Sep 08, 2022
ASCO seeks to advance the education of all oncology professionals and ultimately facilitate and support enhanced patient care. The ASCO Oncology Self-Assessment Series on ASCO Connection consists of free case-based multiple-choice practice questions, educational links, and answer rationales from ASCO-SEP.
Learn more about ASCO’s Educational products, such as the 2022 ASCO-SEP Digital Subscription, which includes the digital book, access to education courses and virtual meeting-related content, plus over 900 practice questions in the Question Bank. Oncology trainees and Training Program Directors can visit Education Essentials for Oncology Fellows (EEOF) to learn more and register for the new 2022-23 cycle.
Correct answers are listed at the bottom of the page.
Question 1: Gastrointestinal Cancer
A 45-year-old man presents to the emergency department with early satiety and unintentional weight loss over the past several months. An upper endoscopy reveals an infiltrative mass extending below the gastroesophageal junction to the antrum. A biopsy of the mass shows poorly differentiated adenocarcinoma with signet ring features. He reports that his brother was diagnosed and treated for gastric cancer in his 50s, and that his father died of gastric cancer in his 60s. The patient is worried that his 18-year-old daughter will also develop gastric cancer. Genetic testing reveals CDH1 mutation in both the patient and his daughter.
Which of the following is the best recommendation for this patient's daughter?
- Yearly upper endoscopy screening starting at age 30 years
- Prophylactic total gastrectomy in her 20s
- Prophylactic total gastrectomy only after childbearing is completed (if desired)
- No additional screening
Question 2: Leukemia
A 75-year-old man with a history of coronary artery disease, hypertension, and diabetes presented with a two-week history of progressive shortness of breath and fatigue. His physical examination was significant for pallor and petechia on the lower extremities. A complete blood count revealed a hemoglobin level of 7 g/dL, leukocyte count of 600/mm3, and platelet count of 20,000/mm3. A bone marrow biopsy showed 60% cellularity with 40% myeloblasts. A cytogenetic analysis testing of bone marrow showed no abnormalities. TP53 mutation was noted on molecular testing. An echocardiogram revealed an ejection fraction of 45%. The patient's Eastern Cooperative Oncology Group performance score was 1.
What is the most appropriate treatment for this patient?
- Gemtuzumab ozogamicin
- Liposomal daunorubicin-cytarabine (3+7)
- Azacitidine and venetoclax
- Single-agent azacytidine
Question 1 Rationale and References
Correct answer: B. Prophylactic total gastrectomy in her 20s
Rationale: In families with hereditary diffuse gastric cancer, the risk of developing gastric cancer is 70% for men and 56% for women. Only 10% of patients present with resectable disease, and mortality remains high. A screening endoscopy is not reliable for detecting signet-ring adenocarcinoma; therefore, prophylactic gastrectomy is often recommended between the ages of 20 and 30 years. While malnutrition and weight loss can affect fertility, total gastrectomy does not preclude future pregnancies, and the patient should be counseled appropriately.
- Cisco RM, Ford JM, Norton JA. Hereditary diffuse gastric cancer: implications of genetic testing for screening and prophylactic surgery. Cancer. 2008;113(7 Suppl):1850-6. DOI: https://doi.org/10.1002/cncr.23650
- Blair V, Martin I, Shaw D, et al. Hereditary diffuse gastric cancer: diagnosis and management. Clin Gastroenterol Hepatol. 2006;4(3):262-75. DOI: https://doi.org/10.1016/j.cgh.2005.12.003
- van der Post RS, Vogelaar IP, Carneiro F, et al. Hereditary diffuse gastric cancer: updated clinical guidelines with an emphasis on germline CDH1 mutation carriers. J Med Genet. 2015;52(6):361-74. DOI: https://doi.org/10.1136/jmedgenet-2015-103094
Question 2 Rationale and References
Correct answer: C. Azacitidine and venetoclax
Rationale: The FDA has approved venetoclax (BCL2 inhibitor) in combination with hypomethylating agents to treat newly diagnosed acute myeloid leukemia (AML) for patients aged 75 years and older, or those with comorbidities who are ineligible for standard induction therapy. Results from the phase 3 study, a combination of azacitidine and venetoclax, showed improvement in overall survival and increased remission rate compared to single-agent azacitidine. Gemtuzumab ozogamicin is added to standard frontline therapy (daunorubicin and cytarabine) of newly diagnosed AML. In an older adult patient with comorbidities, including coronary artery disease, standard induction with 3+7 is not appropriate. An anthracycline-containing induction regimen is not appropriate for an older adult patient with coronary artery disease and reduced ejection fraction.
- DiNardo CD, Jonas BA, Pullarkat V, et al. Azacitidine and venetoclax in previously untreated acute myeloid leukemia. N Engl J Med. 2020;383(7):617-29. DOI: https://doi.org/10.1056/NEJMoa2012971