Review Question - QID 210574

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Islet Cell Tumors Somatostatinoma Treatment QID: 210574 (M1.OMB.18.4736)

QID 210574 (Type "210574" in App Search)

A 51-year-old woman presents to her primary care doctor with diarrhea. She has had 3-10 malodorous and loose bowel movements daily for the last 6 months, though she recalls that her bowel movements started increasing in frequency nearly 2 years ago. She was otherwise healthy until 2 years ago, when she had multiple elevated fasting blood glucose levels and was diagnosed with type 2 diabetes mellitus. She was also hospitalized once 6 months ago for epigastric pain that was determined to be due to cholelithiasis. She is an avid runner and runs 3-4 marathons per year. She is a vegetarian and takes all appropriate supplements. Her body mass index is 19 kg/m^2. She has lost 10 pounds since her last visit 18 months ago. On exam, she has dry mucous membranes and decreased skin turgor. A high-resolution spiral computerized tomography scan demonstrates a 5-cm enhancing lesion in the head of the pancreas. Additional similar lesions are found in the liver. Further laboratory workup confirms the diagnosis. The patient is offered surgery but refuses as she reportedly had a severe complication from anesthesia as a child. This patient should be treated with a combination of octreotide, 5-fluorouracil, and which other medication?

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Streptozotocin

9/105

Insulin

42%

44/105

Paclitaxel

11%

12/105

Glucagon

11%

12/105

Methotrexate

18%

19/105

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The patient in this vignette presents with steatorrhea, weight loss, cholelithiasis, and diabetes mellitus with multiple lesions in the head of the pancreas and liver. This presentation is suggestive of metastatic somatostatinoma, which can be treated with the chemotherapeutic agent streptozotocin.

Somatostatinomas are neoplasms of delta cells from the pancreatic islets. These cells produce somatostatin, an inhibitor of multiple hormones like gastrin, cholecystokinin (CCK), secretin, and gastric inhibitory peptide (GIP). Clinically, somatostatinomas may present with slow-onset achlorhydria (due to blockage of gastrin release), cholelithiasis (due to blockage of CCK release), steatorrhea (due to blockage of secretin release), and diabetes mellitus (due to blockage of GIP release). Surgery is the first-line treatment for isolated somatostatinomas and for metastatic tumors to the liver. For patients in whom surgery is contraindicated, a combination of octreotide, 5-fluorouracil, and streptozotocin is considered the optimal treatment. Streptozotocin is an alkylating chemotherapeutic agent that targets cells arising from pancreatic islets.

Incorrect Answers:
Answer 2: Insulin is an endogenous hormone secreted by beta cells in the islets of Langerhans. Insulin has multiple important functions including inhibiting glucagon release, stimulating synthesis of glycogen, triglycerides, and proteins.

Answer 3: Paclitaxel is a chemotherapeutic agent that blocks microtubule breakdown by hyperstabilizing microtubules in M phase. It is used to treat solid tumors such as ovarian and breast cancer.

Answer 4: Glucagon is an endogenous hormone secreted by alpha cells in the islets of Langerhans. Glucagon has multiple important functions including stimulating glycogenolysis and gluconeogenesis and inhibiting insulin release.

Answer 5: Methotrexate is a folate analog that is used as both a chemotherapeutic agent and anti-rheumatic medication. Although methotrexate is used to treat several hematologic and solid neoplasms, it is not used to treat somatostatinomas.

Bullet Summary:
Metastatic somatostatinomas can be treated with octreotide, 5-fluorouracil, and streptozotocin, a chemotherapeutic agent used to treat certain neoplasms arising from pancreatic islets.

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