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Case series of choroid plexus papilloma in children at uncommon locations and review of the literature.

Prasad GL, Mahapatra AK - Surg Neurol Int (2015)

Bottom Line: Two cases presented with features of obstructive hydrocephalus while the latter presented with compressive effects.Complete removal leads to excellent long-term survival rates.Adjuvant treatment is not required.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT

Background: Choroid plexus papillomas (CPPs) comprise around 1% of intracranial neoplasms. The most common location is atrium of the lateral ventricle in children and fourth ventricle in adults. Other rare locations include third ventricle, cerebellopontine (CP) angle and cerebral parenchyma, with only a few cases reported. Authors report three cases of CPP at uncommon locations in pediatric patients. The rarity of these locations, diagnostic dilemma and management aspects are discussed along with an extensive review of the literature.

Methods: Retrospective institutional data analysis of histopathologically confirmed pediatric CPPs from 2010 to 2014.

Results: Authors noted three cases of CPP in children in uncommon locations-one each in the posterior third ventricle, fourth ventricle, and CP angle. All were males in the first decade. Two cases presented with features of obstructive hydrocephalus while the latter presented with compressive effects. Complete excision was achieved in two cases while subtotal removal was performed in one case (fourth ventricular) because of excess blood loss. Mean follow-up duration was 24.6 months (range 20-30 months). One case (of subtotal removal) had fair recovery while other two had excellent outcomes.

Conclusions: Posterior third ventricle, fourth ventricle, and CP angle are uncommon locations for these tumors in children. Complete surgical removal is the treatment of choice and approach needs to be tailored according to the site and size of the lesion. Blood loss is a major concern in young children as they are highly vascular tumors. Complete removal leads to excellent long-term survival rates. Adjuvant treatment is not required.

No MeSH data available.


Related in: MedlinePlus

Case 2. Noncontrast computed tomography showing a heterogeneously hyperdense lesion in the midline posterior fossa with small peripheral cysts (black arrow) (a). A mild extension to the left cerebellopontine angle can be noted. Axial T2-weighted magnetic resonance images showing the lesion to be heterogeneously hyperintense (b). Axial and sagittal postcontrast magnetic resonance images showing intense, homogeneous enhancement of the lesion (c and d)
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Figure 3: Case 2. Noncontrast computed tomography showing a heterogeneously hyperdense lesion in the midline posterior fossa with small peripheral cysts (black arrow) (a). A mild extension to the left cerebellopontine angle can be noted. Axial T2-weighted magnetic resonance images showing the lesion to be heterogeneously hyperintense (b). Axial and sagittal postcontrast magnetic resonance images showing intense, homogeneous enhancement of the lesion (c and d)

Mentions: A 5-year-old boy presented with gait ataxia of 7 months duration and 2 months of headache and nasal regurgitation. Child was drowsy, but following commands; had a visual acuity of finger counting close to face, papilledema, and impaired gag reflex bilaterally. A noncontrast CT head showed a hyperdense midline posterior fossa mass lesion involving the fourth ventricle extending into the left foramen of Lushka with obstructive hydrocephalus. On MRI, the lesion was isointense on T1 and heterogeneously hyperintense on T2 sequences, showed intense enhancement and measured 4.8 cm × 3.5 cm × 5.2 cm [Figure 3a-d]. A VP shunt was inserted in view of his alteration in sensorium. With a presumptive diagnosis of an ependymoma, the patient underwent midline suboccipital approach in the prone position. Intra-operatively, the tumor was friable, very vascular, and reddish-pink in appearance. Only, a subtotal excision could be achieved because of excessive blood loss. Imaging (noncontrast CT) done in the postoperative period showed operative site hematoma which had to be evacuated on an emergency basis. Child showed gradual improvement and was doing well at the last follow-up of 20 months after surgery, with no regrowth of the residual lesion. Histopathological features were compatible with a diagnosis of CPP.


Case series of choroid plexus papilloma in children at uncommon locations and review of the literature.

Prasad GL, Mahapatra AK - Surg Neurol Int (2015)

Case 2. Noncontrast computed tomography showing a heterogeneously hyperdense lesion in the midline posterior fossa with small peripheral cysts (black arrow) (a). A mild extension to the left cerebellopontine angle can be noted. Axial T2-weighted magnetic resonance images showing the lesion to be heterogeneously hyperintense (b). Axial and sagittal postcontrast magnetic resonance images showing intense, homogeneous enhancement of the lesion (c and d)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4596056&req=5

Figure 3: Case 2. Noncontrast computed tomography showing a heterogeneously hyperdense lesion in the midline posterior fossa with small peripheral cysts (black arrow) (a). A mild extension to the left cerebellopontine angle can be noted. Axial T2-weighted magnetic resonance images showing the lesion to be heterogeneously hyperintense (b). Axial and sagittal postcontrast magnetic resonance images showing intense, homogeneous enhancement of the lesion (c and d)
Mentions: A 5-year-old boy presented with gait ataxia of 7 months duration and 2 months of headache and nasal regurgitation. Child was drowsy, but following commands; had a visual acuity of finger counting close to face, papilledema, and impaired gag reflex bilaterally. A noncontrast CT head showed a hyperdense midline posterior fossa mass lesion involving the fourth ventricle extending into the left foramen of Lushka with obstructive hydrocephalus. On MRI, the lesion was isointense on T1 and heterogeneously hyperintense on T2 sequences, showed intense enhancement and measured 4.8 cm × 3.5 cm × 5.2 cm [Figure 3a-d]. A VP shunt was inserted in view of his alteration in sensorium. With a presumptive diagnosis of an ependymoma, the patient underwent midline suboccipital approach in the prone position. Intra-operatively, the tumor was friable, very vascular, and reddish-pink in appearance. Only, a subtotal excision could be achieved because of excessive blood loss. Imaging (noncontrast CT) done in the postoperative period showed operative site hematoma which had to be evacuated on an emergency basis. Child showed gradual improvement and was doing well at the last follow-up of 20 months after surgery, with no regrowth of the residual lesion. Histopathological features were compatible with a diagnosis of CPP.

Bottom Line: Two cases presented with features of obstructive hydrocephalus while the latter presented with compressive effects.Complete removal leads to excellent long-term survival rates.Adjuvant treatment is not required.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT

Background: Choroid plexus papillomas (CPPs) comprise around 1% of intracranial neoplasms. The most common location is atrium of the lateral ventricle in children and fourth ventricle in adults. Other rare locations include third ventricle, cerebellopontine (CP) angle and cerebral parenchyma, with only a few cases reported. Authors report three cases of CPP at uncommon locations in pediatric patients. The rarity of these locations, diagnostic dilemma and management aspects are discussed along with an extensive review of the literature.

Methods: Retrospective institutional data analysis of histopathologically confirmed pediatric CPPs from 2010 to 2014.

Results: Authors noted three cases of CPP in children in uncommon locations-one each in the posterior third ventricle, fourth ventricle, and CP angle. All were males in the first decade. Two cases presented with features of obstructive hydrocephalus while the latter presented with compressive effects. Complete excision was achieved in two cases while subtotal removal was performed in one case (fourth ventricular) because of excess blood loss. Mean follow-up duration was 24.6 months (range 20-30 months). One case (of subtotal removal) had fair recovery while other two had excellent outcomes.

Conclusions: Posterior third ventricle, fourth ventricle, and CP angle are uncommon locations for these tumors in children. Complete surgical removal is the treatment of choice and approach needs to be tailored according to the site and size of the lesion. Blood loss is a major concern in young children as they are highly vascular tumors. Complete removal leads to excellent long-term survival rates. Adjuvant treatment is not required.

No MeSH data available.


Related in: MedlinePlus