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Eur Radiol (2002) 12:883±888 DOI 10.1007/s003300101030
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Preoperative and early postoperative magnetic resonance imaging in two cases of childhood choroid plexus carcinoma
S. E. J. Connor C. Chandler I. Bodi S. Robinson J. M. Jarosz
Received: 5 March 2001 Revised: 29 May 2001 Accepted: 5 June 2001 Published online: 11 September 2001 Springer-Verlag 2001
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S. E. J. Connor ( ) ´ J. M. Jarosz Department of Neuroradiology, King's College Hospital, Denmark Hill, London SE5 9RS, UK E-mail:
[email protected] Phone: +44-2 08-7 61 83 44 Fax: +44-2 07-3 48 31 20 C. Chandler Department of Neurosurgery, King's College Hospital, Denmark Hill, London SE5 9RS, UK I. Bodi ´ S. Robinson Department of Neuropathology, King's College Hospital, Denmark Hill, London SE5 9RS, UK
Abstract We present and illustrate the MRI appearances of two children with choroid plexus carcinoma. The MRI characteristics of these rare tumours are reviewed. Since total surgical resection is a significant prognostic factor, early postoperative MRI was performed in both cases to ensure surgical clearance. In one case a complete resection was documented and this patient remains well at short-term follow-up. Residual tumour was noted in the second case, but despite ªsecond lookº surgery there was subsequent local relapse. Keywords Choroid plexus carcinoma ´ Magnetic resonance imaging
Introduction Choroid plexus neoplasms account for 0.4±0.6 % of intracranial tumours and 1.5±6.4 % of all paediatric intracranial tumours [1, 2, 3]. Choroid plexus carcinoma (CPC) represents between 8 and 17 % of all choroid plexus tumours [1, 4]. The MRI appearances of CPC have rarely been reported [2, 3, 4, 5, 6, 7, 8, 9]. These tumours are difficult surgical targets due to their large size, invasive nature, vascularity and location, and a complete resection is achieved in only 40±60 % of cases [1, 10]; however, the extent of surgery is of major importance to clinical outcome [10, 11]. We used early postoperative MRI to evaluate the completeness of tumour removal and hence the need for ªsecond lookº surgery.
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We describe the MRI appearances of CPCs in two children and review previous MRI descriptions. We also illustrate the use of early postoperative MRI in the management of these patients.
Case reports Case 1 A 4-year-old girl was admitted to her local hospital with a 2-week history of intermittent vomiting, drowsiness and confusion. She had previously been well and had developed normally. Physical examination was normal apart from a subtle left-sided facial palsy. While waiting for a CT scan she became suddenly bradycardic and hypertensive. Following resuscitation, intubation and ventilation, she was managed in the intensive care unit. Fundoscopy was nor-
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b Fig. 1 a±c Case 1. a T2-weighted axial, b T1-weighted coronal and c T1-weighted coronal post-gadolinium images demonstrate the heterogeneous mass, isointense in signal on T1- and T2-weighted images, which extends medially from the left lateral ventricular trigone. There is heterogenous marked gadolinium enhancement and surrounding vasogenic oedema with associated blood degradation products and a posterior cyst. Bulky enhancing choroid plexus is seen within the body of the left lateral ventricle (arrow)
mal and her pupils were equal and reactive to light. Non-enhanced CT revealed an isodense mass lesion related to the choroid plexus within the left lateral ventricle, with an adjacent low-density area posteriorly and high-density haemorrhage at its periphery. The mass extended medially from the left ventricular trigone; the left temporal horn was dilated and there was marked surrounding vasogenic oedema. It underwent marked heterogeneous enhancement. Intravenous dexamethasone was commenced and the child was transferred to the neurosurgical centre where she underwent craniospinal MRI (Fig. 1). This demonstrated a 3.5 3 2.5-cm mass lesion to be contiguous with the choroid plexus within the left ventricular trigone. It was of slightly heterogeneous T1 and T2 signal isointense to grey matter and underwent heterogeneous enhancement. There was a well-marginated non-enhancing T2 hyperintense component posteriorly. Vasogenic oedema and blood degradation products were again noted. No other abnormality was noted within the intracranial or spinal subarachnoid spaces. Through a left temporoparietal craniotomy, a biopsy of the lesion was performed which was reported as ªmalignant tumourº. Extensive internal debulking of the tumour was performed which revealed a deep attachment to a vascular pedicle at the trigone of the lateral ventricle. There was a clear plane between the tumour and the brain which allowed a macroscopic clearance to be achieved. Histological examination of the operative specimen demonstrated poorly differentiated polygonal tumour cells (Fig. 2) with extensive necrosis, high cellularity and a high mitotic index. Some well differentiated areas with a papillary pattern were also present.
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Fig. 2 Case 1. Moderately pleomorphic, polygonal tumour cells arranged haphazardly around blood vessels with mitoses (arrows)
There was immunohistochemical positivity with epithelial markers (CAM 5.2, EMA), but it was largely negative for markers of glial differentiation (GFAP). The morphological appearances and immunophenotype were typical of CPC grade 3 (WHO) [12]. In view of the histopathological findings, a postoperative MRI (Fig. 3) was performed 30 h after surgery. High T1 signal was seen within the surgical cavity, related to the choroid plexus of the left lateral ventricle and along the surgical tract, but there was no pathological enhancement on immediate post-gadolinium T1weighted imaging. Postoperative recovery was uneventful, although there was a residual left facial weakness and a right sixth nerve palsy. Adjuvant chemotherapy was commenced and 3 months later the child remained well.
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Fig. 3 a, b Case 1. a T1-weighted coronal and b T1-weighted coronal post-gadolinium images reveal increased T1weighted signal within the surgical cavity, along the surgical tract and related to the left choroid plexus, but there is no residual enhancing tumour
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b
Case 2 A previously healthy 20-month-old boy presented to his local hospital with vomiting and poor appetite of 2 weeks duration and 1 day of increasing lethargy. Neurological and general examination revealed a somnolent boy who was otherwise normal. A CT scan revealed a heterogeneous mass with peripheral high density and enhancement, which was centred on the trigone of the left lateral ventricle. There was extensive adjacent vasogenic oedema and midline shift to the right. Intravenous dexamethasone was commenced. Following transfer to the neurosurgical centre, a craniospinal MRI was performed (Fig. 4). This demonstrated a 5 5 4-cm left lateral ventricle mass, in continuity with a prominent choroid plexus within the left trigone, which extended anteriorly and inferiorly into the brain parenchyma. It had a heterogeneous intermediate T1 and low T2 signal periphery with a low-T1 and high-T2 signal centre. There was a rim of thick irregular gadolinium enhancement. There was extensive surrounding oedema, midline shift to the right and a dilated left temporal horn. The was no MRI evidence of subarachnoid or intraventricular dissemination. A left temporal craniotomy was performed and, initially, a brain cannula was inserted into the mass. Approximately 20±30 ml of haemorrhagic, necrotic tumour and fluid was aspirated. The smear of this sample was reported as ªmalignant tumourº. Vascular, grey tumour was debulked and the vascular pedicle arising from a hypertrophied choroid plexus was identified and divided. There was no macroscopic evidence of tumour at the completion of surgery. Microscopic examination revealed an undifferentiated carcinoma with a papillary pattern recognisable in places. There was high cellularity and a high mitotic index with multiple areas of necrosis. Tumour cells were focally strongly positive for CAM 5.2 and GFAP. The appearances were of a CPC grade 3 (WHO) [12]. A specimen of macroscopically normal choroid plexus which was sampled from proximal to the tumour at surgery revealed no evidence of tumour infiltration. A postoperative MRI was performed 20 h after surgery. This revealed a thick rim of enhancement laterally within the resection cavity on immediate post-gadolinium T1-weighted images which was interpreted as residual tumour. There was a persistent marked enhancement of the left choroid plexus. Surgical re-exploration identified and removed the residual tumour. At discharge 4 days
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later the child was asymptomatic and there was no residual neurological deficit. Adjuvant chemotherapy, which included carboplatin, procarbazine, cisplatin, etoposide, vincristine and cyclophosphamide, was commenced on a 3-week cycle; however 2 months later the child again developed symptoms of lethargy and vomiting. Magnetic resonance imaging revealed areas of solid enhancement centred at the lateral and posterior margins of the resection cavity with adjacent rim-enhancing cystic areas. The tumour cysts were aspirated and a macroscopic surgical clearance of the solid tumour was again achieved. Histological appearances were similar to the previously reported CPC.
Discussion Choroid plexus carcinomas are large invasive tumours which have a tendency to recur [13] and usually present in patients of a young age [1, 2, 9, 13], so therapy is particularly challenging. Modern neurosurgical techniques and perioperative diagnostic imaging have contributed to an enhanced outcome [10, 14]; however, CPCs are still associated with only a 26±40 % 5-year survival rate [10, 13]. The most important factor relating to the overall prognosis remains the completeness of surgical excision [2, 10, 11, 14]. There are few descriptions of the MRI appearances of these tumours. There are seven case descriptions in the international literature accompanied by MRI illustrations and documentation of both T1 and T2 characteristics (Table 1). Due to their origin in non-eloquent sites, most of these tumours reach a substantial size prior to detection. A median volume at presentation has been recorded as 45 cm3 [10]. In children, the lateral ventricle is the predominant site of involvement [4]; however, there is usually parenchymal invasion with extensive surrounding vasogenic oedema. Ventricular dilatation may be limited to the obstructed portion of
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Fig. 4 a±c Case 2. a T2-weighted axial, b T1-weighted coronal and c T1-weighted coronal post-gadolinium images demonstrate the left lateral ventricular trigone mass extending anteriorly and inferiorly into the brain parenchyma. It had a heterogeneous intermediate-T1 and low-T2 signal periphery with a low-T1 and high-T2 signal centre. There is marked surrounding vasogenic oedema. There is a rim of thick serpiginous gadolinium enhancement. There is bulky gadolinium enhancement of the adjacent choroid plexus (arrow)
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the ventricular system [2, 6] which was the pattern we observed, or there may be a communicating hydrocephalus due to oversecretion, haemorrhage or subarachnoid dissemination [2, 9]. These tumours tend to be of heterogenous signal due to necrosis, haemorrhage and calcification. The T1 signal is typically hypointense or isointense to grey matter. The T2 signal is most frequently isointense to grey matter and may be of low signal, which may be due to high cellularity, calcification or haemorrhage. Gadolinium enhancement may be homogeneous or heterogeneous, and there may be substantial non-enhancing components usually due to central necrosis but also due to associated cysts and haemorrhage [9]. The tumour is incontiguous with the adjacent choroid plexus. We and others [8] have observed the ipsilateral choroid plexus to be enlarged with prominent enhancement due to increased vascularity. Bulky
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enhancing choroid plexus was present in case 2 and surgical biopsy distant to the CPC confirmed this to be free of tumour. This was witnessed to a lesser extent in case 1 and also in a further adult patient with choroid plexus malignancy (not illustrated). A leash of vascular flow voids extending to the tumour and representing the vascular pedicle is commonly seen [4, 5, 6, 9]. Local subependymal extension has also been noted in two cases [5, 9] and CPC has a propensity to produce metastatic seeding within the ventricular system, intracranial subarachnoid space and spinal axis [5, 8, 14]. Therefore, if the MRI appearances are suggestive of CPC at the time of diagnosis, craniospinal imaging is advised as part of the initial staging. The most decisive parameter for survival is total surgical resection [2, 10, 11, 14]. The role of adjuvant chemotherapy and radiotherapy in the primary manage-
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Table 1 Cases of choroid plexus carcinoma in which clinical data is accompanied by MRI illustrations and documentation of T1 and T2 characteristics. NA: information not available in report or from Ref- Age, Site of erence gender origin
Maxi- Ventri- Paren- Contigumum cles en- chymal ity with dimen- larged invachoroid sion sion plexus (cm)
illustrations. Note that the assessment of MRI features was based on limited information and illustrations in some cases
ExtenVascu- T1 sion along lar flow signal adjacent voids ependyma
T2 signal
Hetero- Engeneous hanceprement gadolinium
Nonenhancing component
Adjacent T2 highsignal oedema
[2]
17 Left 4 months, body F and trigone
Dilated Yes occipital horns
Yes
NA
No
Hypo-, Hyper- Yes iso-
NA
Associat- Yes ed blood degradation products
[3]
9 Fourth NA months, ventricle M
Four ventricles
No
NA
NA
No
Hypo- Iso-
NA
NA
[4]
31 years, F
No
Yes
Yes
No
Yes
Iso-
Hyper- Yes
[5]
9 Both Right Four months, trigones 4.3, left ventriF bilateral 2.2 cles lesions
Yes (rightsided lesion)
Yes
Yes (rightsided lesion)
Yes
Iso-
Iso-
Yes (right sided lesion)
[6]
15 Right 5 months, trigone F
Right Yes occipital, temporal horns
Yes
NA
Yes
Iso-
Iso-
Yes
[7]
1 year, F
Left Yes lateral ventricle
Yes
No
No
NA
Iso-, NA hyper-
[8]
2 Right 3 months, trigone F
Four ventricles
Yes, Yes choroid plexus enlarged
No
Hypo- Iso-
NA
Marked No
Yes
Yes
Yes
Iso-, hypo-
Iso-
Yes
Marked Necrosis Yes
Yes
No
No
Iso-
Iso-
Yes
Marked Cyst and Yes blood degradation products
Yes, No choroid plexus enlarged
Yes
Iso-, hypo-
Iso-, Yes hyper-
Marked Necrosis Yes
[9]
28 months, M Case 1 4 years, F
Right NA trigone
Right NA trigone
Left NA trigone Left 3 trigone
Case 2 20 Left 5 months, trigone M
Yes
Yes Left lateral ventricle Left Yes temporal horn Left Yes temporal horn
ment of these tumours remains uncertain [9, 10, 14, 15]. The completeness of resection is difficult to estimate at the time of surgery [16], particularly in the case of CPC where there is frequently restricted exposure. Confirmation of complete excision on postoperative imaging is the aim as such patients have a 78±86 % survival rate [10, 14]. The timing of postoperative MRI is critical in reducing the number of inconclusive scans which result from
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Yes
NA
Marked Multiple Yes peripheral cystic areas NA NA Yes
NA
NA
Yes
Marked Peripher- Yes al cystic areas and necrosis Yes
the inability to distinguish between residual tumour, haemorrhage and postoperative contrast enhancement. High T1 signal methaemoglobin related to a vascular and sometimes haemorrhagic CPC may obscure the enhancement of small residual tumour elements on postoperative imaging. Accelerated formation of methaemoglobin is well recognised in patients undergoing neurosurgery [17] and was witnessed along the surgical tract in case 1. Confounding enhancement patterns
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which may interfere with the delineation of residual tumour intraoperatively and in the early postoperative period [16, 18] include linear enhancement of the resection margin resulting from leakage of contrast media out of surgically opened vessels, intraparenchymal enhancement adjacent to the surgical cavity due to transient blood brain barrier disruption [19, 20] and enhancement of the choroid plexus and ependyma [16, 20] secondary to intraventricular surgery. The best timing of early postoperative imaging is presently unclear [17, 18, 19, 20]; however, MRI must be performed prior to neovascularization which results in contrast enhancement of the tumour bed during the second postoperative week [19]. The principal reason for day 1±2 postoperative imaging in these children was to enable early ªsecond lookº surgery if required.
In conclusion, many of the MRI features of CPC are non-specific and they may be difficult to distinguish from other intraventricular lesions such as choroid plexus papillomas, primitive neuroectodermal tumours, gliomas, meningiomas or metastases; however, intermediate T2 signal, and a leash of vascular flow voids extending from an enlarged ipsilateral choroid plexus in a child, should suggest the diagnosis and prompt preoperative spinal MRI. Early postoperative MRI allowed a confident diagnosis of complete and potentially curative tumour resection in case 1. The subsequent local recurrence in case 2 suggests that repeat postoperative imaging may also be useful after ªsecond lookº surgery in order to ensure surgical clearance.
References 1. Pascual-Castroviejo, Villarejo F, PerezHigueras A, Morales C, Pascual-Pascual SI (1983) Childhood choroid plexus neoplasms. A study of 14 cases less than 2 years old. Eur J Pediatr 140: 51±56 2. Tekkok IH, Palaoglu S, Erbengi A (1992) Primary choroid plexus carcinoma in an infant. Report of a case evaluated by MRI. J Neurosurg Sci 36: 227±231 3. Vazquez E, Ball WS Jr, Prenger EC, Castellote A, Crone KR (1991) Magnetic resonance imaging of fourth ventricular choroid plexus neoplasms in childhood. Pediatr Neurosurg 17: 48±52 4. Matsuda M, Uzura S, Nakasu S, Handa J (1991) Primary carcinoma of the choroid plexus in the lateral ventricle. Surg Neurol 36: 294±299 5. Cila A, Ozturk C, Senaati S (1992) Bilateral choroid plexus carcinoma of the lateral ventricles. US, CT and MR findings. Pediatr Radiol 22: 136±137 6. Coates TL, Hinshaw DB, Peckman N, Thompson JR, Hasso AN, Holshouser BA, Knierim DS (1989) Pediatric choroid plexus neoplasms: MR, CT and pathologic correlation. Radiology 173: 81±88 7. Jinkins JR, Leite C da C (1998) Neurodiagnostic imaging: pattern analysis and differential diagnosis. Lippincott-Raven, Philadelphia, p 436 8. Kang H-S, Wang Y-M, Kim Y-M, Kim I-O, Kim S-K, Chi JG, Cho B-K (1997) Choroid plexus carcinoma in an infant. J Korean Med Sci 12: 162±167
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9. St. Clair SK, Humphreys RP, Pillay PK, Hoffman HJ, Blaser SI, Becker LE (1991) Current management of choroid plexus carcinoma in children. Pediatr Neurosurg 17: 225±233 10. Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A, Bouffet E (1998) Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery 42: 470±475 11. Shinoda J, Kawaguchi M, Matsuhisa T, Deguchi K, Sakai N (1998) Chorid plexus carcinoma in infants: report of two cases and review of the literature. Acta Neurochir 140: 557±563 12. Aguzzi A, Brander S, Paulus W (2000) Choroid plexus tumours. In: Kleihnes P, Cavenee WK (eds) World Health Organization classification of tumours. Pathology and genetics. Tumours of the nervous system. IARC Press, pp 83±86 13. Pencalet P, Sainte-Rose C, LellouchTubiana A, Kalifa C, Brunelle F, Sgouros S, Meyer P, Cinalli G, Zerah M, Pierre-Kahn A, Renier D (1998) Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 88: 521±528 14. Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA, Ryan J, Reaman G, Schut L (1992) Choroid plexus in childhood. Cancer 69: 580±585
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15. McEvoy AW, Harding BN, Phipps KP, Ellison DW, Elsmore AJ, Thompson D, Harkness W, Hayward RD (2000) Management of choroid plexus tumours in children: 20 years experience at a single neurosurgical centre. Pediatr Neurosurg 32: 192±199 16. Albert FK, Forsting M, Sartor K, Adams HP, Kunze S (1994) Early postoperative magnetic resonance imaging after resection of malignant glioma: objective evaluation of residual tumor and its influence on regrowth and prognosis. Neurosurgery 34: 45±60 17. Oser AB, Moran CJ, Kaufmann BA, Park TS (1997) Intracranial tumor in children: MR findings within 24 hours of craniotomy. Radiology 205: 807±812 18. Forsyth PA, Petrov E, Mahallati H, Cairncross JG, Brasher P, MacRae ME, Hagen NA, Barnes P, Sevick RJ (1997) Prospective study of postoperative magnetic resonance imaging in patients with malignant gliomas. J Clin Oncol 15: 2076±2081 19. Henegar MM, Moran CJ, Silbergeld DL (1996) Early postoperative magnetic resonance imaging following nonneoplastic cortical resection. J Neurosurg 84: 174±179 20. Knauth M, Aras N, Wirtz CR, Dorfler A, Engelhorn T, Sartor K (1999) Surgically induced intracranial contrast enhancement: potential source of diagnostic error in intraoperative MR imaging. Am J Neuroradiol 20: 1547±1553
