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NEUROSAE 2018 ANNUAL MEETING EDITION (VOLUME 10, ISSUE 3)
QUESTION 23 OF 100
A 51-year-old man presents with a 6-month history of headaches characterized by sharp, stabbing pain, pressure sensations, and occasional throbbing. The headaches occur at any location and on both sides of the head. He reports having headaches 15 days a month during which time he also is lightheaded, dizzy, and sensitive to light. The pattern and frequency have not changed substantially since onset, and he denies a history of headaches before now. Ibuprofen and naproxen provide some relief. The patient has a history of anxiety, asthma, and hypothyroidism, and current medications include levothyroxine, trazodone, and an albuterol inhaler. Vital signs are as follows: BP 129/74 mm Hg, HR 98 beats/min, temperature 98°F (36.6 C). Results of neurologic examination are unremarkable. An MRI scan with gadolinium is shown. Which of the following is the most likely diagnosis?
D. Ganglioglioma **
** = Your answer
The patient’s MRI findings suggest hemangioblastoma, a benign tumor composed of an overgrowth of capillaries. These tumors most commonly develop in the posterior fossa and may cause increased intracranial pressure (including headache) and cerebellar dysfunction. According to the World Health Organization classification of CNS tumors, hemangioblastomas are considered grade I and may be associated with von Hippel-Lindau syndrome, in which case they can occur throughout the CNS and in the retina. On MRI scans, the cystic component of the tumor is T1 hypointense and T2 hyperintense; the solid pial component is T1 isointense and slightly T2 hyperintense. Inside the solid component, serpiginous flow voids may be seen. Following administration of contrast, marked enhancement of the subpial nodule can be seen without enhancement of the cyst wall, as shown in the patient’s MRI scan. The patient underwent surgical removal, and results of tumor biopsy confirmed the diagnosis. Meningiomas are highly vascular, extra-axial tumors that typically show uniform, avid enhancement on MRI scans with contrast. The patient’s lesion is not extra-axial. Glioblastomas, commonly known as grade IV astrocytomas, are the most common and most lethal type of astrocytomas, with a median survival of 15 months. These tumors are slightly more common in men age > 50 years and almost always occur in the cerebral hemispheres. Imaging studies at the time of diagnosis may show a discrete mass with neoplastic cells spread along white matter pathways throughout the brain. MRI scans with contrast show heterogeneous enhancing patterns with central nonenhancing areas representing necrotic tissue. Gangliogliomas are slow-growing tumors that usually occur in children and young adults. These tumors typically are cystic, with or without a solid component, and have a variable degree of enhancement and calcifications. Oligodendrogliomas usually have some calcification and show less avid enhancement on MRI scans with contrast.
* Baig MA, Klein JP, Mechtler LL. Imaging of brain tumors. Continuum Lifelong Learning Neurol. 2016;22(5):1529-1552.