patient case presentation
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Patient Case Presentation
Neurosurgery Red Service
Gabriel Zada, MD
Sean McNatt, MD
LAC-USC Medical Center
May 3, 2006
Patient J.A.
• History of Present Illness:– 22 month old female I– Irritability, nausea/vomiting, decreased p.o.
intake for 6 days prior to admission– Low grade fever per parents– Multiple visits to emergency room + clinics
over last week, got intravenous fluids and parents told her illness was viral
• History of Present Illness (continued):– Presented again to Pediatrics ER with lethargy, altered
mental status– Bradycardic to pulse of 70s, hypertensive– Intubated and sedated in ER for airway protection– No recent seizures, no sick contacts
• Past Medical History:– Past medical and surgical history unremarkable– Term birth, uncomplicated– Normal developmental milestones
Patient J.A.
• Physical Exam:– Patient intubated, sedated– Somnolent but arousable– Regards examiner, not following commands– Pupils briskly reactive and equal– Extraocular movements intact– Tone normal– Moving all extremities with full power– Normocephalic, no external signs of trauma
Patient J.A.
Initial Management
• Patient admitted to Neurosurgical ICU• Right ventriculostomy placed• Initial ICPs in the 20s, normalized with drainage• CSF yellow, proteinaceous • High ventriculostomy output (150 cc/12 hours)• Patient transferred to CHLA for definitive
management
Operative Management
• Right parieto-occipital craniotomy
• Intraoperative ventriculostomy used to cannulate ventricle
• Gross Total Resection
Choroid Plexus Tumors: Epidemiology• Comprise 0.5% of all brain neoplasms• Comprise 6% of primary pediatric neoplasms• 45% occur within the first year of life• 70% occur within first 2 years of life• Median age at diagnosis is 3.5 years• 1.2 : 1 male to female ratio• Location:
– 50% in lateral ventricles– 37% in 4th ventricle– 9% in third ventricle– Remainder in other locations
• Most present with hydrocephalus secondary to CSF overproduction (78-95%)
• Tumor hemorrhage found in 2 of 21 patients, in one study• Most common symptoms:
– Nausea,vomiting– Irritability– Headaches– Visual changes– Seizures
• Most common signs:– Craniomegaly– Papilledema– Stupor or coma in 25% of presentations
Choroid Plexus Tumors: Clinical presentation
• Believed to arise spontaneously• Many tumors demonstrated to harbor
chromosomal aberrations (usually chromosome 22)
• CPP has been linked experimentally to the SV40 DNA primate virus
• Large T antigen is the major regulator of the SV40 virus protein products, and interacts with the product of the p53 and RB tumor suppressor genes
• When expressed in mice, T antigen induces formation of CPPs
Choroid Plexus Tumors: Pathophysiology
Choroid Plexus Papilloma: Pathology
• “Cauliflower-like” appearance• Evidence of prior hemorrhage often observed• Tumor surface is frond-like, similar to normal choroid
plexus• Stroma has a fibrous consistency• Can differentiate CPP from papillary ependymoma
based on histology:– Ependymoma has stroma composed of neuroglia and epithelial
cells with cilia
• Immunohistochemistry not extremely helpful for these lesions
Choroid Plexus Carcinoma: Pathology
• 29-39% of choroid plexus neoplasms are carcinoma• Differentiating features: (per WHO criteria)
– Nuclear atypia, N/C ratio, mitotic figures– Loss of normal papillary architecture– Invasion of brain parenchyma through ependyma
• Immunohistochemistry with higher Ki67 index labeling
• Variant of CPP with stromal invasion ???– Branching papillae with thin-walled, ectactic blood vessels– More complex architecture than standard CPPs
Choroid Plexus Tumors: Pathology
Choroid Plexus Papilloma-Normal papillary architecture-Columnar + Cuboidal cells-Single layer stalks
Choroid Plexus Carcinoma-Piled up epithelium-Loss of papillary architecture
• Study by M Levy et al, Neurosurgery, 2001• Many other series of CPCs have a wide variability in survival times
and outcomes• Variant of (benign) CPP with invasive characteristics• Retrospective review of 12 patients• 8 patients with traditional CPPs, 4 patients with variant CPPs yet
invasive (patchy, local invasion) yet benign histology• Only one subtotal resection in patient with variant • Five year survival rate: 100%• Summary: Stromal invasion may not be as useful a criterion of
carcinoma as nuclear features and loss of architecture• Gross total resection is the key in all cases
Choroid Plexus Tumors: Significance of Stromal Invasion
Typical CPPVariant (Invasive) CPP: -Replacement of epithelium, -Invasion into surrounding brain
•From M Levy et al, Neurosurgery, 2001
• CT often demonstrates punctate calcification• MRI:
– Isodense to brain on T1 imaging
– Brightly enhancing lesions
– Enlarged choroidal artery can be noted
– CP carcinomas with necrosis, calcification, hemorrhage, homogeneous enhancement
– CP papillomas with “mottled” appearance
Choroid Plexus Tumors: Radiographic Features
• Study by Krieger et al, Neurosurgical Focus, 2005• MR Spectroscopy analysis of 6 children with newly
diagnosed intraventricular brain tumors• Retrospectively, 3 with CP papilloma, 3 with CP
carcinoma• CP papilloma:
– Significant peak of myoinositol (mI) (20.4 vs. 4.1, p<0.01)– Elevated mI/Cho and Glx/Cho ratios
• CP carcinoma:– Lack of mI elevation– Elevated Choline– Low NAA/Cho, Cr/Cho, mI/Cho ratios (significant)
Choroid Plexus Tumors: MR Spectroscopy
Choroid Plexus Tumors: MR Spectroscopy
CP Papilloma:Prominent mI Peak
CP Carcinoma:Prominent Cho Peak
From Krieger et al, Neurosurgical Focus, 2005
• Hydrocephalus– Requirement for VP shunting ranges from 37% to
78%– Raimondi and Gutierrez recommend initial of
shunting all patients with 3rd or 4th ventricular tumors– Hydrocephalus can resolve completely with gross
total resection– High likelihood of VP shunt obstruction:
• Secondary to high protein, debris, blood
Choroid Plexus Tumors: Treatment
• Operative Treatment– Focus on exposure of feeding artery– Avoiding eloquent cortical regions– Third ventricular lesions:
• Approach is midline transcallosal
– Fourth ventricular lesions:• Approach is midline posterior fossa craniectomy
– Emphasis on minimizing blood loss (papilloma versus carcinoma)
Choroid Plexus Tumors: Treatment
• CP Carcinomas:– GTR achieved in less than 50% of cases given
hemorrhagic tumor, invasiveness– No definitive guideleines following surgery– Postoperative chemotherapy:
• Cyclophosphamide, etoposide, vincristine, platinum agent• Low response rate (8 of 22 in one study)
– Postoperative radiation therapy:• One study: 5 year survival following GTR was 68% with
subsequent XRT versus 16% without XRT• Recommended even following GTR given high relapse rates
Choroid Plexus Tumors: Adjuvant Treatment
• CP Papillomas:– 5 and 10 year survival: 81% and 77%– Mortality usually in younger patients (less than one
year)– Up to 33% with significant morbidity– Postoperative subdural fluid collections may require
subdural-peritoneal shunting• CP Carcinomas:
– 5 and 10 year survival: 41% and 35%• Most important prognostic factor is extent of
surgical resection
Choroid Plexus Tumors: Outcomes
References• 1. Gupta N. Choroid Plexus tumors in children. Neurosurg Clin
N AM. 14 (2003) 21-631• 2. Levy M et al. Choroid Plexus Tumors in Children:
Significance of Stromal Invasion. Neurosurgery 48: 303-309, 2001
• 3. Krieger MD et al. Neurosurgical Focus. 18(6a):E4, 1-4, 2005• 4. Ellenbogen RG et al. Tumors of the choroid plexus in
children. Neurosurgery 25: 327-335, 1989• 5. Wolff JE et al. Radiation therapy and survival in choroid
plexus carcinoma. Lancet 1999; 353:2126• 6. Wolff JE et al. Choroid Plexus Tumors. Br J Cancer.
2002;87:1086-1091
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