Neuro-oncology Board Review 5/14/2018

Question 1 A 62-year old man presents with a first time seizure to the emergency room, and is found to have weakness, hyperreflexia in his right extremities, and right homonymous hemianopsia. After MRI showed an enhancing mass, the patient underwent resection, which showed the following. Which of the following pathology tests will portend a BETTER prognosis.

MGMT unmethylated p53 mutation 1p and 19q maintained (no deletion) IDH1 wildtype variant (no mutation) IDH1 R132H mutation

Question 2: Which of the following is clearly associated with formation of gliomas? Use of cell phones Immunocompromise Viral infections Treatment with Ocrelizumab Radiation Exposure

Question 3) A 50-year old man presents with progressive left leg clumsiness, and is found to have the following on MRI. Which of the following is correct regarding this tumor?

This tumor is more common in men It may be associated with neurofibromatosis Type 2 These are rapidly-growing infiltrative tumors A dural tail on MRI is not likely to be seen with these type of tumors Previous radiation is not associated with this type of tumor.

More than half of meningiomas are associated with loss of chromosome 22

Question 4 A 42-year-old-man presents with a seizure, and imaging showed a partially calcified lesion in the L frontal lobe. Resection shows the pathology below. What is the diagnosis?

Primary CNS Lymphoma DNET (Dysembryoplastic Neuro-ectodermal Tumor) Oligodendroglioma Grade II Astrocytoma Further molecular testing needs to be performed.

2016 WHO Classification

The neurological manifestations may respond to ACTH. Question 5 A 5 year-old boy is brought in for evaluation of abnormal jerks and chaotic EOM in all directions. He is also ataxic when attempting to walk and has sudden brief truncal and limb jerks. Which of the following is incorrect regarding his condition? This paraneoplastic syndrome is most commonly associated with medulloblastoma. In adults, this condition may be associated with breast and small cell lung cancer. The neurological manifestations may respond to ACTH. Resection of the primary tumor will lead to resolution of this syndrome In adults, it may be associated with anti-Ri antibodies

Paraneoplastic Opsoclonus myoclonus syndrome In children, it is most commonly associated with neuroblastoma (not medulloblastoma), and may be mediated by anti-Hu antibodies. Neuroblastoma is responsive to ACTH. Treatment of the tumor resolves symptoms. This syndrome can present in adults with breast, ovarian, and small lung cancer. Anti-Ri (aka ANNA-2) antibodies can be seen in this syndrome, and are associated with breast cancer, and a small percentage of small cell lung cancer. Anti-Hu (aka ANNA-1) antibodies have been reported in patients with small cell lung cancer.

Question 6 A patient presents with aphasia, and MRI shows the following. SWI sequence (not shown) shows dropout within the lesion. What kind of tumor is this most likely. Glioblastoma Prostate Ependymoma Lung metastasis Teratoma

Hemorrhagic metastases: M: melanoma: metastatic melanoma to brain R: renal cell carcinoma C: choriocarcinoma T: thyroid carcinoma, teratoma B: bronchogenic carcinoma B: breast carcinoma *lung mets are less frequently hemorrhagic, but are more common, so can still be a hemorrhagic met

Question 7 A 35 year-old man with HIV presents with headaches and altered mental status, and is found to have an intracranial mass. A biopsy is obtained, and shows the following. Which of the following is incorrect regarding this condition?

It is most commonly a diffuse, large B-cell tumor Primary tumors of this type usually involve the parenchyma rather than the leptomeninges. Steroids should be avoided prior to biopsy. It is associated with EBV. Primary therapy involves whole-brain radiation.

Question 8 A 9-year old boy with intractable epilepsy undergoes temporal lobectomy, and pathology is shown as below. Which of the following is incorrect regarding this tumor?

The most common location is the temporal lobe. This lesion often has a cortical or juxtacortical location Floating neurons are characteristic of this lesion. It is a WHO grade I tumor. On MRI-is has a heterogenous contrast-enhancing pattern.

DNET (Dysembryoplastic neuroepithelial tumor) Characteristics: benign tumor that is can cause seizures in children. Imaging: nodular or cystic lesion that is T2-hyperintense and does not enhance. Pathology: prominent clear spaces that contain ganglion cells, and a glial component that resembles oligodendroglioma. Ganglion cells appear to “float” within mucin-filled spaces. At the edges of these tumors there may be cortical dysplasia.