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|Year : 2021 | Volume
| Issue : 3 | Page : 729-732
Cerebello-pontine and Cerebello-medullary Fissure Choroid Plexus Papilloma in a Child – Case Report and Review of Literature
K Sridhar, Sushanth Subramaniam
Department of Neurosurgery, Institute of Neurosciences and Spine, MGM Healthcare, Chennai, Tamil Nadu, India
|Date of Submission||28-May-2020|
|Date of Decision||15-Sep-2020|
|Date of Acceptance||03-Oct-2020|
|Date of Web Publication||24-Jun-2021|
Dr. K Sridhar
Department of Neurosurgery, Institute of Neurosciences, Room 123, First Floor, MGM Healthcare, 72, Nelson Manickam Road, Chennai, Tamil Nadu - 600 029
Source of Support: None, Conflict of Interest: None
Choroid plexus papillomas (CPP) are commonly seen in the supratentorial compartment in children and only very rarely in the posterior fossa. CPP in the cerebello-pontine angle and cerebello-medullary fissure (CPA) in the pediatric age group are extremely rare with only seven previous cases reported in literature. The authors present the case of a 7-year-old girl who presented with neck tilt, imbalance, and headache. The MRI showed a lesion with a frond-like appearance in the CPA with heterogeneous enhancement. The tumor was excised radically using a cerebello-medullary fissure approach. The authors review and analyze the literature on this rare pediatric tumor, with specific attention to radiology and the surgical aspects.
Keywords: Cerebello-medullary fissure, cerebello-pontine angle, choroid plexus papilloma, pediatricKey Messages: CPP of the CPA are rare, especially in the pediatric age. They can have a varied appearance on radiology, with a few specific characteristic features. They rarely cause hydrocephalus and can be removed relatively easily as they are not as vascular as their supratentorial counterparts.
|How to cite this article:|
Sridhar K, Subramaniam S. Cerebello-pontine and Cerebello-medullary Fissure Choroid Plexus Papilloma in a Child – Case Report and Review of Literature. Neurol India 2021;69:729-32
|How to cite this URL:|
Sridhar K, Subramaniam S. Cerebello-pontine and Cerebello-medullary Fissure Choroid Plexus Papilloma in a Child – Case Report and Review of Literature. Neurol India [serial online] 2021 [cited 2021 Jul 25];69:729-32. Available from: https://www.neurologyindia.com/text.asp?2021/69/3/729/319229
Choroid plexus papillomas (CPP) are uncommon tumors occurring more commonly in children than in adults.,,, Posterior fossa CPP are almost exclusively seen in adults. CPP in the cerebello-pontine and cerebello-medullary fissures (CPP-CPA) are very rare in children with only seven cases reported in literature. We describe the case of a child with a CPP CPA and review the relevant literature.
| » Case History|| |
A 7-year-old girl presented with head tilt and increasing imbalance for 18 months and occipital headache for 9 months. On examination, the child had bilateral coarse nystagmus with imbalance on walking and left-sided incoordination.
MRI brain plain and contrast [Figure 1] showed a well-defined lobulated mass lesion in the left cerebello-medullary cistern, extending below the level of the foramen magnum, with a frond-like pattern in T2W images. Contrast scans showed heterogeneous enhancement of the lesion, with the hyperintense areas in T2W showing intense enhancement, and T2W isointense areas showing moderate enhancement.
|Figure 1: (a)MRI brain plain showed a well-defined lobulated mass lesion in the left cerebello-medullary cistern, hypointense on T1W images with mass effect over the medulla. (b,c) The lesion is heterogeneous in T2W images with isointense and hyperintense regions. The lesion extends below the level of the foramen magnum. (d,e,f) Post-gadolinium axial and coronal T1-weighted images show moderate to intense heterogeneous enhancement of the lesion, with the hyperintense appearing areas in T2W showing intense enhancement, while the T2W isointense areas showing moderate enhancement|
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A left posterolateral approach with C1 hemilaminectomy reaching the vertebral artery was sued to approach the lesion [Figure 2]. On opening the dura, the lesion was seen as a reddish tumor with a multilobulated surface typical of a CPP [Figure 3]. The lesion was followed into the foramen of Lushka and removed. There was no definite connection to the choroid plexus. Radical excision of the tumor was done. Postoperatively, she developed difficulty in swallowing, which resolved in 8 weeks when she became asymptomatic.
|Figure 2: The surgery was done in the prone position. An inverted L-shaped incision was used to allow a left unilateral low subocciptal craniotomy including the foramen magnum and a unilateral removal of the posterior arch of Atlas More Details|
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|Figure 3: Intraoperative photograph showing tumour exposed using the unilateral cerebello-medullary fissure approach. The typical mulberry type appearance of a CPP tumour (T) is seen. The lesion is relatively avascular and has been dissected off the XI cranial nerve (XIn)|
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Histological examination [Figure 4] showed a histology and IHC typical for a CPP. The child on follow-up of 8 months is neurologically normal with the last MRI done at 6 months follow-up showing no residual or recurrent tumor [Figure 5].
|Figure 4: (a) Microphotograph (x10) showing a papillary tumour with delicate fibrovascular connective tissue fronds lined by cuboidal to columnar epithelial cells with round to oval basally located monomorphic nuclei, (b) the same at × 40, (c) reticulin staining showing the fibrovascular strands, and (d) IHC S-100 positivity|
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|Figure 5: MRI scan taken at 6-month follow-up shows radical excision of the tumor with no recurrence|
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| » Discussion|| |
CPP form approximately 0.4–0.6% of all intracranial tumors and 10–20% of all infant brain tumors.,,,,, Only 9% of all choroid plexus tumors are found in the cerebello-pontine angle and almost exclusively in adults., CPP- CPA is extremely rare in children with only seven cases reported in the literature.,,,,, Lena et al. reported on 24 children with Choroid plexus tumors; none of which were in the CPA. Luo et al. reported on a series of 21 cases of CPP-CPA of which only two were children. Mehta et al. reported a 19-year-old mentally challenged girl with a CPP-CPA.
Hammock et al. in 1976 reported the first child with a CPP-CPA. Since then, seven cases have been reported in the literature [Table 1]. The children were between 5 and 14 years of age. Headache, vomiting, cranial nerve palsies, and cerebellar signs were the common presentations. Piguet and Triboulet reported on a 5-year-old child who was presented with subarachnoid haemorrhage. The radiological features in children are similar to that in adults., CPPs present on the CT scan mostly as homogeneous lesions with intense enhancement, with or without hydrocephalus. Only 1 out of 12 patients of Talachhi et al. had a nonhomogeneous CPP with nonhomogeneous enhancement on the CT scan. Calcification on CT scan which has been reported in 4–20% of cases may be patchy or dense., On MRI, CPPs present as a well-defined multilobulated cauliflower or mulberry shaped mass, which is homogeneous and T1W iso or hypointense and T2W iso to hyperintense lesions, often associated with adjacent flow voids., While cysts may be seen in up to 20% of CPP, none of the children with CPP-CPA had a cyst. Approximately 25% may be heterogeneous on plain MRI scans. On contrast MRI, they enhance intensely and homogeneously. Heterogeneous enhancement may occasionally be seen., The poor enhancement has been thought to correlate with poor vascularity of the tumor.,, In our patient, the enhancement was heterogeneous; with poor vascularity in the nonenhancing areas and moderate vascularity in the enhancing areas. Hydrocephalus may or may not be seen in CPP-CPA.,, We found some degree of ventricular dilatation in five of the eight children reported, including our patient with only one needing a preoperative shunt. The lower degree of hydrocephalus in the CPP-CPA may be due to the decreased blood supply in these tumors as well as a lesser degree of obstruction of CSF pathways. The mild extent of hydrocephalus is one of the five points that help in the preoperative diagnosis of a primary CPP-CPA. The role of angiography has been debated by authors.,,, While tumor blush may or may not be present, Zhang defined four other characteristic features that are unique to primary CPP-CPA. However, with high-resolution MRI and the risk in children, DSA may not be necessary,
The surgical principle for the excision of these vascular tumors has been centered on the initial step of the devascularization of the tumor by early identification of the feeders. The retromastoid, cerebello-medullary fissure or the far lateral approaches have been utilized to remove these tumors,,, In our patient, the tumor was in the cerebello-medullary cistern extending medially into the foramen of Luschka and inferiorly to the Foramen magnum. We routinely use a postero-lateral skull base approach to the lateral cerebello-medullary cistern that can be extended upwards as needed. Origin of the tumor, from the choroid plexus at the foramen of Luschka, may or may not be evident at the surgery. Radical tumor excision has been recommended and reported by most authors for a good long-term outcome.,, Of the eight children with CPA-CPP, in only one patient was a subtotal excision done due to adherence to cranial nerves and the brain stem [Table 1]. While en-bloc resection to reduce blood loss is possible in smaller tumors, it is impossible in larger lesions especially those seen in the CPA. When operating on children, it is, therefore, necessary to keep in mind that due to low blood volume, life threatening blood loss should be preempted and prevented. Preoperative embolization as well as preoperative chemotherapy has been tried in children mainly in supratentorial tumors but not used., In children with CPP-CPA, the common complications reported after surgery include pneumocephalus, pneumoventricle, subdural effusion worsening of lower cranial nerve palsies and cerebellar signs, and hydrocephalus., Hammock et al. reported postoperative hydrocephalus requiring a shunt procedure.
The prognosis for patients with CPP undergoing radical excision is excellent. All the children with CPP- CPA have shown a good outcome with no recurrence at follow up.
| » Conclusion|| |
The authors report a rare case of a Choroid plexus papilloma of the cerebello-pontine angle in a child, the eighth case to be reported in the literature. These are difficult cases to diagnose, though with high-resolution MRI scans this has become easier. Utilization of appropriate skull base approaches and microsurgical techniques make radical excision with good outcomes possible in a majority of these children.
The authors would like to acknowledge the help extended by Dr Pradeep for the pathological inputs and Dr Parthiban for the radiological inputs of this case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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