Choroid plexuses papilloma: A rare case report
India August 03:
Choroid plexus papillomas (CPPs) are rare intracranial neoplasms of ventricular system of the brain. The fourth ventricle is the most common site of these lesions in adults and lateral ventricles in children. Here we report CPP in a male child presented with the symptoms of increased intracranial pressure. Magnetic resonance imaging show hydrocephalus related to a mass in the lateral ventricle obstructing the foramen of monro. After endoscopic lateral ventriculostomy, tumor was completely excised. Pathological examination shows a typical CPP.
Choroid plexus tumors accounting for less than 1% of all intracranial tumors and 2-4% of all brain tumors in children. it is very rare tumor[1,2]. The most common location in infants and children are the lateral ventricle and in adult are the fourth [2,3]. There is operative challenges to the management of the young child because of the propensity of this tumor for CSF overproduction, and also extreme vascularity which can lead to significant blood loss during surgery [3,4]. We present a case of lateral ventricular choroid plexus papilloma.
A 5-year-old male child presented with moderate to severe headache associated with vomiting for 4 month. On general physical examination, child was conscious, well oriented to time place and person without any neurological deficit. Contrast computed tomography (CT) scan and magnetic resonance imaging show ventriculomegaly and a homogenously enhancing mass in lateral ventricle. Based on the radiographic appearance of this lesion, a presumptive diagnosis of choroid plexus papilloma was made. Hydrocephalus was relieved by Endoscopic left lateral ventriculostomy (ETV) and surgery was done. A tumor was approached by Infratentorial supracerebellar approach by midline sub occipital craniotomy. A proliferating mass was identified at the roof of the left lateral ventricle. The tumor was mildly vascular and non-suckable. Gross total removal of tumor was achieved. In our department we received multiple grey white to red tanned soft tissue bits measuring 1.2X1X0.3 cm altogether amounting to 0.8g. Microscopic examination show normal areas of choroid plexuses papillary fronds [figure 1], some of the papillary fronds show nuclear stratifications along with oncocytic change [figure 2], and along with areas of tubular glandular architecture [figure 3] and xanthomatous change in the papillae of the tumor cells are also seen [figure 4]. These tumor cells were positive for pancytokeratin and negative for GFAP on immunohistochemistry. On the basis of this a diagnosis of choroid plexus papilloma (WHO Grade I) was made.
Figure 1: Photomicrograph shows areas of normal choroid plexuses papillary fronds.
Figure 2: Photomicrograph shows areas of nuclear stratifications along with oncocytic change.
Figure 3: Photomicrograph showing tubular glandular architecture of tumor cells.
Figure 4: Photomicrograph shows xanthomatous change in the papillae.
Choroid plexus papilloma is the neoplasms of the ventricular system, but it is very rare tumor of the brain. In children, 80% of CPP arise in the lateral ventricle, 16% in the fourth ventricle, and 4% in the third ventricle. These tumors are more common in male subjects [3,7,8]. Choroid plexus papillomas are derived from neuroectoderm and considered to be a benign neoplasm, correspond to WHO Grade I; Grade II is reserved for atypical CPPs and grade III for the choroid plexus carcinoma. These tumors are benign in behavior so these tumors are often curative with the complete resection and there is little chances of recurrence. These tumors require special challenge in management due to younger age of presentation, high potential for CSF overproduction and the vascularity of these lesions.[2,4,9] The vascularity of these lesions is often due to the rich anastmotic bed of choroidal blood vessels. Due to the rich vascular supply a preoperative endovascular Embolisation has been used in an attempt to reduce blood loss intraoperatively. surgical excision is the treatment of choice of this tumor. Total excision is usually achieved as many as 96% of cases. Mortality rates related to surgery attributed to blood loss have been reported to be as high as 12%. [2,4,9,10] this tumor is approached by various methods but the most preferred approach for midline tumor is infratentorial supracerebellar approach.
CPPs have an excellent prognosis with long term survival after gross total resection, varies from 90% to 100%. Malignant tumors are treated aggressively by adjuvant chemotherapy.
- Buckle C, Smith JK. Choroid plexus papilloma of the third ventricle. Pediatr Radiol. 2007;37:725. [PubMed]
- Ogiwara H, Dipatri AJ, Jr, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. Br J Neurosurg. 2012;26:32–7. [PubMed]
- Kroppenstedt SN, Golfinos J, Sonntag VK, Spetzler RF. Pineal region lesion masquerading choroid plexus papilloma: Case report. Surg Neurol. 2003;59:124–7. discussion 127. [PubMed]
- Krishnan S, Brown PD, Scheithauer BW, Ebersold MJ, Hammack JE, Buckner JC. Choroid plexus papillomas: A single institutional experience. J Neurooncol. 2004;68:49–55. [PubMed]
- Tsumoto T, Nakai E, Uematsu Y, Nakai K, Itakura T. Choroid plexus papilloma in the posterior third ventricle in infancy: A case report. No Shinkei Geka. 1999;27:673–8. [PubMed]
- Wind JJ, Bell RS, Bank WO, Myseros JS. Treatment of third ventricular choroid plexus papilloma in an infant with embolization alone. J Neurosurg Pediatr. 2010;6:579–82. [PubMed]
- Carson BS, Weingart JD, Guarnieri M, Fisher PG. Third ventricular choroid plexus papilloma with psychosis. Case report. J Neurosurg. 1997;87:103–5. [PubMed]
- Karim A, Fowler M, McLaren B, Cardenas R, Patwardhan R, Nanda A. Concomitant choroid plexus papillomas involving the third and fourth ventricles: A case report and review of the literature. Clin Neurol Neurosurg. 2006;108:586–9. [PubMed
- Menon G, Nair SN, Baldawa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV. Choroid plexus tumors: An institutional series of 25 patients. Neurol India. 2010;58:429–35. [PubMed]
- Sarkar C, Sharma MC, Gaikwad S, Sharma C, Singh VP. Choroid plexus papilloma: A clinicopathological study of 23 cases. Surg Neurol. 1999;52:37–9. [PubMed]