Craniopharyngioma in an Adult
Kevin Pierre, BS1 • Laura Magnelli, MD1,2 • Priya G. Sharma, MD1,2
1University of Florida College of Medicine
2University of Florida Department of Radiology
Pierre K, Magnelli L, Sharma PG. Craniopharyngioma in an adult. Consultant. 2020;60(8):22-23. doi:10.25270/con.2020.05.00015
Received January 2, 2020. Accepted April 9, 2020.
The authors report no relevant financial relationships.
Priya G. Sharma, MD, Assistant Professor, Department of Radiology, Divisions of Pediatric and Abdominal Imaging, University of Florida College of Medicine, 1600 SW Archer Rd, PO Box 100374, Gainesville, FL 32608-0374 (firstname.lastname@example.org)
A 30-year-old woman presented with a syncopal episode 3 days prior. She reported having stood up from the kitchen table, becoming dizzy, and falling to the floor. She felt that she was “out for seconds.” Family members who were present noted no seizures. She also reported chronic headaches and a 2-week history of nausea and vomiting.
Physical examination. Her lungs were clear to auscultation. Her heart had a normal rate and regular rhythm. Her abdomen was soft and nontender. She was oriented to person, place, and time.
In the right eye, there was grade 3 papilledema and an enlarged blind spot. In the left eye, there was a relative afferent pupillary defect, a dense scotoma, and visual acuity worse than 20/400 in the periphery. Extraocular movements were intact, and pupils were equal, round, and reactive to light. Facial sensation was intact, and her face moved symmetrically. Hearing was normal, and her palate elevated symmetrically. There were no abnormalities in head rotation against resistance or in shoulder shrug against resistance. All muscle groups were 5/5 in strength. She followed complex commands.
Diagnostic tests. Magnetic resonance imaging (MRI) of the brain was obtained. T1-weighted imaging without contrast showed a hypointense lesion within the third ventricle (Figure 1). T1-weighted imaging with contrast showed an enhancing third ventricular mass with nonenhancing intratumoral hemorrhage and peripheral calcifications; there was also ventriculomegaly from obstruction of the third ventricle (Figures 2 and 3).
Figure 1. T1-weighted MRI without contrast showed a hypointense lesion within the third ventricle.
Figures 2 and 3. T1-weighted MRI with contrast showed an enhancing third ventricular mass with nonenhancing intratumoral hemorrhage (curved arrows) and peripheral calcifications (line arrows). There was also ventriculomegaly from obstruction of the third ventricle.
A biopsy was performed, the results of which showed a craniopharyngioma, papillary type, World Health Organization (WHO) grade I.
Treatment and outcome. An external ventricular drain was placed to relieve the hydrocephalus. She had a ventriculoperitoneal shunt placed and subsequently underwent a bifrontal craniotomy for resection of the craniopharyngioma, followed by radiation therapy.
Shortly after discharge, the patient developed endocrinologic dysfunction with hyperglycemia, along with new-onset bilateral frontal lobe seizures. She was stabilized with an insulin drip, and her seizures were well controlled with lacosamide.
She was left with anosmia and decreased vision in the left eye, but otherwise has been doing well. She has scheduled follow-up visits with neurology, ophthalmology, and endocrinology specialists. She is on a medication regimen for treatment of her hypopituitarism.
Discussion. Craniopharyngiomas occur in a bimodal distribution, with peak incidences from 5 to 14 years of age and at 50 to 74 years.1 The papillary subtype generally only presents in adults, whereas the adamantinomatous subtype presents in all ages. These tumors behave similarly and have similar survival and quality of life outcomes in adults and children.2,3
Craniopharyngiomas are tumors of the epithelial remnants of the Rathke pouch,4 a division of the oral ectoderm, that develop in the sellar, suprasellar, or parasellar region.2,4 Although they are benign WHO grade I tumors,5 they often cause significant morbidity due to their potentially large sizes. Craniopharyngiomas, particularly the adamantinomatous subtype,6 cause invasion of surrounding structures, including the pituitary stalk, pituitary gland, optic nerve and cerebrospinal fluid outflow tracts. Patients therefore often present with signs of hypopituitarism, menstrual disorders, visual field deficits, decreased visual acuity, headache, and nausea/vomiting.7
Diagnosis initially involves computed tomography (CT) and MRI with and without contrast enhancement. CT best assesses the calcification, while MRI best assesses the extent of the tumor and allows for neurosurgical planning.8 Both craniopharyngioma subtypes normally show solid components that vividly enhance with gadolinium. They also show cystic components, although cysts are a less prominent feature in the papillary subtype, since they are more solid in nature. The adamantinomatous subtype is more likely to show calcified components, although this is a less reliable finding in adults, since calcification occurs less often in that age group.9 The papillary subtype is generally isointense to slightly hypointense on T1-weighted MRI,10 as in our patient’s case, while the adamantinomatous subtype is more likely to be hyperintense.9 Generally, however, the subtypes are not reliably distinguished on imaging, leaving biopsy as the gold standard.11 Our patient’s case further exemplifies this in that it shows calcifications, which are generally only seen in the adamantinomatous subtype.9
Treatment involves surgery followed by radiotherapy.8 The tumor can be accessed by transsphenoidal approach or craniotomy, depending on the size and location. The patient must then follow care with a multidisciplinary team, including endocrinologists, ophthalmologists, neurosurgeons, and primary care providers to treat for tumor- or treatment-related issues.7 These tumors have a good prognosis with an 85% to 90% 10-year survival rate.2,3
- Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998;89(4):547-551. doi:10.3171/jns.1998.89.4.0547
- Karavitaki N, Brufani C, Warner JT, et al. Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol (Oxf). 2005;62(4):397-409. doi:10.1111/j.1365-2265.2005.02231.x
- Van Effenterre R, Boch A-L. Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 2002;97(1):3-11. doi:10.3171/jns.2002.97.1.0003
- Jane JA Jr, Laws ER. Craniopharyngioma. Pituitary. 2006;9(4):323-326. doi:10.1007/s11102-006-0413-8
- Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114(2):97-109. doi:10.1007/s00401-007-0243-4
- Weiner HL, Wisoff JH, Rosenberg ME, et al. Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery. 1994;35(6):1001-1011. doi:10.1227/00006123-199412000-00001
- Zoicas F, Schöfl C. Craniopharyngioma in adults. Front Endocrinol (Lausanne). 2012;3:46. doi:10.3389/fendo.2012.00046
- Garnett MR, Puget S, Grill J, Sainte-Rose C. Craniopharyngioma. Orphanet J Rare Dis. 2007;2:18. doi:10.1186/1750-1172-2-18
- Lee IH, Zan E, Bell WR, Burger PC, Sung H, Yousem DM. Craniopharyngiomas: radiological differentiation of two types. J Korean Neurosurg Soc. 2016;59(5):466-470. doi:10.3340/jkns.2016.59.5.466
- Sartoretti-Schefer S, Wichmann W, Aguzzi A, Valavanis A. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am J Neuroradiol. 1997;18(1):77-87.
- Eldevik OP, Blaivas M, Gabrielsen TO, Hald JK, Chandler WF. Craniopharyngioma: radiologic and histologic findings and recurrence. AJNR Am J Neuroradiol. 1996;17(8):1427-1439.