Oncology Board Review

1. Oncology Board Review Molly McVey 2014

2. A 31-year-old woman is evaluated for a palpable left breast mass that has been present for 6 months. She is nulliparous and is of Ashkenazi Jewish descent. Her medical history is unremarkable. Her mother was adopted; her father is 72 years old and has a history of prostate cancer. Her paternal aunt was diagnosed with ovarian cancer at age 48 years. Another paternal aunt was diagnosed with breast cancer at age 49 years. Her paternal grandmother died of complications from breast cancer at age 60 years.On physical examination, there is a 4-cm mass in the left breast affixed to the chest wall and a 1-cm, freely movable left axillary lymph node. She undergoes core needle biopsy of the breast mass and lymph node. Pathologic examination of the node and mass reveals moderately differentiated estrogen receptor-positive and progesterone receptor-positive, HER2-negative invasive ductal carcinoma. CT and bone scan do not show metastatic disease. Echocardiogram shows a normal ejection fraction.She will receive preoperative chemotherapy followed by surgery. She undergoes counseling for fertility preservation.Which of the following will be most helpful in determining the best surgical approach? • Counseling and genetic testing • Genomic profile assay • PET scan • Tumor marker testing

3. Breast Cancer • Risk Factors: • Nulliparity • first childbirth after age 30 years • early menarche • late menopause • older age • postmenopausal obesity • alcohol use • lack of physical activity • maternal and paternal family history of breast cancer • early exposure to ionizing radiation • 5% to 10% of all women with breast cancer have a germline mutation of BRCA1, BRCA2, p53, or other mutations

4. When to test:

5. A 42-year-old woman is evaluated following a partial mastectomy and postoperative radiation therapy for intermediate-grade ductal carcinoma in situ that was estrogen receptor-positive and progesterone receptor-positive. The patient is premenopausal. Before surgery, she had been taking an oral contraceptive that has since been discontinued.On physical examination, vital signs are normal. The breast shows a well-healing surgical scar and cutaneous signs of radiation treatment. The remainder of her physical examination is normal.Which of the following is the most appropriate additional therapy? • Anastrazole • Chemotherapy • Raloxifene • Tamoxifen

6. Type of Breast Cancer • Ductal Carcinoma in Situ • Treatment: breast-conserving treatment (lumpectomy plus radiation therapy) OR mastectomy • Tamoxifen: treat for 5 years • Reduce risk of recurrence and development of new primary tumor in ipsilateral or contralateral breast • Invasive Breast Cancer • Treatment: mastectomy and sentinel lymph node evaluation OR lumpectomy and sentinel lymph node evaluation followed by whole-breast radiation therapy

7. Prognosis • Favorable prognostic variables: • expression of estrogen • progesterone receptors • small tumor size • low pathologic grade • Poor prognostic variables • large tumor size • lymph node involvement • overexpression of HER2/neu • hormone receptor negativity.

8. Staging • Stage I: tumors less than 2 cm in diameter and negative lymph nodes. • Stage II: • tumors smaller than 2 cm who also have one to three positive lymph nodes • tumors between 2 and 5 cm with zero to three positive lymph nodes • tumors greater than 5 cm and negative lymph nodes • Stage III: • four or more positive axillary lymph nodes • tumors greater than 5 cm with one to three positive lymph nodes • tumors that extend into the chest wall or skin • Stage IV: distant metastatic disease

9. A 45-year-old woman undergoes evaluation after a recent diagnosis of stage II hormone receptor-positive, HER2-negative breast cancer. She is premenopausal. She was treated with modified radical mastectomy and just completed adjuvant chemotherapy. She had a deep venous thrombosis associated with oral contraceptive pill use 20 years ago. Her family history is negative for venous thromboembolism. She is a nonsmoker and is very physically active.On physical examination, temperature is 37.0 °C (98.6 °F), blood pressure is 100/60 mm Hg, pulse rate is 72/min, and respiration rate is 14/min. No jugular venous distention is present. The lungs are clear to percussion and auscultation.Laboratory studies show a hemoglobin level of 12.8 g/dL (128 g/L) and a leukocyte count of 5600/µL (5.6 × 109/L).Which of the following is the most appropriate next step in management? • Adjuvant aromatase inhibitor therapy • Adjuvant trastuzumab therapy • Baselin imaging with whole-body CT scan or PET scan • Ovarian ablation

10. Treatment • Endocrine therapy (ER+ or PR+) • Tamoxifen – premenopausal • 5 yr course, if contraindicated then ovarian ablation is treatment of choice • Aromatase inhibitor – postmenopausal • 5 yr course primary, or additional 5 yr after 5 yr course of tamoxifen • Anastrozole and examestane are examples • Trastuzumab – HER2 receptor targeted • Impairs systolic ventricular function – get an echo before starting

11. Follow up • Early stage • Clinical eval every 6 months for 5 yrs and annually thereafter • Month self breast exam • Annual mammogram • Vasomotor symptoms side effects: venlafaxine

12. 59-year-old woman is evaluated for the recent onset of persistent lower abdominal pressure. She notes no other pelvic symptoms or problems with bowel function. Her appetite is decreased, but she has not lost weight. She reports no fever, shortness of breath, or chest pain. She has never been pregnant and is a former cigarette smoker with a 25-pack-year history. Breast cancer developed in her maternal grandmother at the age of 75 years.On physical examination, vital signs are normal. No lymphadenopathy is noted. The chest is clear to auscultation. The abdomen is soft with a positive fluid wave and shifting dullness. Pelvic examination discloses nontender fullness in the right adnexa with a normal-appearing cervix. The remainder of the examination is unremarkable.Abdominal ultrasound reveals a right adnexal mass and moderate ascites.Which of the following is the most appropriate next step in establishing a diagnosis? • BRCA1 and BRCA 2 genetic testing • Laparotomy and biopsy • Pap smear • Paracentesis with cytologic analysis • Serum CA-125 measurement

13. Ovarian Cancer • Risk factors: • BRCA1/BRCA2 gene mutations (most significant) • hereditary nonpolyposis colorectal cancer syndrome • high-fat diet • Nulliparity • delayed menopause ***Oral contraceptives decrease risk****

14. Ovarian Cancer • Screening – not recommended • High risk patient – recommend prophylactic bilateral salpingo-oophorectomy • After childbearing • Before 40

15. Treatment • Surgery: • Tumor debulking, TAH, BSO, omenectomy, selective lymphadenectomy and appendectomy • Chemotherapy: • Not indicated in low-grade, well-differentiated CA • Indicated: • High-risk, early-stage disease • advanced disease (Patients with high-grade cancer, positive cytology in ascites or pelvic washings, or pelvic extension have a 30% to 40% relapse rate following surgery alone and should receive three to six cycles of adjuvant chemotherapy – taxane with cisplatin and carboplatin)

16. Follow Up • History and physical examination (including pelvic exam) and measurement of serum CA-125 levels performed every 2 to 4 months for the first 2 years, then every 3 to 6 months for 3 years, and then annually.

17. A 41-year-old woman undergoes follow-up evaluation for stage II invasive squamous cell cervical cancer.She recently underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy with pelvic lymph node sampling. Pathologic examination reveals a 3.3-cm tumor with multiple high-risk features.Which of the following is the most appropriate next step in management? • Human papillomavirus testing • Pelvic radiation therapy • Pelvic radiation therapy and concurrent chemotherapy • Observation with serial CT scan and pelvic examinations every 4 months

18. Cervical Cancer • Most important risk factor for the development of cervical cancer is persistent HPV infection • Other risk factors: • early-onset sexual activity • multiple sexual partners • sexually transmitted diseases • Smoking • Multiparity • prolonged use of oral contraceptive agents • immunosuppression

19. Cervical Cancer • Signs: vaginal bleeding, abnormal discharge • Diagnosis: punch biopsy or coposcopy • Stage: • Ia:confined to uterus (microscopic lesions) • Ib: confined to uterus (macroscopic lesions) • II: extends beyond uterus (not invading pelvic wall or lower third of vagina) • III: extension into side wall or lower vagina or urethral obstruction • IV: invades bladder or rectum, beyond pelvis or distant metastasis

20. Cervical Cancer Surveillance • Pelvic and pap every 3-6 month x 2 years, then every 6 month for next 3 years, then annually

21. A 62-year-old woman is evaluated for a 6-month history of intermittent pain and occasional small amounts of bright red blood on defecation. She is otherwise well with no significant medical or family history.On physical examination, temperature is 36.8 °C (98.2 °F), blood pressure is 125/85 mm Hg, pulse rate is 80/min, and respiration rate is 14/min. The abdomen is soft with no distention or organomegaly, and bowel sounds are normal. The remainder of the examination is normal.Colonoscopy is performed, and a 3-cm mass is identified starting 7 cm from the anal verge. A biopsy of the mass reveals invasive adenocarcinoma. An endorectal ultrasound shows involvement of the mucosa and submucosa. The mass penetrates into, but not fully through, the rectal wall with no lymph node metastases (T2N0). A high-resolution, contrast-enhanced CT scan of the chest, abdomen, and pelvis demonstrates only rectal wall thickening and no evidence of metastatic disease.Which of the following is the most appropriate treatment? • Chemotherapy • Concurrent chemo and radiation, followed by surgery and then further chemotherapy • Radiation therapy • Radiofrequency ablation • Surgery

22. GI Cancer • Colorectal cancer • Anal cancer • Pancreatic Cancer • Esophageal Cancer • Gastric Cancer • GI Neuroendocrine tumors

23. GI Cancer • Colorectal cancer • Anal cancer • Treat with chemo and radiation, no chemo • Pancreatic Cancer • Poor prognosis • Esophageal Cancer • Risk factors and symptoms • Gastric Cancer • Risk factors, association with H.pylori • GI Neuroendocrine tumors

24. Colorectal Cancer • Screening • Types: stool-based test, flex sig or colonoscopy • Time: • Average risk: age 50 for men and women • High risk • age 40 with family history of colorectal cancer, adenomatous polyps or symptoms of concern • 10 years younger than the age at which the youngest affected relative was diagnosed

25. Staging • I: does not invade full thickness of bowel wall, no lymph nodes involved • II: invades full thickness of bowl wall and may invade into pericolonic or perirectal fat, no lymph nodes involved • III: 1+ lymph nodes involved • IV: metastatic

26. Treatment • Surgery: local and localregional disease • Chemo: determined by stage • All stage III should receive adjuvant chemo • FOLFOX • Know side effects of different chemo • Metastases: if single or in one organ – can be resected

27. Surveillance • Physical exam and CEA every 3-6 month for first 3 years, every 6 months during years 4 and 5 • CT scan annually x 3 years • Colonoscopy – 1 year after resection, then in 3 years, then every 5 years

28. 57-year-old woman is evaluated in the emergency department for shortness of breath associated with wheezing and pain with inspiration. The patient has a 25-pack-year history of cigarette smoking. She takes no medications.On physical examination, temperature is normal, blood pressure is 138/82 mm Hg, and respiration rate is 18/min. Wheezing is heard on pulmonary examination. The remainder of the examination is normal.Laboratory studies are unremarkable. A chest radiograph is normal, and a spiral CT scan reveals multiple pulmonary emboli in addition to several bilateral pulmonary nodules, each measuring 2 to 4 mm.The patient is admitted to the hospital, and heparin and warfarin therapy are begun.Which of the following is the most appropriate next diagnostic step in the evaluation of the patient’s pulmonary nodules? • Bronchoscopy with cytologic analysis • CT –guided biopsy of the largest nodule after withdrawal of anticoagulation • Follow-up CT in 12 months • PET/CT now

29. Lung Cancer • SCLC (most all are smokers!) • Associated with hyponatremia 2/2 SIADH • CANNOT be surgically resected • NSCLC • Associated hypercalcemia 2/2 bone metastases and PTHr • CAN be surgically resected • Can be adeno (peripheral), squamous cell (central) and large cell (peripheral)

30. NSCLC • Stage: • Ia: <3cm • Ib: >3cm • II: regional lymph nodes/local invasion/near carina • III: mediastinal lymph nodes • IV: mets or ipsilateral pleural effusion • Treatment: • Stage I and II – surgery • Stage III – radiation and chemo

31. A 65-year-old man is evaluated for a 3-week history of hemoptysis and a recent 4.5-kg (10-lb) weight loss. He has a 90-pack-year smoking history.On physical examination, vital signs are normal. The pulmonary examination reveals occasional crackles at the posterior right midlung field. The remainder of the physical examination is normal.A chest radiograph demonstrates a large right hilar mass. A CT scan of the chest shows a 5-cm right hilar mass with bulky mediastinal lymphadenopathy. Bronchoscopic examination reveals small cell lung cancer. Results of staging studies with an MRI of the brain and a bone scan are negative.The patient receives six cycles of cisplatin and etoposide chemotherapy with radiation to the lung mass and regional disease concurrent with the first cycle of chemotherapy. A follow-up CT scan of the chest shows a residual 1.5-cm right hilar abnormality.Which of the following is the most appropriate next step in this patient’s management? • Biopsy of the residual mass • Three additional cycles of chemo • Whole-brain radiation • Observation

32. SCLC • Stage: • Limited: confined to one hemithorax, all disease confined to one radiation field • Extensive: beyond one hemithorax, ipsilateral malignant effusion, mets (brain, liver , bone) • Treatment: • Limited: chemo and radiation • Extensive: chemo • Prophylactic brain radiation: used in both!!

33. A 58-year-old man is evaluated for a sore throat and neck pain of 5 weeks' duration. He has a 50-pack-year smoking history.On physical examination, vital signs are normal. A firm mass at the base of the tongue and a 3-cm firm mass at the angle of the jaw on the right are noted. The remainder of the physical examination is normal.Fiberoptic examination reveals a biopsy-proven squamous cell carcinoma of the tongue base. An MRI suggests invasion into adjacent cortical bone. PET/CT demonstrates several enlarged lymph nodes in the right neck, the largest measuring 3.3 cm.Which of the following is the most appropriate treatment? • Chemotherapy • Chemotherapy and radiation therapy • Radiation • Surgical resection

34. Head and Neck Cancer • Risk factors: ALCOHOL, TOBACCO, EBV, HPV • Staging: (varies by location) • I and II: smaller without LN involvement • III: small-volume ipsilateral LN involvement • IV: larger tumors with local invasion, more extensive LN involvement or mets • Treatment: • Early (stage I and II): surgical resection or radiation • Advanced (stage III and IV): radiation and chemo +/- resection

35. An 80-year-old man undergoes an annual physical examination. He has had mild stable nocturia for many years. He reports no bone pain, weight loss, fever, chest pain, or shortness of breath. Medical history is notable for hypertension and type 2 diabetes mellitus for which he takes antihypertensive and diabetic medications.On physical examination, blood pressure is 148/84 mm Hg, pulse rate is 60/min, and respiration rate is 15/min. No lymphadenopathy is noted. The lungs are clear to auscultation, and the abdomen is soft, with normal bowel sounds. Rectal examination reveals an enlarged prostate gland with a nodule on the right side.Prostate-specific antigen level is 6.4 ng/mL (6.4 µg/L).Prostate biopsy reveals several small foci of adenocarcinoma in 2 of 12 cores on the right side, with a Gleason score of 6.Which of the following is the most appropriate management? • Androgen deprivation therapy • Radiation with androgen deprivation therapy • Radical prostatectomy • Observation

36. Prostate Cancer • Know symptoms and diagnosis (abnl DRE and/or elevated PSA) • Treatment: • Radical prostatectomy: disease confined to prostate and life expectancy >10years • Know side effects (ED, incontinence..) • Radiation • Androgen deprivation therapy: GnRH agonist/antagonist and antiandrogen

37. Prostate Treatment

38. Testicular Cancer • Seminomas • Better prognosis • Dx: B-hCG elevated • Nonseminomas (germ cell) • Dx: AFP and B-hCG elevated

39. Renal Cell Cancer • Know risk factors • Staging • Treatment: • Stage I-III: partial (tumor <4cm) or radical nephrectomy • Chemo – mostly reserved for metastatic disease

40. A 48-year-old man is evaluated for chronic dyspepsia and intermittent midabdominal pain of 6 months' duration. He has had no fatigue, vomiting, weight loss, fever, or night sweats. He has otherwise been well and takes no medications.On physical examination, vital signs are normal. The abdominal examination reveals midepigastric tenderness to palpation but no evidence of masses or peritoneal signs. The remainder of the physical examination is normal. • cyclophosphamide, vincristine, and prednisone • Involved-field radiation therapy • Metronidazole, amoxicillin and omeprazole • Single-agent rituximab • Observation with repeat endoscopy in 3-6 months Upper endoscopy shows a small gastric ulcer. Small clonal mucosa-associated B cells expressing CD20 antigen are identified on biopsy along with Helicobacter pylori organisms. CT scans of the chest, abdomen, and pelvis are unremarkable. Which of the following is the most appropriate management?

41. Lymphoma • Dx: excisional biopsy and molecular testing • Classification: indolent, aggressive, and highly aggressive • Types: • NHL: B-cell, T-cell and NK-cell • Hodgkin's • Indolent types • Follicular: 3 grades based on lymphocyte size – determines treatment and prognosis.

42. Indolent NHL • Follicular: 3 grades based on lymphocyte size-determines treatment and prognosis • Tx(wait until symptomatic/organ dysfxn) • Rituximab, cyclophosphamide, vincristine and prednisone (R-CVP) • Rituximab, doxorubicin, vincristine and prednisone (R-CHOP) • MALT: associated with H.pylori • Tx: metronidazole, amoxicillin/clarithromycin plus omeprazole

43. A 45-year-old man is evaluated for gradually worsening fatigue and pallor of 7 months' duration, as well as fever, weight loss, night sweats, and pain.On physical examination, temperature is 38.0 °C (100.4 °F), blood pressure is 140/70 mm Hg, pulse rate is 110/min, and respiration rate is 28/min. He has bilateral cervical and axillary lymphadenopathy. Abdominal examination discloses a palpable spleen 4 cm below the left costal margin. • Allogeneic hematopoietic stem cell transplantation • Cyclophosphamide, vincristine and prednisone • Fludarabine • rituximab The peripheral blood smear shows excessive small lymphoid cells and multiple smudge forms. Flow cytometry of the peripheral blood reveals monoclonal B cells expressing CD20, CD52, CD10, CD5, and ζ-chain-associated protein 70. Cytogenetic analysis reveals a deletion of 17p, and the heavy-gene mutational status is unmutated. Which of the following is the most appropriate treatment of this patient?

44. CLL • Clinical stages: • I: LAD • II: splenomegaly • III: anemia • IV: thrombocytopenia • Criteria for initiating tx: B symptoms (fevers, WL and night sweats), symptoms 2/2 LAD, HSM, worsening cytopenia

45. Hairy Cell Leukemia • Highly curable • Recognize the cell characteristics (below) • “hairy” projections • Tx: cladribine

46. Aggressive Lymphomas • Diffuse large Cell • Tx: RCHOP +/- radiation • relapse should be considered for autologous HSCT • Mantle cell • Hodgkin • Highly curable, mediastinal mass • Local dz: radiation +/- chemo • Advanced: ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) • Cutaneous T-cell Non-Hodgkin • Mycosis fungoides and Sezary syndrome • “Cerebriform” nucleus

47. A 69-year-old woman is evaluated for a lump under her arm found on self-examination. She is otherwise healthy and has no other symptoms. Medical and family histories are unremarkable, and she takes no medications.On physical examination, temperature is 37.4 °C (99.3 °F), blood pressure is 110/70 mm Hg, pulse rate is 72/min, and respiration rate is 14/min. The patient has a hard, fixed, 2-cm mass palpable in the right axilla. The remainder of the examination, including breast examination, is normal.Complete blood count and serum creatinine, total bilirubin, and alkaline phosphatase levels are normal.A needle aspirate of the right axillary mass reveals adenocarcinoma. Bilateral mammography and breast MRI are normal. CT scan of the chest, abdomen, and pelvis demonstrates the enlarged axillary lymph node and no other abnormalities.Which of following is the most appropriate initial treatment? • Breast cancer chemotherapy regimen • Excision of the axillary lymph node • Mastectomy with axillary lymph dissection • Radiation therapy to the right axilla

48. Cancer of Unknown Primary Site • Workup: scans, tumor markers, mammogram and gyneval for women, prostate exam for men, GI symptoms (scopes) • Groups: • Young males poorly differentiated CA: think testicular (order AFP and B-hCGand US testicles), treat with platinum-based chemo • Woman with isolated axillary LAD: think breast cancer (manage as stageII) • Isolated cervical LAD: think head and neck • Women with peritoneal carcinomatosis: ovarian