Hematolymphoid Tumors Involving the CNS Flashcards
A 50-year-old patient presents in OPD with symptoms of mental status changes, increased ICP, generalized seizure, and multiple cranial palsies. Risk factors for CNS lymphomas are immunosuppression (AIDS, transplants), Epstein-Barr virus, and collagen vascular disease. What is the best statement regarding management of these tumors?
● A. Treatment is usually with radiotherapy and steroids with very good response initially to steroids (short-lived disappearance also known as“Ghost Tumors”)
● B. There can be a need for placement of ventricular access reservoir for chemotherapy
● C. Biopsy should be taken prior to commencement of treatment to confirm the diagnosis
● D. Debulking of extremely large tumors causing symptoms should be done
● E. All of the above
E. All of the above
The essential diagnostic criteria for primary diffuse large B-cell lymphoma of the CNS are biopsy-proven mature large B-cell lymphoma confined to the CNS at presentation and expression of one or more B-cell markers (CD20, CD19, CD22, CD79a, PAX5).
Which of the following are other types of CNS lymphoma?
● A. Immunodeficiency-associated CNS lymphoma (AIDS related, EBV related, lymphomatoid granulomatosis)
● B. Intravascular large B-cell lymphoma
● C. Miscellaneous rare lymphomas in the CNS (low grade B-cell lymphomas, T-cell and NK/T-cell lymphomas, anaplastic large lymphoma)
● D. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) of the dura
● E. All of the above
E. All of the above
Following are the conditions with increased risk of CNS lymphoma except?
● A. Collagen vascular disease (SLE, Sjogren’s syndrome, rheumatoid arthritis)
● B. Organ transplant recipients (post-transplant lymphoproliferative disease) and AIDS patient
● C. Low incidence in elderly due to good immune system in these patients
● D. Severe congenital immunodeficiency syndrome
● E. Epstein-Barr virus related
C. Low incidence in elderly due to good immune system in these patients
What are the common sites of primary CNS lymphomas (PCNSL)?
● A. Temporal and occipital lobes of brain
● B. Corpus callosum, basal ganglia, periventricular region, and cerebellum
● C. Brainstem, leptomeninges, and spinal cord
● D. Suprasellar region and pineal region
● E. None of the above
B. Corpus callosum, basal ganglia, periventricular region, and cerebellum
A patient presents with painful skin nodules and plaques in the abdomen and lower extremities, fever, cranial neuropathies, and multifocal cerebrovascular events. Biopsy showed malignant lymphoid cells in the small arteries, veins, and capillaries with little or no parenchymal extension. What is the most likely diagnosis in this patient?
● A. Fungal infection of CNS blood vessels
● B. Inflammation of CNS blood vessels due to chronic infection
● C. Intravascular lymphomatosis or angioendotheliomatosis
● D. B cell lymphoma of CNS vasculature
● E. T cell lymphoma of the CNS
C. Intravascular lymphomatosis or angioendotheliomatosis
Following are the investigations and their findings in PCNSL except?
● A. Multiple hyperdense lesions on CT of brain which are contrast enhancing and shows as fluffy cotton balls
● B. Bright on DWI and isointense to hypointense on ADC map
● C. Low levels of serum lactate dehydrogenase indicates rapid cell turnover and is an independent poor prognosticator
● D. Sensitivity of CSF analysis is higher when more than 10.5 mL of CNS is taken with up to 3 LPs
● E. Immunoglobulin analysis shows a single elevated immunoglobulin heavy chain band with lymphoma
C. Low levels of serum lactate dehydrogenase indicates rapid cell turnover and is an independent poor prognosticator
Radiation therapy for PCNSL consists of whole brain radiation after biopsy with 40 to 50 gray total dose in 1.8 to 3 gray daily fraction while chemotherapeutic agents are methotrexate, which is given through intraventricular access device with
6 doses of 12 mg twice a week combined with IV leucovorin, and rituximab. What is the management for overdosage of methotrexate?
● A. Ventricular lumbar perfusion over several hours with 240 mL of warm saline, 2000 U of carboxypeptidase G2 intrathecally with IV dexamethasone and IV leucovorin
● B. Stopping the drug and start draining CSF through LP
● C. Draining the CSF and IV steroids
● D. Stopping the drug and giving IV steroids with intrathecal (IT) leucovorin
● E. None of the above
A. Ventricular lumbar perfusion over several hours with 240 mL of warm saline, 2000 U of carboxypeptidase G2 intrathecally with IV dexamethasone and IV leucovorin
With no treatment of PCNLS, median survival is 1.8 to 3.3 months following diagnosis. Which statement is correct regarding median survival after radiotherapy?
● A. There is no effect of radiotherapy on median survival
● B. Median survival is 10 months with 47% at 1 year, 16% at 2 years, 8% at 3 years, and 3 to 4% at 5 years
● C. Median survival is 87% at 1 year, 47% at 2 years, 16% at 3 years, 8% at 5 years
● D. Increase in median survival is according to the age of the patient
● E. There is no role of chemotherapeutic agent on recurrence of the tumor
B. Median survival is 10 months with 47% at 1 year, 16% at 2 years, 8% at 3 years, and 3 to 4% at 5 years
The essential diagnostic criteria of immunodeficiency-associated CNS lymphomas are a B cell lymphoma confined to the CNS at presentation, in an immunodeficient patient and EBV positivity of tumor cells. What is the median survival of patients with this disease?
● A. 1 to 3 months
● B. 3 to 5 months
● C. 5 to 7 months
● D. 7 to 9 months
● E. 9 to 11 months
B. 3 to 5 months
A patient presents with tender enlarging skull mass. Skull X-ray shows round or oval, sclerotic, punched-out skull lesion with sharply defined margins, involving both inner and outer tables (the disease begins in the diploic space) often with beveled edges, and a central bone density is occasionally noted. CT of brain showed characteristic appearance of a soft tissue mass
within area of bony destruction having a central density. What is the location of Langerhans cell histiocytosis?
● A. Craniofacial bones and skull base favoring the parietal and frontal bones
● B. Hypothalamic pituitary region
● C. Meninges
● D. Choroid plexus
● E. All of the above
E. All of the above
What is the pathologic difference between primary and secondary CNS lymphomas?
● A. N/C ratio
● B. Mitotic activity
● C. Ki67 index
● D. Hyper-/hypochromasia
● E. No difference
E. No difference
What is the most common supratentorial location of PCNSL?
● A. Frontal lobe
● B. Temporal lobe
● C. Parietal lobe
● D. Occipital lobe
● E. Cerebellum
A. Frontal lobe
Which virus is most frequently associated with PCNSL in AIDS patient?
● A. Epstein Barr
● B. H. influenzae
● C. Human papilloma virus
● D. Norovirus
● E. Respiratory syncytial virus
A. Epstein Barr
What is the histopathologic workup to differentiate B-cell from T-cell variant of PCNSL?
● A. Gram staining
● B. Light microscopy
● C. Frozen section
● D. Electron microscopy
● E. Immunohistochemical stains
E. Immunohistochemical stains
A patient presented with PCNSL. He was sent to ophthalmologist for visual problems and an intraocular lymphoma was diagnosed. At what dose of radiotherapy the ocular lymphoma can be managed?
● A. 5 Gy
● B. 7 Gy
● C. 15 Gy
● D. 45 Gy
● E. 54 Gy
B. 7 Gy
