You are in: eMedicine Specialties > Radiology > BRAIN/SPINE CraniopharyngiomaArticle Last Updated: Feb 21, 2007AUTHOR AND EDITOR INFORMATIONAuthor: Jeffrey R Wasserman, DO, Staff Physician, Department of Diagnostic Radiology, Medical College of Pennsylvania-Hahnemann University Hospital Jeffrey R Wasserman is a member of the following medical societies: American Medical Association Coauthor(s): Robert A Koenigsberg, DO, MSc, FAOCR, Director of Neuroradiology, Professor, Department of Radiology, Drexel University College of Medicine; Kiran Batra, MD, DNB, Fellow, Department of Neuroradiology, Hahnemann University Hospital, Drexel University College of Medicine Editors: Mahesh R Patel, MD, Chief of MRI, Department of Radiology, Santa Clara Valley Medical Center; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Robert L DeLaPaz, MD, Director, Professor, Department of Radiology, Division of Neuroradiology, Columbia University; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences Author and Editor Disclosure Synonyms and related keywords: Rathke pouch tumor, craniopharyngeal duct tumor, hemangioblastoma, ameloblastoma, adamantinoma, dysodontogenic epithelial tumor, adamantinomatous tumor, papillary tumor, sellar craniopharyngioma, prechiasmatic craniopharyngioma, retrochiasmatic craniopharyngioma INTRODUCTIONBackgroundCraniopharyngioma is a histologically benign, extra-axial, slow-growing tumor that predominately involves the sella and suprasellar space. Despite its histologic appearance, craniopharyngiomas occasionally behave like malignant tumors, they can metastasize, and patients can have severe symptoms that usually require surgery and/or radiation therapy (with intracystic chemotherapy in some pediatric patients). Recurrence, both local and along surgical tracts, has been reported, as has meningeal seeding. Characteristic radiographic findings help in differentiating craniopharyngiomas from other tumors that can occur in the same anatomic region. Zenker first described craniopharyngioma in 1857. PathophysiologyCraniopharyngiomas are dysodontogenic epithelial tumors derived from the Rathke cleft, which is the embryonal precursor to the adenohypophysis. The craniopharyngeal duct is the embryonal structure along which the eventual adenohypophysis and infundibulum migrate. Tumors can occur anywhere along the course of this duct from the pharynx to the sella turcica and third ventricle, which partially explains the location of the tumor (see Anatomy). The trigger for tumor growth is not clear; however, 3 distinct subtypes primarily based on histologic appearance have been described: adamantinomatous, papillary, and mixed. Regarding adamantinomatous tumor (pediatric type), the classic and most common appearance of this tumor is that of a cystic tumor, usually with a solid component. A wide variation in size is observed. The cyst contains fluid that can vary in color, but it usually has a tan appearance similar to that of motor oil. The color is the result of suspended blood products, protein contents, and cholesterol crystals within the cyst fluid and may be secondary to repeated hemorrhage within the cystic cavity. Histologically, the cyst has a multistratified squamous epithelium with nuclear palisade, and the solid component demonstrates clumps of wet keratin, dystrophic calcifications, trabeculae, nests, and squamous or columnar epithelium. An extensive fibrosis and inflammation (which is considerably more severe with recurrent tumors than with others) are also observed. These findings may result in dense adhesion to adjacent structures and vessels, which accounts for the difficulty in resecting craniopharyngiomas. Intimate interdigitation of the tumor tissue and encasement of the vasculature of the circle of Willis are often present. Gliosis can make the differentiation of these tumors from a primary glial tumor difficult. The tumoral interdigitation also explains why, recurrence is frequent in these tumors that are histologically benign and that appear to have been totally resected on intraoperative visual inspection. The classic appearance of the papillary variant (adult type) is different from that of the other forms and involves only a solid component, which is typically seen without calcifications. The papillary type is frequently located in the third ventricle. These tumors are usually more encapsulated than the others and therefore more amenable to surgical resection. They demonstrate extensive squamous differentiation with the formation of pseudopapillae. Unless otherwise indicated, the more common adamantinomatous type is the subtype referred to in this article. FrequencyUnited StatesCraniopharyngioma represents approximately 3-5% of intracranial tumors and 6-10% of pediatric brain tumors. In pediatric patients, craniopharyngiomas represent the most common intracranial tumor of nonglial origin and account for approximately 54% of all sellar and prechiasmatic tumors. InternationalThe international frequency is identical to that reported in the United States. Mortality/MorbidityA 5-year survival rate of more than 80% is observed in children when the tumor is treated with surgery and radiation. The overall 10-year survival rate is 64-96%.
RaceNo racial predominance is recognized. SexThe male-to-female ratio is equal. AgeA bimodal age distribution is seen, with the first peak occurring in childhood and early adolescence, predominately at age 5-10 years. The second peak (for papillary types) occurs at age 40-60 years. A nearly even distribution in incidences is observed between both age groups. AnatomyCraniopharyngiomas have been surgically divided into 3 groups: sellar, prechiasmatic, and retrochiasmatic. The tumors occur in suprasellar (75%), suprasellar and infrasellar (21%), or entirely intrasellar (4%) locations. Adamantinomatous-type tumors commonly can grow entirely within the sella, the third ventricle, the sphenoid sinus, or the nasal cavity (in rare cases). Craniopharyngiomas are usually avascular on angiography and may encase or displace vessels forming the circle of Willis. The internal carotid artery (ICA) is displaced laterally, the anterior cerebral artery (ACA) is displaced anteriorly, and the basilar artery is displaced posteriorly. Three specific growth categories based on the relationship of the tumor to the vascular structures and the optic chiasm have been described: type A, type B, and type C. In type A, the anterior communicating artery and the A1 segment of the anterior cerebral artery are not disturbed. This appearance occurs in tumors with little or no suprasellar bulge (ie, they are contained almost entirely within the sella). In type B, the anterior communicating artery and the A1 segment of the anterior cerebral artery are elevated, but no posterior displacement of the basilar artery is observed. The tumor protrudes anteriorly between the optic nerves and pushes the optic chiasm posteriorly. In type C, the anterior communicating artery and the A1 segment of the anterior cerebral artery are elevated, with posterior displacement of the basilar artery and stretching of the posterior communicating arteries. The tumor protrudes posteriorly, pushing the chiasm forward and causing it to abut the tuberculum sellae. These tumors typically obstruct the third ventricle and cause hydrocephalus. Clinical DetailsThe clinical presentation of patients with craniopharyngioma consists of a broad spectrum of symptoms that range from asymptomatic to endocrine, visual, or psychological disorders. Many patients remain asymptomatic, while others have a progressively deteriorating clinical course. Because of the associated and frequent endocrine dysfunction, a complete endocrine pretreatment workup is frequently performed. The most common presenting symptoms are headache, nausea, vomiting, and visual disturbances. The most common visual disturbances are bitemporal hemianopsia, homonomous hemianopsia, and amblyopia. Other common findings include oculomotor palsies, bizarre scotomas, blindness, asymmetric acuity deficiencies, and optic atrophy. Hydrocephalus may result from a tumor that obstructs the third ventricle. Papilledema frequently occurs. Visual disturbances usually improve after treatment, whereas most endocrine dysfunctions do not. In children, growth failure and headaches are part of the most common presentation of pediatric patients with craniopharyngioma, and 5 years is the most common age when children present. Other presenting symptoms are those of pituitary and adrenal hypofunction, diabetes insipidus, obesity, weakness, ataxia, coma, chemical meningitis (from rupture of cyst contents into subarachnoid space), and seizures. In particular, children can present with growth failure, obesity (one third to one half), and hypothyroidism (two thirds). Precocious puberty has been reported, as has diabetes insipidus (which affects up to one fifth of patients). Poor school performance is also common, as are psychological problems. Tumors larger than 5 cm have higher postoperative recurrence and morbidity rates than those of smaller tumors. Preferred ExaminationCT and MRI are the complementary examinations of choice. Today, the best imaging tool is MRI without and with contrast enhancement. CT can clearly demonstrate the characteristic calcifications and size of the tumor, while MRI exquisitely demonstrates the size and extent of the tumor and involvement of the third ventricle. MRI results can confirm cystic features of the tumor. Sequences such as fluid-attenuated inversion recovery (FLAIR), gradient-echo (GRE), diffusion-weighted imaging, as well as MR spectroscopy, can be used to make a confident and correct diagnosis. Plain radiography may show abnormalities; however, CT or MRI is still needed regardless of the plain radiographic findings. CT and MRI have supplanted angiography as the primary diagnostic technique; and today, magnetic resonance angiography (MRA) or CT angiography (CTA) may be helpful in differentiating the tumor from an aneurysm of the anterior communicating artery. In the later postoperative period, CT can be performed to establish the baseline for future follow-up scans and to determine the number and size of residual flecks of calcification. In the immediate postoperative period (first 48 h) and later, gadolinium-enhanced MRI may be performed to establish a baseline appearance and to determine whether residual tumor is present. Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans. As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble movingor straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape. Limitations of TechniquesNonenhanced CT may be required to detect calcifications if typical MRI findings are absent. A papillary-type lesion can be missed on MRI or CT when no characteristic cystic component is present or when lesions are not enhancing after the administration of intravenous (IV) contrast material (as in approximately 10% of patients). MRI cannot be used in patients with pacemakers or implanted ferromagnetic metallic objects or in those with metallic foreign bodies in the brain, spinal cord, or soft tissues near important vascular structures. The use of MRI also is limited in patients with claustrophobia and in those unable to remain stationary for the required time. Both CT and MRI evaluations require IV contrast enhancement; therefore, IV access is needed. DIFFERENTIALSArachnoid Cyst Astrocytoma, Brain Epidermoid, Brain Meningioma, Brain Pituitary Adenoma Rathke Cleft Cyst
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| Media file 1: Contrast-enhanced T1-weighted image demonstrates a complex cystic mass (arrow) in the suprasellar space. | |
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| Media file 2: Sagittal contrast-enhanced T1-weighted MRI demonstrates a complex cystic, suprasellar mass that is heterogeneously enhancing (arrow). | |
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| Media file 3: Axial contrast-enhanced T1-weighted MRI demonstrates enhancement of the solid component (arrows) of the lesion. | |
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| Media file 4: T1-weighted MRI in a 23-year-old woman (same patient as in Images 5-7) demonstrates a suprasellar mass with characteristic intermediate-to-high signal material in the cystic material (arrows). | |
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| Media file 5: Sagittal T1-weighted MRI in a 23-year-old woman (same patient as in Image 4) demonstrates the high signal intensity of the cystic material (yellow arrow). | |
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| Media file 6: Gadolinium-enhanced parasagittal T1-weighted MRI in a 23-year-old woman (same patient as in Image 4) demonstrates the characteristic enhancement of the solid component (arrow) of craniopharyngioma. | |
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| Media file 7: Digital radiograph in a 23-year-old woman (same patient as in Image 4) demonstrates characteristic calcifications (arrow) in the suprasellar space. This appearance can easily be misinterpreted as that of an aneurysm. | |
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| Media file 8: Axial CT scan in a 39-year-old man (same patient as in Images 9-10) obtained without contrast enhancement demonstrates a large, cystic mass (arrow) in the suprasellar space that has predominately fluid attenuation. | |
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| Media file 9: CT scan in a 39-year-old man (same patient as in Image 8) obtained with intravenous contrast agent shows enhancement of the anterior, solid component (arrows). | |
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| Media file 10: Digital radiograph in a 39-year-old man (same patient as in Image 8) demonstrates characteristic expansion of the sella turcica (arrows). | |
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| Media file 11: Angiogram obtained at the same time as Image 11 in anteroposterior projection clearly shows elevations of the A1 segment of the anterior cerebral artery (arrows) and anterior communicating artery (in the same patient as in Image 8). | |
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| Media file 12: Axial contrast-enhanced CT scan in a 65-year-old man demonstrates a large, calcified suprasellar mass with anterior displacement of the A1 segment of the anterior cerebral arteries (yellow arrows). The anterior communicating artery is not well depicted. | |
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| Media file 13: Coronal T1-weighted image in a 65-year-old man (same patient as in Image 12) obtained through the sella turcica. Image demonstrates a predominantly sellar lesion (arrows) with some suprasellar extension. | |
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| Media file 14: Contrast-enhanced T1-weighted image in a 66-year-old woman obtained in a slightly lateral parasagittal plane demonstrates irregular enhancement of the solid components (arrow) and the outer rim of the tumor, which has a predominantly cystic composition. | |
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| Media file 15: Sagittal nonenhanced T1-weighted image demonstrates a heterogeneous, cystic mass (arrows) in the suprasellar space (in the same patient as in Image 14). | |
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Article Last Updated: Feb 21, 2007