Oncology

Question 1

Surveillance for Asian man above age 40 with HCC

Answer 1

6 monthly ultrasound

Question 2

Sickle-cell-anemia: why do they not have splenomegaly?

Meta: rationale

Answer 2

Even mild disease: splenic infarcts from childhood

Question 3

Sickle-cell disease: sickle trait:

Clinical phenotype

Answer 3

Asymptomatic

Question 4

Sickle-cell: Stroke prophylaxis

Answer 4

Exchange transfusion

Hydroxyurea takes several weeks to reduce sickle hemoglobin concentrations. Mechanism of stroke in sickle: sickled cells.

Question 5

90 to 95% of urothelial cancers

Answer 5

Transitional cell

Question 6

A group of patients with NSCLC have tumors with inversion in chromosome 2 that juxtaposes the 5’ end of the echinoderm microtubule-associated protein-like 4 (EML4) gene with the 3’ end of the anaplastic lymphoma kinase (ALK) gene, resulting in the fusion oncogene EML4-ALK.

This fusion oncogene rearrangement is transforming both in vitro and in vivo.

Rx?

Answer 6

Crizotinib

ALK inhibitor

“ALK-positive” tumors are highly sensitive to tALK-targeted inhibitors.

Question 7

alpha fetoprotein

Answer 7

1. nonseminomatous GCTs
2. HCC

AFP is not elevated in patients with pure seminomas.

Question 8

Bladder cancer biopsy: Quality Criterion

Answer 8

should include bladder wall muscle

Question 9

Bladder cancer: DxStagingEval

Answer 9

1. Excretory urography/intravenous pyelogram followed by cystoscopy
2. Retrograde pyelography ( upper tract lesions)
3. Urine cytology; brush biopsies may increase the diagnostic yield
4. Biopsies of any lesion must be of adequate size to include bladder wall muscle
5. CT abdomen: Evaluate for local extension and nodal involvement
6. bone scan if bony symptoms
7. CXR staging evaluation

Question 10

Bladder cancer: DxMethod

Answer 10

Excretory urography/intravenous pyelogram followed by cystoscopy

Question 11

When cancer of the upper urinary tract is diagnosed, there is a _% to _% chance of cancer of the bladder.

When bladder cancer is diagnosed, there is a _% to _% chance of developing cancer of upper urinary tract.

Answer 11

When cancer of the upper urinary tract is diagnosed, there is a 30% to 50% chance of cancer of the bladder.

When bladder cancer is diagnosed, there is a 2% to 3% chance of developing cancer of upper urinary tract.

Question 12

Exposure to aryl amines increases risk for _ cell cancer, while Schistosoma haematobium infection is associated with _ cell cancer of the bladder

Answer 12

Exposure to aryl amines increases risk for transitional bladder cancer, while Schistosoma haematobium infection usually associated with squamous cell carcinoma of the bladder

Question 13

Bladder cancer: symptoms

Answer 13

1. Hematuria: painless
2. frequency, urgency, or dysuria
3. Vesical irritation without hematuria: Common in carcinoma in situ of bladder
4. Symptoms of advanced disease: Pain from metastatic sites, edema of lower extremities, cough or dyspnea from lung metastases

Question 14

CA 125

Answer 14

Epithelial ovarian cancer

Question 15

CA 19-9

Answer 15

Pancreatic cancer

Question 16

Cancer associated with: membranous nephropathy, MAHA, PAN

Answer 16

Gastric cancer

OpenQuestion -what drives autoimmunity?

Question 17

CEA

Answer 17

1. GI tumors
2. Some lung
3. Some breast
4. Some MCT

Question 18

Chronic diarrhea after pancreatectomy

Rx

Answer 18

Oral pancreatic enzyme

Question 19

When should first degree relatives of patients who have colon cancer before age 60 be screened?

Answer 19

40 or 10 years prior to dx whichever is first

Question 20

Cryptorchidism: risk of testicular cancer is bilateral

Answer 20

True

Question 21

Majority of testicular cancer in cryptorchid testicles

Answer 21

Pure seminoma

Question 22

Diarrhea associated with carcinoid and VIP

Answer 22

octreotide

Question 23

A familial nephropathy of unknown cause that results in progressive inflammation of the renal parenchyma, leading to renal failure and multifocal, superficial, low-grade cancers of the renal pelvis and ureters

Answer 23

Balkan nephropathy

Question 24

Extracolonic cancer in Lynch syndrome

Answer 24

Endometrial

Question 25

Febrile neutropenia: low risk vs high risk Low risk neutropenia: MxL

Answer 25

1. Expected duration of neutropenia of 7 days or less 2. No evidence of sepsis 3. No co-existing conditions (what does that mean?) 4. No hepatic or renal dysfunction Low risk neutropenia: 1. No need to admit 2. home on ciprofloxacin + Augmentin

Question 26

ß-HCG: marker for

Answer 26

Germ cell tumor gestational trophoblastic disease (GTD), testicular tumors, ovarian germ cell tumors, teratomas, and, rarely, other human chorionic gonadotropin (hCG)-secreting tumors

Question 27

Irregular vaginal bleeding after sex

Answer 27

Cervical cancer

Question 28

LCIS: ER status

Answer 28

Nearly always ER+

Question 29

LCIS: Mx

Answer 29

1. Surgical excision 2. Annual mammogram 3. Risk reduction counseling 4. Tamoxifen

Question 30

55 to 74 year old patient with 30 pack year history, currently smoke or have quit in 15 years

Answer 30

Yearly low dose CT

Question 31

Lynch syndrome

Answer 31

1. Autosomal dominant 2. Error in DNA mismatch repair system 3. AKA HNPCC 4. Adenocarcinoma of the proximal colon, splenic flexure 5. Amsterdam criteria: all have to be met

Question 32

Lynch syndrome: lifetime risk of cancer

Answer 32

70%

Question 33

Malignant tumor: most common men 25-35

Answer 33

Testicular

Question 34

Morphine dosing rule in palliation

Answer 34

10% of daily total dose every hour (PO) or every 30 minutes IV

Question 35

Morphine: time to peak effect

Answer 35

30 to 60 minutes

Question 36

Nonsmoker with metastatic adenocarcinoma: OnDx

Answer 36

Test for EGFR, ALK If EGFR +ve, erlotinib or geftinib If ALK+ve: crizotinib

Question 37

Palliation: morphine failure at high doses Severe, continued pain in spite of high dose long acting, frequent high dose short acting

Answer 37

Switch to methadone

Question 38

Post menopausal bleeding + Hx tamoxifen use: Mx

Answer 38

In-office endometrial biopsy

Question 39

Postmenopausal bleeding: imaging

Answer 39

Pelvic USG, saline sonography Endometrial thickness \< 4 mm has NPV 99% for endometrial cancer

Question 40

Prostate cancer: castration resistant, metastatic

Answer 40

docetaxel

Question 41

Prostate cancer: life expectancy \< 10 years

Answer 41

1. No symptoms: Active surveillance 2. + Symptoms: Radiation (same efficacy as surgery)

Question 42

Risk of lung cancer in smokers | (Model derived from CARET study)

Answer 42

1. duration of smoking, 2. number of cigarettes smoked per day, 3. duration of abstinence 4. age http://nomograms.mskcc.org/Lung/Screening.aspx 10 year risk: 1. 51-year-old woman who smoked one pack per day for 29 years but stopped smoking 9 years : 0.8% 2. 68-year-old man who smoked two packs a day for the past 50 years and continued to smoke: 15%

Question 43

Suspected ovarian cancer: first investigation?

Answer 43

Transvaginal ultrasound

Question 44

Tamoxifen: can cause which cancer?

Answer 44

Uterine carcinoma and sarcoma (Because it has both antiestrogenic and pro-estrogenic effects; can cause endometrial proliferation)

Question 45

HIT: when does it occur?

Answer 45

Typically 5-14 days after exposure

Question 46

HIT: Pre-test probability assessment

Answer 46

The score assesses the degree of **Thrombocytopenia**, the **Timing** relative to heparin exposure, the presence of **Thrombosis**, and **oTher** causes for thrombocytopenia. Points are assigned as follows:

Question 47

Microcytosis: mild anemia MCV 60s

Answer 47

Beta-Thal trait

Question 48

Budd-Chiari: Commonest risk factor

Answer 48

MPN Myeloproliferative neoplasm

Question 49

Acute porphyria: FirstTest Meta: FirstTest

Answer 49

Rapid urine screen for porphobilinogen

Question 50

Sickle-cell: Acute chest syndrome: Severe: Rx other than supportive

Answer 50

Exchange transfusion

Question 51

β-thalassemia intermeda: why are they prone to venous thrombosis?

Answer 51

Abnormal erythrocytes which express prothrombotic phospholipids; activated clotting factors and activated platelets. Meta: never seen a case Several reports have described an increased risk of thromboembolic complications in thalassemia [68-70]. In a retrospective series of 584 individuals with beta thalassemia intermedia, thrombosis was observed in 82 (14 percent) [71]. (See "Clinical manifestations and diagnosis of the thalassemias".) The mechanisms are incompletely understood. It has been suggested that increased phosphatidylserine (PS) on the outer leaflet of the RBC membrane may promote thrombosis, similar to the role of PS in promoting coagulation on the surface of activated platelets [72]. (See "Overview of hemostasis", section on 'Multicomponent complexes'.) Other hemostatic changes may include alterations in the levels of procoagulant or anticoagulant factors, and/or chronic activation of platelets, endothelial cells, or white blood cells [68]. Splenectomy may also increase the risk of thrombosis [73,74]. Similarity to the increased risk of thromboembolism in sickle cell disease has also been suggested. However, as high quality evidence for the increased risk of thrombophilia is lacking, we manage patients with thalassemia similar to the general population. Further study of this issue is warranted

Question 52

β-thalassemia: classification-basis

Answer 52

Degree of beta-globin reduction Beta thalassemia is further classified as: 1. beta thalassemia major, 2. beta thalassemia intermedia, or 3. beta thalassemia minor (also called beta thalassemia trait) according to the degree of reduction in beta globin

Question 53

β-thalassemia: causes of anemia

Answer 53

1. Intramedullary hemolysis 2. Reduced RBC survival Beta thalassemia is caused by one or more mutations in the beta globin gene (figure 1) that result in an imbalanced ratio of alpha to beta globin. This imbalance in turn leads to impaired red blood cell (RBC) maturation and destruction of developing RBC precursors in the bone marrow, called ineffective erythropoiesis or intramedullary hemolysis; as well as hemolysis, with reduced RBC survival in the peripheral blood. The severity of beta thalassemia may also be affected by other mutations that affect the ratio of alpha globin to beta globin, including concomitant alpha thalassemia, hereditary persistence of fetal hemoglobin, concomitant sickle hemoglobin mutation, or concomitant hemoglobin E. (See 'Imbalanced ratio of alpha to beta globin' above.)

Question 54

β-thalassemia: causes of organ damage Meta: PathophysiologicMechanism

Answer 54

Organ damage in beta thalassemia (renal disease, cardiomyopathy, diabetes) is due to the combined effects of anemia, chronic hypoxia, iron overload, and possibly other disease features such as a chronic inflammatory state. (See 'Organ damage' above.)

Question 55

CGD

Answer 55

recurrent, life-threatening bacterial and fungal infections and granuloma formation. Most diagnosed before age of five years. Caused by defects in phagocyte enzyme complex which is responsible for the phagocyte respiratory burst. lifelong antifungal plus antibacterial prophylaxis +/- immunomodulatory therapy

Question 56

ITP: DxMethod Meta: DxOfExclusion

Answer 56

Ruling out other causes of thrombocytopenia ## Footnote The pathogenesis of immune thrombocytopenia (ITP) is related to a combination of increased platelet destruction and impaired platelet production caused by anti-platelet autoantibodies.

Question 57

ITP: Rx Meta: FirstLine, SecondLine

Answer 57

1. FirstLine: prednisone/IVIG 2. SecondLine: splenectomy, rituximab, thrombopoietin receptor agonists or immunosuppressive therapy

Question 58

GM-CSF: evidence for use during sepsis + neutropenia

Answer 58

Not that great Granulocyte colony stimulating factors (G-CSFs) have been widely evaluated to minimize the extent and duration of neutropenia associated with myelosuppressive cytotoxic chemotherapy or radiation therapy (RT) for conditions other than acute leukemia, myelodysplastic syndrome, and in the setting of hematopoietic cell transplantation (HCT). Despite their effects on neutropenia, prophylactic use of G-CSFs has not been shown to have an impact on survival in most clinical situations.

Question 59

Aplastic anemia vs MDS: difference

Answer 59

In Myelodysplastic Syndrome (MDS), there is impaired production of myeloid class of blood cells by the bone marrow whereas aplastic anemia is a condition in which the bone marrow is damaged leading to decreased new blood cell production. In MDS, the bone marrow produces new blood cells but they are abnormal and deformed whereas in aplastic anemia, the bone marrow stops producing new blood cells. Read more: Difference Between Aplastic Anemia and Myelodysplastic Syndrome | Difference Between http://www.differencebetween.net/science/health/disease-health/difference-between-aplastic-anemia-and-myelodysplastic-syndrome/#ixzz4eqINRJn6

Question 60

MDS vs myelofibrosis

Answer 60

1. Myelofibrosis: leukoerythroblastic picture 2. Myelofibrosis: splenomegaly

Question 61

MPN: Common MPNs

Answer 61

1. CML 2. PV 3. PMF 4. ET CML = chronic myeloid leukemia; PV = polycythemia vera; PMF = primary myelofibrosis; ET = essential thrombocythemia; IG = immature granulocytes; Megas = megakaryocytes; EPO = erythropoietin See: http://surgpathcriteria.stanford.edu/mdsmps/primary-myelofibrosis/differential-diagnosis.html

Question 62

Leukoerythroblastic

Answer 62

Neutrophilia and nucleated red blood cells may also be associated with teardrop shaped RBCs and early granulocytes in the blood, the so-called leukoerythroblastic blood picture (picture 7A-B). This combination suggests the presence of infiltrative marrow disease (eg, myelofibrosis, malignancy, granulomatous disease, inflammation or infection in the marrow itself).

Question 63

5q minus syndrome

Answer 63

MDS subtype: severe anemia, normal platelet counts, and del(5q) ## Footnote Approximately 5 percent of patients with MDS present with "5q minus syndrome" characterized by severe anemia, preserved platelet counts, and del(5q) as the sole cytogenetic abnormality. Patients with 5q minus syndrome with or without other cytogenetic abnormalities have demonstrated high response rates to treatment with low-dose **lenalidomide**.

Question 64

MDS: Rx

Answer 64

1. No standard Rx 2. symptomatic anemia and an erythropoietin level \>500 mU/mL who are not good candidates for immunosuppressive Rx, azacitidine, decitabine, or lenalidomide 3. serum erythropoietin level \>500 mU/mL, and a good probability of responding to immunosuppressive therapy, we suggest a trial of antithymocyte globulin (ATG) plus cyclosporin