Clinico-Pathologic Conference Pediatrics Borela-Cotaoco 17 February 2010

Case summary

Course in the wards

Laboratories Chest X-ray: normal Chemistry Calcium2.62 (N: mg/dl) Magnesium1.0 (N: mmol/L) Creatinine61 (N: umol/L) Uric acid281 (N: mmol/L) Sodium143 (N: mg/dl) Potassium3.7 (N: mmol/L) Chloride105 (N: mmol/L)

Laboratories Blood Chemistry Hgb (N: mg/dl) Hct (N: ) Platelet count260 WBC10.9 Neu0.66 Lymphocytes0.24 Eosinophils0.05 Basophils Stabs0.01 ESR (N: 0-15 mm/hr)21 Blood type B+

Laboratories CSF (ventricular) – 5 cc of clear, colorless liquid – Ph: 7.5 – SG: – RBC: 514 x 10^6 (N: 0-10 cells) – WBC: 1 x 10^6 – Total protein: 0.11 (N: mg/dl) – Glucose: 4.7 (N: 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 – 2 nd 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 tumor 3. Diffuse astrocytomas 4. Ependymomas 5. 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: % overall survival rate – Partial: 50-95% overall survival rate Low metastatic potential Rarely invasive Leptomeningeal spread

References Nelson’s Textbook of Pediatrics 18 th ed.

Thank you!