[MCQ] Pheochromocytoma- Part 2

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Pheochromocytoma- Part 2

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Question 1

Which out of the following has maximum specificity in the diagnosis of pheochromocytoma ?

A	Plasma-free metanephrines
B	Plasma catecholamines
C	Urinary-fractionated metanephrines
D	VMA

Question 2

Radioactive tracer used to localize phaeochromocytomas is ?

A	131I- or 123I-metaiodobenzylguanidine (MIBG)
B	111In-somatostatin analogues
C	18F-dopa (or dopamine
D	All of the above

Question 2 Explanation:

phaeochromocytoma tumor can be localized by radioactive tracers like 131I- or 123Imetaiodobenzylguanidine (MIBG), 111In-somatostatin analogues, or 18F-dopa (or dopamine) positronemission tomography (PET). 131I-MIBG is also used in treatment of malignant phaeochromocytoma using 200-mCi doses at monthly intervals, over three to six cycles.

Question 3

Which of the following provides better diagnostic sensitivity in the diagnosis of phaeochromocytomas ?

A	CT
B	MRI
C	18F-fluorodopamine PET
D	131I-MIBG

Question 4

Which of the following is an -adrenoceptor blocker ?

A	Prazosin
B	Doxazosin
C	Urapidil
D	All of the above

Question 4 Explanation:

-adrenoceptor blockers are phenoxybenzamine, prazosin, doxazosin & urapidil.

Question 5

In pheochromocytoma, phenoxybenzamine should be administered for at least how many days prior to surgery ?

A	2 to 5 days
B	5 to 10 days
C	10 to 14 days
D	14 to 21 days

Question 5 Explanation:

Adequate alpha adrenergic blockade with phenoxybenzamine generally requires 10 - 14 days, with a typical final dose of 20 - 30 mg three times per day.

Question 6

Malignant phaeochromocytoma can metastasize to ?

A	Bones
B	Lungs
C	Liver
D	All of the above

Question 6 Explanation:

Term malignant pheochromocytoma is restricted to tumors with distant metastases, most commonly to lungs, bone or liver, suggesting a vascular pathway of spread.

Question 7

Which of the following features of phaeochromocytoma have a higher risk for malignant disease ?

A	Large size (5 cm)
B	Paragangliomas with SDHB mutations
C	Increased plasma/urinary dopamine and dopa
D	All of the above

Question 8

Averbuch’s chemotherapy protocol for treatment of malignant pheochromocytoma consists of all except ?

A	Dacarbazine
B	Methotrexate
C	Cyclophosphamide
D	Vincristine

Question 8 Explanation:

Averbuch’s chemotherapy protocol includes dacarbazine, cyclophosphamide, and vincristine, repeated every 21 days for three to six cycles.

Question 9

Classic features of neurofibromatosis include ?

A	Café au lait spots
B	Axillary freckling of skin
C	Lisch nodules of iris
D	All of the above

Question 9 Explanation:

Classic features of Neurofibromatosis type 1 (NF 1) or Von Recklinghausen’s Disease include multiple benign Schwann cell neurofibromas, pigmented café au lait macules (CALM), fr

Question 10

Lisch nodules of iris best relates to which of the following ?

A	Pigmentation
B	Hamartoma
C	Prolapse
D	Dysplasia

Question 10 Explanation:

Lisch nodules refers to hamartomas of the iris.

Question 11

NF1 gene that causes von Recklinghausen’s disease is on which chromosome ?

A	6
B	12
C	17
D	18

Question 11 Explanation:

NF1 gene on chromosome 17 causes von Recklinghausen’s disease. NF1 gene is a tumor-suppressor gene & encodes a neurofibromin which modulates signal transduction through the ras GTPase pathway.

Question 12

Sipple’s syndrome is also called ?

A	MEN type 1A
B	MEN type 1B
C	MEN type 2A
D	MEN type 2B

Question 13

Multiple endocrine neoplasia type 2A is characterized by ?

A	Medullary thyroid carcinoma (MTC)
B	Pheochromocytoma
C	Hyperparathyroidism
D	All of the above

Question 13 Explanation:

MEN 2A is characterized by MTC, pheochromocytoma & hyperparathyroidism

Question 14

Multiple endocrine neoplasia type 2B is characterized by ?

A	Medullary thyroid carcinoma (MTC)
B	Pheochromocytoma
C	Multiple mucosal neuromas
D	All of the above

Question 14 Explanation:

MEN 2B includes MTC, pheochromocytoma & multiple mucosal neuromas. It typically lacks hyperparathyroidism. Both types of MEN 2 are caused by mutations in RET (rearranged in transfection) that encodes a tyrosine kinase.

Question 15

Which of the following is not a feature of pheochromocytomas in MEN 2 ?

A	Benign
B	Located in adrenals
C	MTC may be symptomatic before pheochromocytoma
D	Bilateral

Question 15 Explanation:

Pheochromocytomas in MEN 2 are benign, located in the adrenals, and bilateral. Pheochromocytoma may be symptomatic before MTC.

Question 16

Which of the following is not a feature of Von Hippel-Lindau syndrome ?

A	Renal clear-cell carcinoma
B	Retinal hemiangioblastoma
C	Testicular tumour
D	Pancreatic islet cell tumour

Question 16 Explanation:

VHL is an autosomal dominant disorder that predisposes to retinal & cerebellar hemangioblastomas, which also occur in the brain stem & spinal cord. Other features of VHL are clear cell renal carcinomas, pancreatic islet cell tumors, endolymphatic sac tumors (ELSTs) of the inner ear, cystadenomas of the epididymis & broad ligament, and multiple pancreatic or renal cysts.

Question 17

VHL gene encodes an E3 ubiquitin ligase that regulates expression of ?

A	Insulin-like growth factor (IGF) I
B	Basic fibroblast growth factor (bFGF)
C	Tissue-specific transcription factor
D	Hypoxia-inducible factor-1 (HIF-1)

Question 17 Explanation:

VHL gene encodes an E3 ubiquitin ligase that regulates expression of hypoxia-inducible factor- 1 (HIF-1). Loss of VHL is associated with increased expression of vascular endothelial growth factor (VEGF), which induces angiogenesis

Question 18

Type of mutation in VHL gene in pheochromocytoma is ?

A	Point
B	Missense
C	Transition
D	Frameshift

Question 18 Explanation:

Patients with pheochromocytoma predominantly have missense mutations in VHL gene. Mutations involving single nucleotides are referred to as point mutations. Substitutions are called transitions if a purine or pyrimidine is replaced by another purine or pyrimidine base. Changes from a purine to a pyrimidine, or vice versa, are called transversions. If the DNA sequence change occurs in a coding region and alters an amino acid, it is called a missense mutation. Polymorphisms are sequence variations that have a frequency of at least 1%. Small nucleotide deletions or insertions cause a shift of the codon reading frame (frameshift). Most commonly, reading frame alterations result in an abnormal protein segment of variable length before termination of translation occurs at a stop codon (nonsense mutation).

Question 19

Mutations of which of the following gene causes paraganglioma syndrome 1 (PGL1) ?

A	SDHA
B	SDHB
C	SDHC
D	SDHD

Question 19 Explanation:

Succinate dehydrogenase (SDH) is formed by four subunits (A - D). Mutations of SDHB (PGL4), SDHC (PGL3), and SDHD (PGL1) predispose to three of the paraganglioma syndromes.

Question 20

Mutations of which of the following do not predispose to paraganglioma tumors ?

A	SDHA
B	SDHB
C	SDHC
D	SDHD

Question 20 Explanation:

Mutations of SDHA do not predispose to paraganglioma tumors but cause Leigh disease, a form of encephalopathy.

Question 21

Which is most frequent paraganglioma syndrome ?

A	PGL1
B	PGL2
C	PGL3
D	PGL4

Question 21 Explanation:

PGL1 is most frequent, followed by PGL4. PGL3 is rare. Adrenal, extraadrenal abdominal & thoracic pheochromocytomas are components of PGL1 & PGL4, but not of PGL3.

Question 22

Abdominal extraadrenal pheochromocytoma are located in association with ?

A	Celiac ganglia
B	Superior mesenteric ganglia
C	Inferior mesenteric ganglia
D	All of the above

Question 23

Hereditary phaeochromocytomas occur in ?

A	MEN type 2
B	Von Hippel-Lindau syndrome
C	Neurofibromatosis type 1
D	All of the above

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See all quizzes of Pheochromocytoma at here:

Pheochromocytoma- Part 1| Pheochromocytoma- Part 2| Pheochromocytoma- Part 3

1.Which out of the following has maximum specificity in the
diagnosis of pheochromocytoma ?
A. Plasma-free metanephrines
B. Plasma catecholamines
C. Urinary-fractionated metanephrines
D. VMA
2. Radioactive tracer used to localize phaeochromocytomas is ?
A. 131I- or 123I-metaiodobenzylguanidine (MIBG)
B. 111In-somatostatin analogues
C. 18F-dopa (or dopamine)
D. All of the above

3. Which of the following provides better diagnostic sensitivity in
the diagnosis of phaeochromocytomas ?
A. CT
B. MRI
C. 18F-fluorodopamine PET
D. 131I-MIBG
4. Which of the following is an -adrenoceptor blocker ?
A. Prazosin
B. Doxazosin
C. Urapidil
D. All of the above

5. In pheochromocytoma, phenoxybenzamine should be
administered for at least how many days prior to surgery ?
A. 2 to 5 days
B. 5 to 10 days
C. 10 to 14 days
D. 14 to 21 days

6. Malignant phaeochromocytoma can metastasize to ?
A. Bones
B. Lungs
C. Liver
D. All of the above

7. Which of the following features of phaeochromocytoma have a
higher risk for malignant disease ?
A. Large size (5 cm)
B. Paragangliomas with SDHB mutations
C. Increased plasma/urinary dopamine and dopa
D. All of the above
8. Averbuch’s chemotherapy protocol for treatment of malignant
pheochromocytoma consists of all except ?
A. Dacarbazine
B. Methotrexate
C. Cyclophosphamide
D. Vincristine

9.Classic features of neurofibromatosis include ?
A. Café au lait spots
B. Axillary freckling of skin
C. Lisch nodules of iris
D. All of the above

10.Lisch nodules of iris best relates to which of the following ?
A. Pigmentation
B. Hamartoma
C. Prolapse
D. Dysplasia

11. NF1 gene that causes von Recklinghausen’s disease is on which
chromosome ?
A. 6
B. 12
C. 17
D. 18

12. Sipple’s syndrome is also called ?
A. MEN type 1A
B. MEN type 1B
C. MEN type 2A
D. MEN type 2B
13. Multiple endocrine neoplasia type 2A is characterized by ?
A. Medullary thyroid carcinoma (MTC)
B. Pheochromocytoma
C. Hyperparathyroidism
D. All of the above

14. Multiple endocrine neoplasia type 2B is characterized by ?
A. Medullary thyroid carcinoma (MTC)
B. Pheochromocytoma
C. Multiple mucosal neuromas
D. All of the above
.
15. Which of the following is not a feature of pheochromocytomas in
MEN 2 ?
A. Benign
B. Located in adrenals
C. MTC may be symptomatic before pheochromocytoma
D. Bilateral

16. Which of the following is not a feature of Von Hippel-Lindau
syndrome ?
A. Renal clear-cell carcinoma
B. Retinal hemiangioblastoma
C. Testicular tumour
D. Pancreatic islet cell tumour

17. VHL gene encodes an E3 ubiquitin ligase that regulates expression
of ?
A. Insulin-like growth factor (IGF) I
B. Basic fibroblast growth factor (bFGF)
C. Tissue-specific transcription factor
D. Hypoxia-inducible factor-1 (HIF-1)

18.Type of mutation in VHL gene in pheochromocytoma is ?
A. Point
B. Missense
C. Transition
D. Frameshift

19. Mutations of which of the following gene causes paraganglioma
syndrome 1 (PGL1) ?
A. SDHA
B. SDHB
C. SDHC
D. SDHD

20.Mutations of which of the following do not predispose to
paraganglioma tumors ?
A. SDHA
B. SDHB
C. SDHC
D. SDHD

21. Which is most frequent paraganglioma syndrome ?
A. PGL1
B. PGL2
C. PGL3
D. PGL4

22. Abdominal extraadrenal pheochromocytoma are located in
association with ?
A. Celiac ganglia
B. Superior mesenteric ganglia
C. Inferior mesenteric ganglia
D. All of the above
23. Hereditary phaeochromocytomas occur in ?
A. MEN type 2
B. Von Hippel-Lindau syndrome
C. Neurofibromatosis type 1
D. All of the above

Pheochromocytoma

[MCQ] Pheochromocytoma- Part 2

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Pheochromocytoma- Part 2

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[MCQ] Pheochromocytoma- Part 1

[MCQ] Pheochromocytoma- Part 3

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