Radiol Oncol 2004; 38(3): 181-5.
Choroid plexus carcinoma: A case report
Primož Strojan1, Mara Popovic2, Katarina Šurlan3, Berta Jereb1
1Department of Radiotherapy, Institute of Oncology, 2Institute of Pathology, Medical Faculty University of Ljubljana, 3Institute of Radiology, University Clinical Centre, Ljubljana, Slovenia
Background. The opinions on the value of adjuvant therapy in choroid plexus carcinomas vary. The aim of present report is to present a case of successful therapy of this rare tumor.
Result. A fourteen-year-old girl with third ventricle tumor had non-radical surgery and adjuvant chemother-apy and irradiation. She is alive with no evidence of disease 8.5 years after diagnosis. The role of adjuvant therapy in the context of literature data is discussed.
Conclusion. For choroids plexus carcinomas, adjuvant multiagent chemotherapy and craniospinal radio-therapy following surgery should be considered.
Key words: choroid plexus neoplasms; chemotherapy, adjuvant; radioteraphy; survival analysis
Introduction
Choroid plexus tumor (CPT) is a rare neo-plasm, arising from the neuroepithelial lin-ing.1 After its first description in 1832, more than 500 CPT patients have been described in literature.2 Three quarters of the patients are children, with tumors most often found in the lateral ventricles. In adults, the fourth ventri-cle and its recesses are the most common sites of origin.1 The histopathology of CPT ranges from a well - demarcated benign pa-pilloma (WHO grade I) to highly anaplastic,
Received 8 June 2004
Accepted 5 July 2004
Correspondence to: Assist. Prof. Primož Strojan, M.D, Ph.D., Department of Radiotherapy, Institute of Oncology, Zaloška 2, SI-1000 Ljubljana, Slovenia. Phone: +386 1 5879 110; Fax: +386 1 5879 400; E-mail: pstrojan@onko-i.si
infiltrative carcinoma (WHO grade III, choroid plexus carcinoma [CPC]). Surgical re-section alone is curative for benign tumors, but the optimal adjuvant therapy for the ma-lignant ones has not yet been defined and the prognosis is poor.1,3 Our experience in suc-cessfully treating such a patient is therefore of interest.
Case report
The patient was previously healty 13.8-year old girl who was admitted to the hospital in January 1996, with a six months complaint of headache and double vision when reading. Two months prior to admission she became progressively lethargic and dull resulting in a deterioration of school performance. Neurologic examination revealed bilateral pa-pilledema, ataxia, positive bilateral Babinski
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Figure 1. T1-weighted transverse postcontrast MRIs of the patient.
(A) Before surgery: partially cystic tumor mass of the third ventricle with the extension to thalamus, and dilatation of ventricular system.
(B) Two weeks after surgery and before chemotherapy: residual tumor in the third ventricle.
(C) After the second cycle of multiagent chemotherapy: marked regression of residual tumor.
(D) Five months after radiotherapy: even after gadolinium application, there is no pathologic enhancement suspi-cious for residual tumor mass.
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Figure 2. Histologic characteristic of resected tumor.
(A) Solid growth of carcinoma with focal papillary structures. Mitotic figures are present (arrowheads). H&E, mag-nification 190x.
(B) Strong cytokeratin immunoreaction is evident in all tumor cells. Immunohistochemistry, DAKO’s CK 18 mon-oclonal antibody, magnification 190x.
signs and right-sided hyperreflexia. Two days before surgery, Parinaud syndrome devel-oped. Computed tomography (CT) and mag-netic resonance (MR) scans of the brain showed a contrast-enhancing lesion in the pineal region with extension to the thalamus and the lamina quadrigemina and dilatation of the supratentorial ventricles (Figure 1A).
External ventricular drainage was intro-duced first. No tumor cells were found in the cerebrospinal fluid. Tumor resection was per-formed one week later and a reddish granular tumor tissue overgrowing the posterior part of the third ventricle was found. It infiltrated locally and extended to the lamina quadrige-mina. Histologic examination revealed a highly cellular, focally necrotic tumor compo-sed of polygonal cells with moderately poly-morphic nuclei and scattered mitoses. Tumor cells were organized in sheets or formed pap-illary structures lined with multiple epithelial layers. Due to pineal location, embryonal car-cinoma was also taken into consideration. Strong cytokeratin and NSE labelling of tumor cells, in addition to negative AFP and PLAP, confirmed the diagnosis of CPC (Figure 2). Two days after surgery, external ventricular drainage was removed. On postoperative imaging, tumor residue was seen in
the postero-inferior part of the third ventricle (Figure 1B).
Postoperatively, multiagent chemotherapy was introduced according to the so-called BEP protocol (bleomycin 15 mg/m2 I.V., days 1-3; etoposide 100 mg/m2 I.V., days 1-3; cisplatin 20 mg/m2 I.V., days 4-8). After the second cycle of chemotherapy, the MRI showed a marked regression of the residual tumor (Figure 1C), and an additional two cycles of these drugs resulted in further tumor reduction. The treatment con-cluded with craniospinal radiotherapy of 31.5 Gy/21 fx and a local tumor boost of 22 Gy/20 fx b.i.d. The patient was irradiated five days per week, using 5 MV linear accelerator photon beams. The treatment technique consisted of posterior spinal fields with moving junction and a combination of two lateral opposing portals and one posterior portal for the brain. Two-field technique was used for boosting the tumor. Complete disappearance of the tumor was con-firmed by a subsequent MR scan (Figure 1D).
To date, 8.5 years after diagnosis, there has been no evidence of local tumor recurrence or metastasis. She has no severe neurologic im-pairment and her Karnofsky index is 100. She finished high school, got married, and gave birth to her first child. So far, the child is normal as was the endocrine testing of his mother.
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Discussion
Experience with this extremely rare malig-nant tumor is scanty. Surgery is, however, unequivocally considered to be the first-line therapy for all histological variants of CPTs. Surgical techniques and possible complica-tions have been widely described in litera-ture.1 In CPCs, the most significant predictive factor for survival is the extent of surgery. This has been confirmed in several single-institution analyses4-6 as well as in literature re-views.2,3 To obtain complete tumor removal, Ellenbogen et al. and others have advocated as many surgical procedures as required.4-6
One of the key debates in CPC therapy re-lates to the value of adjuvant therapy after gross tumor resection. There are both oppo-nents and proponents of the combined treat-ment approach.2-5,7,8 A recent literature review by Wolff et al. found adjuvant radiother-apy to be of benefit over surgery alone.2 Similar conclusions with regard to radiother-apy and/or chemotherapy can be drawn from numerous single-institution reports.5,9,10
The need for aggressive adjuvant therapy is widely recognized in the patients with residual CPC following surgery since the expected survival is half of that after gross tumor resec-tion.2,3,8 Even though there are anecdotal reports on successful adjuvant radiotherapy7,9 or chemotherapy,10 it is our impression that aggressive combined radio-chemotherapy offers the best chance for survival. Examples of beneficial effect of combined therapy, some-times incorporating second-look surgery, can be found in the literature.8,9,11
The radiotherapy target volume is dictated by the propensity of CPC for subarachnoid seeding which, when confirmed, calls for ad-juvant therapy per se, irrespective of the de-gree of completeness of surgical procedure. Subarachnoid seeding was found in 43% of those reported cases that were investigated for dissemination.3 Thus, craniospinal axis irradiation to a dose of 30 Gy and a boost to the
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tumor bed up to 50 Gy is indicated if radio-therapy is used,12 and seems to be more ef-fective than chemotherapy.3
Due to the high risk of severe adverse in-tellectual and endocrinologic sequelae in very young children, early radiotherapy is an option in older age groups only. Two treatment strategies were described in the literature for young patients, both placing emphasis on multiagent chemotherapy, although this also is not entirely free of long-term side-effects.13 To facilitate complete tumor resection as a main prognostic determinant in CPCs, St Clair et al. used preoperative chemotherapy to reduce tumor vascularity and bulk after initial biopsy or limited surgery. Not specifying the postresection therapy, if any, two out of four children from this program were reported to be free of disease for 30 and 39 months from diagnosis.6 The second option was described by Duffner et al. Using chemotherapy for 24 months in children 0-23 months of age and 12 months in those 24-36 months of age and delayed radiotherapy, the autors reported a 3-year survival of 75%, with five of the eight patients surviving a minimum of 48 months following diagnosis.10 The variety of chemo-therapeutic agents and their combinations used by these two groups as well as in other reports indicates that an optimal chemothera-py regimen is yet to be defined.
In conclusion, our recommendation would be that for CPCs, after as extensive surgery as possible, adjuvant therapy consisting of multiagent chemotherapy and craniospinal radio-therapy, delayed in very young children, should be considered. The BEP regimen de-tailed above plus radiotherapy gave a good re-sult in the patient described.
References
1. Ellenbogen RG, Scott RM. Choroid plexus tumors. In: Kaye AH, Laws ER Jr, editors. Brain tumors: An encyclopedic approach. 2nd ed. London: Churchill Livingstone; 2001. p. 552-62.
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2.   Wolff JEA, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. Br J Cancer 2002; 87: 1086-91.
3.   Geerts Y, Gabreëls F, Lippens R, Merx H, Wesseling P. Choroid plexus carcinoma: A report of two cases and review of the literature. Neuropediatrics 1996; 27: 143-8.
4.   Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery 1989; 25: 327-35.
5.   Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, Kalifa C, Brunelle F, Sgouros S, et al. Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 1998; 88: 521-8.
6.   St Clair SK, Humphreys RP, Pillay PK, Hoffman HJ, Blaser SI, Becker LE. Current management of choroid plexus carcinoma in children. Pediatr Neurosurg 1991-1992; 17: 225-33.
7.   Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA, et al. Choroid plexus carcinoma of childhood. Cancer 1992; 69: 580-5.
8.   Fitzpatrick LK, Aronson LJ, Cohen KJ. Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resect-ed? J Neuro-Oncol 2002; 57: 123-6.
9.   Pierga JY, Kalifa C, Terrier-Lacombe MJ, Habrand JL, Lemerle J. Carcinoma of the choroid plexus: A pediatric experience. Med Pediatr Oncol 1993; 21: 480-7.
10. Duffner PK, Kun LE, Burger PC, Horowitz ME, Cohen ME, Sanford RA, et al. Postoperative chemotherapy and delayed radiation in infants and very young children with choroid plexus car-cinomas. Pediatr Neurosurg 1995; 22: 189-96.
11. Aricň M, Raiteri E, Bossi G, Giordana MT, Corbella F, Locatelli D, et al. Choroid plexus carci-noma: report of one case with favourable response to treatment. Med Pediatr Oncol 1994; 22: 274-8.
12. Palazzi M, Di Marco A, Campostrini F, Grandinetti A, Bontempini L. The role of radio-therapy in the management of choroid plexus neo-plasms. Tumori 1989; 75: 463-9.
13. Duffner PK, Krischer JP, Horowitz ME, Cohen ME, Burger PC, Friedman HS, et al. Second malignancies in young children with primary brain tumors fol-lowing treatment with prolonged postoperative chemotherapy and delayed irradiation: A Pediatric Oncology Group study. Ann Neurol 1998; 44: 313-6.
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