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Answer to Case of the Week: Feb 25-Mar 4, 2010

9 year with celiac disease presents with vomiting and headache


 




There is a midline posterior fossa neoplasm with cystic and solid components showing irregular contrast enhancement

Diagnosis: Medulloblastoma

Posterior fossa neoplasms in children are usually due to either medulloblastoma, ependymoma, pilocytic astrocytoma or brain stem glioma. 

Clues to the specific diagnosis include cystic neoplasm with enhancing nodule in pilocytic astrocytoma and inhomogeneous mass more often with calcification and extrusion into foramen with ependymoma.

 Unfortunately, the diagnosis must still be made histologically as many tumors do not adhere to such criteria. 

Medulloblastoma usually presents with headache, emesis and lethargy as patient is usually hydrocephalic at time of diagnosis.

 Occasionally, patient has symptoms of brainstem compression such as head tilt, hearing loss or facial weakness. 

 Medulloblastoma has an increased association:

Turcot’s syndrome

Ataxia-telangectasia

Gorlin syndrome (autosomal dominant basal cell nevus syndrome)Gardner syndrome

Cowden syndrome

Taybi and Coffin-Siris syndrome. 

Imaging usually shows a fairly homogeneous mass due to the homogeneous nature of the lesion histologically. It may therefore be hyperdense (90%) on noncontrast CT.  

Metastases are direct extension through the subarachnoid space in the ventricular system, basal cisterns and thecal sac and are imaged with post contrast MRI.

 Calcification, cystic change, necrosis or hemorrhage in less than 15% of patients. 

MR Spectroscopy shows $$ NAA and ##increased Choline and lactate usually present. 

More than 50% of patients are disease free at 5 years with complete resection, more than 80% if no metastases are present at presentation. 

MNEMONIC FOR POSTERIOR FOSSA TUMORS IN CHILDREN: 

GAME

G-Brian stem glioma

A-Pilocytic astrocytoma

M- Medulloblastoma

E- Ependymoma

  Multiple PNET and rhabdoid tumors can also occur in the posterior fossa. 

Differential Diagnosis of DROP METS IN CHILDREN:

Medulloblastoma

Ependymoma

Hypothalamic astrocytoma

Thalamic glioma

Choroid plexus carcinoma

Pineal tumors

Lymphoma, Leukemia 

Take Home Message:

Rhabdoid tumors of the kidney are associated with posterior fossa brain tumors. 

Medulloblastoma arises from the roof ( superior medullary velum) of fourth ventricle. 

Ependymoma arises from the floor of the fourth ventricle 


Residents Submitting Correct Diagnosis - Case of the Week
Radiology
Pediatrics
VCU Resident
  • Aaron Nordgren
  • Charlotte Cockrell
  • Jeremy Camden
  • Karen Gerlach
  • John Fahrner
  • Jamil Muasher
  • Shep Morano
  • Joseph Eason
  • Kathryn Jones
  • Brian Deuell
  • Sarah Johnson
  • Gwin Tennis
  • Shadi Jurdi
    Others
  • Yang TangUnited States of America
  • Brian TrottaUnited States of America
  • Flavia GaspariniBrazil
  • Jose HidalgoColombia
  • Sayed GhoneimEgypt
  • Nicole KelleherUnited States of America
  • Nagendar RaoIndia
  • Yeshodha YennaIndia
  • Nishard AbdeenCanada
  • Wael NemattallaEgypt
  • Gitanjali BajajIndia
  • JAMAL ABAZIDSyrian Arab Republic
  • R HeiserCanada
  • Cephus SimmonsUnited States of America
  • Faisal ShadabIndia
  • Joshua BallUnited States of America
  • Chad St. GermainUnited States of America
  • Mantosh RattanUnited States of America
  • Manoj JohnIndia
  • Rasha ElshafeyEgypt
  • Khalid KhashoggiSaudi Arabia
  • Ezzeldin Abdel KaderSaudi Arabia
  • Naganathan ManiUnited States of America
  • Shanaree MuzinichUnited States of America
  • Robert PalmerUnited States of America
  • Sema YildizTurkey
  • BOUTHINA IBRAHIMEgypt
  • Syed AminUnited States of America
  • Abirami MahadevanIndia
  • John KirkhamUnited States of America
  • Anup GuptaIndia
  • Prakash MasandUnited States of America
  • Simon DupreAustralia
  • Mufudzi MavikiZimbabwe
  • AHMED EIDSaudi Arabia
  • Aiser GlayaCanada
  • Pankaj AgarwalIndia
  • Rajesh PahwaIndia
  • Gita KarandeIndia
  • Fahri AyzitTurkey
  • Gaurav KumarUnited States of America
  • Kshipra HemalUnited States of America
  • PRAGATI KUMARUnited States of America
  • Christopher FrancisUnited States of America
  • OLIVA GONZALEZMexico
  • Eisha TahirPakistan
  • Archana LaroiaUnited States of America
  • Mohammadreza RastegariIslamic Republic of Iran
  • Rajesh SIndia

    Disclaimer: This information is intended solely for resident review of presented cases which may or may not be pathologically proven. Information is derived from a number of published sources of varying reliability and does not represent original research from the institution. It is not intended to be comprehensive and should therefore not substitute for careful review of the literature.