Board Preparation: Gynecologic Cancers, Leukemia

May 01, 2017

Test your knowledge of gynecologic cancers and leukemia with questions from a past edition of ASCO-SEP®, ASCO’s self-evaluation program in oncology.

The fifth edition of ASCO-SEP is available for purchase in the ASCO University® bookstore. Featuring 21 updated chapters and more than 180 new self-assessment questions in the book, as well as a 120-question comprehensive mock exam online, this resource is perfect for board preparation, and can be used to earn Maintenance of Certification and continuing medical education credit. Visit ASCO University for information about the latest edition of ASCO-SEP and other self-assessment resources.

Correct answers, rationales, and suggested reading are listed at the bottom of the page.

 

  1. A 50-year-old woman with newly diagnosed endometrial adenocarcinoma comes to you for consultation. The pathology report states that there is loss of MSH2 by immunohistochemistry.

    In addition to discussing the management of her endometrial cancer, you recommend genetic counseling, given that she may be at increased genetic risk for which of the following cancers:

A. Lobular breast cancer
B. Clear cell renal cell carcinoma
C. Medullary thyroid carcinoma
D. Adenocarcinoma of the colon

 

  1. A 48-year-old woman presents for gynecologic evaluation, reporting mild, persistent pelvic pressure and bloating. On pelvic examination, the cervix appears normal. A Pap smear yields cells suspicious for adenocarcinoma with features suggestive of serous carcinoma; HPV testing is negative for all high-risk subtypes. Sonography shows small pelvic ascites and a right adnexal mass. CA125 is 1536 u/mL.  Her family history is negative for colon cancer and endometrial cancer; a paternal aunt had breast cancer at age 50. The patient’s body mass index is 24.

Which of the following is the most likely diagnosis?

A. HPV-negative adenocarcinoma of the cervix, with metastasis to the right ovary
B. Endometrial adenocarcinoma with endocervical and right ovarian involvement
C. Fallopian tube carcinoma
D. Vaginal adenocarcinoma

 

  1. A 32-year-old man was diagnosed with B-cell acute lymphoblastic leukemia (ALL) 5 months ago. At the time of diagnosis, the complete blood cell count revealed a white blood cell count of 13.8 x 109/L, hemoglobin of 8.8 g/dL, and platelets of 18 x 109/L. The bone marrow examination at the time of diagnosis revealed 76% lymphoblasts. The patient had an uncomplicated induction course and experienced a complete remission. He is currently receiving his second month of consolidation therapy with vincristine/dexamethasone/methotrexate/asparaginase. Over the last few days, he has developed increasing abdominal pain radiating to the back. On physical exam, he has epigastric abdominal pain with guarding, but no rebound and normal active bowel sounds.

What is the most likely cause of this patient's abdominal pain?

A. Acute cholelithiasis
B. Erosive gastritis due to corticosteroids
C. Asparaginase toxicity
D. Vincristine toxicity

 

  1. A 55-year-old woman is weak, fatigued, and has night sweats. Her spleen is 8 cm below the left coastal margin. Her white blood count is 75,000 with 30% polymorphonuclear leukocytes, 7% bands, 8% eosinophils, 15% basophils, 10% myelocytes, 15% metamyelocytes, and 15% blasts. Hemoglobin is 10 and platelets are 346,000. The patient began receiving 100 mg of dasatinib once daily. After 12 months her white blood count has normalized and her spleen is no longer palpable, but a bone marrow examination reveals 20/20 Ph+ and ABL kinase domain mutation showed a T315I mutation. 

Which of the following would be the most appropriate immediate treatment for this patient?

A. Human leukocyte antigen typing for immediate allogeneic stem cell transplantation
B. Switch to nilotinib at a dose of 400 mg twice daily    
C. Increase the dose of dasatinib to 140 mg once daily
D. Switch to ponatinib at a dose of 45 mg once daily
E. Continue with current therapy and repeat the bone marrow examination in 6 months

 

Rationales

1: D

Endometrial carcinoma is part of Hereditary Non-Polyposis Colon Cancer (Lynch) syndrome and is often the first-diagnosed cancer among women with Lynch syndrome. Certain histologic characteristics, and patient age, may suggest the possibility of a Lynch syndrome endometrial cancer. Demonstrating loss of one of the mismatch repair enzyme proteins by immunohistochemisty (IHC) can further corroborate the possibility of Lynch syndrome.  Patients should be referred for genetic counseling and testing. Patients with endometrial carcinoma who have Lynch syndrome are at increased risk for colon cancer.

A genetic increased risk for lobular breast cancer is found in patients with E-cadherin germline mutations. Such patients are also at risk for gastric cancer. Lynch syndrome patients are at increased risk for urothelial cell tumors but not renal cell carcinomas. Medullary thyroid carcinoma is part of the Multiple Endocrine Neoplasia 2 familial syndrome.

Suggested Readings

Vasen HF, Stormorken A, Menko FH et al. MSH2 mutation carriers are at higher risk of cancer than MLH1 mutation carriers: a study of hereditary nonpolyposis colorectal cancer families. J Clin Oncol. 2001 Oct 15;19(20):4074-80.

Bonadona V, Bonaïti B, Olschwang S, et al. Cancer risks associated with germline mutations in MLH1, MSH2, and MSH6 genes in Lynch syndrome. JAMA. 2011 Jun 8;305(22):2304-10.

2: C

Fallopian tube cancer may present with adenocarcinoma cells on Pap smear due to shedding of the malignant cells from the tubal lumen. The high CA125 suggests an ovarian or fallopian tube cancer, although it may be elevated in other Mullerian cancers. The serous features suggest ovarian or fallopian tube primary, although some endometrial carcinomas may be classified as serous. The family history supports the possibility of a fallopian tube or ovarian primary. Cervix cancer, both adenocarcinomas and squamous cell carcinomas, are nearly always associated with HPV. Endometrial carcinoma may also present with a positive Pap smear but the patient’s low BMI, young age, and lack of family history make this diagnosis less likely than a tubal or ovarian primary.

Suggested Reading

Mulvany NJ, Mitchell G, Allen DG. Adenocarcinoma cells in Pap smears. Pathology. 2009;41(5):411-8.

3: C

This question relates to toxicities of the common agents that are used in treatment of ALL. This patient has developed pancreatitis, which is a common side effect of asparaginase.  Other asparaginase–related side effects include allergic reaction, coagulopathy, lethargy, apathy, and asthenia. These side effects are usually reversible with cessation of treatment and time.  Although all of the choices can present as abdominal pain, the most likely diagnosis of the pain is pancreatitis.  Asparaginase is the most likely cause of the pancreatitis and therefore of the patients symptoms.

Suggested Readings

Barry E, DeAngelo DJ, Neuberg D, et al. Favorable outcome for adolescents with acute lymphoblastic leukemia treated on Dana-Farber Cancer Institute Acute Lymphoblastic Leukemia Consortium Protocols. J Clin Oncol. 2007;25:813–9.

Pui C-H, Evans WE. Drug therapy: treatment of acute lymphoblastic leukemia. N Engl J Med. 2006;354:166–78.

Stock W, Douer D, DeAngelo DJ, et al. Prevention and management of asparaginase/pegasparaginase toxicities in adults and older adolescents: recommendations of an expert panel. Leuk Lymphoma. 2011; 52(12): 2237–53.

4: D

Although the majority of patients with stable phase CML will obtain a complete cytogenetic remission (CCyR), it is important to ensure that patients follow response milestones. The DASISON trial demonstrated significantly faster responses for both complete cytogenetic response and major molecular response among patients treated with dasatinib compared with those in the imatinib arm; however, there was no improvement in either progression free or overall survival.  This patient has failed to achieve a CCyR at 12 months on upfront dasatinib therapy, and therefore a change to an alternative tyrosine kinase inhibitor (TKI) is indicated. The presence of a T315I ABL mutation has rendered the patient resistant to dasatinib.  In addition, neither imatinib, nilotinib or bosutinib will be effective. Ponatinib is a new third-generation TKI recently approved by the FDA that is effective against the T315I mutation and would be a reasonable choice.  Although the durability of clinical remissions remains high, long-term follow up is lacking and it would also be reasonable to refer the patient for stem cell transplantation and perform HLA typing in order to determine donor availability.

Suggested Readings

Kantarjian HM, Shah NP, Cortes JE,et al. Dasatinib or imatinib in newly diagnose chronic-phase chronic myeloid leukemia: 2-year follow-up from a randomized phase 3 trial (DASISION). Blood. 2012;119:1123-9.

Cortes J, Kim D, Pinilla-Ibarz J, et al. A phase II trial of ponatinib in Philadelphia-positive leukemias. N Engl J Med. 2013;369:1783-96.

Back to Top