Clinico-Pathologic ConferencePediatrics

Borela-Cotaoco17 February 2010

Case summary

Course in the wards

Laboratories• Chest X-ray: normal• Chemistry

Calcium 2.62 (N: 8.8-10.5 mg/dl)Magnesium 1.0 (N:0.650-1.050 mmol/L)Creatinine 61 (N: 59-104 umol/L)Uric acid 281 (N: 0.160-0.43 mmol/L)Sodium 143 (N: 135-144 mg/dl)Potassium 3.7 (N: 3.6-5.2 mmol/L)Chloride 105 (N:104-108 mmol/L)

Laboratories• Blood Chemistry

4-4-09 4-9-09Hgb (N: 14-16 mg/dl) 141 128Hct (N: 0.40-0.54) 0.42 0.38Platelet count 260WBC 10.9Neu 0.66Lymphocytes 0.24Eosinophils 0.05

BasophilsStabs 0.01ESR (N: 0-15 mm/hr) 21Blood type B+

Laboratories• CSF (ventricular)

– 5 cc of clear, colorless liquid– Ph: 7.5– SG: 1.010– RBC: 514 x 10^6 (N: 0-10 cells)– WBC: 1 x 10^6– Total protein: 0.11 (N: 20-40 mg/dl)– Glucose: 4.7 (N: 60-160 mg/dl)– Pandy’s: negative

• MRI of the spine (Post-operative)– normal cervical, thoracic and lumbar spine

Laboratories• Audiometry– unremarkable

• CT scan– Slight enhancing heterogenous hyperdense lesion in the

cerebellar vermis with perilesional edema and mass effect. Moderate extraventricular obstructive hydrocephalus.

differentials

Differentials• Pseudotumor cerebri• Medulloblastoma• Ependymoma• Brainstem glioma

Pseudotumor Cerebri• RULE IN

– Overweight– Headache– Vomiting– Absent focal neurologic signs– Mentation and laertness

preserved– Ataxia– Limited lateral eye

movements– Normal CSF total protein

content

• RULE OUT– Papilledema not

mentioned– No visual field defect– Increased CSF RBC count– (+) hyperdense lesion in

the cerebellar vermis on CT Scan

Medulloblastoma• Rule In– Headache– Vomiting– (+) Romberg’s sign– Nystagmus– Limited lateral eye

movement on the left– Lesion in the cerebellar

vermis on CT scan

• Rule Out– Potential for

metastasis

tuberculoma• RULE IN– Increased ICP– Infratentorial signs,

esp cerebellar– CT scan findings:

lesion in the cerebellar vermis with perilesional edema

• RULE OUT– Clear CXR– No TB symptoms– (-) Kernig’s and

Brudzinski’s signs

Ependymoma• RULE IN– Projectile vomiting– Hydrocephalus

• RULE OUT– Hyperdense lesion

Brainstem glioma• RULE IN– Headache and vomiting– Age of the patient (10

y/o) – Horizontal nystagmus

• RULE OUT– No gait disturbances– No ataxia– Papilledema

Primary impression

Epidemiology• Brain tumors– 2nd most common childhood malignancy– Mortality as high as 45 %– 5 categories of tumors comprise 80 % of all brain tumors

in children:1. Juvenile pilocytic astrocytoma,2. Medulloblastoma/primary neuroectodermal tumor3. Diffuse astrocytomas4. Ependymomas5. Craniopharyngomas

• Brain tumors with a male predominance would be: Medulloblastoma and Ependymoma

Epidemiology

• Astrocytomas– the most common intracranial neoplasm– low grade astrocytomas occuring mainly in

childhood and with a excellent prognosis

• Associated environmental risk factors are not known, except for IONIZING RADIATION

• Loss of DNA on chromosomes 10p, 17p, 13q and 9.

Etiology

Clinical Manifestations• Progressive headache– No history of infection– Lack of fever, GI and urinary problems– Unremarkable laboratory work-up– Increasing intensity, frequency, association with

vomiting, unresponsive to medications– ↑ ICP secondary to an underlying tumor

• Juvenile pilocytic astrocytoma– Localized signs and symptoms of cerebellar

dysfunction• Positive Romberg’s sign, intact motor strength, no gait

disturbance• Horizontal nystagmus, no auditory problems• CT scan findings

– Others• Headache• Vomiting, with or without nausea• No visual problems• Anemia• Hyperreflexia of the lower extremities• No pathologic reflexes, supple neck

Diagnosis• Biopsy– Bundles of compact fibrillary tissue with loose, microcystic

spongy areas – Rosenthal fibers

• MRI– Contrast-enhancing nodule

• Lumbar Puncture• -very strongly contraindicated

• Molecular/Cytogenetic and serum evaluation– Not necessary

imaging

Treatment• Surgery• Radiation therapy• Chemotherapy

Prognosis• After surgical resection– Complete: 80-100% overall survival rate– Partial: 50-95% overall survival rate

• Low metastatic potential• Rarely invasive• Leptomeningeal spread

References• Nelson’s Textbook of Pediatrics 18th ed.

Thank you!