NEURORADIOLOGY H.R. Haghighatkhah MD1 M. Sanei Taheri MD1 Imaging of Monstrous M. Haghighi MD2 S. Shahzadi MD3 Craniopharyngioma: A Pictorial Sh. Birang MD4 Essay Craniopharyngioma is usually a mixed solid and cystic suprasellar tumor, which usually occurs in children and adults in the fourth and fifth decades of life. The tumors are histologically benign and slow growing, but focal invasion and peritumoral fibrosis leads to high tumoral recurrence after surgery. We had 11 patients admitted with different symptoms such as headache, nausea, vomiting and visual loss in whom extensive craniopharyngioma was proved after surgery. In some patients, primary imaging studies were reviewed. In others who presented with new onset symptoms several years after surgery, brain CT scan and MRI studies were performed. On brain CT scan of most cases, a mixed cystic and solid suprasellar mass with calcifications in both solid and cystic parts was detected. In this article, we present some cases of large cra- niopharyngioma with interesting extensions and invasions to adjacent brain structures. Keywords: Craniopharyngioma, Computed Tomography, Magnetic Reson- ance Imaging Introduction raniopharyngioma is a histologically benign slow-growing tumor (WHO grade Ι),1,2 which accounts for approximately 3% of all intracranial tumors.3,4 C It has a bimodal age distribution with the first peak in children and the second in 1 adults at age 40-60 years. It is not predominant in any sex or race. It originates from the embryonic precursor of adenohypophysis which is called 1. Associate Professor, Department of 1,5,6 Radiology, Shohada Tajrish Hospital, Rathke’s pouch. The adenohypophysis and infandibulum migrate from the Shahid Beheshti University of Medical pharynx to the sella turcica during fetal life. This fact shows that craniopharyn- Sciences, Tehran, Iran. 2. Resident of Radiology, Shohada giomas may occur anywhere along this migration tract.1 Tajrish Hospital, Shahid Beheshti Craniopharyngioma has three histological types: adamantinomatous, papillary University of Medical Sciences, Tehran, Iran. and mixed.1 The adamantinomatous type predominantly occurs in children. 3. Professor of Neurosurgery, Shohada- 1,2,7 Tajrish Hospital, Shahid Beheshti Uni- They are classically cystic tumors with a solid component. The fluid in the versity of Medical Sciences, Tehran, cyst is usually similar to motor oil.8,9 It contains high protein materials, choles- Iran. 4. Associate Professor, Department of terol crystals and blood products. The solid part contains calcification, squamous Radiology, Loghman Hospital, Shahid- or columnar epithelium, fibrosis and inflammation. Complete resection of the Beheshti University of Medical Sciences, Tehran, Iran. tumor is difficult because of this fibrosis and dense adhesion to adjacent struc- tures. This issue leads to frequent recurrence of tumor in patients treated with Corresponding Author Shirin Birang surgery. The papillary type predominantly occurs in adults. It has only a solid Department of Radiology, Loghman component typically without calcification.1,7 This type is more encapsulated and Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran. has less recurrence after surgical resection.1 Tel: +9821 5541 1411 Fax: +9821 5541 1411 Because of the slow-growing nature of tumors, the symptoms develop insi- E-mail: [email protected] diously and the common interval between onset of symptoms and diagnosis is 1- 2 Received September 2, 2009; 2 years. The most frequent reported symptoms are headache, nausea, vomiting Accepted after revision March 8, 2010 and visual disturbance. Growth failure and hypogonadism are also encountered Iran J Radiol 2010;7(2):79-89 frequently. Bitemporal hemianopia is the most frequent disturbance of the visual Iran J Radiol 2010, 7(2) 79 Haghighatkhah et al. field. Other presentations include seizures, preco- showed a tumoral lesion in the suprasellar region cious puberty, inappropriate secretion of ADH, hear- with extension to the middle and posterior cranial ing loss and epistaxis.10 fossa. The tumor is predominantly cystic with solid In patients with raised intracranial pressure or rapid components. Eggshell calcification is seen in the cys- visual function deterioration, we must focus on sur- tic part. The solid part is also calcified in both pop- gical treatment of hydrocephalus or tumor cyst de- corn and solid lump appearances. Right temporal cra- compression. The main management of tumor is sur- niotomy and an intracystic catheter are noted as well. gery with an attempt to gross total resection of tu- On brain MRI study, the mass is seen with both sol- mor, but in cases with extensive tumors in which to- id and cystic parts. The cystic part is hypointense on tal resection is impossible, limited resection with tu- T1W and hyperintense on T2W images. After con- moral debulking is recommended to reduce the tu- trast injection, homogeneous enhancement is seen in moral compression of optic pathways and to help CSF the solid part and rim enhancement is also seen in the drainage. Limited surgical resections are followed by cystic part. The tumor extends from the suprasellar radiotherapy.2 region to sella and then from the perimesencephalic One of the most interesting issues in craniopharyn- cisterns to the posterior fossa, superior cerebellar cis- gioma imaging is its extension and invasion to mul- tern and cerebellum. It also extended to the right lat- tiple brain structures, which is best appreciated on eral ventricle and third ventricle and produces biven- MRI. These tumors have different patterns of exten- tricular hydrocephalus due to compression of fora- sion such as superior extension from the suprasellar men of Monro. Pathologic examination revealed an region with compression of the third or lateral ven- adamantinomatous craniopharyngioma (Fig. 1). tricles, lateral extension to parasellar areas, cavernous sinuses and temporal lobes, anterior extension to Patient 2 frontal lobes with superior displacement of A1 seg- The second patient is an 11-year-old boy who pre- ment of the anterior cerebral artery and anterior sented with visual loss 1.5 years ago. Brain CT scan communicating artery, posterior extension to perime- revealed a tumoral lesion with both solid and cystic sencephalic cisterns, brain stem, compression of the parts in the suprasellar and sellar regions. Calcifica- basilar trunk and involvement of cerebellum and su- tion is seen in the lesion, which has eggshell configu- perior cerebellar cistern and finally inferior extension ration in the cystic part and lumpy appearance is also to sella, sphenoid and ethmoidal sinuses and nasopha- seen in the solid part. The tumor extends to the post- rynx. Identifying these patterns of extension can help erior cranial fossa. It also extends to the third ven- us in the surgical management of craniopharyngi- tricle from its superior margin. On MRI study, a T1 omas to decide whether total or partial resection is isointense and T2 hyperintense cystic lesion is seen in recommended and to differentiate these tumors from the suprasellar region with extension to sella turcica other extensive skull-base tumoral lesions. and involvement of the left parasellar region with left We encountered 11 patients with surgically con- carotid artery encasement and left cavernous sinus firmed craniopharyngioma. These are extensive tu- invasion. The tumor extended to the posterior fossa mors with unusual extension to their neighboring and compressed the brain stem at its posterior mar- brain structures. Their brain CT scan and MR images gin. It deviated the pons, midbrain and fourth ven- are reviewed. tricle to the right. In addition, it compressed the basi- lar trunk at its posterior margin. At the superior mar- Case Presentation gin, the tumor extended to the third ventricle and produced biventricular hydrocephalus. After contrast Patient 1 injection, thin rim enhancement was seen in the cys- tic component and nodular enhancement was en- The first patient is a six-year-old boy who presented countered in the solid part (Fig. 2). with nausea, vomiting and ataxia 2 years after surgic- al treatment of craniopharyngioma. Brain CT scan 80 Iran J Radiol 2010, 7(2) Monstrous Craniopharyngioma: A Pictorial Essay A B C D E F Fig. 1. A six-year-old boy with nausea, vomiting and ataxia 2 years after surgical treatment of craniopharyngioma. G A. Axial contrast enhanced CT scan of the brain, supra and parasellar cystic tumor with rim enhancement, extension to the posterior fossa and encasement of the basilar artery. B. Axial contrast enhanced CT scan of the brain, cystic and solid tumor with calcification and enhancement of solid and cystic parts, tumoral extension to the third ventricle and posterior fossa is seen. C. Axial contrast enhanced CT scan of the brain, extension of craniopharyngioma to the su- pratentorial region with compression of Monro's foramen. Drainage catheter is seen in the cystic part. D. Sagittal T1W MRI with contrast, low-signal cystic tumor with rim enhancement, extension to prepontine cistern and invasion to the midbrain and cerebellum. E. Sagittal T1W MRI with contrast, tumoral extension from perimesencephalic cisterns to the posteriorfossa and supratentorial area with biventricular hydrocephalus induction. F. Coronal T1W MRI with contrast, tumoral extension to cerebellum and lateral ventricles. G. Coronal T2W MRI, high signal cystic tumor with compression of the third ventricle. was seen in the wall of the cystic part after contrast