Uncommon presentation of choroid plexus papilloma in an infant.

Choroid plexus tumors are relatively rare primary brain tumors that arise from the epithelial differentiated tissue, majority being well-differentiated papillomas. In adults, fourth ventricle and in children, lateral ventricles are the most common site of these tumors. We reported a case of choroid plexus papilloma in the temporal horn of lateral ventricle in a female child who presented with the uncommon symptoms of sudden intraventricular hemorrhage and multiple episodes of seizure without symptoms of raised intracranial tension.

Introduction

Falling under the category of rare intraventricular tumors, choroid plexus tumors account for <1% of all intracranial tumors, of which it forms only 2–4% of brain tumors in pediatric age group.[12] The majority of these tumours occurs in children (70%), of which at least 50% present before 2 years of age.[3] Fourth ventricle is the most common site in adults whereas in infants and children, it is most commonly seen in lateral ventricles.[24] On histology, a majority of these neoplasms are well-differentiated choroid plexus papillomas (CPPs). In the emergency department, we witnessed a case of CPP of temporal horn of lateral ventricle with an unusual presentation of sudden spontaneous hemorrhage, which was managed using a superior temporal sulcus approach to the lateral ventricle and complete resection of the tumor with left temporoparietal craniotomy.

Case Report

A 9-month-old female child presented with sudden loss of consciousness and multiple episodes of seizure in the emergency. Her vitals were within normal limits. Glasgow Coma Scale was E3V3M5. Computed tomography (CT) imaging showed hyperdense lesion measuring 3.6 cm × 3 cm in the left temporal horn of lateral ventricle [Figure 1] with features suggestive of left temporal horn hemorrhagic space occupying lesion with hemorrhage in left lateral ventricle. Contrast-enhanced CT angiography showed evidence of well-defined, strongly homogenous, enhancing polypoidal intraventricular mass lesion measuring approximately 30 mm × 38 mm × 33 mm in anterior-posterior, transverse, and craniocaudal axis, respectively, in temporal horn of left lateral ventricle with punctuate calcification and intense homogenous enhancement in arterial phase with persistent enhancement in venous phase. Evidence of mild to moderate dilatation of ventricles with intraventricular hemorrhage in left lateral ventricle was seen [Figure 2]. CT angioghaphy was suggestive of choroid plexus papilloma with intraventricular hemorrhage.

Figure 1

Computed tomography imaging showing hyperdense hemorrhagic lesion in the left temporal horn of lateral ventricle with hemorrhage in left lateral ventricle

Figure 2

Contrast-enhanced angiography showing evidence of well-defined, strongly homogenous, enhancing polypoidal intraventricular mass lesion both in arterial phase and venous phase with hemorrhage in left lateral ventricle

The patient underwent left temporoparietal craniotomy via superior sulcus approach to the lateral ventricle. A cauliflower-like mass was observed in the temporal horn of lateral ventricle with an attachment to the choroid plexus of the lateral ventricle which was vascular and nonaspirable [Figure 3]. There was hemorrhagic clot with fresh bleed in the ventricle. Total excision of tumor was done. Postoperatively, her condition did not improve in the next 3–4 days. A repeat noncontrast CT showed evidence of small extra-axial hypodense cerebrospinal fluid (CSF) attenuating area overlying frontotemporal lobe suggestive of subdural hygroma, dilated both lateral, third and fourth ventricles suggestive of moderate communicating hydrocephalus with evidence of bilateral cerebellar hemisphere encephalomalacia [Figure 4]. This was taken care by medium pressure ventriculoperitoneal shunting, and postoperative period was uneventful as the patient did not develop any neurological deficits. Histopathological examination was consistent with the diagnosis of CPP [Figure 5].

Figure 3

A cauliflower-like vascular mass in the temporal horn of lateral ventricle with an attachment to the choroid plexus

Figure 4

Noncontrast computed tomography showing dilated both lateral, third and fourth ventricles suggestive of moderate communicating hydrocephalus

Figure 5

(a) Papillary fronds lined by single layer of cells (H and E, ×40). (b) Delicate fibrovascular connective tissue fronds covered by single layer of uniform cuboidal to columnar epithelial cells with round to oval, basally situated nuclei (H and E, ×200)

Discussion

CPPs originate from neuroectodermal tissues are benign in nature, assigned as WHO Grade I. Choroid plexus tumors (CPTs) commonly present with features of raised intracranial tension.[567] Excessive production of CSF by the tumor leads to raised intraventricular pressure.[7] Besides this, obstruction of CSF flow due to large tumors[8] or impaired CSF absorption at the subarachnoid space secondary to hemorrhage or proteinaceous material from these highly vascular tumors can be the cause of hydrocephalus. Although radiological findings were observed as expected, clinically this was an unusual presentation with sudden spontaneous hemorrhage and seizure episodes without any features of raised intracranial tension.

As far as treatment of CPP is concerned, complete surgical excision of the tumor should be the aim. Several surgical approaches have been defined for lateral ventricle CPPs. We preferred a transsulcal approach from the most posterior portion of the superior temporal sulcus. Advantage of using this approach was direct access to the posterior one-third of the lateral ventricle, called the trigone. Due to large size and high vascularity of these tumors, achieving total resection is difficult in young children as the circulating blood volume is proportionately small. Importantly, mortality rates due to blood loss have been reported to be as high as 12%.[91011] To reduce the blood loss during surgery, in pediatric patients, gentle and repetitive bipolar coagulation of the tumor surface with constant irrigation so as to shrink the tumor and remove it completely is preferred compared to excision in piecemeal. Surgical complications adding to mortality range from 0% to 25%.[1011] Since CPTs are supplied by a rich anastamotic bed of choroidal vessels role of endovascular embolization as a preoperative adjunct has also been tried to reduce blood loss intraoperatively.[12]

Long-term survival rates range from 90% to 100% with gross total resection. For malignant tumors and tumors with leptomeningeal spread adjuvant therapy is employed.[1011] Adjuvant therapy role is still controversial and reserved for recurrent and residual lesions.

Thus, CPTs are rare intracranial tumors. Since CPPs have good prognosis, timely surgical intervention aimed at complete surgical excision with efforts to reduce blood loss and regular follow-up is, therefore, the best treatment strategy to manage these patients.

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Conflicts of interest

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