Endocrine Tumors

Endocrine tumors account for what percentage of all pediatric malignancies??
1. 1 percent
2. 5 percent
3. 10 percent
4. 20 percent
What tumors cause diencephalic syndrome (wasting, pallor, vomiting, nystagmus)?
1. Gliomas in the hypothalamus
2. Craniopharyngioma
3. Pinealoma
4. Medulloblastoma
What is the most common tumor involving the hypothalamus or pituitary area in children?
1. Gliomas in the hypothalamus
2. Craniopharyngioma
3. Pinealoma
4. Medulloblastoma
Which hormone is NOT decreased in patients with a craniopharyngioma?
1. TSH
2. ACTH
3. ADH
4. Growth hormone
5. All are decreased
Pituitary adenoma is rare in children, but when present what percent are functional?
1. 10 percent
2. 25 percent
3. 50 percent
4. 75 percent
What is the treatment of a pituitary adenoma associated gigantism?
1. TS hypophysectomy and radiation
2. Radiation therapy
3. Hypophysectomy only
What is the treatment of Prolactinoma?
1. TS hypophysectomy
2. Radiation therapy
3. Bromcirptine
4. All of the above
What percentage of childhood adrenal tumors are from the adrenal medulla?
1. 90 percent
2. 75 percent
3. 50 percent
4. 25 percent
What is the mean age of adrenocortical tumors in children, their male to female ratio, their malignant versus benign ratio, and what percent are bilateral?
1. Less than 2 years
2. 4 to 6 years
3. More than 10 years
Which is the most common hormonal symptom adrenocortical tumors produce?
1. Feminization
2. Virilization
3. Cushing's syndrome
What is the most common presenting symptom of adrenal tumors?
1. Homronal symptoms
2. Mass
3. Hypertension
4. Weight loss and anorexia
Which factor is NOT associated with increased risk of adrenocortical tumors?
1. Brain tumors
2. Fetal alcohol syndrome
3. Hemihypertrophy
4. Hirschsprungs disease
Which is NOT a symptom of Conn's syndrome in children?
1. HTN
2. Polyuria
3. Hypokalemia
4. Hypernatremia
Which is a nonspecific measure of increased androgenic metabolism (leading to virilization in children)?
1. Androstanedione
2. Testosterone
3. 17 ketosteroids
T or F: 17 ketosteroids are increased in adrenocortical carcinoma, but not in benign adrenal tumors?
1. True
2. False
Two thirds of 17 ketosteroids are produced by gonadal sources; and another one-third is produced by the adrenal glands?
1. True
2. False
The most common cause of virilization at birth is what?
1. CAH - congenital adrenal hyperplasia
2. Neuroblastoma
3. Exogenous steroids
The most common cause of virilization after infancy is what?
1. Exogenous steroids
2. Adrenal hyperplasia
3. Tumor
Which is associated with increase 17 ketosteroids in the urine (in the differential diagnosis of virilization in girls)?
1. Sertoli Leydig cell tumors in the ovary
2. Adrenal tumors
3. Neither
4. Both
In the differential diagnosis of virilization in boys, which of the following causes bilateral testicular enlargement?
1. Leydig cell tumor of the testis
2. Adrenal tumor
3. Ectopic HCG producing tumor
4. Isosexual precocity
Which is the most common cause of Cushing's syndrome in children?
1. Pituitary tumor
2. Adrenal tumor
3. Exogenous steroids
4. Adrenal hyperplasia
T or F: One of the reasons children with Cushing's syndrome are short is the suppression of GH release by elevated cortisol?
1. True
2. False
Which is NOT a test for hypercortisolism?
1. Plasma cortisol
2. Urine 17 hydroxycorticosteroids
3. Urinary free cortisol
4. DHEA
True or false: dexamethasone suppression tests are used to differentiate benign from malignant tumors; and are quite accurate?
1. True
2. False
True or false: in patients with a nonfunctional adrenocortical carcinoma, the 17 ketosteroids and 17 hydroxysteroid levels may be entirely normal?
1. True
2. False
Adrenal scans with with radiolabeled cholesterol compounds demonstrate which?
1. Malignant adrenal tumors
2. Benign adrenal tumors
3. Both
4. Neither
What does bilaterally symmetric increased uptake on adrenal scan mean in conjunction with the presence of elevated 17 hydroxysteroids?
1. Adrenal hyperplasia
2. Adenoma
3. Ectopic hormone production
4. Adrenal carcinoma
What does bilateral nonvisualization mean on adrenal scan?
1. Adrenal hyperplasia
2. Adenoma
3. Ectopic hormone production
4. Adrenal carcinoma
T or F: cholesterol adrenal scans take 4 to 7 days to perform?
1. True
2. False
In stage 1 versus stage 2 adrenal tumors in children, the distinguishing factor is BLANK?
1. Size above 5 cm
2. Nodal metastasis
3. Complete resectability
True or false: adrenocortical tumors are usually considered radiosensitive?
1. True
2. False
T or F: Mitotane chemotherapy for adrenocortical carcinoma results in regression in 30 - 60%?
1. True
2. False
What fraction of patients experience toxicity with mitotane?
1. 20 percent
2. 40 percent
3. 60 percent
4. 90 percent
Who has better survival: those with nonfunctional adrenal cancer or those with functional tumor?
1. Functional
2. Non-functional
3. Equivalent
What is the cause of hypertension in 0.1 percent of all hypertensive patients?
1. CHOICE
2. CHOICE
3. CHOICE
4. CHOICE
What is the autopsy incidence of pheochromocytoma?
1. 0.2 to 0.9 percent
2. 2 - 3 percent
3. 5 percent
What percentage of pheochromocytomas are benign versus malignant?
1. 90 percent benign
2. 60 percent benign
3. 40 percent benign
4. 10 percent benign
What percentage of pheochromocytomas are familial?
1. 90 percent benign
2. 60 percent benign
3. 40 percent benign
4. 10 percent benign
Extra-adrenal tumors and multiple pheochromocytomas are (more or less) frequently seen in children?
1. More
2. Less
3. Equal
True or false: familial pheochromocytoma may be sporadic or associated with MEN II A or MEN IIB?
1. True
2. False
What percent of childhood pheochromocytomas are malignant?
1. 90 percent
2. 50 percent
3. 25 percent
4. 10 percent
Pheochromocytoma is an exceptionally rare cause of hypertension overall; however, what percent of hypertension in children is accounted for by pheochromocytoma?
1. 50 percent
2. 25 percent
3. 10 percent
4. 1 percent
Which is NOT part of MEN IIA?
1. Pheochromocytoma
2. Parathyroid adenoma
3. Medullary thyroid carcinoma
4. Pancreatic islet tumor
Which is NOT part of MEN IIB?
1. Pancreatic islet tumor
2. Ganglioneuroma of the visceral plexus
3. Medullary carcinoma of thyroid
4. Mucocutaneous neuroma
What percent of patients with neurofibromatosis have pheochromocytoma?
1. Less than 5 percent
2. 10 - 15 percent
3. 25 - 30 percent
4. 50 percent
What percent of pheochromocytomas rise from the neck and mediastinum?
1. Less than 5 percent
2. 15 percent
3. 25 percent
What percent of patients with pheochromocytoma have hypertension?
1. 90 percent
2. 50 percent
3. 25 percent
4. 10 percent
What disease can cause syncope with micturition?
1. Bladder pheochromocytoma
2. Organ of Zuckerkandl pheochromocytoma
3. Thalamic tumors
4. Extra-adrenal pheochromocytoma
True or false: the hallmark of hypertension associated with pheochromocytoma is that is very commonly orthostatic?
1. True
2. False
Which is produced more often by adrenal pheochromocytomas - epinephrine or norepinephrine?
1. Epinephrine
2. Norepinephrine
3. Equivalent
Pheochromocytomas that produce exclusively WHAT are usually malignant?
1. Norepinephrine
2. Dopamine
3. Substance P
4. EGF
Which hormone produced by pheochromocytomas is associated with an increased chance of malignancy?
1. VIP
2. Substance P
3. Somatostatin
What studies are NOT done to establish the diagnosis of pheochromocytoma?
1. VMA
2. Catecholamine
3. Dopamine
4. Metanephrine
5. All of the above
In the clonidine test for pheochromocytoma, it suppresses plasma catecholamines in normal patients and / or those with pheochromocytoma?
1. Pheochromocytoma
2. Normals
3. Both
4. Neither
What percent of pediatric pheochromocytomas or extra adrenal?
1. 5 - 20 percent
2. 20 - 30 percent
3. 50 - 60 percent
4. 75 percent
What is the best test for localization of extra-adrenal pheochromocytoma - CAT scan, I-131 MIBG, or MRI?
1. MRI
2. CT
3. MIBG
If arteriography is needed for pheochromocytoma, what must be used before the study?
1. Beta Blockade
2. Alpha Blockade
3. Steroids
4. Nipride drip
MIBG is concentrated in what vessels?
1. Cholinergic
2. Adrenergic
3. Dopaminergic
What drug provides a better alpha blockade for pheochromocytoma - phenoxybenzamine or phentolamine?
1. Phenoxybenzamine
2. Phentolamine
3. Equivalent
What should be ligated first during resection of a pheochromocytoma?
1. Adrenal artery(s)
2. Adrenal vein(s)
3. Either
What is the surgical treatment of pheochromocytoma associated with MEN II?
1. Bilateral adrenalectomy
2. Unilateral adrenalectomy
3. Radical nephrectomy and adrenalectomy
4. Medical adrenalectomy with glutehamide
What clinical findings suggest the presence of malignancy in pheochromocytoma?
1. Metastases
2. Direct invasion of surrounding structures
3. Positive lymph node
4. All of the above
True or false: pheochromocytoma grows rapidly?
1. True
2. False
What is the five-year survival for malignant pheochromocytoma?
1. 10 to 15 percent
2. 20 to 30 percent
3. 40 to 50 percent
4. 65 to 75 percent
What islet cells make what somatostatin?
1. Alpha cells
2. Beta cells
3. D cells
4. G cells
What is the most common islet cell tumor?
1. Glucagonoma
2. VIPoma
3. Zollinger-Ellison syndrome
4. Insulinoma
A drug is used to treat malignant insulinoma?
1. Streptomycin
2. Mitotane
3. Glutethamide
4. Streptozocin
What age does insulinoma usually occur?
1. Less than 1 year of age
2. Under 3 years
3. Greater than 4 years
4. Greater than 13 years
Most older children with insulinoma present with Whipple's triad - which is NOT a component?
1. Symptoms releived by glucose
2. With fasting, symptoms of hypogylcemia
3. Hypoglycemia with fasting
4. IRI to glucose level of more than 0.3
What is thetypical plasma insulin to glucose ratio in insulinoma?
1. 0.1
2. 0.3
3. 0.4
4. More than 1
Why should C-peptide levels be checked in a patient with insulinoma?
1. Elevation implies exogenous hyperinsulinism
2. Elevation implies endogenous hyperinsulinism
3. Marker for severity of disease
4. May indicate malignancy
What does elevated proinsulin mean in the patient with insulinoma?
1. Elevation implies exogenous hyperinsulinism
2. Elevation implies endogenous hyperinsulinism
3. Marker for severity of disease
4. May indicate malignancy
A CT scan is diagnostic of insulinoma in what percent?
1. 25 percent
2. 50 percent
3. 75 percent
Most insulinomas can be enucleated; what do you do if all techniques to localize the tumor are negative?
1. Distal 80% pancreatectomy
2. 95% pancreatectomy
3. Medical management with somatostatin
4. Medical management with diazoxide
In contrast to adults, most VIPomas in children are associated with what primary tumor?
1. Pancreatic primary
2. Intestinal primary
3. Hepatic primary
4. Neuroblastoma or ganglioneuroma
Whic of the following abnormalities are NOT typically associated with VIPomas?
1. Metabolic alkalosis
2. Dehydration
3. Hypokalemia
4. Hypochloremia
In what percentage of MEN I are gastrinomas present?
1. 10 percent
2. 25 percent
3. 50 percent
4. 75 percent
T or F: In children, gastrinomas have not been reported to have MEN I?
1. True
2. False
In what percentage of children with gastrinoma are the tumors multiple?
1. 10 percent
2. 30 percent
3. 60 percent
4. 90 percent
What is a surgical treatment of gastrinoma?
1. Total gastrectomy
2. Resect the primary
3. Medical management alone
4. Vagotomy and antrectomy
True or false: aldosterone secreting tumors generally produce much higher levels of aldosterone (> 100 micrograms per deciliter) than are produced by adrenal hyperplasia?
1. CHOICE
2. CHOICE
3. CHOICE
4. CHOICE
What cholesterol analogue scanning test is used for hyperaldosteronism?
1. MIBG
2. NP 59
3. Gladolinium
4. T2 weighted MRI with gallium
What is the treatment for adrenal hyperplasia causing hyperaldosteronism?
1. Unilateral adrenalectomy
2. Bilateral adrenalectomy
3. Spironolactone
4. Aminoglutethamide
Is Conn's syndrome due to hyperplasia or an adenoma?
1. Adenoma
2. Hyperpasia
What does the combination of hypertension and low serum potassium in a patient on no medications suggest?
1. Hyperaldoseronislm
2. Addison's disease
3. SIADH
4. ATN
What type of cells do carcinoid tumors arise from?
1. APUD cells
2. Kulchitsky cells
3. Kupfer cells
4. Hurthle cells
In children, what is the most common location for carcinoid tumor?
1. Ileum / Jejunum
2. Bronchial
3. Rectum
4. Appendix
What type of reaction can intestinal carcinoid tumors cause in the mesentery?
1. Desmoplastic
2. Fibrosarcomatous
3. Hyperplastic
4. Metaplastic
Is carcinoid associated with any MEN syndrome?
1. MEN 1
2. MEN 2
3. MEN 2B
4. None
What site typically has argentaffin positive carcinoids?
1. Foregut
2. Midgut
3. Hindgut
In midgut tumors, carcinoid syndrome is not manifest until what?
1. Lymphatic metastases
2. Hepatic metastases
3. Size of primary more than 5 cm
What heart lesions are associated with carcinoid tumors?
1. Septal hypertrophy
2. Atrial septal defects
3. Mitral valve prolapse
4. Right-sided endocardial fibrosis
What is the treatment for an appendiceal carcinoid tumor less than 2 cm?
1. Right hemicolectomy
2. Wedge resection of 5 cm margin of bowel on either side
3. Appendectomy
What is the five-year survival in adults for carcinoid with hepatic metastasis?
1. 10 percent
2. 20 percent
3. 40 percent
4. 60 percent
What is the most common primary lung tumor in children?
1. Cylindroma
2. Adenoma
3. Adenocarcinoma
4. Bronchial carcinoid
At bronchoscopy in a child with recurrent pneumonia, a lesion suspicious for a bronchial carcinoid is identified. What should be done?
1. Biospy bronchoscopically
2. Brush biopsy
3. Resection - segmental or even lobectomy
4. Angiography

Endocrine Tumors

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