Number of questions: 26

PDF File Each question has only one answer.

1.   Which of the following(s) is(are) true about childhood meningiomas? Answer

1.      They are more likely to behave more aggressive and recur more frequently.

2.      They are associated with neurofibromatosis 2 (NF2).

3.      They are more likely to be found at infratentorial, intraventricular, or intraparenchymal locations.

4.      Meningioms are common tumors in infants and children.

A.  1, 2, and 3 are true.

B.  1 and 3 are true.

C.  2 and 4 are true.

D.  Only 4 is true.

E.  All of the above.

2.   This primary cerebral tumor was removed from an 18 month-old boy. The most likely diagnosis is: Answer

A.  Ependymoma.

B.  Choroid plexus papilloma.

C.  Pilomyxomoid astrocytoma.

D.  Medulloepithelioma.

E.  Medulloblastoma.

3.   Which type of meningioma is most likely to be found in patients under 20 years of age? Answer

A.  Fibrous meningioma.

B.  Transitional meningioma.

C.  Secretory meningioma.

D.  Chordoid meningioma.

E.  Psammomatous meningioma.

4.   Which of the followings is not true about optic nerve glioma? Answer

A.  Optic nerve gliomas in children tend to behave in a indolent, slowing growing fashion.

B.  Optic nerve gliomas are associated with neurofibromatosis 1 (NF1).

C.  Optic nerve gliomas in adults tend to be non-familial and behave in a benign fashion.

D.  Spontaneous regression has been described in childhood cases of optic nerve gliomas.

E.  Optic nerve gliomas tend to grow outside the optic nerve and expand the subarachnoid and subdual space.

5.   This primary tumor is removed from the cerebellum of a 2 year-old boy. The most likely molecular changes that would associate with this tumor is: Answer

A.  Isochromosome 17.

B.  Deletion of chromosome 22q.

C.  t(11;22)(q24;q12) translocation.

D.  Human homologue of the Drosophilia segment polarity gene (PTCH gene) on chromosome 9q22.3.

E.  None of the above.

6.   Which of the following(s) is(are) true about atypical teratoid/rhabdoid tumor (AT/RT) of the brain? Answer

1.      These rare tumors are equally common in children and in adults.

2.      These tumors typically occur in a pure form without association with non-rhabdoid components.

3.      These tumors share similar molecular aberrations with rhabdoid tumors arising in other locations in adults.

4.      AT/RT are grade IV tumor in the 4 tier system as per the year 2000 Classification of the World Health Organization (WHO).

A.  1, 2, and 3 are true.

B.  1 and 3 are true.

C.  2 and 4 are true.

D.  Only 4 is true.

E.  All of the above.

7.   This intraoperative cytologic preparation is prepared from a primary intracranial tumor. This type of tumor would most likely produce? Answer

A.  Anton syndrome.

B.  Walker-Warburg sundrome.

C.  Wallenberg syndrome.

D.  Parinaud syndrome.

E.  Smith-Magenis syndrome.

8.   Which of the following(s) is(are) true about primary intracranial germinoma? Answer

1.      The most common location is the pineal gland.

2.      Extensive granulomatous changes mimicking infection can occur in these tumors.

3.      The tumor cells are immunoreactive for placental alkaline phosphatase (PLAP).

4.      The tumor cells are immunoreactive for CD117 (c-kit) and OCT4.

A.  1, 2, and 3 are true.

B.  1 and 3 are true.

C.  2 and 4 are true.

D.  Only 4 is true.

E.  All of the above.

9.   In this preparation with Masson's trichrome stain, the most likely diagnosis is: Answer

A.  Desmoplastic medulloblastoma.

B.  Pleomorphic xanthoastrocytoma.

C.  Dysembryoplastic neuroepithelial tumor (DNET).

D.  Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease).

E.  Desmoplastic infantile ganglioma (DIG).

10. Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is associated with? Answer

A.   Ataxia-telangiectasia.

B.  Currarino triad.

C.  Meckel-Gruber Syndrome.

D.  Aicardi syndrome.

E.  Cowden syndrome.

11. Which of the following syndromes are not associated with increased risk for developing malignancy? Answer

A.   Miller-Dieker Syndrome.

B.  Gorlin syndrome.

C.  Turcot syndrome.

D.  Bloom syndrome.

E.  Down syndrome.

12. This photo is taken from a tumor in an enucleated globe. Which of the following is not true regarding this type of tumor? Answer

A.  This type of tumors are positive for neuroendocrine antigens such as synaptophysin.

B.  The familial type of this tumor is associated with germ line mutation of the RB1 gene..

C.  "Trilateral tumor" involving both eyes and pineal gland can occur in patients with this type of tumor.

D.  The ultrastructures of this type of tumor share similarities with photoreceptor cells.

E.  Spontaneous regression is unknown to this type of tumors.

13. This extradural tumor is removed from the spine of a 15 year-old boy and is positive for CD99 on immunohistochemistry. Additional findings would include: Answer

1.      PAS positive, diastase sensitive cytoplasmic substance.

2.      Positive for synaptophysin.

3.      Demonstration of t(11;22)(q24;q12) by fluoresent in situ hybridization (FISH).

4.      Positive for neurofilament protein.

A.  1, 2, and 3 are true.

B.  1 and 3 are true.

C.  2 and 4 are true.

D.  Only 4 is true.

E.  All of the above.

14. Which of the following is true about melanotic neuroectodermal tumor of infancy? Answer

A.  These tumors typically occur as solitary, solid tumor in the brain.

B.  Recurrence is is uncommon and metastasis is practically unknown.

C.  Most tumors occur in older children.

D.  These tumors contain large, pigmented, melanotic cells and small primitive neuroectodermal cells. Synaptophysin is positive in both cell populations.

E.  The large, melanotic cells are usually positive for HMB45 and S100 protein.

15. The most common organ for extracranial metastatic medulloblastoma is(are)? Answer

A.  Lung and lymph node.

B.  Liver.

C.  Skin and soft tissue.

D.  Bone.

E.  Pleura.

16. Which of the followings is not ture about choroid plexus carcinoma? Answer

A.  They occur almost exclusively in adults.

B.  Immunohistochemistry could demonstrate transthyretin.

C.  Brain invasion is common.

D.  The tumor cells are positive for vimentin, cytokeratin, and S100 protein.

E.  Junctional membrane complex can be demonstrated by electron microscopy.

17. Tumors that contain the pattern of cell arrangement as illustrated here: Answer

1.    Can be seen in medulloblastoma and neuroblastomas.

2.    Can be seen in retinoblastoma.

3.    Can express neuronal markers such as neurofilament protein.

4.    Can express proteins specific for photoreceptors such as S-antigen and rod-opsin.

A.  1, 2, and 3 are true.

B.  1 and 3 are true.

C.  2 and 4 are true.

D.  Only 4 is true.

E.  All of the above.

18. Which of the followings is not true for pilocytic astrocytoma? Answer

A.  They are indolent, slow growing tumors and are of histologic grade I in the year 2000 World Health Organization (WHO) classification.

B.  They do not enhance on MRI images.

C.  Rosenthal fibers and eosinophilic granular bodies are often but not always present.

D.  Many of them are cystic tumor with a mural nodule.

E.  The most common location is the cerebellum.

19. Which of the following is true about pilomyxoid astrocytoma? Answer

A.  They behave in an indolent fashion comparable to that of pilocytic astrocytoma..

B.  They are most commonly found in the hypothalamus-chiasma area.

C.  They do not have angiocentric or perivascular arrangement of tumor cells.

D.  They are histologic grade I tumor as per the year 2000 Classification of the World Health Organization (WHO).

E.  They are seen almost exclusively in infants and children.

20. This primary tumor is obtained from a 12 year-old boy. The most likely syndrome that is associated with this boy is: Answer

A.  Von Hippel-Lindau syndrome.

B.  Neurofibromatosis 1 (NF1).

C.  Turcot syndrome.

D.  Gorlin syndrome.

E.  Neurofibromatosis 2 (NF2).

21. Which of the following is not true about dysembryoplastic neuroepithelial tumor? Answer

A.  They are typically associated with intractable seizure.

B.  They are composed exclusively of glial cells differentiating along different lineages.

C.  Many of them are associated with cortical dysplasia in the adjacent tissue.

D.  They are biologically benign.

E.  They are typically found in the medial aspect of the temporal lobe.

22. Subependymal giant cell astrocytoma can be immunoreactive for? Answer

A.  Glial fibrillary acidic protein (GFAP).

B.  Neurofilament.

C.  Vimentin.

D.  All of the above.

E.  None of the above.

23. Which of the followings is not true for endolymphatic sac tumor? Answer

A.  These tumors are more common in children then adult.

B.  These tumors are associated with von Hippel-Lindau disease.

C.  These tumors are morphologically very similar to, if not indistinguishable from, choroid plexus papilloma.

D.  These tumors are of osseous origin and often invade into the cerebellopontine angle with possible extension into the posterior fossa.

E.  These tumors are positive for carcinoembryonic antigen, epithelial membrane origin, and cytokeratin.

24. Which of the followings is not true about meningioangiomatosis? Answer

A.  They are associated with neurofibromatosis 1 (NF1).

B.  It is a rare, benign, focal, hamartomatous type of lesion of leptomeninges and the underlying cerebral cortex, characterized by leptomeningeal and meningiovascular proliferation.

C.  A majority of them are found in the frontal-temporal region.

D.  A schwannian component can be present.

E.  Mitotic figures are rare and the Ki67 (MIB-1) labeling index is low.

25. The most likely diagnosis of this cerebellar tumor is: Answer

A.  Cerebellar liponeurocytoma.

B.  Classic medulloblastoma.

C.  Medulloblastoma with advanced neuronal differentiation.

D.  Desmoplastic medulloblastoma.

E.  Large cell medulloblastoma.

26. The most common location for primary extraosseous, intra-parenchymal (intra-axial) location for Langerhans' cell histiocytosis is: Answer

A.  Pineal gland.

B.  Hypothalamus.

C.  Basal ganglia.

D.  Cerebellum.

E.  Brainstem.