Pheochromocytoma and paraganglioma — MCQs

A 27-year-old man comes to the physician because of worsening abdominal pain over the last several months. He has also had recent feelings of sadness and a lack of motivation at work, where he is employed as a computer programmer. He denies suicidal thoughts. He has a history of multiple kidney stones. He has a family history of thyroid cancer in his father and uncle, who both underwent thyroidectomy before age 30. His temperature is 37°C (98°F), blood pressure is 138/86 mm Hg, and pulse is 87/min. Physical examination shows diffuse tenderness over the abdomen and obesity but is otherwise unremarkable. Serum studies show: Na+ 141 mEq/L K+ 3.6 mEq/L Glucose 144 mg/dL Ca2+ 12.1 mg/dL Albumin 4.1 g/dL PTH 226 pg/mL (normal range 12–88 pg/mL) Results of a RET gene test return abnormal. The physician refers him to an endocrine surgeon. Which of the following is the most appropriate next step in diagnosis?

A previously healthy 61-year-old man comes to the physician because of a 6-month history of morning headaches. He also has fatigue and trouble concentrating on his daily tasks at work. He sleeps for 8 hours every night; his wife reports that he sometimes stops breathing for a few seconds while sleeping. His pulse is 71/min and blood pressure is 158/96 mm Hg. He is 178 cm (5 ft 10 in) tall and weighs 100 kg (220 lb); BMI is 31.6 kg/m2 . Which of the following is the most likely cause of this patient's hypertension?

A patient presents with periods of severe headaches and flushing however every time they have come to the physician they have not experienced any symptoms. The only abnormal finding is a blood pressure of 175 mmHg/100 mmHg. It is determined that the optimal treatment for this patient is surgical. Prior to surgery which of the following noncompetitive inhibitors should be administered?

A 30-year-old man comes to the physician for follow-up evaluation for hypertension. He reports a 1-month history of episodic throbbing headaches, palpitations, and paroxysmal sweating. Blood pressure is 160/90 mm Hg. He appears pale but physical examination is otherwise unremarkable. Laboratory studies show elevated urine and plasma metanephrines. A CT scan of the abdomen shows a mass in the left adrenal gland. Which of the following is the most appropriate initial pharmacotherapy for this patient?

A 27-year-old man comes to the physician because of a 4-month history of recurrent episodes of headaches, palpitations, and sweating. He was adopted shortly after birth and does not know his biological parents. His pulse is 103/min and blood pressure is 160/105 mm Hg. Physical examination shows multiple soft, yellow papules on the tip of the tongue. There is a 2-cm, firm thyroid nodule. He has long and slender upper extremities, and his elbows and knees can be hyperextended. The most likely cause of this patient's condition is a mutation in which of the following genes?

A 21-year-old male college student is very anxious about having thyroid cancer as he found a lump in his neck a few days ago. He has also had diarrhea and a feeling of warmth on his face for 3 days now. His father and uncle both have been diagnosed with thyroid cancer. The lump is about 1 cm in diameter and is fixed and nontender on palpation. Physical examination is otherwise unremarkable. Ultrasound shows a non-cystic cold nodule that requires fine needle aspiration for diagnosis. Thyroid functions tests are normal, and his calcitonin level is 346 ug/ml. Which of the following genetic mutations would warrant thyroidectomy in this patient?

A 51-year-old African American man with a history of poorly controlled hypertension presents to the emergency room with blurry vision and dyspnea. He reports rapid-onset blurred vision and difficulty breathing 4 hours prior to presentation. He takes lisinopril, hydrochlorothiazide, and spironolactone but has a history of poor medication compliance. He has a 50 pack-year smoking history and drinks 4-6 shots of vodka per day. His temperature is 99.2°F (37.3°C), blood pressure is 195/115 mmHg, pulse is 85/min, and respirations are 20/min. On exam, he is ill-appearing and pale. He is intermittently responsive and oriented to person but not place or time. Fundoscopic examination reveals swelling of the optic disc with blurred margins. A biopsy of this patient’s kidney would most likely reveal which of the following?

A 38-year-old woman with type 1 diabetes for 20 years presents with diabetic ketoacidosis. She is treated and recovers. Six months later, she develops progressive fatigue, nausea, and hyperpigmentation. Laboratory studies show morning cortisol 3 μg/dL, ACTH 180 pg/mL, TSH 8.2 mIU/L, free T4 0.6 ng/dL, and positive anti-thyroid peroxidase antibodies. She also has positive 21-hydroxylase antibodies. Her 12-year-old daughter was recently diagnosed with type 1 diabetes. Evaluate the pathologic process and most critical monitoring recommendation for the daughter.

A 29-year-old man presents with severe headaches and episodic palpitations, sweating, and anxiety. During an episode, blood pressure is 240/130 mmHg. Between episodes, blood pressure is 135/85 mmHg. 24-hour urine shows metanephrines 4.2 mg (normal: <1.0). MRI reveals a 4 cm right adrenal mass. His brother died suddenly at age 25 from an intracranial hemorrhage, and his father had thyroid cancer. Genetic testing reveals a RET proto-oncogene mutation. Evaluate the pathologic syndrome and preoperative management priority.

A 62-year-old man presents with confusion and severe muscle weakness. He recently started treatment for small cell lung cancer. Laboratory studies show sodium 118 mEq/L, serum osmolality 245 mOsm/kg, urine osmolality 520 mOsm/kg, and urine sodium 85 mEq/L. He is clinically euvolemic with blood pressure 125/80 mmHg. Serum cortisol and TSH are normal. Evaluate the pathophysiologic mechanism and most appropriate initial management given the clinical context.