Melanocytic Tumors and CNS Germ Cell Tumors Flashcards
A patient presents to neurosurgical OPD with MRI showing hyperintensity of leptomeninges with subarachnoid spaces on T1WI and same lesions hypointense on T2WI, with multifocal nodularity and invasion of Virchow-Robin (perivascular) spaces. On histology, there are diffuse, multifocal, primary me-
ningeal melanocytic lesion with invasion of CNS parenchyma, marked cytological atypia, absence of mitotic activity, and necrosis. What is the location of diffuse meningeal melanocytic neoplasms in the brain?
● A. Temporal lobes
● B. Cerebellum
● C. Pons
● D. Medulla
● E. All of the above
E. All of the above
A well-developed, solid, noninfiltrating tumor arises from the leptomeninges, characterized by epithelioid, fusiform, polyhedral, or spindled melanocytes with no evidence of anaplasia, necrosis, or increased mitoses. These lesions are most commonly found in cervical and thoracic spine. What is the most likely diagnosis in this scenario?
● A. Meningeal melanoma
● B. Meningeal melanocytoma
● C. Meningeal melanomatosis
● D. Meningeal melanocytosis
● E. None of the above
B. Meningeal melanocytoma
All of the following are included in the essential diagnostic criteria of meningeal melanoma except?
● A. Circumscribed/localized primary melanocytic neoplasm in the meninges
● B. Mitotic count more than 1.5 mitosis/mm2
● C. Demonstration of GNAQ, GNA11, PLCB4, or CYSLTR2 mutation
● D. Necrosis
● E. Marked cytological atypia
C. Demonstration of GNAQ, GNA11, PLCB4, or CYSLTR2 mutation
Meningeal melanomas are pleomorphic, anaplastic, mitotically active, and often invade CNS tissue and may demonstrate necrosis. These tumors may spread through CSF pathways, and occasionally systemic metastases outside the CNS do occur.
Which statement regarding management of these tumors is correct?
● A. These are not aggressive tumors
● B. With GTR, prognosis is better than with cutaneous melanoma metastatic to the CNS
● C. These tumors respond well to radiotherapy
● D. Peak age of these tumors is in 4th decade
● E. All of the above
B. With GTR, prognosis is better than with cutaneous melanoma metastatic to the CNS
Germ cell tumors primarily affect children and adolescents with peak age of 10 to 14 years. Which of the following statements is incorrect regarding these tumors?
● A. 80 to 90% of these tumors occur in the midline usually in the suprasellar and pineal region
● B. These tumors are not sensitive to radiotherapy
● C. Simultaneous suprasellar and pineal region lesions is diagnostic of so-called synchronous germ cell tumors which constitutes 13% of germ cell tumors
● D. In the pineal region, these tumors occur predominantly in males while in females germ cell tumors are more common in the suprasellar region
● E. All intracranial germ cell tumors are malignant and may metastasize via CSF and systemically
B. These tumors are not sensitive to radiotherapy
Germ cell tumors can (but not always) give rise to tumor markers in the CSF. Which of the following statements is correct?
● A. Choriocarcinoma and 50% of germinoma show elevated levels of beta human chorionic gonadotropin levels in the CSF
● B. Yolk sac tumors, embryonal carcinoma, and occasionally teratoma show elevated levels of alpha-fetoprotein
● C. Intracranial germinomas show elevated serum or CSF levels of placental alkaline phosphate
● D. Many of these tumors are mixed cell type and many pineal region tumors do not give rise to markers
● E. All of the above
E. All of the above
A patient presents with tumor that has germ cell tumor with components exhibiting differentiation along at least two of the three somatic tissue lines (ectoderm, endoderm, mesoderm) and fully differentiated adult type histology and absence of
germ cell tumor components. What is the most likely diagnosis in this patient?
● A. Mature teratoma
● B. Immature teratoma
● C. Teratoma with somatic type malignancy
● D. Germinoma
● E. Embryonal carcinoma
A. Mature teratoma
Which statement is incorrect regarding germinoma?
● A. These contain large undifferentiated-appearing cells resembling primordial germ cells with ample glycogen-rich clear cytoplasm containing round, vesicular, and centrally placed nuclei
● B. Necrosis is common in these tumors
● C. Mitotic activity is variable
● D. The cells are arranged in sheets, lobules, or cords with trabeculae
● E. Some may have syncytiotrophoblastic giant cells
B. Necrosis is common in these tumors
Which of the following is not included in the essential diagnostic criteria of embryonal carcinoma?
● A. A germ cell tumor with large epithelioid cells
● B. Cytokeratin expression
● C. CD30 or OCT4 expression
● D. Absent or only focal nonmembranous KIT expression
● E. Absence of hCG expression, absence of AFP expression, and absence of other germ cell tumor components
B. Cytokeratin expression
Which of the following is a feature if choriocarcinoma?
● A. Only syncytiotrophoblasts are seen
● B. Only cytotrophoblastic elements are visible
● C. Both syncytiotrophoblasts and cytotrophoblasts are seen
● D. It shows APD expression
● E. There is CD30 expression
C. Both syncytiotrophoblasts and cytotrophoblasts are seen
How are primary CNS melanocytic tumors distinguished from other pigmented CNS neoplasms like melanotic nerve sheath tumors?
● A. Location
● B. Negri bodies
● C. Tissue histology
● D. Molecular analysis
● E. Alkylation profiling
D. Molecular analysis
What is the most common location of germ cell tumors in females?
● A. Suprasellar
● B. Pineal region
● C. CP angle
● D. Cisterna magna
● E. Interpeduncular cistern
A. Suprasellar
Regarding germ cell tumors, which CSF tumor marker is expressed in almost 100% of choriocarcinomas?
● A. Beta HCG
● B. ALP
● C. PLAP
● D. AFP
● E. INF
A. Beta HCG
What is the diagnostic criterion for mature teratoma?
● A. A germ cell tumor with components exhibiting differentiation along all three somatic tissue lines
● B. Fully differentiated
● C. Dedifferentiated
● D. Fetal histology
● E. Presence of embryonal germ cell components
B. Fully differentiated
Absence of all of the following is essential for diagnosis of choriocarcinoma except?
● A. KIT
● B. AFP
● C. CD30
● D. OCT4
● E. β-HCG
E. β-HCG
