NEUROSURGERY CLINICOPATHOLOGICAL CONFERENCE

(CPC)
POSTERIOR FOSSA TUMOUR
6TH May 2022
Dr. Samuel OLUKA
Department of Surgery & Department of
Anatomic Pathology, School of Medicine,
Neurosurgical Unit, Kenyatta National Hospital,
University of Nairobi, NAIROBI, KENYA.
 NEUROSURGERY
CLINICOPATHOLOGICAL
CONFERENCE

 Welcome to the weekly Neurosurgery CPC.

 Our Neurosurgery Residents led by Dr Oluka will take us
through this conference. Dr Bett will give the case history
and radiological findings, Dr. Oluka will take us through the
surgery after which this case will be open for discussion.
 After the brief discussion and comments from the
participants, Dr Abdalla will present the histopathological
findings followed by a brief review of the topic by Dr Oluka,
discussion and closing remarks.
 Biodata

 MMM

 23 years

 Female

 From Kimomori, Murang’a

 Informant: Mother and self

 Presenting Complaints

 Headache 3 months

 Unsteady gait- 6 weeks

 Vomiting- 2 weeks
 HPI

Headache- 3 months
 Gradual onset of intermittent global headache that
was progressive over time in nature and intensity

 Worse in the morning with no known aggravating

factors but relieved by over-the-counter analgesic

 Over time was associated with nausea and vomiting,

notably in the last 2 weeks of presentation

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 HPI

Unsteady gait- 6 weeks

 Insidious onset unsteady gait and loss of balance with
tendency to fall more toward the right

 Mother reports subtle weakness and numbness on

the right side that started with the right upper limb
then progressed to involve the right lower limb

 No hearing difficulty, earache, otalgia, tinnitus or

vertigo
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 Important Negatives

 No visual symptoms

 No facial asymmetry dysphagia or voice hoarseness

 No history of constitutional symptoms

 No history of convulsion

 No history of trauma
 Clinical Follow-up

 Was on follow up in Murang’a where imaging was done and was

referred to Kenyatta National hospital for further management

 Admitted to ward 4C on 5/4/2022

 Past Medical & Surgical
History
 Index admission

 No history of chronic illness or tumour

 No prior history of surgery or blood transfusion

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 Family Social History

 Single

 No family history of malignant or similar illness

 Does not take alcohol nor smoke cigarette

 NHIF upto date

 Summary

MMM, is a 23 year old lady who presented with 3 months history of

headache associated with vomiting and 6 weeks history of unsteady gait
with subtle right sided weakness
 Physical examination

Lady in fair general condition

Not pale, No jaundice, No cyanosis, Not dehydrated
BP:121/87 mmHg
PR: 82 bpm
Temp: 36.4oC
RR: 18 cpm
 Nervous System Exam
Higher functions
 GCS: 15/15 (E4,V5,M6)
 Alert and oriented in time, place and person
 Memory: intact
 Intelligence: normal abstract reasoning
 Speech: coherent, spontaneous and of good volume
 Cranial nerves
CN I: Not assessed
CN II: Intact
CN III,IV and VI: Intact
CN V: Intact
CN VII: Intact
CN VIII: Intact
CN IX/X: Intact
CN XI: Intact
CN XII: Intact
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 Motor examination

RUL LUL RLL LLL

Bulk Normal Normal Normal Normal

Tone Increased Normal Increased Normal

Power 3 5 3 5

Reflexes Normal Normal Normal Normal

 Sensory exam

Pain Temperature Vibration/ Fine touch

Proprioception

Right Normal Normal Normal Normal

Left Normal Normal Normal Normal

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 Cerebellar Exam

 Romberg’s test positive

 No truncal ataxia
 No titubation
 Right dysmetria??
 Right dysdiadochokinesia??
 No nystagmus
 No hypotonia
 No dysarthria

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 Cont..

Normal cortical sensation: stereognosis,

graphasthesia, two-point discrimination and sensory
inattention

Systemic inquiry was unremarkable

 Summary

MMM, is a 23 year old lady who presented with 3

months history of headache associated with vomiting
and 6 weeks history of unsteady gait with subtle right
sided weakness

On examination, she had right upper motor neuron

lesion with right eccentric cerebellar signs
 Clinical Impression

Raised intracranial pressure secondary to a space

occupying lesion:

Likely in the:
● Posterior fossa
 INVESTIGATIONS

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 Laboratory investigation

 FHG: WBC- 12.41*109/L; HB- 13.4g/dl; PLT- 268*109/L

 Urea, Electrolyte and Creatinine: Normal

 Coagulation profile: Normal

 Covid- Negative

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RADIOLOGY
 CT Scan

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 CT Scan

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 Coronal CT Scan

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 Sagital CT scan

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 Axial T1W MRI

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 Axial T2W MRI

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 FLAIR

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 Axial T1W with Contrast

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 T1W Pre and Postcontrast

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 Coronal T1W with
Contrast

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 Sagital T1W with
Contrast

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 Impression

 Medulloblastoma
 Ependymoma

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NEUROSURGERY CLINICOPATHOLOGICAL
CONFERENCE, UNIVERSITY OF NAIROBI,
KENYATTA NATIONAL HOSPITAL

THANK YOU
 Intra-op and post –operative followup
 Date of surgery 19/4/2022
 Consultant: Dr. Magoha
 Residents: Dr. Oluka, Munialo, Wanyoike et al
 Dx: Posterior fossa tumor
 Precautions: Neuromonitoring- SSEP, MEP, Individual CN VII, V, IX, X, XII
 Positioned: prone, Mayfield clamp used to hold the head in flexion.
 Sterility: Aseptic techniques strictly adhered to
 Incision: midline incision from 2 cm
above the inion to the C2 spinous
process
 Dissection done through the midline
layers until the inion on the rostral
end and C2 spinous process as the
caudal extent
 Exposed the occipital bone C1
posterior arch and c2 verterbra
 Standard suboccipital craniotomy
done
 C1 laminectomy done
 Y-shaped durotomy done
 Exposing the cerebellum and
cerebellar tonsils with an intact
arachnoid membranes and cisterna
magna containing CSF
 I identified the PICA branches
between the 2 cerebellar tonsils
 Release of CSF done from the
subarachnoid space
 Found the right tonsil was displaced
to the far right
 Continued the dissection into the
tonsilo-medullary fissure
 Gently retracted the left tonsil
revealing the tumor surface which
was highly vascular
 Cauterization of the tumor surface
done.
 De-dressing was circumferentially
done while cauterizing the tumoral
feeders.
 This was followed by debulking of
the freed tumor.
 Largely solid tan grey tumor with a
rubbery and nodular appearance
was seen
 Advanced the brain swabs between
the tumor and the floor of the 4th
ventricle with no resistance
 Be slightly tilted to the left and
tumor in the left lateral recess was
dissected out.
 Visualized the dura on the left CPA,
CN XII, VII, V
 Achieved GTR of the tumor
 Tumor freed from, the roof, floor
and lateral recesses
 Hemostasis on the tumor bed was
achieved by low powered cautery
and point coagulation, and was
thoroughly washed to remove
obvious blood products
 Clossure
 Dura closed using proline 5/0 in a
water tight fashion.
 Tumor cavity filled with warm saline
 Craniotomy bone secured in place
 Closure of the overlying soft tissues
done in layers
 Wound dressed.
post-op
 Wheeled to CCU, self-extubated
 Maintained on dexamethasone ,
antibiotics and critical monitoring of
vital function.
 Discharged after 5 days of ICU stay
 Power improved on the left and
right side
 Patient’s biodata

NAME: M.M
AGE : 23
GENDER: FEMALE
SPECIMEN NUMBER: S/1166/22
RECEIVED ON : 19.04.22
REPORTED ON: 5th May 2022
 Clinical History

 Right sided weakness, headache and acute hydrocephalus

 MRI- posterior fossa tumor
 Gross appearance

 Multiple fragments brown in color.

 Aggregate measurement of the largest 50 by 20 by 10 mm.
MICROSCOPY- x10 papillary architecture
Solid sheet pattern
Brain invasion
Calcification
High power x40
 Microscopy description

Sections show multiple fragments of densely cellular papillary epithelial

neoplasm with foci of solid sheets having areas of calcifications. The
neoplastic cells are pseudostratified around a central fibrovascular core
exhibiting moderate to marked pleomorphism with round to spindly deeply
hyperchromatic nuclei. Occasional binucleated forms are noted. Mitotic
count is 2-3/HPF. There is evidence of invasion into the adjacent brain tissue.
Diagnosis: Atypical choroid plexus papilloma (aCPP)
WHO-II
 Choroid Plexus Tumors

 Are rare neoplasms derived from the choroid plexus epithelium.

 It occurs within the ventricular system of the brain.
 They are classified into 3 histological subtypes:
 WHO grade I- Choroid plexus papilloma (CPP)
 WHO grade II- Atypical choroid plexus papilloma (aCPP)
 WHO grade III- Choroid plexus carcinoma (CPC)
 The histologic classification is based on:
 The architecture/ papillary pattern
 The cellularity
 Cytology-nuclear pleomorphism
 Proliferation-mitosis
 Necrosis and brain invasion
 Based on DNA methylation profiling they are classified into 3
prognostically relevant epigenetics subgroups:
 Supratentorial pediatric low risk CPP/aCPP.
 Infratentorial adult low risk CPP/aCPP.
 Supratentorial pediatric high risk CPP/aCPP/CPC.
 Epidermiology

 Predominantly in children
 2-5% of all pediatric brain tumors
 High incidence in infants
 M:F= 1.2:1 (slight male preponderance)
 Associated with Li Fraumeni syndrome and X chromosome abnormalities
 Tumor location

 Lateral ventricle in children

 Fourth ventricle in all ages groups
 Rarely in the spine and ectopic sites
 Clinically

 Presents with increased intracranial pressure, head enlargement,

hydrocephalus, vomiting, headache, papilledema
 Gross appearance

 Papillomas appear well circumscribed cauliflower like, can be friable.

 Carcinoma appear invasive, solid or with necrotic areas
Microscopy : WHO-I
WHO-II
WHO-III
 ImmunoHistoChemistry

 Positive stains- Transthyretin(pre-albumin) majorly synthesized in the

choroid plexus , Kir7.1 an inward rectifier potassium channel , EAAT-1 a
excitatory amino acid transporter, S100, pancytokeratin CK7/CK20, Ki67
 Negative stains are CEA carcino embryonic antigen and EMA-epithelial
membrane antigen
Choroid plexus tumors
Dr. samuel Oluka
 Choroid plexus tumors

 Defn: Benign or malignant neoplasms that arise from the choroid plexus, which is a
specialized tissue that develops along certain segments of the neural tube and is
present within each of the four ventricles

 Benign CPTs rarely convert to malignant phenotypes,

 it is believed that both benign and malignant tumors arise de

novo
 The benign tumors can be cured with surgical resection alone, but the
treatment can be associated with increased morbidity related to the
intraventricular location and their predominance in very young children
 Epidemiology

 Account for <1% of all brain tumors

 They occur in all age groups, but the majority occur in children, and they account
for approximately 14% of all brain tumors in the first year of life.

 Reports have also been described of CPTs being

 Male-female ratio of 1.2:1, a median age at diagnosis of 3.5 years, and a striking
difference in tumor location and age
 Epidemiology
 Supratentorial tumors occur mostly in infants, median age 1.5 years

 CPTs occur almost exclusively in the infratentorial compartment in adults. 1

 The median ages at diagnosis of tumors in the fourth ventricle and

cerebellopontine angle, in comparison, were 22.5 and 35.5 years, respectively
1

1. Hosmann A, et al. Management of choroid plexus tumors-an institutional experience. Acta Neurochir (Wien). 2019;161(4):745–
754
 Laurence did note that in the cases reviewed, 50% of tumors were situated in the lateral
ventricles, 37% in the fourth ventricle, 9% in the third ventricle, and the remainder in other
locations. 1

 More unusual tumor locations such as the pineal region have been described

 CPPs account for approximately 75% of all CPTs, with a slight male
preponderance seen for CPCs. 2

1. Laurence KM. The biology of choroid plexus papilloma and carcinoma of the lateral ventricle. In: Vinken PJ, Bruyn GW, eds. Handbook of
Clinical Neurology. Elsevier; 1974:555–595
2. Cannon DM, et al. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic
management. J Neurooncol. 2015;121(1):151–157.
 PATHOLOGY- Gross Appearance
 CPPs recapitulate the gross appearance of the tissue of their origin, in
this case the normal choroid plexus.

 The surface of the tumor is similar to the soft fronds of the choroid
plexus, and the overall shape is approximately globular.

 The well-differentiated secondary structures result in a cauliflower-like

appearance.

 The stroma can be fibrous and tough in consistency, and evidence of

previous haemorrhage is sometimes apparent
 Growth pattern
 CPPs tend to expand the ventricle rather than invade adjacent brain
tissue

CPC
 The boundary between CPCs and the surrounding brain parenchyma is
not as well developed, and brain invasion is frequently observed.

 The tumor itself appears less differentiated and is often very vascular
 Classification
Choroid plexus papilloma………………………………… Grade 1
 CPPs usually have fewer than 2 mitotic figures per 10 HPF

Atypical choroid plexus papilloma…………………….Grade 2

 More than 2 mitotic figures but fewer than 5 per 10 HPF

Choroid plexus carcinoma…………………………………Grade 3

 CPCs have more than 5 mitotic figures per 10 HPF
 Microscopic appearance
 CPP-
 The prominent papillary structure is composed of simple cuboidal or columnar
epithelia
 The internal stroma of these fibrovascular structures consists of connective tissue
and small blood vessels

NB: choroid epithelial cells do not contain cilia or blepharoplasts, as do ependymal

cells.

Unusual pathologic features: mucinous degeneration, tubular differentiation,

and formation of structures such as bone are sometimes observed.
 CPC

 The cytologic criteria of malignancy, such as nuclear atypia, increased

nucleus-cytoplasm ratio, and prominent mitotic figures

 The loss of the normal papillary architecture

 Evidence of brain invasion with extension of the tumor through the

ependymal lining of the ventricle.
 Immunohistochemistry

 CPPs usually express cytokeratin, vimentin, the calcium-binding protein

S100, and podoplanin

 When less than 50% of the cells in a given tumor stained heavily for S100,
the prognosis was poor.

 Absence of transthyretin-positive cells was also correlated with poor

prognosis.

 Cellular proliferation, as measured by Ki-67/MIB1 labeling, is low in CPPs and

significantly higher in CPCs
 Genetics and Molecular Biology - CPP
 Genetics. Genomic analysis of CPPs suggests a role of genes involved in the
development and biology of plexus epithelium (i.e., OTX2 and TRPM3).

 Choroid plexus tumors—especially carcinomas—occur in patients with Li-Fraumeni

syndrome, a cancer predisposition syndrome caused by TP53 germline mutation.

 SMARCB1 mutations with INI1 protein alterations and CPPs have been described in
the rhabdoid predisposition syndrome.

 Both mutations are very rarely identified in sporadic CPPs.

 CPPs also occur as part of Aicardi syndrome, an X-linked dominant syndrome that
occurs almost exclusively in female patients
 The prevalence of CPPs in Aicardi syndrome is estimated at 3-5%.

 Bilateral and triventricular CPPs occur in 1% of cases

 Clinical Features
 The most common presentation of choroid plexus neoplasms is related
to increased intracranial pressure (ICP) secondary to obstructive
hydrocephalus, CSF overproduction or both.

 Historical reports have noted that hydrocephalus resolves after complete

tumor removal, which suggests that CSF hypersecretion was responsible
for ventriculomegaly

 In infants and young children, the characteristic symptoms of increased

ICP are nausea/vomiting, irritability, headache, visual difficulty, and
seizure. 1
1. Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery. 1989;25(3):327–335
 The duration of symptoms reported in this series varied from 2 months in
patients younger than 2 years of age to 6 months on average in patients
older than 2 years of age.

 In contrast, 68% of adults present with headache and 50% with gait
ataxia. 1

1. Humphreys RP, NS, Hendrick EB. Childhood choroid plexus tumors. Con Pedia Neurosurg. 1987;7:1–18.
 Diagnostic Imaging CT
 The tumours are usually well-defined lobulated masses, either iso- or somewhat
hyperdense compared to the adjacent brain.

 There is associated hydrocephalus.

 They usually homogeneously enhance, demonstrating an irregular frond-like

pattern, resulting in a cauliflower-like appearance.

 If there is markedly heterogeneous contrast enhancement, a choroid plexus

carcinoma should be suspected

 Fine, speckled calcification is seen within the tumour in approximately 25% of cases
 MRI

 A sharply marginated lobular mass that is iso- to slightly hypointense

relative to brain is seen on T1WI.

 CPPs are iso- to hyperintense on T2WI and FLAIR.

 Linear and branching internal "flow voids" reflect the increased

vascularity common in CPPs.

 T2* (GRE, SWI) may show hypointense foci secondary to calcification or

intra-tumoral haemorrhage
 Intense homogeneous enhancement is seen following contrast
administration.

 CPPs generally do not restrict on DWI.

 MRS may show elevated myoinositol

 Rare CPP variants include purely cystic CPP and cystic extraaxial
metastases from an intraventricular CPP.

 In purely cystic CPP, a large, often mobile cyst with intensely enhancing
mural nodules is attached to the choroid plexus.

 It can cause sudden obstructive hydrocephalus.

 Purely cystic extraaxial metastases from CPP are seen as non-enhancing

cisternal CSF-like cysts that resemble multiple parasitic cysts, most
commonly neurocysticercosis
 CPP vs aCPP vs CPC
 Distinguishing between CPP and aCPP/CPC on the basis of MRI alone remains
challenging, although both aCPP and CPC have been associated with larger tumor
size, greater extent of peritumoral edema, irregular morphology, and tumor
necrosis, all of which are nonspecific features that suggest greater invasiveness

 Tumor infiltration beyond the ventricles may also suggest malignant pathology

 MRS of CPP and CPC is characterized by a prominent choline peak and absence of
N-acetyl aspartate.

 The myo-inositol level is also reported to be specifically increased in CPPs

 Ultrasound.

 CPPs appear as well-defined, lobular, hyperechoic

 intraventricular masses on transcranial US

 Ddx
 Choroid plexus hyperplasia, aka villous hypertrophy of the choroid
plexus, is a very rare cause of CSF overproduction and shunt-resistant
hydrocephalus.

 Choroid plexus xanthogranulomas are benign incidental lesions that

occur commonly in the lateral ventricular choroid plexus..

 Choroid plexus metastasis occurs in middle-aged and older adults and is

not in the differential diagnosis of a pediatric CPP.
Treatment
 Management of Hydrocephalus
 Because up to 90% of patients present with symptoms of increased ICP secondary to
hydrocephalus, the initial step is to decide whether CSF drainage is required. 1

 In the presence of a decline in neurologic function, however, an EVD should be inserted

immediately, before definitive tumor management.

 Placement of a VPS , although an acceptable alternative, does not allow external CSF
drainage and ICP monitoring in the intraoperative and postoperative setting.

 Furthermore, the presence of intraventricular blood and debris after tumor resection may
lead to blockage of the shunt

 Zhou WJ, et al. Clinical features and prognostic risk factors of choroid plexus tumors in children. Chin Med J (Engl). 2018;131(24):2938–2946.
 37% of surviving patients required a CSF shunt. 1

 2 OTHER STUDIES reported much higher rates of shunt dependency,

ranging from 57% to 78%. 2

1. Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery. 1989;25(3):327–335
2. Humphreys RP, NS, Hendrick EB. Childhood choroid plexus tumors. Con Pedia Neurosurg. 1987;7:1–18
 Preoperative Considerations
 High-resolution MRI before and after gadolinium administration in all three planes is invaluable for
surgical planning and determining the relationship of the tumor to various structures within the
ventricles. Conventional catheter

 Angiography often demonstrates that the vascular supply of CPPs arises from normal choroidal vessels,
which often enlarge as the tumors grow.

 Tumors of the lateral ventricle or third ventricle are generally supplied by branches of the anterior or
posterior choroidal arteries.

 A fourth ventricular tumor receives its blood supply from medullary or vermian branches of the posterior
inferior cerebellar artery.

 Supraselective catheter angiography and embolization of choroidal vessels, when possible, facilitate
surgical resection. 
 Operative treatment
 For both CPP and CPC, GTR is associated with the most favorable outcome in
patients with such tumors.

Operative principles
1. Temporary or permanent resolution of hydrocephalus,
2. Identification of the arterial supply
3. Planning the surgical approach to allow access to the vascular supply and
maximal exposure of the tumor

 Vascularity and large size make resection difficult

 4th ventricular CPT
 Fourth ventricular tumors almost always produce triventricular obstructive hydrocephalus and
may necessitate preoperative CSF drainage.

 Tumors in this location arise from the caudal part of the roof of the fourth ventricle
 The tumor may extend into the lateral recesses or through the foramen of Magendie.
 The approach is through a standard midline posterior fossa craniotomy that exposes the vermis
and tonsils.
 The blood supply from branches of the posterior inferior cerebellar artery are visualized from a
medial vantage point.

 In certain situations, the tumor may extend through the foramina of Luschka, in which case the
tonsil can be reflected laterally to allow access to this area.

 Alternatively, at that sitting or during a staged procedure, a retrosigmoid approach can be used
to reach the space anterolateral to the brainstem
 CHOROID PLEXUS CARCINOMA AND ATYPICAL
CHOROID PLEXUS PAPILLOMA
Surgical Considerations
 Progressive decrease in the likelihood of successful GTR with CPP(79%), aCPP (63%),
and CPC (47%). 1

 Technical considerations with CPC include the expected increased vascularity of the
tumor, the lack of a well-developed plane between the brain and tumor, and
excessive friability of the tumor tissue.

 The rate of recurrence associated with GTR alone suggests that combination therapy,
consisting of chemotherapy and radiation, leads to improved survival, although
definitive guidelines are not available

1. Wrede B, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. 2009;95(3):383–392
 Adjuvant Therapy
 Approximately 20% of patients with CPTs undergo some form of adjuvant therapy,
predominantly in the form of chemotherapy, radiation, or both.

 Although a number of trials have involved the use of various chemotherapy regimens, no
regimen is standard.

 In an international consortium, patients with CPC and atypical CPC were treated with etoposide
(100 mg/m2 daily for 5 days), vincristine (1.5 mg/m2 on day 5), and random assignment to
receive either carboplatin (350 mg/m2 daily for 2 days) or cyclophosphamide (1 g/m2 daily for 2
days).

 In one metaanalysis, of these drugs, etoposide was shown to have the highest response rate. 1

1. Berrak SG, et al. Which therapy works better in choroid plexus carcinomas? J Neurooncol. 2011;103(1):155–162
 Radiation therapy
 Postoperative radiation is usually recommended for children older than 3 years of
age, although this therapy has not been subjected to a clinical trial.

 Radiation is also used in the presence of leptomeningeal dissemination, subtotal

resection, and drop metastases.

 In one series, 10 patients with CPC were treated with chemotherapy, craniospinal
irradiation, or both.
 Some of these patients demonstrated no evidence of disease after chemotherapy alone, but
some required radiation treatment to achieve disease control
 Fitzpatrick and associates noted that after subtotal resection, radiation therapy, either
alone or in combination with chemotherapy, offered a survival advantage. 1

 For aCPPs, the pathologic features and preliminary data suggest that GTR can result in
favourable outcomes, but the disease can recur, sometimes years after the initial
diagnosis. 2
 It is not clear whether chemotherapy for aCPPs after GTR will reduce the likelihood of
recurrence, particularly inasmuch as some CPPs may also recur despite GTR.

 Indeed, more aggressive treatment for CPPs is indicated if features such as metastasis
are present at the time of diagnosis

1. Fitzpatrick LK, Aronson LJ, Cohen KJ. Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely
resected? J Neurooncol. 2002;57(2):123–126.
2. Wrede B, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. 2009;95(3):383–392
 OUTCOMES

 In general, most patients with CPP can expect long-term survival after surgical
resection alone, with an estimated 5-year OS of >90% and a 5-year progression-free
survival (PFS) reported as high as 100%

 Since aCPP is a relatively new diagnostic entity, outcomes are more difficult to
measure. (2007)
Nevertheless, the survival outcomes appear to be in between that
of CPP and CPC
 In a large meta-analysis, the rates of 1-, 5-, and 10-year survival were 90%, 81%, and 77%,
respectively, with CPP, in comparison with only 71%, 41%, and 35%, respectively, with CPC. 1

 Some institutions, however, have reported more favorable outcomes in patients with
aCPP and CPC, with 5-year OS of 96.2% and 64.7%, respectively. 2

1. Wolff JE, et al. Choroid plexus tumours. Br J Cancer.2002;87(10):1086–1091

2. Siegfried A, et al. A French retrospective study on clinical outcome in 102 choroid plexus tumors in children. J
Neurooncol. 2017;135(1):151–160.
 Treatment Outcomes

 Morbidity and mortality associated with treatment are of significant outcome

concern (even in CPPs), especially in children less than 12 months

 Operative mortality: 8 – 10%

 Morbidity :
 Persistent motor sequelae & psychomotor retardation (33%)
 Significant neurocognitive deficits (up to 75%)