Surgical Removal of a Huge Atypical Choroid Plexus Papilloma

KISEP J Korean Neurosurg Soc 35 : 517-519, 2004

Surgical Removal of a Huge Atypical Choroid Case Report Plexus Papilloma

Sang-Kook Lee, M.D., Kyu-Man Shin, M.D ., Do-Sang Cho, M.D., Myung-Hyun Kim, M.D. Department of Neurosurgery, Mokdong Hospital, Ewha Medical Center, Seoul, Korea

The authors present a case of huge atypical choroid plexus papilloma in both lateral and third ventricles. A 2-year- old girl presented with lethargy and vomiting. Magnetic resonance images disclosed a huge enhancing mass, which probably arose from the left lateral ventricle and extended to the right lateral and the third ventricles. Total removal of the mass via the interhemispheric transcallosal approach was done. Histologically, an atypical choroid plexus papilloma was identified. Second operative treatment, subduro-peritoneal shunt, was performed due to postoperative subdural effusion. The patient was discharged two weeks after the second surgical procedure without a neurological deficit.

KEY WORDS : Choroid plexus papilloma Transcallosal approach Subdural effusion.

Introduction Case Report

horoid plexus tumors are rare, composing 0.4 to 0.6% of 2-year-old girl presented with vomiting and lethargy. C all intracranial tumors in all ages11,13). Choroid plexus A She had a history of poor oral intake for three months. neoplasms are primary tumors of childhood. They occur more On a neurological examination, the patient was drowsy. She than 70% in children, and at least 50% presented in less than 2 had long tract signs, including an increased deep tendon reflex years5). They are most often found in the lateral ventricles and and positive bilateral ankle clonus. less commonly in the fourth ventricle in children, and these A preoperative computed tomography(CT) scan revealed a tumors generally localized in the fourth ventricle and its lateral huge lobulated mass lesion in the lateral ventricles extended to recess in adults1, 15). the third ventricle with hydrocephalus. She underwent an Some rare cases of bilateral choroid plexus papilloma have extraventricular drainage due to a progressive deterioration of been published6,14). Matson and Crofton14) did note that bilateral her mental status. choroid plexus papillomas represent 7% of their cases. Third Magnetic resonance imaging(MRI) disclosed a huge ventricular choroid plexus neoplasms in pediatric series intraventricular tumor, which probably arose from the left represent average 10% with ranging from 7% to 29%5,10,14). lateral ventricle and extending to the right lateral and the third Choroid plexus tumors arise from the neuroepithelial lining ventricle(Fig. 1). The mass lesion was enhanced densely and of the ventricular choroid plexus and their histologic and homogeneously and linear enhancement was noted in biologic properties demonstrate widely from the benign prepontine cistern suggesting leptomeningeal seeding. A papillomas to highly anaplastic and infiltrative carcinoma. presumptive diagnosis of a choroid plexus papilloma was We report a 2-year-old patient with atypical choroid plexus made. papilloma of the lateral and third ventricle with literature The mass lesion was resected via anterior interhemispheric review. transcallosal approach. At surgery, a huge whitish to pinkish lobulated soft tissue mass lesion was exposed, encapsulated by a thin layer of fibrous tissue. A gross total removal was Received：July 28, 2003 Accepted：December 26, 2003 achieved by piecemeal Address for reprints：Kyu-Man Shin, M.D., Department of Histologically, an atypical choroid plexus papilloma was Neurosurgery, Mokdong Hospital, Ewha Medical Center, 911-1 Mok-6-dong, Yangcheon-gu, Seoul, Korea identified(Fig. 2). The microscopic examination of the tumor Tel : 02) 2650-2651, Fax : 02) 2655-0948 showed the histological features of a choroid plexus tumor. E-mail : shin47ns @ orgio.net However, the slightly higher number of hyperchromatic nuclei and frequent mitoses were identified. There were also

VOLUME 35 May, 2004 517 Atypical Choroid Plexus Papilloma

complex combination of CSF overproduction and limited outflow may be the cause in many patients. Also in our case, a combination of CSF overproduction and outflow obstruction may be the contributional factor to the hydrocephalus. Matson and Crofton14) have reported that the successful removal of a tumor obviate A B C the need for shunt. However, other factors, such as blood in the ventricle, arachnoid Fig. 1. T1-weighted magnetic resonance axial(A) and sagittal(B) images with gadolinium enhancement, and T2-weighted coronal image(C) revealing a huge intraventricular scarring, and postoperative meningitis may enhancing mass lesion with linear thin enhancement in prepontine cistern suggesting render the patient shunt dependent. leptomeningeal seeding, which probably arose from the left lateral ventricle and Ellenbogen and colleagues5) reported that 37 extending to the right lateral and the third ventricle. percent of surviving patients require psammoma bodies. Immunostaining of S-100, and cytokeratin shunting in his series. Lena et al12) reported much higher rates showed diffuse positive reactions with focal immunoreac- of shunt placement. tivities to vimentin. Plain films may show the usual evidence of increased A postoperative CT scan demonstrated intraventricular intracranial pressure and calcification. The introduction of CT hemorrhage, which was resolved by extraventricular drainage. and MRI improved the safety and accuracy of diagnosis of On the 11th postoperative day, a slight mental deterioration these tumors. Especially, MRI is the diagnostic imaging study developed and MRI showed a subdural fluid collection in the of choice because of the detailed anatomical delineation and left fronto-parietal area(Fig. 3). Therefore, a subduro-peritoneal triplanar imaging ability. Choroid plexus papilloma could have shunt was performed. The patient was discharged 2 weeks after either homogeneous or heterogeneous intensity on MRI and the second surgical procedure without a neurological deficit. usually enhances intensely and homogeneously3,7). The gross appearance of choroid plexus papilloma appears a Discussion cauliflower like surface and generally well-circumscribed mass arising from within the ventricle. Microscopically, choroid n 1832, Guerard8) described the first case of a choroid plexus papillomas typically appear as a single layer of cuboidal I plexus tumor in an autopsy specimen of 3-year-old girl. epithelial cells surrounding a delicate fibrovascular stalk, Rare case of bilateral ventricles was also noted6). In our patient, arranged in papillary configuration with finger-like projection a huge lobulated mass was located in the lateral ventricle body of tissues. On occasion, a choroid plexus tumors may demonst- and frontal horn, more on the left than right side, involving rate some atypical histopathological features without frank third ventricle. The lateral and third ventricles were enlarged evidence of invasion. Microscopic features include increased but aqueduct and fourth ventricle appeared to be normal, which cellularity, mitoses, nuclear pleomorphism and poorly formed suggests the obstruction at foramen of Monro and proximal papillary structures. These intermediate tumors may poses one aqueduct. The tumor in the left lateral ventricle seems to be or two of these feat- extension of tumor tissues through foramen to right lateral and ures and are called third ventricle. or termed atypical The hydrocephalus is cardinal manifestation of choroid pap-illoma, but do plexus neoplasm. Probably it is the result of obstruction of CSF not necessarily have pathway or over-production of CSF. Over production of CSF is a more aggressive major contribution factor9,15). The resolution of hydrocephalus na-tural history and has been reported after complete resection of choroid plexus are not classified as tumor, supporting the basis that over secretion of CSF was carcinoma4). The hi- responsible for the hydrocephalus. Nevertheless, hydroce- stologic examination Fig. 2. Photomicrograph shows the phalus persists in one-third to one-half of patients with choroid of our patient was histology of the atypical choroid plexus papilloma. There are slightly higher plexus neoplasms and require CSF shunt procedure after concordant with the number of hyperchromatic nuclei and complete tumor removal5,17). Rekate et al16) demonstrated that a features of these in- frequent mitoses(arrow) (H & E, 400).

518 J Korean Neurosurg Soc 35 SK Lee, et al.

ternediate tum- removal of the choroid plexus papilloma is the most important ors. So it was di- determinant of the prognosis. agnosed as a atypical choroid ple- Conclusion xus papilloma. In general, tran- he authors report a case of huge atypical choroid plexus scortical and tr- T papilloma and suggest the extent of a surgical excision of anscallosal app- a choroid plexus papilloma may be the most important factor roaches have be- for preventing post-surgical complications and determining the en used for the prognosis. surgical removal of the choroid pl- References exus tumors. The 1. Baek KH, Kim TY, Kim JM : Encapsulated Choroid Plexus Fig. 3. T2-weighted magnetic resonance main determining Papilloma with Surrounding Large Cyst. J Korean Neurosurg Soc coronal image on the 11th postoperative day 28 : 1727-1731, 1999 showing a subdural fluid collection in the left factors of choice 2. Boyd MC, Steinbok P : Choroid plexus tumors : Problems in fronto-temporo-parietal area and marked of surgical app- diagnosis and management. J Neurosurg 66 : 800-805, 1987 midline shifting. roach were preo- 3. Coates TL, Hinshaw DB, Peckman N, Thompson JR, Hasso AN, Holshouser BA, et al : Pediatric choroid plexus neoplasms. MR, CT, perative condition of patient, location of tumor, age of patient and pathologic correlation. Radiology 173 : 81-88, 1989 and vascular supply. Velasco-Siles and Raimondi18) provided a 4. Ellenbogen RG, Donovan DJ : Choroid plexus tumors in Keating RF, Goodrich JT, Packer RJ(eds) : Tumors of the Pediatric detailed outline of surgical approaches and pitfalls in the Central Nervous System, New York : Thieme, 2001, pp339-350 resection of choroid plexus papilloma. In selected patients, 5. Ellenbogen RG, Winston KR, Kupsky WJ : Tumors of the choroid transcallosal approach is useful, because this permits the access plexus in children. Neurosurgery 25 : 327-335, 1989 6. Erman T, Gocer AI, Erdogan S, Tuna M, Ildan F, Zorludemir S : to the mass in the ventricle with low risk of neuropsychological Choroid plexus papilloma of bilateral lateral ventricle. Acta sequelae and seizure. Total and en bloc tumor excision is Neurochir 145 : 139-143, 2003 14) 7. Girardot C, Boukobza M, Lamoureux JP, Sichez JP, Capellel, Zoua- currently performed and recommended . En block excision oui A, et al : Choroid plexus papilloma of the posterior fossa in can't be done in large tumors because sometimes it is very adults : MR imaging and gadolinium enhancement. J Neuroradiol 15,17) 17 : 303-318, 1990 difficult to mobilize a large mass . Therefore the authors 8. Guerard M : Tumeur Fongeuse dans le ventricle droit ducerveau performed gross total resection with peacemeal removal. chez une petite fille de trois ans. Anat Paris 8 : 211-214, 1832 One of the most problematic complication of intraventricular 9. Johnson DL : Management of choroid plexus tumors in children. Pediatr Neurosci 15 : 195-206, 1989 surgery for the choroid plexus neoplasm is the development of 10. Jooma R, Grant D : Third ventricle choroid plexus papillomas. symptomatic subdural fluid collections2,14). Koos et al11) thought Childs Brain 10 : 242-250, 1983 11. Koos W, Miller M : Intracranial Tumors of Infants and Children. that a preoperative shunt procedure allows resolution of the St. Louis : Mosby, 1971, pp239-244 hydrocephalus and the decreases the risk of post operative 12. Lena G, Genitori L, Molina J, Legatte JR, Choux M : Choroid plexus subdural fluid collection in the majority of cases. Boyd and tumors in children. Review of 24 cases. Acta Neurochir(Wien) 106 : 68-72, 1990 2) Steinbok have suggested that these subdural collection related 13. Matson D : Tumors of the choroid plexus in Matson D(ed) : to persistence of a ventriculosubdural fistula. They have argued Neurosurgery of Infancy and Childhood, Springfield, IL: Charles C Thomas, 1969, pp581-595 for use of pial stay-suture to close the cortical incision, 14. Matson D, Crofton F : Papilloma of the choroid plexus in childhood. reinforced with Tissel(Baxter Healthcare Corp., Deerfield, IL, J Neurosurg 17 : 1002-1027, 1960 USA), to prevent this complication. In our patient, posto- 15. Pascual-Castroviejo I, Villarejo F, Perez-Higueras A, Morales C, Pascual-Pascual SI : Childhood choroid plexus neoplasms : A study perative intraventricular hemorrhage developed. The of 14 case less than 2 years old. Eur J Pediatr 140 : 51-56, 1983 hematoma was removed with extraventricular drainage. 16. Rekate HL, Erwood S, Brodkey JA, Chizeck HJ, Spear T, Ko W, et al : Etiology of ventriculomegaly in choroid plexus papilloma. Thereafter symptomatic subdural fluid collection appeared in Pediatr Neurosci 12 : 196-201, 1985 CT scan. The subdural collection was resolved with subduro- 17. Tomita T, McLone DG, Flannery AM : Choroid plexus papillomas peritoneal shunt. of neonates, infants and children. Pediatr Neurosci 14 : 23-30, 1988 18. Velasco-Siles JM, Raimondi AJ : Pediatric Neurosurgery, ed 2. Incidence of postoperative neurological sequelae of choroid New York : Grune & Stratton, 1982, pp 451-460 plexus papilloma is between 29% and 63%, despite these tumors are benign. Despite the treatment of choroid plexus papilloma still results high morbidity rate, the successful total

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