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Ependymoblastoma


Presentation

  • These observations and the presentation of our patient suggest that a histological diagnosis of ependymoblastoma of the spinal cord, with or without symptoms of intracranial tumor, should suggest that an intracranial primary lesion might be present.[ncbi.nlm.nih.gov]
  • Clinical presentation Patients present with signs and symptoms of raised intracranial pressure and hydrocephalus. Focal neurological signs may be present in older children.[prod.wiki.cns.org]
Asymptomatic
  • Symptoms - Ependymoblastoma * Asymptomatic in early stages * Pain * Headache * Swelling * Lump Causes - Ependymoblastoma Not supplied. Prevention - Ependymoblastoma Not supplied.[checkorphan.org]
  • .: Asymptomatic recurrence detection with surveillance scanning in children with medulloblastoma. J Clin Oncol 15 (5): 1811-3, 1997. [PUBMED Abstract] Torres CF, Rebsamen S, Silber JH, et al.: Surveillance scanning of children with medulloblastoma.[cancerinfo.tri-kobe.org]
Anorexia
  • Beginning two and a half months before entry, without previous relevant illness, she had had periodic nausea, vomiting, and anorexia. At first the presence of a tumor of the posterior fossa was not recognized.[pubs.rsna.org]
Subcutaneous Mass
  • We describe a case of congenital ependymoblastoma presenting as a subcutaneous mass in the sacrococcygeal area of a newborn male.[ncbi.nlm.nih.gov]
Distractibility
  • Ependymoblastoma: dear, damned, distracting diagnosis, farewell!*. Brain Pathol 2010; 20 :133–9 [ PubMed ] [ Google Scholar ][ncbi.nlm.nih.gov]
Focal Neurologic Deficit
  • There was no history of vomiting, other focal neurological deficit or trauma. On neurological examination, patient was alert, well oriented with no sensori-motor deficit.[ncbi.nlm.nih.gov]
  • Seizures and focal neurological deficits, with features of raised ICT are the symptoms at presentation. CT and MRI reveal a well differentiated, heterogenous, markedly enhancing hemispheric mass with varying degree of cystic or necrotic changes.[thamburaj.com]
Lethargy
  • Symptoms & Signs Some of the symptoms of Ependymoblastoma often includes the following: patients also have symptoms even at an early stages, pain, headache, swell that occur for no reason at all & lump which are usually painful, fever, weight loss, lethargy[signssymptoms.org]
Excitement
  • Note there is diffusion restriction ( D ), but no intratumoral contrast enhancement ( C ) (1.5T Signa Excite; GE Healthcare, Milwaukee, Wisconsin). Fig 5.[ajnr.org]

Workup

Brain Edema
  • Imaging On MRI, ependymoblastomas are large heterogeneously enhancing lesion with associated brain edema and mass effect.[prod.wiki.cns.org]

Treatment

  • Prognosis of children with ependymoblastoma is poor, and data on clinical behavior and optimal treatment strategies are lacking, but sustained remissions have been achieved after multimodal treatment according to existing literature.[ncbi.nlm.nih.gov]
  • Children 3 years or younger: Standard treatment is usually surgery followed by chemotherapy.[secure.ssa.gov]

Prognosis

  • The prognosis seems to be far inferior to other embryonal CNS tumors, and known clinical and MRI characteristics of EBL are based on scattered case reports.[ncbi.nlm.nih.gov]
  • Prognosis of children with ependymoblastoma is poor, and data on clinical behavior and optimal treatment strategies are lacking, but sustained remissions have been achieved after multimodal treatment according to existing literature.[unboundmedicine.com]

Etiology

  • This lets people see incidence and survival patterns, evaluate diagnosis and treatment, facilitate etiologic studies, establish awareness of the disease, and ultimately, prevent brain tumors.[cern-foundation.org]
  • Etiology To theorize the "cell of origin" of ependymomas and related tumors, one needs only to look back through the stages of normal ependymal cell development.[emedicine.medscape.com]

Epidemiology

  • CONCLUSIONS: With this study, we add the largest case collection to the limited published database of MR imaging findings in ependymoblastoma, together with epidemiologic data.[ncbi.nlm.nih.gov]
  • Epidemiology Ependymoblastoma is a rare tumor that occurs in infants and young children in the first two years of life. Both sexes are affected equally. Localization Ependymoblastomas are large lesions that occur in the supratentorial compartment.[prod.wiki.cns.org]
  • […] subclassification of embryonal tumors Rosettes are frequent and characteristic of embryonal tumors Ependymoblastoma lacks a neuropil-like matrix and ganglion cell elements Overexpression of LIN28A protein in C19MC altered embryonal tumors Poor prognosis Epidemiology[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • .: Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. Neurosurgery 37 (5): 885-93, 1995.[cancerinfo.tri-kobe.org]

Prevention

  • In order to prevent recurrence in future and good survival chances, a child may also undergo bone marrow or stem cell transplant. After the surgery, a medical team collects and freezes healthy stem cells of a child.[bookinghealth.com]
  • Prevention - Ependymoblastoma Not supplied.[checkorphan.org]
  • Please find comprehensive information on Ependymoma regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE .[dovemed.com]
  • Prevention There are no sure ways of preventing ependymomas.[knowcancer.com]
  • This lets people see incidence and survival patterns, evaluate diagnosis and treatment, facilitate etiologic studies, establish awareness of the disease, and ultimately, prevent brain tumors.[cern-foundation.org]

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