Random Questions from Practice Tests Flashcards
Mechanism of Action for rolapitant
substance P/neurokinin-1 inhibitor
Risk factors for adenocarcinomas of the nasal cavity/sinuses
Wood dust and leather dust
Cancer thorotrast is associated with
cholangiocarcinoma
Conslidation strategy after induction for CNS lymphoma
auto SCT or whole brain RT
Drugs in CHOP
cyclophosphamide, doxorubicin, vincristine, prednisone
Effects of TKI in pregnancy
increases fetal malformation and spontaneous abortions
In what position does SVC syndrome worsen?
Supine
What is the presenting triad associated with Plummer-Vinson syndrome, and what cancer is it associated with?
Iron deficiency anemia, esophageal webs, dysphagia. Patients are at increased risk of developing Squamous cell carcinoma of the esophagus
Appropriate duration of anticoagulation in women who have DVT during pregnancy
6 weeks after birth
When should chemotherapy be added to adjuvant radiation in patients with head and neck cancer?
Positive margins or extracapsular extension
Hct goal post cardiac surgery
24%
What are the initiator caspases?
2, 8, 9, 10
First line therapy for BRAF WT Melanoma
Pembrolizumab or Ipi/Nivo
What are the mandatory criteria to diagnose POEMS syndrome
Polyneuropathy and Monoclonal plasma cell proliferative disorders (almost always lambda)
What does POEMS stand for?
Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes
What are the major criteria for POEMS and how many do you need for diagnosis?
You need one. Castelman disease, sclerotic bone lesions, and VEGF elevation
Appropriate treatment for locally advanced BCC that cannot be treated with surgery and radiation
Hedgehog pathway inhibitor (vismodegib or sonidegib)
How would you treat a grade 1 or 2 metastatic endometroid endometrial carcinoma not eligible for 1st line chemo?
Sequential megestrol and tamoxifen
What is the mechanism of action and indication for tositumomab?
Monoclonal antibody against CD20 receptor. Used to treat relapsed or treatment refractory Non-Hodgkin Lymphoma (NHL)
What is the mechanism of action and indication for alemtuzumab?
Monoclonal antibody against CD52 receptor; used in management of CLL
What is the mechanism of action and indication for Brentuximab vedotin?
Monoclonal antibody against CD30 receptor. Used in treatment of Hodgkin’s lymphoma and anaplastic large cell lymphoma
How do you calculate an odds ratio?
Odds of exposure with cases divided by odds of exposure within controls
What hereditary condition is associated with an increased risk fo developing desmoid tumors?
Gardner syndrome
What are patients with Cowden syndrome at risk for?
GI hamartomas, breast/thyroid/uterine cancer
How do you define unfavorable disease for early-stage Hodgkin lymphoma according to NCCN guideline?
Bulky mass (>10cm), large mediastinal mass (MMR >.33), ESR>50, presence of any B symptoms, or >3 nodal areas
What percentage of men with high-grade PIN will develop prostate cancer within 5 years?
50%
What is the optimal treatment regimen for primary mediastinal B-cell lymphoma?
DA-EPOCH-R x 6 cycles
What measure do you use to express association between an exposure and outcome in case-control studies?
Odds Ratio
What measure do you use to express association between an exposure and outcome in cohort studies?
Relative Risk
What enzyme is missing in acute intermittent porphyria?
Porphobilinogen deaminase
What is the classic translocation seen in MALT lymphoma?
t(11;18)
What is the alteration in Glanzmann’s thromabsthenia, and what is the resulting problem?
mutation that inactivates GP2B/3A so unable to immobilize fibrinogen and vWF
what is the most common genetic alteration in liposarcoma?
12q amplification involving MDM2 and CDK4 genes
what is the mechanism of action of plerixafor?
CXCR4 antagonist which results in the release of hematopoietic stem cells from the marrow
What cell surface antigens are expressed by hematopoietic stem cells?
CD34, CD90, CD110, CD117, CD133, CD164
What is the cytogenetic abnormality present in >50% of patients with hypereosinophilic syndrome?
del(4)(q12q12): FIP1L1-PDGFRA fusion
What defines early stage Hodgkin lymphoma as unfavorable?
Bulky disease (mediastinal mass ratio >0.33 or any mass >10cm); ESR>50; Presence of B symptoms; >3 lymphoid regions
What is the most commonly reported variant RARA translocation?
t(11;17)(q23;q21.2): PLZF-RARA
What does cryoprecipitate contain?
Fibrinogen, Factor VIII, Factor III, vWF, Fibronectin
What are risk factors for local recurrence in soft tissue sarcoma?
Age >50, Recurrent disease at presentation, Positive margins, histologic subtypes (fibrosarcoma and malignant peripheral nerve tumors)
What are risk factors for distant recurrence in soft tissue sarcomas?
Tumor size >5cm, high tumor grade, deep location, recurrent disease, leiomyosarcoma
What are the 4 most prominent features in dyskeratosis congenita?
aplastic anemia, dystrophic nail changes, reticular skin changes, lingular leukoplakia
In addition to aplastic anemia, what clinical findings characterize fanconi anemia?
developmental delay, short stature, microcephaly
What is the underlying pathophysiologic dysfunction in dyskeratosis congenita?
telomeric dysfunction (abnormally short telomeres)
Which CNS tumor is characterized by 1p and 19q co-deletion?
Oligoastrocytomas
Which CNS tumor frequently has an IDH1 mutation?
low grade astrocytoma
What are the peripartum anticoagulation recommendations for patients heterozygous for Factor V Leiden mutation?
Low dose LWMH for 6 weeks after delivery if C-section or 1st degree relative with VTE under age 50
Which sarcoma histologies are at high risk for lymph node metastasis?
CARE: clear cell, angiosarcoma, rhabdomyosarcoma, epithelioid
Which platelet disorder shows normal aggregation with primary and secondary ADP and collagen, but not with ristocetin?
Bernard-Soulier (also has large platelets)
Which cyclin binds to cdk4 and 6?
Cyclin D- to signal that cell is ready to advance to S phase from G1
Which cyclin regulates progression to M phase?
Cyclin B (by binding to CKD1)
What percentage of metastatic melanoma has a BRAF mutation?
50%
What is on the US black box warning for Mitomycin C?
hemolytic uremic syndrome
What are the most common sites of distant metastases for retroperitoneal sarcoma?
Liver (most common) followed by lung
What are the clinical characteristics of Gardner syndrome?
Multiple colyn polyps, osteomas, desmoid tumors, fibromas, thyroid cancer
how do you calculate negative predictive value?
True negatives divided by total number of people testing negative
What is the mechanism of action of ramucirumab?
IgG monoclonal antibody that targets VEGFR-2
What are the two most common resistance mutations to crizotinib?
L1196M and G1269A
What are the components of the international prognostic score for Hodgkin Lymphoma?
Age >45; WBC >15, Lymphopenia (<600 or <8% of WBCs), Stage IV, Hgb <10.5, Albumin <4.0, Male
What genes are mutated in juvenile hemochromatosis?
hemojuvelin (HJV) or hepcidin (HAMP
What is the classic MRI appearance of CNS lymphoma?
“cotton wool appearance”
What is the classic MRI appearance of paragangliomas?
“salt and pepper” appearance
What percentage of patients with CNS lymphoma have ocular involvement?
15-20%
What is the lymphatic drainage of the upper 2/3 of the vagina?
pelvic nodes including internal and external iliacs, and obturator nodes
what is the lypmhatic drainage of the lower 1/3 of the vagina?
inguinofemoral nodes
Anticoagulation recommendation for pregnancy in FVL homozygous women with no personal/family hx of DVT?
Intermediate dose LMWH post-partum only
Anticoagulation recommendation for pregnancy in FVL homozygous women with personal/family hx of DVT?
Intermediate dose LMWH during pregnancy and post-partum
What are the 4 most common types of soft tissue sarcoma, in order?
1.) Undifferentiated pleomorphic sarcoma, 2.) Liposarcoma 3.) Synovial sarcoma, 4.) Leiomyosarcoma
How many lypmh nodes should be examined in gastrectomy?
15
What is the most common genetic abnormality in clear cell sarcoma?
t(12;22)
What proteins in the HPV genome act as oncoproteins?
E7 and E8 (through destruction of p53 and Rb)
What is the most common subtype of APL?
Hypergranular variant (less common is microgranular)
What medications can be used in ACTH-secreting adenomas?
Ketoconazole, metrapone, mitotane
What medications can be used in GH-secreting adenomas?
somatostatin analogs (somatostatin, octreotide)
What medications can be used for prolactinomas?
Dopamine agonists (bromocriptine, cabergoline)
What are the tumor marker cutoffs for good risk nonseminoma?
AFP <1000, B-hCG <5,000, LDH <1.5x ULN
What are the tumor markers for poor risk nonseminoma?
AFP >10,000, B-hCG >50,000, LDH >10x ULN
What is the most common mutation in NSCLC?
KRAS
Treatment for early-stage unfavorable-risk HL?
ABVD x 4 + 30gy IFRT
What are the non-taxane microtubule inhibitors?
eribulin and ixabepilone
In what setting is pertuzumab approved in the neoadjuvant setting?
Tumor >2cm, positive lymph nodes
What agent can eliminate interference of anti-CD38 targeted therapies on antibody screens?
DTT- but will destroy kell antigens
What percentage of pancreatic cancer patients present with resectable tumors at baseline?
20%
What percentage of pancreatic cancer patients present with metastatic disease at baseline?
50%
What is the ETV6-NTRK3 fusion gene relatively pathognomonic for in salivary gland tumors?
mammary analogue secretory carcinoma (MASC)
Which molecularly targeted agent would be appropriate for a patient with metastatic melanoma and an NRAS mutation?
Binimetinib
What is the flow cytometry phenotype for hairy cell leukemia?
Positive for: CD19, CD20, CD103, CD25, CD11c
What is the flow cytometry phenotype for splenic marginal zone lymphoma?
Positive for: CD20, CD79a. Negative for: CD5, CD10, CD23, CD25.
What is first-line treatment for splenic marginal zone lymphoma?
single agent rituximab
What is the most important factor associated with improved survival in patients with oligodenroglial tumors?
1p and 19q codeletion
BRAF V600E is most commonly seen in what type of low grade glioma?
pleomorphic xanthoastrocytoma
Treatment for 5q deleted MDS
Lenalidomide
In multiple myeloma, is t(4:14) good or poor risk?
poor
In multiple myeloma, is t(14:16) good or poor risk?
poor
In multiple myeloma, is t(14:20) good or poor risk?
poor
In multiple myeloma, are trisomies of odd number chromosomes good or poor risk?
good
What are poor risk cytogenetics in multiple myeloma?
17p13, t(4;14), t(14;16), t(14;20)
What cells does anaplastic thyroid carcinoma arise from?
follicular cells
What condition has normal PT, PTT, TT, with positive urea clot test?
FXIII deficiency
Is translocation 12;21 favorable or poor prognosis in ALL?
Favorable (TEL/AML1)
Is translocation 16;16 favorable or poor prognosis in AML?
Favorable- can do high dose Ara-C for chemotherapy
What are flower cells pathognomonic for?
adult t-cell lymphomas
What is the flow cytometry phenotype of adult t-cell leukemia/lymphoma?
CD2-5, CD25. Important: lacks CD7
Which subtype of vWD mimics hemophilia A?
vWD type 2N
What malignancy is t(8;14)(q24.1;q11.2); MYC-TRA associated with?
T-lymphoblastic leukemia (T-ALL); carries a poor prognosis
What is an antidote to 5-FU?
Uridine triacetate
Is the absence of MGMT promotor methylation a favorable or poor prognostic indicator in primary glioblastoma?
Poor prognostic factor
What is the platelet aggretometry results for Glanzmann thrombasthenia?
Normal aggregation with risotcetin but lack of aggregation with remaining platelet agnosits
What is the preferred screening test for porphyria cutanea tarda?
Urine or plasma porphyrins
Is wild type NPM1 and FLT3-ITD high poor risk or favorable risk in AML?
Adverse risk
What AML risk category is mutated NPM1 with FLT3-ITD High
intermediate
What AML risk category is mutated RUNX1?
Adverse
What AML risk category is mutated ASXL1?
Adverse
What AML risk category is t(6;9) DEK-NUP214?
Adverse
What AML risk category is KMT2A rearranged?
Adverse
What AML risk category is inv(3)?
Adverse
What AML risk category is t(3;3) GATA2,MECOM(EVI1)
Adverse
What AML risk category is t(8;21) RUNX1-RUNX1T1?
Favorable
What AML risk category is inv(16)?
Favorable
What AML risk category is biallelic mutated CEBPA?
Favorable
What is enasidenib?
IDH2 inhibitor
What is ivosidenib?
IDH1 inhibitor
What is the pathognmonic mutation in Waldesntroms?
MYD88
What is first-line therapy for metastatic cholangiocarinoma?
Gemcitabine/Cisplatin
What is second-line therapy for metastatic cholangiocarcinoma?
FOLFOX
Which gene is mutated in congenital gray platelet sydnrome?
NBEAL2
What is the treatment for post transfusion purpura?
IVIG 2g/kg of body weight over 2-5 days
What maintenance regimen is preferred in adverse risk multiple myeloma?
Bortezomib
What is the mechanism of action of plerixafor?
CXCR4 antagonist
Which malignancy commonly produces calcitriol?
Lymphoma
What gene is mutated in Cowden syndrome?
PTEN (hamartomatous polyps in GI tract)
What constitutes grade 1 diarrhea?
<4 bowel movements daily more than his baseline
What is the active metabolite of irinotecan?
SN-38
What does increased expression of p170 glycoprotein on cancer cells result in?
Increased drug efflux of many chemotherapeutic agents including irinotecan
Which chemokine is expressed in nearly all cases of angioimmunoblastic T cell cases?
CXCL13 (cytoplasmic)
What regimen should be considered in patients with CNS involvement of multiple myeloma?
Pomalidomide + carfilzomib + dexamethasone
What defect is present in Hermansky-Pudlak syndomre?
Absence of dense granules, most common mutation in HPS1
