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Patient Case Presentation Neurosurgery Red Service Gabriel Zada, MD Sean McNatt, MD LAC-USC Medical Center May 24, 2006.

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Presentation on theme: "Patient Case Presentation Neurosurgery Red Service Gabriel Zada, MD Sean McNatt, MD LAC-USC Medical Center May 24, 2006."— Presentation transcript:

1 Patient Case Presentation Neurosurgery Red Service Gabriel Zada, MD Sean McNatt, MD LAC-USC Medical Center May 24, 2006

2 Patient J.P. History of Present Illness: –22 year old caucasian female –Long history of headaches –Presented with 2 days of: Sinus headache progressing to Bifrontal headache Somnolence Altered mental status Nausea/vomiting –No fevers, chills –No history of trauma

3 Past Medical History: –Headaches x 2 years –Otherwise unremarkable past medical history Medications: –None Allergies: –None Known Social History: –Mother of a 2 year old child –No tobacco, drug, or alcohol use Patient J.P.

4 Physical Exam –Mental Status Patient somnolent, partially arousable Oriented inconsistently to name only Responds inappropriately with one word responses –Cranial Nerve Exam Right partial ptosis Papilledema Right pupil 5  4 mm, sluggish Left pupil 5  3 mm, brisk Extraocular movements intact Cranial Nerves otherwise intact Patient J.P.

5 Motor exam –Normal tone –Follows simple commands intermittently –Squeezes hands, wiggles toes –Diffusely weak in all extremities Sensory Exam –Sensation intact to light touch in all extremities Reflexes –Reflexes 2+, symmetrical –No Hoffman’s sign –Toes downgoing Cerebellar/Gait exam –Mild dysmetria bilaterally on finger-nose test –Gait Deferred Patient J.P.

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16 Initial Management Patient transferred to LAC Medical Center Right ventriculostomy placed –CSF sent for cytology  “atypical cells” Patient’s exam significantly improved: –Awake, alert –Oriented to name only (San Dimas, 1993) –Significant short-term memory deficits –Partial right IIIrd nerve palsy improved –No pronator drift, power 5/5 throughout

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61 Surgery Right interhemispheric transcallosal approach to third ventricle Patient’s right side down Frozen pathology  malignant glial tumor with high cellularity Gross total resection

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66 Transcallosal Approach to the Third Ventricle Position with head in lateral position to allow gravity to facilitate in retraction Bone flap 2/3 anterior to coronal suture and 1/3 posterior to coronal suture –May modify accordingly for anterior versus posterior third ventricular lesions Callosal incision between 2 ACAs Must account for shift involved with lateral positioning Callosotomy approximately 2-3 cm in length –Some authors advocate transverse callosotomy

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68 Postoperative MRI Brain

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92 Postoperative Course Patient with unchanged neurological status following procedure Ventriculostomy left in place yet unable to wean off Left VP shunt placed on postoperative day 7 Short term memory slightly improved over course of week Patient transferred to step-down

93 Pathology

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98 Diagnosis: Intraventricular Anaplastic Oligodendroglioma (WHO Grade III)

99 Oligodendroglioma: Background Two recognized grades: –WHO grade II: oligodendroglioma –WHO grade III: anaplastic oligodendroglioma 4% of all primary brain tumors Mean age: approximately 43 years 6% during infancy and childhood No known patterns of inheritance Most commonly occur in white matter of frontal and temporal lobes Intraventricular oligodendroglioma: –Approximately 20 case reports in the literature

100 Anaplastic Oligodendroglioma: Epidemiology Account for ~3% of all adult supratentorial primary malignant gliomas Account for 20-54% of all oligodendrogliomas Most common in adults (mean age 49 years) –Older than patients with grade II oligodendrogliomas Male to female ratio 1.5 : 1 Preference for frontal lobe (60%) followed by temporal lobe (33%)

101 Anaplastic Oligodendroglioma: Histopathology Share with oligodendroglioma: –Honeycomb appearance with clear cytoplasm –“Fried egg yolk” appearance –Frequent calcification –Occasional gemistocytes –Often GFAP and S-100 positive –Perinuclear halos Diffuse features of malignancy: –Increased cellularity –Cellular atypia –High mitotic index –Necrosis and microvascular proliferation may be present Occasional multinucleated giant cells of Zulch

102 Anaplastic Oligodendroglioma: Differential Diagnosis All with neoplastic cells with round nucleus and clear cytoplasm (oligodendroglioma-like cells or OLCs) Clear cell ependymoma: –ependymal features (ie rosettes) help differentiate Central neurocytoma: –Synaptophysin positive, more commonly originates in ventricles Clear cell meningioma: –PAS positive, EMA immunoreactivity Metastatic renal cell tumor

103 Oligodendroglioma: Molecular Genetics Chromosome 19: –Loss of heterozygosity (LOH) on long arm of chromosome 19 (19q) –50-80% of cases Chromosome 1: –LOH on short arm (1p) in 67% of cases –Almost always coexists with LOH at 19q Polysomia, deletions on other chromosomes (ie 9,10) Progression to malignancy correlates with EGFR, PDGF overexpression Fluorescence In Situ Hybridization (FISH) used to detect Lack of correlation between histology and molecular markers

104 Oligodendroglioma: Molecular genetics Several molecular subtypes: 1) Combined/isolated loss of 1p/19q: –More likely frontal, parietal –Diffuse enhancement –Close to 100% response rate –Survival greater than 10 years 2) 1p loss without 19q loss –Close to 100% response rate –Survival approximately 6 years 3) No deletion of 1p/19q with TP53 mutation –More likely temporal, insular –Ringe enhancement more likely –33% response rate –Survival approximately 6 years 4) No deletion of 1p/19q, no TP53 mutation, yet other mutations –18% response rate –Survival generally less than 18 months

105 Anaplastic Oligodendroglioma: Multimodal treatment Surgery is still primary treatment: –Gross total resection whenever possible Mixed data regarding adjuvant radiotherapy –Postoperative radiation therapy has been shown to extend survival, yet carries associated morbidity –Delayed XRT as effective as immediate postop XRT in one study –Another study showed no benefit to radiotherapy XRT/chemo current standard in recurrent, high-grade oligodendroglioma Salvage therapy frequently chemotherapy with stem cell rescue

106 Anaplastic Oligodendroglioma: Response to chemotherapy Tumors with combined 1p and 19q deletions are often responsive to chemotherapy –Procarbazine, CCNU, Vincristine (PCV) Many side effects including myelosuppression in 46% –More recently, temozolamide (in trials) –Half of such tumors show complete radiological responses to chemotherapy –Mean survival time 10 years with these deletions compared to patients without these deletions (mean 2 years)

107 Anaplastic Oligodendroglioma: Prognosis Median survival time of 4 years –Five year survival: 41% –Ten year survival: 20% Local tumor recurrence occurs frequently Leptomeningeal spreading (‘oligodendrogliomatosis’) has also been described Metastatic disease uncommon, yet incidence may be increasing –Most common sites bone, lymph nodes, scalp Good prognostic factors: –Younger patient age –Female sex –Seizure as presenting symptom

108 References 1. Merrell R et al. 1p/19q chromosome deletions in metastatic oligodendroglioma. J Neurooncology Waldron JS, Tihan T. Epidemiology and pathology of intraventricular tumors. Neurosurgery Clinical of N America. 14 (2003) Dumont AS et al. Intraventricular gliomas. Neurosurgery Clinical of N America. 14 (2003) Reifenberger G. Anaplastic oligodendroglioma. In Tumours of the Nervous System. (Kleihues P, Cavanee WK, eds.) IARC Press, Kasowski HJ et al. Transcallosal Transchoroidal Approach to Tumors of the Third Ventricle. Neurosurgery 57, Suppl , Engelhard HH. Current diagnosis and treatment of Oligodendroglioma. Neurosurgical Focus. 12(2), 2002.

109 Thank You


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