The Neuroradiology Journal 19: 330-333, 2006
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CT and MR Imaging in Choroid Plexus Carcinoma. Report of Two Cases R. BALAJI*, K. RAMACHANDRAN*, P. KUSUMAKUMARI**, A.S. KRISHNAKUMAR*, M. VENUGOPAL* * Imageology Division, Regional Cancer Centre; ** Pediatric Oncology Division, Regional Cancer Centre; Trivandrum, Kerala, India.
Key words: choroid plexus carcinoma, lateral ventricle, CT, MRI
SUMMARY – This case report describes the CT and MR appearances of choroid plexus carcinomas in children less than two years of age. MR and CT imaging of this rare tumor revealed intensely enhancing mass lesions in an intraventricular location, the atrium of the lateral ventricle being the preferred site in children. The tumors were associated with hydrocephalus and cerebral parenchymal invasion.
Introduction Choroid plexus carcinomas (CPC) are rare, malignant invasive brain tumors which occur predominantly in infants below two years of age and rarely in childhood between the age group of three to ten years 1. Primary choroid plexus neoplasms constitute only 1-2 % of childhood brain tumors 2,3. Of these, choroid plexus carcinomas account for 10-20% and the remainder are benign choroid plexus papillomas 4,5. Primary CPCs most commonly affect the atria of lateral ventricles in children while in adults the commonest site is the fourth ventricle 6,7. There are very few reports on MRI appearances of this rare tumor 5,8-12. Children with CPC usually present with the signs and symptoms of raised intracranial pressure caused by hydrocephalus and as such the clinical diagnosis is difficult since the symptoms are nonspecific. In this report, we present MRI and CT features of CPCs in two infants. Case Reports Case 1 An 18-month-old boy presented with a history of increasing head size, inability to move the right half of the body and vomiting for a 330
period of three months. Fundus examination revealed signs of raised intracranial pressure. No cranial nerve palsy was observed. Plain CT imaging disclosed a large inhomogeneous, hyperdense soft-tissue mass within enlarged atrium and occipital horn of the left lateral ventricle, and invasion of adjacent brain parenchyma (figure 1). A focal calcific density was also noted within the lesion. There was marked inhomogeneous enhancement after the administration of intravenous contrast (figure 2). The right occipital and temporal horns were moderately dilated suggesting obstructive hydrocephalus. At surgery a pinkish, vascular soft tumor was partially excised from the left lateral ventricle. Case 2 A five-month-old girl was admitted with symptoms of increasing head size, vomiting, lethargy and poor feeding of two weeks duration. She had previously good health and normal developmental history. Physical examination revealed bulging anterior fontanelle with “sunset type” eyes and bilateral papilledema with no neurological deficits or cranial nerve palsy. Coronal and sagittal MR imaging of brain revealed a left lateral ventricular mass having intermediate signal on T1 weighted images
R. Balaji
CT and MR Imaging in Choroid Plexus Carcinoma. Report of Two Cases
Figure 1 Nonenhanced CT showing irregular, hyperdense tumor involving the atrium of the left lateral ventricle.
Figure 2 Tumor showing intense enhancement after contrast administration, focal non-enhancing necrotic areas and parenchymal vasogenic edema.
Figure 3 T1-weighted sagittal MR image of brain after IV gadolinium injection revealing lobular intensely enhancing mass in atrium of the left lateral ventricle.
Figure 4 Post gadolinium T1-weighted coronal MR image demonstrates hydrocephalus and vasogenic edema.
arising from the trigone with extension into the cerebral parenchyma, associated with vasogenic edema and moderate hydrocephalus. The mass showed intense enhancement after IV gadolinium injection (figures 3 and 4). The tumor showed intermediate signal intensity on T2 weighted images. At surgery the intraventricular component of the tumor was completely removed while the resection of the parenchymal part was incomplete because of invasion of the cortical tissue. Histogical examination in both cases showed highly cellular stratified cuboidal tumor or columnar epithelial cells with hyperchromatic or mitotic nuclei that formed irregular papillary or tubular pattern of different sizes in single or multiple layers.
Discussion Choroid plexus carcinomas are malignant brain tumors which are usually large and highly invasive occurring in infants and young children. Choroid plexus papillomas constitute approximately 80% of choroid plexus tumors and the carcinomas make up the remainder 2. In children, the atrium of the lateral ventricle is the most commonly affected site. In adults, the common site is the fourth ventricle, although there are some cases reported in children in this site. Most cases occur before the age of two years and patients usually present with symptoms of raised intracranial pressure or focal neurological deficit. The usual plain CT appearance of a choroid 331
CT and MR Imaging in Choroid Plexus Carcinoma. Report of Two Cases
plexus carcinoma is a large, bulky, heterogeneous, lobulated, intraventricular mass appearing relatively hyperdense to the brain parenchyma. Choroid plexus carcinomas show intense enhancement on contrast administration reflecting their highly vascular nature. Variable areas of central low attenuation represent tumoral necrosis. Calcifications are occasionally seen especially in lesions located near the atria of the lateral ventricles. Extensive peritumoral low attenuation vasogenic edema suggests transgression of the ventricular wall and parenchymal invasion. Choroid plexus papillomas tend to be homogeneous at CT while heterogeneity due to hemorrhage, necrosis and calcification may suggest carcinomatous change 4. On MR imaging CPCs are seen as large irregular cauliflower-like masses arising from the atria of the lateral ventricles with intermediate signal intensity on T1 weighted images while appearing hypo- to iso- intense on T2 weighted images. Areas of tumor necrosis show low signal intensity on T1 weighted images and increased intensity on T2 weighted images. Intense enhancement of the tumor is seen on post-gadolinium T1 weighted scans. The tumor may invade the cerebral parenchyma. Peritumoral vasogenic edema within the adjacent white matter shows up as increased signal on T2 weighted images. Because of the location, these tumors are associated with hydrocephalus. Hydrocephalus occurs in approximately 80% of patients, probably due to the overproduction of CSF or
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tumor CSF seeding, bleeding and mechanical obstruction. CPCs often exhibit hydrocephalus of lesser severity than that associated with choroid plexus papilloma 4,13. Histological examination demonstrates hypercellularity of stratified cuboidal or columnar epithelium with bizarre hyperchromatic nuclei which form papillary or tubular structures in single or multiple layers 14. Choroid plexus carcinomas are distinguished from choroid plexus papillomas by their tendency to invade adjacent neural tissue, loss of regular papillary architecture, high mitotic activity and nuclear atypia 15 . Differential diagnoses in this age group include primitive neuroendocrine tumors, malignant gliomas, metastases to choroid plexus and other intraventricular masses like papillary ependyomomas and pilocytic astrocytoma. Imaging features of choroid plexus carcinoma are nonspecific in distinguishing from these intraventricular lesions. However an enlarged ipsilateral choroid plexus with prominent enhancement (due to increased vascularity) can be suggestive of CPC. To conclude though both CT and MR are useful in diagnosis, MR has the added advantage of multiplanar imaging, which not only confirms the exact intraventricular location but also provides more accurate details of cerebral parenchymal invasion and tumor vascularity which will be of greater assistance to the surgeon. The prognosis is poor if there is residual tumor after surgery. Adjuvant radiation and chemotherapy may prolong survival.
www. centauro. it
The Neuroradiology Journal 19: 330-333, 2006
References 1 Allen JC, Wisoff J, Helson L et Al: Choroid plexus carcinomas. Responses to chemotherapy alone in newly diagnosed young children. J Neurooncol 12: 69-74, 1992. 2 Johnson DL: Management of choroid plexus tumors in children. Pediatr Neurosci 15: 195-206, 1989. 3. Cohen ME, Duffner PK: Choroid plexus tumor In: Cohen ME, Duffner PK, eds.Brain tumors in Children: principles of diagnosis and treatment. 2nd ed. Raven, New York 1994: 363-376. 4 Pascual-Castroviejo, Villarejo F,Perez Higueras A et Al: Childhood choroid plexus neoplasms. A study of 14 cases less than 2 years old Eur J Pediatr 140: 51-56, 1983. 5 Coates TL, Hinshaw DB, Peckman N et Al: Pediatric Choroid Plexus Neoplasms: MR, CT and pathologic Correlation, Radiology 173: 81-88, 1989. 6 Shinoda J, Kawaguchi M, Matsuhisa T et Al: Choroid plexus carcinoma in infants: report of two cases and review of the literature. Acta Neurochir 140: 557-563, 1998. 7 Packer RJ, Perilongo G, Johnson D, et Al: Choroid Plexus Carcinoma of Childhood, Cancer 69: 580-585, 1992. 8 Cila A, Ozturk C, Senaati S: Bilateral Choroid plexus carcinoma of the lateral ventricles. US, CT and MR findings. Pediatr Radiol 22: 136-137, 1992. 9 Tekkok IH, Palagolu S, Erbengi A: Primary choroid plexus carcinoma in an infant. Report of a case evaluated by MRI. J Neurosurg Sci 36: 227-231, 1992. 10 Vazquez E, Ball WS, Prenger EC et Al: Magnetic resonance imaging of fourth ventricular choroid plexus neoplasms in childhood. Pediatr Neurosurg 1748-52, 1991. 11 Grygotis LA, Chew FS: Choroid Plexus Carcinoma of the Lateral Ventricle. Am J Radiol 169: 1400, 1997. 12 Connor SEJ, Chandler C, Bodi L et Al: Preoperative and early post operative magnetic resonance imaging in two cases of childhood choroid plexus carcinoma. Eur Radiol 12: 883-888, 2002. 13 Kendall B, Reider-Grosswasser I, Valentine A: Diagnosis of masses presenting within the ventricles on computed tomography. Neuroradiology 25: 11-22, 1983. 14 Coffin CM, Wick MR, Braun JT et Al: Choroid plexus
carcinoma: Clinicopathologic and immunohistochemical studies. Am J Surg Pathol 10: 394-404, 1986. 15 Newbould MJ, Kesley AM, Arango JC et Al: The choroid plexus carcinomas of childhood: histopathology, immunocytotochemistry and clinicopathological correlations. Histopathology 26: 137-143, 1995.
Dr. Ravikanth Balaji Radiologist Imageology Division Regional Cancer Centre Medical College PO Trivandrum 695011 Kerala - India Tel.: Hospital 91 471 2522434 E-mail: [email protected]
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CT and MR Imaging in Choroid Plexus Carcinoma. Report of Two Cases R. BALAJI*, K. RAMACHANDRAN*, P. KUSUMAKUMARI**, A.S. KRISHNAKUMAR*, M. VENUGOPAL* * Imageology Division, Regional Cancer Centre; ** Pediatric Oncology Division, Regional Cancer Centre; Trivandrum, Kerala, India.
Key words: choroid plexus carcinoma, lateral ventricle, CT, MRI
SUMMARY – This case report describes the CT and MR appearances of choroid plexus carcinomas in children less than two years of age. MR and CT imaging of this rare tumor revealed intensely enhancing mass lesions in an intraventricular location, the atrium of the lateral ventricle being the preferred site in children. The tumors were associated with hydrocephalus and cerebral parenchymal invasion.
Introduction Choroid plexus carcinomas (CPC) are rare, malignant invasive brain tumors which occur predominantly in infants below two years of age and rarely in childhood between the age group of three to ten years 1. Primary choroid plexus neoplasms constitute only 1-2 % of childhood brain tumors 2,3. Of these, choroid plexus carcinomas account for 10-20% and the remainder are benign choroid plexus papillomas 4,5. Primary CPCs most commonly affect the atria of lateral ventricles in children while in adults the commonest site is the fourth ventricle 6,7. There are very few reports on MRI appearances of this rare tumor 5,8-12. Children with CPC usually present with the signs and symptoms of raised intracranial pressure caused by hydrocephalus and as such the clinical diagnosis is difficult since the symptoms are nonspecific. In this report, we present MRI and CT features of CPCs in two infants. Case Reports Case 1 An 18-month-old boy presented with a history of increasing head size, inability to move the right half of the body and vomiting for a 330
period of three months. Fundus examination revealed signs of raised intracranial pressure. No cranial nerve palsy was observed. Plain CT imaging disclosed a large inhomogeneous, hyperdense soft-tissue mass within enlarged atrium and occipital horn of the left lateral ventricle, and invasion of adjacent brain parenchyma (figure 1). A focal calcific density was also noted within the lesion. There was marked inhomogeneous enhancement after the administration of intravenous contrast (figure 2). The right occipital and temporal horns were moderately dilated suggesting obstructive hydrocephalus. At surgery a pinkish, vascular soft tumor was partially excised from the left lateral ventricle. Case 2 A five-month-old girl was admitted with symptoms of increasing head size, vomiting, lethargy and poor feeding of two weeks duration. She had previously good health and normal developmental history. Physical examination revealed bulging anterior fontanelle with “sunset type” eyes and bilateral papilledema with no neurological deficits or cranial nerve palsy. Coronal and sagittal MR imaging of brain revealed a left lateral ventricular mass having intermediate signal on T1 weighted images
R. Balaji
CT and MR Imaging in Choroid Plexus Carcinoma. Report of Two Cases
Figure 1 Nonenhanced CT showing irregular, hyperdense tumor involving the atrium of the left lateral ventricle.
Figure 2 Tumor showing intense enhancement after contrast administration, focal non-enhancing necrotic areas and parenchymal vasogenic edema.
Figure 3 T1-weighted sagittal MR image of brain after IV gadolinium injection revealing lobular intensely enhancing mass in atrium of the left lateral ventricle.
Figure 4 Post gadolinium T1-weighted coronal MR image demonstrates hydrocephalus and vasogenic edema.
arising from the trigone with extension into the cerebral parenchyma, associated with vasogenic edema and moderate hydrocephalus. The mass showed intense enhancement after IV gadolinium injection (figures 3 and 4). The tumor showed intermediate signal intensity on T2 weighted images. At surgery the intraventricular component of the tumor was completely removed while the resection of the parenchymal part was incomplete because of invasion of the cortical tissue. Histogical examination in both cases showed highly cellular stratified cuboidal tumor or columnar epithelial cells with hyperchromatic or mitotic nuclei that formed irregular papillary or tubular pattern of different sizes in single or multiple layers.
Discussion Choroid plexus carcinomas are malignant brain tumors which are usually large and highly invasive occurring in infants and young children. Choroid plexus papillomas constitute approximately 80% of choroid plexus tumors and the carcinomas make up the remainder 2. In children, the atrium of the lateral ventricle is the most commonly affected site. In adults, the common site is the fourth ventricle, although there are some cases reported in children in this site. Most cases occur before the age of two years and patients usually present with symptoms of raised intracranial pressure or focal neurological deficit. The usual plain CT appearance of a choroid 331
CT and MR Imaging in Choroid Plexus Carcinoma. Report of Two Cases
plexus carcinoma is a large, bulky, heterogeneous, lobulated, intraventricular mass appearing relatively hyperdense to the brain parenchyma. Choroid plexus carcinomas show intense enhancement on contrast administration reflecting their highly vascular nature. Variable areas of central low attenuation represent tumoral necrosis. Calcifications are occasionally seen especially in lesions located near the atria of the lateral ventricles. Extensive peritumoral low attenuation vasogenic edema suggests transgression of the ventricular wall and parenchymal invasion. Choroid plexus papillomas tend to be homogeneous at CT while heterogeneity due to hemorrhage, necrosis and calcification may suggest carcinomatous change 4. On MR imaging CPCs are seen as large irregular cauliflower-like masses arising from the atria of the lateral ventricles with intermediate signal intensity on T1 weighted images while appearing hypo- to iso- intense on T2 weighted images. Areas of tumor necrosis show low signal intensity on T1 weighted images and increased intensity on T2 weighted images. Intense enhancement of the tumor is seen on post-gadolinium T1 weighted scans. The tumor may invade the cerebral parenchyma. Peritumoral vasogenic edema within the adjacent white matter shows up as increased signal on T2 weighted images. Because of the location, these tumors are associated with hydrocephalus. Hydrocephalus occurs in approximately 80% of patients, probably due to the overproduction of CSF or
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tumor CSF seeding, bleeding and mechanical obstruction. CPCs often exhibit hydrocephalus of lesser severity than that associated with choroid plexus papilloma 4,13. Histological examination demonstrates hypercellularity of stratified cuboidal or columnar epithelium with bizarre hyperchromatic nuclei which form papillary or tubular structures in single or multiple layers 14. Choroid plexus carcinomas are distinguished from choroid plexus papillomas by their tendency to invade adjacent neural tissue, loss of regular papillary architecture, high mitotic activity and nuclear atypia 15 . Differential diagnoses in this age group include primitive neuroendocrine tumors, malignant gliomas, metastases to choroid plexus and other intraventricular masses like papillary ependyomomas and pilocytic astrocytoma. Imaging features of choroid plexus carcinoma are nonspecific in distinguishing from these intraventricular lesions. However an enlarged ipsilateral choroid plexus with prominent enhancement (due to increased vascularity) can be suggestive of CPC. To conclude though both CT and MR are useful in diagnosis, MR has the added advantage of multiplanar imaging, which not only confirms the exact intraventricular location but also provides more accurate details of cerebral parenchymal invasion and tumor vascularity which will be of greater assistance to the surgeon. The prognosis is poor if there is residual tumor after surgery. Adjuvant radiation and chemotherapy may prolong survival.
www. centauro. it
The Neuroradiology Journal 19: 330-333, 2006
References 1 Allen JC, Wisoff J, Helson L et Al: Choroid plexus carcinomas. Responses to chemotherapy alone in newly diagnosed young children. J Neurooncol 12: 69-74, 1992. 2 Johnson DL: Management of choroid plexus tumors in children. Pediatr Neurosci 15: 195-206, 1989. 3. Cohen ME, Duffner PK: Choroid plexus tumor In: Cohen ME, Duffner PK, eds.Brain tumors in Children: principles of diagnosis and treatment. 2nd ed. Raven, New York 1994: 363-376. 4 Pascual-Castroviejo, Villarejo F,Perez Higueras A et Al: Childhood choroid plexus neoplasms. A study of 14 cases less than 2 years old Eur J Pediatr 140: 51-56, 1983. 5 Coates TL, Hinshaw DB, Peckman N et Al: Pediatric Choroid Plexus Neoplasms: MR, CT and pathologic Correlation, Radiology 173: 81-88, 1989. 6 Shinoda J, Kawaguchi M, Matsuhisa T et Al: Choroid plexus carcinoma in infants: report of two cases and review of the literature. Acta Neurochir 140: 557-563, 1998. 7 Packer RJ, Perilongo G, Johnson D, et Al: Choroid Plexus Carcinoma of Childhood, Cancer 69: 580-585, 1992. 8 Cila A, Ozturk C, Senaati S: Bilateral Choroid plexus carcinoma of the lateral ventricles. US, CT and MR findings. Pediatr Radiol 22: 136-137, 1992. 9 Tekkok IH, Palagolu S, Erbengi A: Primary choroid plexus carcinoma in an infant. Report of a case evaluated by MRI. J Neurosurg Sci 36: 227-231, 1992. 10 Vazquez E, Ball WS, Prenger EC et Al: Magnetic resonance imaging of fourth ventricular choroid plexus neoplasms in childhood. Pediatr Neurosurg 1748-52, 1991. 11 Grygotis LA, Chew FS: Choroid Plexus Carcinoma of the Lateral Ventricle. Am J Radiol 169: 1400, 1997. 12 Connor SEJ, Chandler C, Bodi L et Al: Preoperative and early post operative magnetic resonance imaging in two cases of childhood choroid plexus carcinoma. Eur Radiol 12: 883-888, 2002. 13 Kendall B, Reider-Grosswasser I, Valentine A: Diagnosis of masses presenting within the ventricles on computed tomography. Neuroradiology 25: 11-22, 1983. 14 Coffin CM, Wick MR, Braun JT et Al: Choroid plexus
carcinoma: Clinicopathologic and immunohistochemical studies. Am J Surg Pathol 10: 394-404, 1986. 15 Newbould MJ, Kesley AM, Arango JC et Al: The choroid plexus carcinomas of childhood: histopathology, immunocytotochemistry and clinicopathological correlations. Histopathology 26: 137-143, 1995.
Dr. Ravikanth Balaji Radiologist Imageology Division Regional Cancer Centre Medical College PO Trivandrum 695011 Kerala - India Tel.: Hospital 91 471 2522434 E-mail: [email protected]
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