KISEP J Korean Neurosurg Soc 34 : 480-483, 2003

Case Report Huge Choroid Plexus Carcinoma in an Infant

Hae Yoo Kim, M.D., Yong Soon Hwang, M.D., Hyung Shik Shin, M.D., Sang Keun Park, M.D. Department of Neurosurgery, College of Medicine, Inje University, Sanggye Paik Hospital, Seoul, Korea

Choroid plexus carcinoma is a rare malignant brain tumor that occurs predominantly in childhood. A 203-day-old infant was admitted to our hospital with macrocephaly and right hemiparesis. The skull protruded diffusely in the left parietal area. Brain magnetic resonance imaging) revealed a huge mass in the left ventricle. We performed an open biopsy and discovered a choroid plexus carcinoma. The tumor bled very easily and hemostasis was difficult. After three cycles of chemotherapy, we resected the entire mass in a staged operation. Chemotherapy enabled us to resect the entire tumor by reducing its size. We report this case to stress the benefits of preoperative chemo- therapy and review the relevant literature.

KEY WORDS : Choroid plexus carcinoma Perioperative chemotherapy Brain tumor

Introduction

horoid plexus carcinoma(CPC) is very rare, occurs C predominantly in infants, and tends to spread via the cerebrospinal fluid(CSF). The treatment of choice is surgical removal. Poor results are obtained with subtotal CPC resection and no adjuvant therapy. Usually radiation therapy and chemotherapy are used as adjuvants. We treated an infant admitted with macrocephaly and right hemiparesis. A B Preoperative chemotherapy was used as adjuvant therapy, Fig. 1. Brain computed tomography scan revealing a 10×12×10 and the tumor was removed entirely in a 3-stage operation. We cm round mass involving almost the entire left hemisphere. present here a case of an infant with a huge CPC that was treated with surgery and preoperative chemo-therapy, and computed tomography(CT) scan and magnetic resonance(MR) emphasize the benefits of chemotherapy as an adjuvant therapy image showed a huge mass that occupied most of the left in CPC. hemisphere of the brain(Fig. 1,2). The mass was assumed to arise from the left trigon of the lateral ventricle, and it measured Case report 10 12 10cm. The mass enhanced very well, and showed heterogenous intensity. In T1-weighted MRI images (T1WI), 203-day-old infant was admitted to our hospital with a the mass showed isosignal intensity, good enhancement, and A skull deformity and right hemiparesis, which were first multilobulation. The differential diagnosis included primitive noted 1 month earlier. At admission, the left parietal area neuroectodermal tumors (PNET), choroid plexus tumor, and protruded diffusely, and the circumference of the head was 45 ependymoma. A left frontotemporal cran-iotomy was performed cm. The infant was in the 98th percentile on a pediatric growth and a biopsy speci-men was obtained. The pathology indic-ated curve, and could not support its head. There were no specific choroid plexus carcinoma (Fig. 3). findings during delivery and development. The initial brain Few cells were in the papillary config-uration, and the most were irregularly arr-anged. Received：July 21, 2003 Accepted：August 8, 2003 The cells showed the characteristics of a malignant tumor : Address for reprints：Yong Soon Hwang, M.D., Department of nuclear pleomorp-hism, high nuclear/cytoplasmic ratio and Neurosurgery, College of Medicine, Inje University, Sanggye Paik Hospital, 761-1 Sanggye-dong, Nowon-gu, Seoul 139-707, Korea frequent mitosis. As seen radiologically, the tumor was very Tel：02) 950-1035 , Fax：02) 950-1040 large. In addition, it bled easily and hemostasis was very difficult. E-mail：[email protected] Therefore, the initial operation was a simple biopsy. Over time,

480 J Korean Neurosurg Soc 34 HY Kim, et al.

A B A B

Fig. 4. Pre- (A) and post- (B) chemotherapy T1 weighted enhanced coronal magnetic resonance images reveal mild volumetric reduction and consolidation of the tumor after chemotherapy.

C D

Fig. 2. FLAIR (A), T1 enhanced axial (B), T2 axial (C), and T1 pre- enhanced sagittal (D) magnetic resonance images reveal a huge round mass in the left hemisphere. A B

the mass protruded from the skull defect and the cranial deformity worse- ned. Chemotherapy was started to red- uce the size of the C D mass to facilitate resection. The ch- Fig. 5. Postoperative magnetic resonance image(FLAIR (A), T1 emotherapy regi- enhanced axial (B), T2 axial (C), and T1 pre-enhanced sagittal (D)) reveals total resection of the tumor, with a subdural cerebrospinal fluid men involved 8 collection. This is another complication following tumor resection. drugs given in one day to minimize with an interval of two to four weeks between cycles. After bone marrow sup- preoperative chemotherapy, the tumor was 30% smaller pression : vincris- radiologically and showed central consolidation(Fig. 4). A tine, hydroxyurea, second operation was performed after three cycles of chemo- procarbazine, CC- therapy over 3 months. Although there were some change in Fig. 3. Photographs of the pathological NU,cisplatin, cyt- the tumor, it was still large and bled easily, causing many specimen show a loss of papillary differen- tiation and high cellularity in the area osine, arabinoside, difficulties during surgery. Total removal was possible with a surrounded by the white arrows and high-dose methy- staged operation(Fig. 5). A volcano-like skull deformity glandular formation in the area surrounded by the black arrows(H&E100, upper). lprednisolone, and remained, and the dead space was filled with CSF, which Nuclear pleomorphism suggests the cyclophospham- formed a subdural hygroma. A subduroperitoneal shunt was malignant nature of the tumor(H&E400, ide10). Three cycles inserted on the twelfth postoperative day for the subdural lower). The remained papillary architecture in brain tissue suggests the tumor originates of chemotherapy hygroma. Clinically, the patient was well at discharge, although from the choroid plexus. were performed neurological signs remained, including right hemiparesis

VOLUME 34 November, 2003 481 Choroid Plexus Carcinoma and right facial palsy. The skull defect, skull deformity, and more studies of stereotactic radiotherapy and local radiotherapy subdural hygroma were other problems requiring treatment. are necessary. Intraoperative radiotherapy as been reported to have a significant effect in inducing remission following Discussion surgical excision of malignant gliomas8). Radiation therapy as an adjuvant therapy after surgery has been reported to improve horoid plexus tumors(CPT) include choroid plexus survival in CPC if the patients is not too young14). Nevertheless, C papilloma(CPP) and choroid plexus carcinoma(CPC). radiation therapy is not used commonly because it has severe There are clear criteria for differentiating CPC from CPP : intellectual and endocrinological sequelae in the young. As formation of solid sheets of cells, nuclear pleomorphism, high CPC occurs in infants or young children and CSF dissemina- nuclear/cytoplasmic ratio, atypical and frequent mitoses, areas tion is frequent, whole central nervous system (CNS) axis of necrosis and invasion of the surrounding normal sube- radiation is inevitable. Radiation therapy is not used often as pendymal brain tissue5). CPC arises from neuroectoderm and is adjuvant therapy. a very rare tumor with a poor prognosis. Greenberg5) reported There have been many reports of the relative effectiveness of that CPC constitutes 0.4~0.6% of all brain tumors at all ages, chemotherapy. Chemotherapy can reduce the volume and 2~4% of tumors occurring in childhood, and 10~20% of vascularity of a tumor, allowing gross total resection. Therefore, intracranial tumors manifesting in the first year of life. The bulk chemotherapy is the most commonly used adjuvant therapy. of choroid plexus tumors in childhood are papillomas, but However, chemotherapy of a subtotally resected CPC is not 20~40% are carcinomas. About 80% of all cases of CPC occur very effective. Recently, it was recommended that gross total in children, and there have been a few published cases in resection of CPC be achieved or perioperative chemotherapy Korea6,7). The average annual incidence of this disease is 0.3 used to improve the prognosis1,9). The reported 5-year survival per million. Overall, 50, 40, and 5% of CPT occur in the lateral, rate is 26%2). However, patients undergoing gross total fourth, and third ventricles, respectively. Typically, most resection, either as part of the initial surgery or after adjuvant patients present with evidence of raised intracranial pressure. therapy, showed excellent survival with a rate of 86%2). In Infants present with increased head circumference. Young another report, survival was 84% for cases with gross total children present with vomiting, lethargy and strabismus with or resection as compared to 18% for patients with subtotal without a focal deficit5). resection4). Hoffman reported that preoperative chemotherapy The histological criteria of CPC include : 1) transition from reduces the size and vascularity of CPC, facilitating tumor normal to abnormal choroid plexus, 2) cellular (or nuclear) removal, so that most children can be cured of their CPC2). atypia, 3) invasion of adjacent neural tissue, 4) loss of papillary architecture, 5) necrosis, 6) frequent mitosis and 7) a positive Conclusion reaction with cytokeratin3,7,10,11,13). It is very difficult to remove CPC surgically, because the tumor is highly vascular, with is a rare malignant tumor occurring in infants or young choroid plexus hypertrophy and an indistinct boundary with CPC children. Its prognosis is poor in many cases. However, invasion of the surrounding tissue. In childhood or infancy, the prognosis is excellent with gross total resection with or total resection has a high mortality and morbidity. In one without adjuvant therapy. We experienced a case of CPC, contemporary report, morbidity was as high as 30% due to which was very large, and achieved gross total resection using excessive intraoperative bleeding12). preoperative chemotherapy. As demonstrated in this case, we Nonetheless, it is clear that gross total resection has a beneficial can improve the prognosis of CPC using proper adjuvant impact on the outcome5). Therefore, the treatment of choice is therapy when a tumor is very large and initially cannot be as large a surgical resection as possible. In one clinical study, resected totally. the reported survival for cases with gross total resection was 84% compared to 18% for patients with subtotal resection4). Acknowledgement This work was presented as a Poster at 2002 Annual Autumn Meeting of That is, gross total resection is a unique prognostic factor for The Korean Neurosurgical Society success. However, the factors limiting the likelihood of gross total resection include the degree of invasion of the surrounding References brain tissue and the extent of vascularity5). 1. Allen J, Wisoff J, Melson L, Pearce J, Arenson E : Choroid plexus Adjuvant therapy, including radiotherapy or chemotherapy, is carcinoma responses to chemotherapy alone in newly diagnosed young children. J Neurooncol 12 : 69-74, 1992 needed to treat CPC. The benefit of radiotherapy is unclear and 2. Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A,

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