemispheres]] of the brain from the cerebellum.  Another term for medulloblastoma is infratentorial PNET.  Medulloblastoma is the most common PNET originating in the brain.<ref>Chris Hinz, Deneen Hesser, [http://hope.abta.org/mdl Focusing On Brain Tumors: Medulloblastoma], American Brain Tumor Association 2006, ISBN 0-944093-67-1.</ref>
==[[Medulloblastoma classification|Classification]]==


All PNET tumors of the brain are invasive and  rapidly growing tumors that, unlike most brain tumors, spread through the [[cerebrospinal fluid]] (CSF) and frequently metastasize to different locations in the brain and spine.
==[[Medulloblastoma pathophysiology|Pathophysiology]]==


==Incidence==
==[[Medulloblastoma causes|Causes]]==
[[Brain tumor|Brain tumors]] are the second most common malignancy among children less than 20 years of age.  Medulloblastoma is the most common malignant brain tumor, comprising 14.5% of newly diagnosed cases.<ref name="seer">James G. Gurney, Malcolm A. Smith, Greta R. Bunin, [http://seer.cancer.gov/publications/childhood/cns.pdf CNS and Miscellaneous Intracranial and Intraspinal Neoplasms], SEER Pediatric Monograph, National Cancer Institute</ref>  In adults, medulloblastoma is rare, comprising less than 2% of CNS malignancies.<ref>[http://cbtrus.org/2005-2006/tables/2006.table12.pdf Selected Primary Brain and Central Nervous System Tumor Age-Specific Incidence Rates], Central Brain Tumor Registry of the United States, 1998-2002.</ref>


The incidence of childhood medulloblastoma is higher in males (62%) than females (38%). <ref>[http://cbtrus.org/2005-2006/tables/2006.table13.pdf  Selected Childhood Primary Brain and Central Nervous System Tumor Incidence Rates by Major Histology Groupings, Histology and Gender] Central Brain Tumor Registry of the United States, 1998-2002.</ref>  Medulloblastoma and other PNET tumors are more prevalent in younger children than older children.  40% of medulloblastoma patients are diagnosed before the age of 5, 31% are between the ages of 5 and 9, 18.3% are between the ages of 10 and 14, and 12.7% are between the ages of 15 and 19. <ref>[http://cbtrus.org/2005-2006/tables/2006.table15.pdf Selected Childhood Primary Brain and Central Nervous System Tumor Age-Specific Incidence Rates], Central Brain Tumor Registry of the United States, 1998-2002.</ref>
==[[Medulloblastoma differential diagnosis|Differentiating Medulloblastoma from other Diseases]]==


==Pathogenesis==
==[[Medulloblastoma epidemiology and demographics|Epidemiology and Demographics]]==
Medulloblastomas usually form in the fourth ventricle, between the brainstem and the cerebellum.  Tumors with similar appearance and characteristics originate in other parts of the brain, but they are not identical to medulloblastoma. <ref>Roger Packer M.D, [http://virtualtrials.com/medullo.cfm Medulloblastoma] Clinical Trials and Noteworthy Treatments for Brain Tumors 2002.</ref>


Although it is thought that medulloblastomas originate from immature or embryonal cells at their earliest stage of development, the exact cell of origin, or "medulloblast" has yet to be identified.
==[[Medulloblastoma risk factors|Risk Factors]]==
 
It is currently thought that medulloblastoma arises from cerebellar "stem cells" that have been prevented from dividing and differentiating into their normal cell types. This accounts from the varying histologic variants seen on biopsy. '''Rosette''' formation is highly characteristic of medulloblastoma and is seen in up to half of the cases.


Molecular genetics reveal a loss of genetic information on the distal part of chromosome 17, distal to the ''[[p53]]'' gene, possibly accounting for the neoplastic transformation of the undifferentiated cerebellar cells. Medulloblastomas are also seen in [[Gorlin syndrome]] as well as [[Turcot syndrome]]. Another research has strongly implicated the [[JC virus]], the virus that causes [[progressive multifocal leukoencephalopathy|multifocal leukoencephalopathy]].
==[[Medulloblastoma screening|Screening]]==


==Clinical manifestation==
==[[Medulloblastoma natural history|Natural History, Complications and Prognosis]]==
Symptoms are mainly due to secondary increased [[intracranial pressure]] due to blockage of the [[fourth ventricle]] and are usually present for 1 to 5 months before diagnosis is made. 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. Soon, the child will develop a ''stumbling gait'', ''frequent falls'', ''[[diplopia]]'', ''[[papilledema]]'', and ''sixth cranial nerve palsy''. ''Positional dizziness'' and ''[[nystagmus]]'' are also frequent and facial sensory loss or motor weakness may be present. [[Decerebrate]] attacks appear late in the disease.


Extraneural metastases to the rest of the body is rare, but usually only after [[craniotomy]].


==Diagnosis==
==Diagnosis==
The tumor is distinctive on T1 and T2-weighted [[MRI]] with heterogeneous enhancement and typical location adjacent to and extension into the fourth ventricle.
[[Medulloblastoma staging|Staging]] | [[Medulloblastoma history and symptoms|History and Symptoms]] | [[Medulloblastoma physical examination|Physical Examination]] | [[Medulloblastoma laboratory tests|Laboratory Findings]]  | [[Medulloblastoma CT|CT]] | [[Medulloblastoma MRI|MRI]] | [[Medulloblastoma other imaging findings|Other Imaging Findings]] | [[Medulloblastoma other diagnostic studies|Other Diagnostic Studies]]


Histologically, the tumor is solid, pink-gray in color, and is well circumscribed. The tumor is very cellular, many mitoses, little cytoplasm, and has the tendency to form clusters and rosettes.
==Treatment==
[[Medulloblastoma medical therapy|Medical Therapy]] | [[Medulloblastoma surgery|Surgery]] | [[Medulloblastoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Medulloblastoma future or investigational therapies|Future or Investigational Therapies]]


Correct diagnosis of medulloblastoma may require ruling out [[ATRT|atypical teratoid rhabdoid tumor]] (ATRT)<ref>{{cite journal
==Case Studies==
| author = Burger PC
[[Meduloblastoma case study one|Case #1]]
| coauthors = Yu I, Tihan T, et al
| year = 1998
| title = Atypical teratoid rhabdoid tumors of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group Study.
| journal = Am J Surg Pathol 1998
| issue = 22:
| pages = 1083-92
| language = English
}}</ref> and [[primitive neuroectodermal tumor]] (PNET).


===MRI===
==Related chapters==


([http://www.radswiki.net Images courtesy of RadsWiki])
* [[Brain tumor]]


<gallery>
Image:Medulloblastoma-01.jpg|Medulloblastoma
Image:Medulloblastoma-02.jpg|Medulloblastoma
Image:Medulloblastoma-03.jpg|Medulloblastoma
Image:Medulloblastoma-04.jpg|Medulloblastoma
Image:Medulloblastoma-06.jpg|Medulloblastoma
Image:Medulloblastoma-07.jpg|Medulloblastoma
</gallery>


== Treatment and prognosis==
{{Nervous tissue tumors}}
Treatment begins with maximal resection of the tumor. The addition of ''radiation'' to the entire neuraxis and ''chemotherapy'' may increase the disease-free survival. This combination may permit a 5 year survival in more than 80% of cases. The presence of desmoplastic features such as connective tissue formation offers a better prognosis. Prognosis is worse if child is less than 3 years old, inadequate degree of resection, or if presence of any CSF, spinal, supratentorial or systemic spread.


Increase intracranial pressure may be controlled with [[corticosteroids]] or a ventriculoperitoneal [[shunt_(medical)|shunt]]
{{WikiDoc Help Menu}}
{{main|intracranial pressure}}
{{WikiDoc Sources}}
 
==References==
{{Reflist|2}}
 
==See also==
 
*List of notable brain tumor resources
*List of notable childhood cancer resources


==External links==
[[Category:Disease]]
 
* [http://www.medulloblastoma.org Medulloblastoma support resources]
* {{Chorus|00905}}
 
 
 
{{Nervous tissue tumors}}
{{SIB}}
 
[[Category:Disease state]]
[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Neurosurgery]]
[[Category:Neurosurgery]]
[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Mature chapter]]
[[Category:Up-To-Date]]
 
[[Category:Oncology]]
[[de:Medulloblastom]]
[[Category:Medicine]]
[[nl:Medulloblastoom]]
[[Category:Neurology]]
[[ja:髄芽腫]]
[[Category:Neurosurgery]]
[[pl:Rdzeniak]]
 
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

Latest revision as of 12:24, 18 October 2019

For patient information click here

Medulloblastoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Medulloblastoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case studies

Case #1

Medulloblastoma On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Medulloblastoma

All Images
X-rays
Echo and Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Medulloblastoma

CDC on Medulloblastoma

Medulloblastoma in the news

Blogs on Medulloblastoma

Directions to Hospitals Treating Medulloblastoma

Risk calculators and risk factors for Medulloblastoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2] Syed Musadiq Ali M.B.B.S.[3]

Synonyms and keywords: Medulloblastomas; Medulloblastomi; Infratentorial PNET; Infratentorial primitive neuroectodermal tumor

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Medulloblastoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related chapters


Template:Nervous tissue tumors


Template:WikiDoc Sources