Diffuse villous hyperplasia of the choroid plexus and its surgical management

Size: px

Start display at page:

Download "Diffuse villous hyperplasia of the choroid plexus and its surgical management"

Aleesha O’Connor’
5 years ago
Views:

1 J Neurosurg Pediatrics 5: , 5: , 2010 Diffuse villous hyperplasia of the choroid plexus and its surgical management Case report Og u z Ca t a lt e p e, M.D., 1 De b o r a h Liptzin, M.D., 2 Li n d s e y Jo l l e y, P.A.-C., 1 a n d Th o m a s W. Sm i t h, M.D. 3 1 Division of Neurosurgery and 2 Department of Pathology, University of Massachusetts Medical Center, Worcester, Massachusetts; and 2 Department of Pediatrics, University of Pittsburgh, Pennsylvania Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a very rare cause of hydrocephalus in children. Only 12 cases of DVHCP have been reported in the literature. In this report the authors describe a new case of a patient with DVHCP who was diagnosed prenatally with hydrocephalus. In a comprehensive literature review and discussion, the authors also discuss radiological and histological characteristics of the reported cases, treatment approaches, and surgical modalities that have been used in the treatment of these patients. (DOI: / PEDS0960) Ke y Wo r d s choroid plexus hydrocephalus diffuse villous hyperplasia endoscopic coagulation Diffuse villous hyperplasia of the choroid plexus is an extremely rare congenital condition. Twelve cases of DVHCP have been described in the literature 1 3,7,10,12,13,15,16,18 20 since the first description of diffuse villous hypertrophy of the choroid plexus by Davis in Diffuse villous hyperplasia of the choroid plexus is characterized by massively enlarged, but radiologically and histologically normal appearing choroid plexuses, and is always associated with severe hydrocephalus due to excessive CSF production. 1 3,7,10,17,19,20 The true nature of DVHCP whether it is a separate entity or a stage on a continuum from DVHCP to CPP and its diagnostic criteria are still controversial. In this paper we report on another patient with DVHCP and discuss the radiological and histological characteristics of these lesions, as well as treatment approaches. Case Report History and Examination. In this male newborn, Abbreviations used in this paper: CPC = choroid plexus carcinoma; CPP = choroid plexus papilloma; DVHCP = diffuse villous hyperplasia of the choroid plexus; FIESTA = fast imaging employing steady-state acquisition; VA = ventriculoatrial; VP = ventriculoperitoneal. hydrocephalus was diagnosed during a prenatal ultrasonography examination. At birth, his neurological examination was normal, and his head circumference was greater than the 97th percentile. The ultrasound scan of his head after delivery was quite remarkable, showing unusually large ventricles filled with diffusely enlarged, prominent choroid plexuses. After this unusual head ultrasound scan, MR imaging of the brain was obtained. The MR imaging showed large, bulky choroid plexuses with normal-appearing architecture (Fig. 1). No lobulated mass appearance was apparent and the entire choroid plexus was avidly and homogenously enhanced using Gd. At 9 days of age, the patient was taken to surgery for endoscopic coagulation of the choroid plexus in the right lateral ventricle and VP shunt placement in the same session. The postoperative period was uneventful. However, 2 weeks later, he developed ascites and a subgaleal collection with a CSF leak through the incision. A shunt tap was performed, which revealed findings consistent with shunt infection. Therefore, his VP shunt was removed, and an external ventricular drain was placed. External CSF drainage was noted to be > 2000 ml/day with an external ventriculostomy drainage system set at 5 cm Hg. After discussing all management options with the boy s family, including VA shunt placement, the decision was made to resect the choroid plexus of the right lateral ventricle to decrease the massive amount of CSF production. 518 J Neurosurg: Pediatrics / Volume 5 / May 2010

Diffuse villous hyperplasia of the choroid plexus Fig. 2. Postoperative MR images obtained after resection of the right-sided choroid plexus. Left: Axial image using the FIESTA sequence.

Initial brain MR imaging shows very large, homogenously enhancing choroid plexuses on the temporal (A and D) and occipital (C) horns, as well as multiple cysts in the FIESTA sequence (B) and severe

2 Diffuse villous hyperplasia of the choroid plexus Fig. 2. Postoperative MR images obtained after resection of the right-sided choroid plexus. Left: Axial image using the FIESTA sequence. Right: Axial T1-weighted image with Gd enhancement. Fig. 1. Initial brain MR imaging shows very large, homogenously enhancing choroid plexuses on the temporal (A and D) and occipital (C) horns, as well as multiple cysts in the FIESTA sequence (B) and severe hydrocephalus. Operation and Postoperative Course. A small parietooccipital craniotomy was performed, and the atrium was entered through a small cortical incision. A very hypertrophic, reddish-pink choroid plexus was encountered in the atrium. An endoscopically assisted microsurgical technique was used during the resection of the choroid plexus in the temporal horn and atrium. After resection of the choroid plexus (Fig. 2), a VP shunt was placed. The patient experienced an uneventful postoperative course, but was readmitted after developing ascites again 3 months later. A CSF sample revealed a shunt infection, and consequently the shunt was removed, a ventriculostomy was performed, and antibiotic treatment was begun. During this period, external CSF drainage was noted to be 900 ml/day. After completing the antibiotic course, a new VP shunt was placed because the boy s family remained unwilling to proceed with placement of a VA shunt at that time. However, the patient developed a significant amount of ascites within a week, at which time the options were discussed with the family, given as either VA shunt placement or resection of the left-sided choroid plexus. We then proceeded with a resection of the left-sided choroid plexus in the lateral ventricle with the anticipation that the patient might not require a shunt at all. The choroid plexus of the left lateral ventricle was removed using the same technique as described above. A ventriculostomy catheter was left in place to measure the amount of CSF drainage and determine if a shunt would be necessary. The CSF drainage was > 300 ml/day postoperatively. Attempts to gradually elevate and close the external J Neurosurg: Pediatrics / Volume 5 / May 2010 ventriculostomy drainage system to stop the CSF drainage were not tolerated as the boy developed a subgaleal collection. Therefore, we had to place a VA shunt as well. After VA shunt placement, the patient experienced an uneventful clinical course (Fig. 3). At his follow-up examination 2 years after his initial surgery, the boy s developmental status was found to be age appropriate, and his neurological examination was unremarkable except for a possible left-sided visual field defect, although it was difficult to perform a complete visual field examination due to his age. Magnetic resonance imaging showed significantly smaller ventricles (Fig. 4). Histological Examination. The choroid plexus specimens from both ventricles were essentially identical and were consistent with villous hyperplasia of the choroid plexus. These specimens were characterized by large numbers of elongated, slender papillae with a well-defined surface epithelium lying upon thin, delicate, fibrovascular stromal cores (Fig. 5A). The surface epithelium consisted of a single layer of cuboidal cells with central to slightly basilar nuclei (Fig. 5B). The nuclei had very little to no pleomorphism or hyperchromasia. No mitoses were observed. The Ki 67/MIB-1 proliferative index (Fig. 5C) was 3% in the right-sided choroid plexus and 0.5% in the left-sided choroid plexus. Immunohistochemical staining was negative for CK7, CK20, epithelial membrane antigen, and glial fibrillary acidic protein, and positive only for transthyretin and S100 protein. Discussion Diffuse villous hyperplasia of the choroid plexus is a rarely reported pathological entity. The first report of bilaterally hyperplastic choroid plexus was a descriptive postmortem study published by Davis in Since then, only 12 additional cases of DVHCP have been reported (Table 1). 1 3,7,10,12,13,15 17,20 Typical radiological characteristics of these lesions are massively enlarged choroid plexuses throughout the entire ventricular system, with numerous cysts of varying sizes, a normal architectural pattern of the choroid plexus without any nodular appearance, strong enhancement with Gd, and significantly en- 519

O. Cataltepe et al. Fig. 3. Postoperative MR images obtained after resection of the left-sided choroid plexus. sequence. B and C: Axial (B) and coronal (C) T1-weighted images with Gd enhancement.

However, radiological diagnosis of DVHCP remains a challenge in some cases, and bilateral papillomas may also appear as diffuse enlargements of the choroid plexus, similar to DVHCP.

Detailed histological examinations are reported in only 7 of the published cases, 1,7,10,12,16,19,20 just 3 describe immunohistological assessment, and only 1 reports the MIB-1 proliferative index.

3 O. Cataltepe et al. Fig. 3. Postoperative MR images obtained after resection of the left-sided choroid plexus. sequence. B and C: Axial (B) and coronal (C) T1-weighted images with Gd enhancement. A: Axial image using the FIESTA larged ventricles as well as extraaxial subarachnoid spaces. However, radiological diagnosis of DVHCP remains a challenge in some cases, and bilateral papillomas may also appear as diffuse enlargements of the choroid plexus, similar to DVHCP. 5,7,9,11,17 There are no clearly defined histological criteria to diagnose DVHCP. Detailed histological examinations are reported in only 7 of the published cases, 1,7,10,12,16,19,20 just 3 describe immunohistological assessment, and only 1 reports the MIB-1 proliferative index. 7 All published cases reported histologically normal looking choroid plexus without any atypical features, pleomorphism, or mitoses, but in the study by D Ambrosio et al., 7 a high proliferative index was also noted. Microscopic architecture of noninvasive, benign (WHO Grade I) CPPs are virtually identical to that of villous hyperplasia, as described above, and characteristically do not show cellular atypia or mitotic figures. 14 Thus, histological differentiation between bilateral CPP and DVHCP may not always be possible, especially if the radiological findings lack any characteristic of a papilloma, such as a nodular mass appearance. 5,9,11 Because of these diagnostic difficulties, D Ambrosio et al. 7 suggested using the MIB-1 proliferative index to better differentiate DVHCP from CPP. However, only a few studies have compared the proliferative index in DVHCP, CPP, or CPC. 4,6,7,19 In normal choroid plexus, the proliferative index is close to 0. 4,6,18 In CPP, the proliferative index ranges from 0.2 to 17.42, whereas in CPC the index ranges from 4.14 to ,6,19 In DVHCP, the only previously reported proliferative index is 4%. 7 In our case, we found the proliferative index to be 3% on the right side and 0.5% on the left. There is clearly overlap between CPP, CPC, and DVHCP in terms of the MIB- 1 proliferative index, and further studies with additional cases are needed to clarify the role of MIB-1 immunohistochemistry in differentiating between DVHCP, CPP, and CPC. 7 Whether DVHCP is a separate entity or is part of a spectrum of histological pathologies that includes choroid plexus hyperplasia, CPP, and even CPC is not yet clear. Fig. 4. Axial (A), sagittal (B), and coronal (C) T1-weighted MR images with Gd enhancement, and an axial T2-weighted image (D), obtained 2 years after surgery. Note residual choroid plexuses in the third and lateral ventricles (C and D). Fig. 5. Photomicrographs of choroid plexus specimens. A: Hyperplastic choroid plexus consisting of slender, elongated papillae. H & E. Original magnification 20. B: Papilla with uniform cuboidal epithelium and fibrovascular stroma. H & E. Original magnification 40. C: Epithelial cells reactive for Ki 67/MIB-1. Original magnification J Neurosurg: Pediatrics / Volume 5 / May 2010

4 Diffuse villous hyperplasia of the choroid plexus TABLE 1: Summary of previously reported cases of DVHCP* Authors & Year Age at Diagnosis, Sex Initial Procedure Complication Further Procedures Shunt Placement CSF Production Rate (ml/day) Davis, mos, M none NA none NA unknown Welch et al., 1983 in utero, F VPS ascites VAS, BCPR no unknown Bucholz & Pittman, mo, F VPS ascites EC NA (patient died) unknown Hirano et al., yrs, F VPS shunt malfunction BCPR no 2000 Britz et al., mos, M VPS ascites VAS VAS 900 Philips et al., mos, F VPS ascites VPS, EC VPS unknown D Ambrosio et al., mos, F VPS ascites VPS, BCPR no 1200 Fujimoto et al., mos, M VPS ascites UCPR VPS unknown Aziz et al., yrs, M VPS ascites VPS, BCPR no 2650 Iplikcioglu et al., yrs, F VPS ascites VAS VAS unknown Tamburrini et al., mos, F VPS ascites EC, BCPR, VPS VPS 2000 Smith et al., mos, F VPS ascites BCPR, VPS VPS 1400 Warren et al., days, M VPS ascites EC, BCPR VPS unknown present study in utero, M VPS & EC ascites VPS, BCPR VAS 2000 * BCPR = bilateral choroid plexus resection; EC = endoscopic coagulation; NA = not applicable; UCPR = unilateral choroid plexus resection; VAS = VA shunt; VPS = VP shunt. D Ambrosio et al. 7 suggested that DVHCP may be a stage along a disease continuum, ranging from villous hyperplasia, to papilloma, and finally to carcinoma. If this is the case, close follow-up to check for recurrence or transformation to more aggressive forms is needed in patients with villous hyperplasia and a high mitotic index, such as our patient in this case. All published DVHCP cases presented with hydrocephalus and almost all underwent VP shunt placement as initial treatment. 1 3,7,10,12,13,15 17,19,20 All these patients, except 1, suffered from VP shunt failure by developing ascites, due to the massive overproduction of CSF, and DVHCP was diagnosed only after VP shunt failure. Massive amounts of CSF production were documented in many of the published cases, ranging from 900 to 2650 ml/day. 1 3,7,12,16,17 In our patient, the CSF production rate was > 2000 ml/day after endoscopic coagulation, decreased to > 900 ml/day after resection of the right choroid plexus, and became > 300 ml/day after resection of the left choroid plexus. These massive CSF production rates and absorption difficulties make the treatment of these patients quite challenging. Therefore, multiple strategies have been attempted to manage the excess CSF, beginning with VP shunt placement. 1 3,7,10,12,13,15 17,20 Alternative treatment attempts have varied from VA shunt placement, 2,13,20 to endoscopic coagulation of the choroid plexus, 3,7,17 to use of acetazolamide, 2,10 and finally to resection of the choroid plexus. 1,7,10,12,16,17,19,20 Unfortunately, all these approaches have different shortcomings. The success of VP shunt placement in these cases is limited by the resorption capacity of the peritoneum of an infant. Tamburrini et al. 17 have suggested avoiding VP shunt placement as first-line treatment in infants once DVHCP is diagnosed. Ventriculoatrial shunt placement for DVHCP management has been used successfully as the definitive treatment in 2 previous J Neurosurg: Pediatrics / Volume 5 / May 2010 cases, 2,13 although it failed in another case. 20 Endoscopic coagulation of choroid plexus is a very attractive, minimally invasive option, although its efficacy is closely related to the size of the choroid plexus. Endoscopic coagulation was found to be successful in only 1 of 3 published cases as a definitive treatment for DVHCP, but it needed to be used in combination with VP shunt placement. 15 Of the 2 unsuccessful cases, 1 patient did not survive postoperatively, 3 and the other also required resection of the choroid plexuses. 17 We also used endoscopic coagulation in our patient by combining it with VP shunt placement as an initial surgical approach, but the patient developed ascites. Choroid plexectomy in the management of choroid plexus hyperplasia was performed by Welch et al. 20 for the first time, and since then several more cases were managed with this approach, either uni- or bilaterally. 1,7,10,12,16,17,19 Although 4 of these cases did not require additional shunt placement after choroid plexus resection, 1,7,12,20 the remaining cases did need additional shunt placement. 10,16,17,19 Our literature review, including the present case, demonstrates that although choroid plexus resection might be a definitive treatment in some cases, shunt placement might still be needed in others, as in our patient. Based on the existing literature and our experience with the present case, we believe that if the radiological characteristics of the patient are strongly suggestive of DVHCP, it is highly likely that a VP shunt would fail and other management options should be considered. Endoscopic coagulation combined with a VP or VA shunt might be a reasonable option in some cases, such as those with less bulky and moderately enlarged choroid plexuses. Unilateral or bilateral resection of choroid plexuses is an efficient technique to decrease the CSF production rate in massively enlarged choroid plexuses, and to make VP or VA shunt placement feasible in these cases. Although 521

5 O. Cataltepe et al. choroid plexus resection was reported as a definitive treatment in some published cases, other patients needed shunt placement, which occurred in our case. Overall, VA shunt placement may play a larger role in the management of these cases, and can be considered as a first-line treatment option alone or in combination with endoscopic coagulation or choroid plexus resection. Disclosure Author contributions to the study and manuscript preparation include the following. Conception and design: O Cataltepe. Acquisition of data: O Cataltepe, D Liptzin, L Jolley. Analysis and interpretation of data: O Cataltepe, TW Smith. Drafting the article: O Cataltepe, D Liptzin. Critically revising the article: O Cataltepe, TW Smith. Reviewed final version of the manuscript and approved it for submission: O Cataltepe, D Liptzin, L Jolley, TW Smith. Administrative/technical/material support: L Jolley. Study supervision: O Cataltepe. References 1. Aziz AA, Coleman L, Morokoff A, Maixner W: Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children? Pediatr Radiol 35: , Britz GW, Kim DK, Loeser JD: Hydrocephalus secondary to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature. J Neurosurg 85: , Bucholz RD, Pittman T: Endoscopic coagulation of the choroid plexus using the Nd:YAG laser: initial experience and proposal for management. Neurosurgery 28: , Carlotti CG Jr, Salhia B, Weitzman S, Greenberg M, Dirks PB, Mason W, et al: Evaluation of proliferative index and cell cycle protein expression in choroid plexus tumors in children. Acta Neuropathol 103:1 10, Ceddia A, Di Rocco C, Carlucci A: [Hypersecretive congenital hydrocephalus due to choroid plexus villous hypertrophy associated with controlateral papilloma.] Minerva Pediatr 45: , 1993 (Italian) 6. Centeno BA, Louis DN, Kupsky WJ, Preffer FI, Sobel RA: The AgNOR technique, PCNA immunohistochemistry, and DNA ploidy in the evaluation of choroid plexus biopsy specimens. Am J Clin Pathol 100: , D Ambrosio AL, O Toole JE, Connolly ES Jr, Feldstein NA: Villous hypertrophy versus choroid plexus papilloma: a case report demonstrating a diagnostic role for the proliferation index. Pediatr Neurosurg 39:91 96, Davis LE: A physio-pathological study of the choroid plexus with the report of a case of villous hypertrophy. J Med Res 44: , Di Rocco C, Iannelli A: Poor outcome of bilateral congenital choroid plexus papillomas with extreme hydrocephalus. Eur Neurol 37:33 37, Fujimoto Y, Matsushita H, Plese JP, Marino R Jr: Hydrocephalus due to diffuse villous hyperplasia of the choroid plexus. Case report and review of the literature. Pediatr Neurosurg 40:32 36, Fujimura M, Onuma T, Kameyama M, Motohashi O, Kon H, Yamamoto K, et al: Hydrocephalus due to cerebrospinal fluid overproduction by bilateral choroid plexus papillomas. Childs Nerv Syst 20: , Hirano H, Hirahara K, Asakura T, Shimozuru T, Kadota K, Kasamo S, et al: Hydrocephalus due to villous hypertrophy of the choroid plexus in the lateral ventricles. Case report. J Neurosurg 80: , Iplikcioglu AC, Bek S, Gökduman CA, Bikmaz K, Cosar M: Diffuse villous hyperplasia of choroid plexus. Acta Neurochir (Wien) 148: , Levy ML, Goldfarb A, Hyder DJ, Gonzales-Gomez I, Nelson M, Gilles FH, et al: Choroid plexus tumors in children: significance of stromal invasion. Neurosurgery 48: , Philips MF, Shanno G, Duhaime AC: Treatment of villous hypertrophy of the choroid plexus by endoscopic contact coagulation. Pediatr Neurosurg 28: , Smith ZA, Moftakhar P, Malkasian D, Xiong Z, Vinters HV, Lazareff JA: Choroid plexus hyperplasia: surgical treatment and immunohistochemical results. Case report. J Neurosurg 107 (3 Suppl): , Tamburrini G, Caldarelli M, Di Rocco F, Massimi L, D Angelo L, Fasano T, et al: The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia. Childs Nerv Syst 22: , Vajtai I, Varga Z, Aguzzi A: MIB-1 immunoreactivity reveals different labelling in low-grade and in malignant epithelial neoplasms of the choroid plexus. Histopathology 29: , Warren DT, Hendson G, Cochrane DD: Bilateral choroid plexus hyperplasia: a case report and management strategies. Childs Nerv Syst 25: , Welch K, Strand R, Bresnan M, Cavazzuti V: Congenital hydrocephalus due to villous hypertrophy of the telencephalic choroid plexuses. Case report. J Neurosurg 59: , 1983 Manuscript submitted January 30, Accepted December 8, Address correspondence to: Oguz Cataltepe, M.D., Division of Neurosurgery, University of Massachusetts Medical Center, Suite S2-848, 55 Lake Avenue North, Worcester, Massachusetts oguz.cataltepe@umassmemorial.org. 522 J Neurosurg: Pediatrics / Volume 5 / May 2010

A Case of Difficult Management of Fluid-Electrolyte Imbalance in Choroid Plexus Papilloma

Case Report doi: 10.2176/nmc.cr2012-0380 Neurol Med Chir (Tokyo) xx, xxx xxx, 20xx A Case of Difficult Management of Fluid-Electrolyte Imbalance in Choroid Plexus Papilloma Atsushi Saito, 1 Shinjitsu Nishimura,