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T1 - Phase 2 - Systems Modules (I) > Neoplasia/Hematology - Mechanisms of Disease - Acute Leukemias; Myelodysplastic Syndromes; CML; Myeloproliferative Diseases > Flashcards

Neoplasia/Hematology - Mechanisms of Disease - Acute Leukemias; Myelodysplastic Syndromes; CML; Myeloproliferative Diseases Flashcards

1
Q

Acute leukemias can be thought of as disorders characterized by a block in leukocyte _____________.

A

Acute leukemias can be thought of as disorders characterized by a block in leukocyte differentiation.

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2
Q

Chronic leukemias can be thought of as disordered/increased leukocyte ____________.

A

Chronic leukemias can be thought of as disordered/increased leukocyte proliferation.

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3
Q

How could you easily differentiate between an acute and a chronic leukemia via a review of the peripheral blood smear?

A

Presence of >20% blasts = acute leukemia

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4
Q

AML is mostly a disease of what median age group?

A

Median: 67 years of age

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5
Q

True/False.

AML is typically characterized by concurrent leukocytosis, thrombocytosis, and variable erythrocyte levels.

A

False.

AML is typically characterized by pancytopenia with elevated blasts.

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6
Q

Is splenomegaly typically seen in AML or CML or both?

A

CML

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7
Q

True/False.

Splenomegaly and lymphadenopathy are somewhat unusual in most types of AML.

A

True.

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8
Q

The pancytopenia seen in AML means that patients may present with what sort of S/Sy?

A

Pallor/fatigue/SoB (anemia);

infection (leukopenia);

bleeding (thrombocytopenia)

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9
Q

Why is AML characterized by pancytopenia?

A

Bone marrow infiltration by immature (clonal) malignant cells.

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10
Q

The peripheral blood in AML usually shows a high number of what?

A

Blasts (>20%)

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11
Q

The bone marrow in AML is almost always ______cellular.

A

The bone marrow in AML is almost always hypercellular (filled with blasts).

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12
Q

True/False.

If you do not see Auer rods on peripheral smear, it is not AML.

A

False.

Auer rods are pathognomonic for AML; however, their absence does not rule out AML.

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13
Q

In most instances, blasts need to comprise at least ___% of white blood cells in the peripheral blood or at least ___% of bone marrow aspirate cellularity to make a diagnosis of AML.

A

In most instances, blasts need to comprise at least 20% of white blood cells in the peripheral blood or at least 20% of bone marrow aspirate cellularity to make a diagnosis of AML.

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14
Q

Patients with AML typically require what in addition to their chemotherapy?

A

Antibiotics + transfusions

(for leukopenia and thrombocytopenia)

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15
Q

Chemotherapy for AML, the mainstay of therapy, is divided into what two phases?

A
  1. An early induction phase (to establish remission)
  2. A later consolidation phase (to maintain remission)
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16
Q

What can be used in AML treatment either as consolidation or if the disease relapses?

A

Bone marrow transplantation (autologous or allogeneic)

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17
Q

AMLs with t(8;21), t(16;16), or t(15;17) have a(n) _________ prognosis.

AMLs with t(9:11) have a(n) _________ prognosis.

AMLs with no translocations have a(n) _________ prognosis.

A

AMLs with t(8;21), t(16;16), or t(15;17) have a good prognosis.

AMLs with t(9:11) have an intermediate prognosis.

AMLs with no translocations have a poor prognosis.

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18
Q

True/False.

The overall long-term survival of patients diagnosed with AML is only ~25-30%.

A

True.

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19
Q

What medication makes up an especially major portion of AML treatment?

A

Cytarabine

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20
Q

Describe the WHO classification of AML:

  1. AML with recurrent ________ abnormalities
  2. AML with ________-related changes
  3. Therapy-related myeloid neoplasms
  4. AML, not otherwise specified.
A

Describe the WHO classification of AML:

  1. AML with recurrent genetic abnormalities
  2. AML with myelodysplasia-related changes
  3. Therapy-related myeloid neoplasms
  4. AML, not otherwise specified.
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21
Q

Describe the WHO classification of AML:

  1. AML with recurrent genetic abnormalities
  2. AML with myelodysplasia-related changes
  3. ________-related myeloid neoplasms
  4. AML, not otherwise ________.
A

Describe the WHO classification of AML:

  1. AML with recurrent genetic abnormalities
  2. AML with myelodysplasia-related changes
  3. Therapy-related myeloid neoplasms
  4. AML, not otherwise specified.
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22
Q

Which form of AML is susceptible to treatment with all-trans retinoic acid (tretinoin)?

A

Acute promyelocytic leukemia

(AML subtype M3 in FAB classification)

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23
Q

Acute promyelocytic leukemia is almost always characterized by a t(15;17) translocation, resulting in fusion of what genes?

A

RARA-PML fusion protein from RARA and PML genes

(Retinoic acid receptor-alpha; promyelocytic leukemia)

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24
Q

Acute promyelocytic leukemia is almost always characterized by production of the RARA-PML fusion protein. This results from translocation of what chromosomes?

A

t(15;17)

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25
Q

What is commonly seen within the blasts seen in a peripheral smear of a patient with AML?

What is overly expressed in these cells to produce this finding?

A

Auer rods;

myeloperoxidase

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26
Q

What does the RARA-PML fusion protein of acute promyelocytic leukemia do?

A

Inhibits differentiation

(acts as a DNA regulator)

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27
Q

What is the most common leukemia seen in children?

A

ALL

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28
Q

ALL is most commonly seen in what populations?

A

Children;

the elderly

(peaks at <6 years and >60 years)

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29
Q

What are the two main subtypes of ALL?

A
  1. B-cell ALL
  2. T-cell ALL
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30
Q

Clinically, patients with ALL present identically to patients with ____ except that splenomegaly and lymphadenopathy are more common.

A

Clinically, patients with ALL present identically to patients with AML except that splenomegaly and lymphadenopathy are more common.

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31
Q

Patients with ALL present clinically identical to patients with AML except that ___________ and ___________ are more common.

A

Patients with ALL present clinically identical to patients with AML except that splenomegaly and lymphadenopathy are more common.

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32
Q

Which form of ALL is much more likely to present with anterior mediastinal masses?

A

T-cell ALL

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33
Q

True/False.

B-ALL is typically a disease of the peripheral blood +/- bone marrow blood +/- extramedullary sites: CNS, lymph nodes, spleen, liver, and testis.

A

False.

Typically a disease of the bone marrow +/- peripheral blood +/- extramedullary sites: CNS, lymph nodes, spleen, liver, and testis.

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34
Q

What are the two main WHO categories of ALL?

Which is associated with a worse prognosis (answer bolded)?

A
  1. Non-specified
  2. Recurrent genetic abnormalities (t(9;22))
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35
Q

You note that a patient has an elevated number of blasts in their bone marrow and on peripheral smear. How do you differentiate whether these are myeloblasts or lymphoblasts?

A

Immunohistochemistry

and/or

flow cytometry

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36
Q

True/False.

Pediatric cases of either ALL or AML tend to be very treatable.

A

True.

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37
Q

True/False.

Adult cases of either ALL or AML tend to be very treatable.

A

False.

Much worse prognosis than pediatric cases.

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38
Q

ALL has a predilection for sanctuary sites such as the ____, making intra________ treatment necessary.

A

ALL has a predilection for sanctuary sites such as the CNS, making intrathecal treatment necessary.

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39
Q

ALL with a t(9;22) is associated with a ________ outcome.

ALL with a t(12;21) is associated with a ________ outcome.

A

ALL with a t(9;22) is associated with a poor outcome.

ALL with a t(12;21) is associated with a good outcome.

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40
Q

Identify which of the following are mutations relevant to evaluation/prognosis/treatment of AML:

NPM1

FLT3 ITD

FLT3 TKD

IDH1

IDH2

P53

C-Kit

A

All of them

NPM1

FLT3 ITD

FLT3 TKD

IDH1

IDH2

P53

C-Kit

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41
Q

__________ syndrome is a disorder of bone marrow stem cells in which the cells do not normally differentiate into mature cells.

A

Myelodysplastic syndrome is a disorder of bone marrow stem cells in which the cells do not normally differentiate into mature cells.

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42
Q

What syndrome is characterized by defective stem cell maturation in the bone marrow and an increased risk of AML development?

A

Myelodysplastic syndrome

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43
Q

___________ myelodysplastic syndrome follows such things as chemotherapy (especially alkylating agents), radiation exposure, benzene exposure, or Fanconi anemia.

A

Secondary myelodysplastic syndrome follows such things as chemotherapy (especially alkylating agents), radiation exposure, benzene exposure, or Fanconi anemia.

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44
Q

What is the median age for diagnosis with myelodysplastic syndrome?

A

70 years

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45
Q

Most cases of myelodysplastic syndrome are __________ but shorten the affected patient’s life in the long-run via an increase in risk of infection, bleeding, and AML.

A

Most cases of myelodysplastic syndrome are asymptomatic but shorten the affected patient’s life in the long-run via an increase in risk of infection, bleeding, and AML.

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46
Q

The bone marrow in myelodysplastic syndrome is usually ______cellular; but, due to changes in hematopoiesis, the cell counts are __________ in the peripheral blood.

A

The bone marrow in myelodysplastic syndrome is usually hypercellular; but, due to changes in hematopoiesis, the cell counts are decreased in the peripheral blood.

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47
Q

A 73 year old male presents with a megaloblastic anemia refractory to B12 and/or folate administration. Orotic acid levels are normal. Analysis of the bone marrow shows ringed sideroblasts and no increase in blasts.

What is the likely diagnosis?

A

Myelodysplastic syndrome

(specifically, refractory anemia with ringed sideroblasts (RARS))

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48
Q

A 73 year old male presents with a megaloblastic anemia refractory to B12 and/or folate administration. Orotic acid levels are normal. Analysis of the bone marrow shows ringed sideroblasts and an increase in blasts.

You diagnose the patient with myelodysplastic syndrome (specifically, refractory anemia with elevated blasts (RAEB)).

Does this patient have an increased risk of AML development?

A

Yes (high-risk group).

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49
Q

What is the most common chromosomal abnormality seen in myelodsyplastic syndrome?

A

Trisomy 8

(also, monosomies of 5 or 7)

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50
Q

How is myelodysplastic syndrome typically treated?

A

Supportive care

(antibiotics and blood transfusions)

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51
Q

Myelodysplastic syndrome is characterized by ______cellular bone marrow that produce _______ cells.

Aplastic anemia is characterized by ______cellular bone marrow that produce _______ cells.

A

Myelodysplastic syndrome is characterized by hypercellular bone marrow that produce defective cells.

Aplastic anemia is characterized by hypocellular bone marrow that produce normal (albeit very few) cells.

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52
Q

________ syndrome is characterized by hypercellular bone marrow that produce defective cells.

________ anemia is characterized by hypocellular bone marrow that produce normal (albeit very few) cells.

A

Myelodysplastic syndrome is characterized by hypercellular bone marrow that produce defective cells.

Aplastic anemia is characterized by hypocellular bone marrow that produce normal (albeit very few) cells.

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53
Q

What myeloproliferative disorder mainly leads to an increase in erythrocytes?

A

Polycythemia vera

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54
Q

What myeloproliferative disorder mainly leads to an increase in platelets?

A

Essential thrombocytosis

55
Q

What myeloproliferative disorder mainly leads to an increase in granulocytes?

A

CML

56
Q

What myeloproliferative disorder mainly leads to an increase in fibrous tissue?

A

Myeloid metaplasia

57
Q

The following myeloproliferative disorder typically leads to an increase in what cell type in particular?

Polycythemia vera

A

Erythrocytes

58
Q

The following myeloproliferative disorder typically leads to an increase in what cell type in particular?

Essential thrombocytosis

A

Platelets

59
Q

The following myeloproliferative disorder typically leads to an increase in what cell type in particular?

Chronic myelogenous leukemia

A

Granulocytes

60
Q

The following myeloproliferative disorder typically leads to an increase in what substance/cell/tissue/product in particular?

Myeloid metaplasia

A

Fibrous tissue

61
Q

About ____% of patients with polycythemia vera are asymptomatic and the disorder is diagnosed by routine blood counts.

A

About 70% of patients with polycythemia vera are asymptomatic and the disorder is diagnosed by routine blood counts.

62
Q

Secondary polycythemia vera results from what example causes?

A

Tumors, hypoxia, hemoglobinopathies, etc.

63
Q

True/False.

Most patients with polycythemia vera have a shortened life expectancy, with bleeding being the most common cause of death.

A

False.

Most patients with polycythemia vera have a normal life expectancy, with thrombosis being the most common cause of death.

64
Q

A 65 year old man presents with headache, bleeding, and thrombosis. Upon examination, you note splenomegaly and pruritis. The man’s hemoglobin, hematocrit, and RBC size are all highly elevated, with some elevation in neutrophil and platelet counts.

What gene is likely mutated in this man?

A

JAK2

(polycythemia vera)

65
Q

How is polycythemia vera treated?

A

RBC reduction

(e.g. via phlebotomy, chemotherapy, etc.)

66
Q

Essential thrombocytosis is characterized by excessive proliferation of ___________ and increased numbers of circulating platelets.

A

Essential thrombocytosis is characterized by excessive proliferation of megakaryocytes and increased numbers of circulating platelets.

67
Q

True/False.

Platelet counts in essential thrombocytosis can get as high as 1 million / mm3!

A

True.

68
Q

The major complications of essential thrombocytosis are those related to either ________ or ________.

A

The major complications of essential thrombocytosis are those related to either thrombosis (due to hugely increased platelet count) or bleeding (due to defective nature of produced platelets).

69
Q

How is essential thrombocytosis treated?

A

Platelet count reduction

(e.g. cytoreductive therapy, platelet pheresis, etc.)

70
Q

CML is characterized by extreme blood _______cytosis, ____philia, and _______megaly.

A

CML is characterized by extreme blood granulocytosis, basophilia, and splenomegaly.

71
Q

CML usually results due to what mutation?

A

t(9;22)

(the Philadelphia chromosome)

72
Q

The t(9;22) seen in CML results in synthesis of what fusion product?

A

BCR-ABL

73
Q

Patients with CML may be asymptomatic or may present with symptoms due to ________________ (high WBC count impedes intravascular flow in the brain, lungs, or heart — include dizziness, delirium, shortness of breath, or blurred vision or slurred speech).

A

Patients with CML may be asymptomatic or may present with symptoms due to hyperleukocytosis (high WBC count impedes intravascular flow in the brain, lungs, or heart — include dizziness, delirium, shortness of breath, or blurred vision or slurred speech).

74
Q

Hyperleukocytosis (high WBC count impeding intravascular flow in the brain, lungs, or heart — leads to dizziness, delirium, shortness of breath, or blurred vision or slurred speech) is associated with what malignancy?

A

CML

(typically occurs when counts are ~300,000 - 800,000)

75
Q

True/False.

CML typically occurs after the 4th decade of life.

A

True.

76
Q

Arrange the following phases of CML in typical order of occurrence:

Accelerated phase

Blast crisis

Chronic phase

A
  1. Chronic phase
  2. Accelerated phase
  3. Blast crisis (culminates in acute leukemia)
77
Q

True/False.

AML often results in a culminating CML.

A

False.

CML often results in a culminating blast crisis / acute leukemia.

78
Q

True/False.

Once CML patients enter the blast crisis phase, they usually die from acute leukemia.

A

True.

79
Q

The CML progression occurs over ___ - ___ years.

Treatment of CML is directed at initial symptoms and reduction of the WBC count.

CML is potentially curable with ______________ performed before the blast crisis.

A

The CML progression occurs over 3 - 5 years.

Treatment of CML is directed at initial symptoms and reduction of the WBC count.

CML is potentially curable with bone marrow transplantation performed before the blast crisis.

80
Q

Myeloid metaplasia is characterized by ________ of the bone marrow and a resulting increase in _________ hematopoeisis.

A

Myeloid metaplasia is characterized by fibrosis of the bone marrow and a resulting increase in extramedullary hematopoeisis.

81
Q

True/False.

Myeloid metaplasia is a disorder of progressively fibrotic bone marrow associated with hepatomegaly, a lack of immature cells in the peripheral blood, and echinocytes due to increased intramedullary hematopoeisis.

A

False.

Myeloid metaplasia is a disorder of progressively fibrotic bone marrow associated with splenomegaly, an increase in immature cells in the peripheral blood, and dacrocytes due to increased extramedullary hematopoeisis.

82
Q

True/False.

Treatment options for myeloid metaplasia include chemotherapy, interferon therapy and splenectomy.

The major causes of death are infection, hemorrhage and (rarely) the development of acute leukemia.

A

True.

83
Q

What are the main symptoms of splenomegaly?

A

Early satiety,

left side abdominal pain

84
Q

Mastocytosis is associated with what mutation?

A

KIT

85
Q

What feature is most important in differentiating a malignant from a benign tumor?

A. Size

B. Mitotic rate

C. Metastasis

D. Necrosis

A

C. Metastasis

86
Q

Cancers that commonly occur in tissues that have gone through the sequence of metaplasia - dysplasia - malignancy include all except:

A. Squamous cell carcinoma of cervix

B. Squamous cell carcinoma of bladder

C. Squamous cell carcinoma of lung

D. Squamous cell carcinoma of esophagus

A

D. Squamous cell carcinoma of esophagus

87
Q

A 65-year-old hypertensive man presented complaining of right costovertebral angle pain and gross hematuria. On physical examination a mass was palpated in the right flank. During the work-up for these problems, a solitary lesion was found in his right lung. Subsequently he had a trivial accident and fractured his right femur. Can you tie together the seemingly disparate findings in this case?

A. Metastatic cancer

B. Adenomas

C. Neuroendocrine tumor

D. Hamartomas

A

A. Metastatic cancer

88
Q

A 79-year-old man was admitted to the hospital with complaint of suprapubic pain associated with hematuria. Workup for hematuria was pursued. Urine analysis showed numerous red blood cells, many neutrophils, and bacteria. Urine cytology showed hyperchromatic neoplastic cells consistent with transitional cell carcinoma. Subsequent bladder biopsy showed a high-grade transitional cell carcinoma.

All of the following statements about the above neoplasm are correct except:

A. It has been associated with p53 gene mutation.

B. It arises only in the bladder.

C. It is regarded as clonal in nature

D. It has been etiologically associated with cigarette smoking.

A

B. It arises only in the bladder.

89
Q

The process of grading and staging of cancer is an attempt to estimate the aggressiveness or degree of malignancy of a cancer. Which one of the following statements is correct?

A. Grading of cancer has greater clinical value.

B. Staging is based on the cytologic differentiation of tumor cells and the number of mitoses within the tumor.

C. Staging is based on the size of the primary lesion, its extent of spread to regional lymph nodes, and the presence or absence of metastases.

D. Grading is based on the degree of local invasion of the primary tumor.

A

C. Staging is based on the size of the primary lesion, its extent of spread to regional lymph nodes, and the presence or absence of metastases.

90
Q

All of the following oncogenic viruses are correctly paired with associated tumors except:

A. HTLV-1 – Adult T cell leukemia/lymphoma

B. Hepatitis C virus (HCV) – hepatocellular carcinoma

C. Human papilloma virus – laryngeal papillomas

D. Epstein-Barr virus – carcinoma of cervix.

A

D. Epstein-Barr virus – carcinoma of cervix.

91
Q

A 22-year-old woman went for a routine visit to her gynecologist, who identified a well delimited mass in her right ovary. The left ovary felt enlarged, but did not show signs of a discrete mass. X-ray and cat scan were done to confirm the abnormality in the right ovary. The tumor was excised and grossly it was cystic and filled with greasy tan, viscous jelly-like material containing hair. This tumor is best classified as:

A. Teratoma (dermoid)

B. Mixed tumor

C. Carcinoma

D. Sarcoma

A

A. Teratoma (dermoid)

92
Q

A 62-year-old man complained of headaches for the past 5-6 months. Lately, the pain had become intolerable. He was vomiting frequently and complained of decreased visual acuity. Physical exam revealed the presence of papilledema. Following an MRI scan, which revealed a “butterfly” cerebral mass, craniotomy was preformed. The tissue was represented by irregularly arranged atypical glial cells, which had markedly pleomorphic, hyperchromatic nuclei. Multi-lobulated nuclei with coarse irregular chromatin were also present along with prominent mitotic activity. There was palisading necrosis with angioplasia present. Based on the location, history, radiographic features and histology, this tumor is best represented as:

A. Malignant brain tumor (glioblastoma multiforme)

B. Benign

C. Metastatic carcinoma

D. Infarction

E. Low-grade glioma

A

A. Malignant brain tumor (glioblastoma multiforme)

93
Q

This 75-year-old man went for a routine medical check-up. On digital rectal examination (DRE), an unusually hard nodule was palpated in the prostate gland. Blood was drawn in a red top tube, and serum sent for prostatic-specific antigen (PSA) showed a value of 80 ng/ml (expected, 0.1-4.0 ng/ml). A biopsy was performed, followed by suprapubic prostatectomy. Which one of the following statements is not true?

A. Prostate specific antigen is a tumor marker commonly used to screen for prostate cancer.

B. The three most common cancers in men are prostate, lung and colorectum

C. There is 20% greater prevalence of cancer in African Americans.

D. Cancers that are decreasing in incidence are lung, breast, and prostate.

E. Risk factor for cancer include smoking, high fat, low fiber diet, and obesity.

A

D. Cancers that are decreasing in incidence are lung, breast, and prostate.

94
Q

A 41-year-old woman has a peripheral blood WBC count of 182,800/µL, with mature and immature myeloid cells predominating. Cytogenetic analysis of cells obtained through bone marrow aspiration reveals a t(9;22) translocation. This has resulted in formation of a hybrid gene, resulting in increased tyrosine kinase activity. Which one of the following genes was translocated from chromosome 9?

A. p53

B. Rb

C. c-abl

D. K-ras

E. NF-1

A

C. c-abl

95
Q

A rectal polyp is found by colonoscopy of a 58-year-old woman. The excised polyp histologically reveals well-differentiated glands with no stalk invasion. Which one of the following investigational research procedures best distinguishes whether the polyp represents hyperplasia of the colonic mucosa or a tubular adenoma (adenomatous polyp)?

A. Determination of clonality by pattern of X chromosome inactivation

B. Immunoperoxidase staining for cytokeratin (AE1/AE3)

C. Histochemical staining for neutral mucin

D. Flow cytometry to determine the percentage of cells in the S phase (growth fraction)

E. Immunoperoxidase staining for factor VIII and CD31

A

A. Determination of clonality by pattern of X chromosome inactivation

96
Q

At birth, a child has a single functional copy of a tumor suppressor gene. At the age of 8 years, the remaining normal allele is lost through a mutation. As a result, the ability to control the transition from G1 to the S phase of cell cycle is lost. Which one of the following neoplasms is most likely to arise by this mechanism?

A. Adenocarcinoma of colon

B. Ductal carcinoma of breast

C. Retinoblastoma

D. Cerebral glioblastoma multiforme

E. Chronic myeloid leukemia

A

C. Retinoblastoma

97
Q

An immunoperoxidase stain for the protease cathepsin D is performed on tissue from a ductal breast carcinoma in a 69-year-old woman. There is pronounced cytoplasmic expression in the tumor cells by prominent staining. The presence of this marker is most likely to predict tumor:

A. Differentiation

B. Aneuploidy

C. Angiogenesis

D. Heterogeneity

E. Invasiveness

A

E. Invasiveness

98
Q

A 62-year-old woman has a granulosa-theca cell tumor of her right ovary that produces estrogen. She subsequently develops endometrial carcinoma. The coexistence of these two tumors is an example of which one of the following?

A. Genetic susceptibility to tumorigenesis

B. A paraneoplastic syndrome

C. Tumor de-differentiation

D. Promotion of carcinogenesis

E. Mutation of a tumor suppressor gene

F. Tumor heterogeneity

A

D. Promotion of carcinogenesis

99
Q

A 29-year-old woman presents for her yearly gynecologic examination. A cervical Pap smear is obtained revealing “koilocytic atypia” characterized by perinuclear halos hyperchromasia and wrinkled nuclei. A cervical biopsy reveals invasive moderately differential squamous cell carcinoma. Molecular tests for human papillomavirus (HPV) reveals positive tumor cells. Which one of the following mechanisms of disease best explains the role of HPV in the pathogenesis of this patient’s neoplastic condition?

A. Episomal viral replication

B. Insertional mutagenesis

C. Activation of cellular oncogenes

D. Inactivation of tumor suppressor proteins

E. Enhanced transcription of telomerase gene

A

D. Inactivation of tumor suppressor proteins

100
Q

A 65-year-old woman presents with a 4-month history of increasing weight loss, and shortness of breath. She has smoked three packs of cigarettes a day for 40 years. Her past medical history is significant for emphysema and chronic bronchitis. A chest x-ray shows a 6-cm mass in her right lung. Bronchoscopy discloses obstruction of the right main stem bronchus. A biopsy reveals squamous cell carcinoma. Immunohistochemical studies of this biopsy specimen would most likely show strong expression of which one of the following tumor markers?

A. Cytokeratins

B. Synaptophysin

C. CDX-2

D. Alpha-fetoprotein

E. Calretinin

A

A. Cytokeratins

101
Q

A patient with cervical carcinoma undergoes a hysterectomy. In addition to a focus of deeply invasive carcinoma, the pathologist identifies dysplastic squamous cells occupying the entire thickness of the cervical epithelium, with no evidence of maturation. The basement membrane in these areas appears intact. Which one of the following terms best describes this cervical lesion?

A. Carcinomatosis

B. Carcinoma in situ

C. Atypical hyperplasia

D. Complex hyperplasia

E. Microinvasive carcinoma

A

B. Carcinoma in situ

102
Q

A 38-year-old man presents with a 6-month history of multiple reddish nodules on his foot. Biopsy of the nodule discloses a poorly demarcated lesion composed of fibroblasts and atypical endothelial-like cells lining vascular spaces. Further work-up identifies similar lesions in the lymph nodes and liver. The neoplastic cells contain sequences of human herpesvirus-8 (HHV-8). This patient most likely has which one of the following diseases?

A. Neurofibromatosis type I

B. Xeroderma pigmentosum

C. Li-Fraumeni syndrome

D. Acquired immunodeficiency

E. Ataxia telangiectasia

A

D. Acquired immunodeficiency

103
Q

A 62-year-old woman presents with increasing pigmentation of the skin. Physical examination shows hyperkeratosis and hyperpigmentation of the axilla, neck, flexures, and anogenital region. Endocrinologic studies reveal normal serum levels of adrenal corticosteroids, glucocorticoids, TSH, and PTH levels. If this patient’s skin pigmentation represents a paraneoplastic syndrome, the primary tumor would most likely be found in which of the following anatomic locations?

A. Pleura

B. Cervix

C. Stomach

D. Bladder

E. Esophagus

A

C. Stomach

104
Q

A 59-year-old woman presents with a 2-year history of upper truncal obesity and moderate depression. Physical examination shows hirsutism and moon facies. A CT scan of the thorax displays a hilar mass bilateral adrenal masses and multiple enlarged peri-aortic lymph nodes. A transbronchial lung biopsy reveals small-cell carcinoma. Electron microscopy of this tumor will most likely reveal which one of the following morphologic features?

A. Hyperplasia of rough endoplasmic reticulum

B. Hyperplasia of smooth endoplasmic reticulum

C. Mitochondrial calcification

D. Neuroendocrine granules

E. Myelin figures in lysosomes

A

D. Neuroendocrine granules

105
Q

A 59-year-old woman presents with a 2-year history of upper truncal obesity and moderate depression. Physical examination shows hirsutism and moon facies. A CT scan of the thorax displays a hilar mass bilateral adrenal masses and multiple enlarged peri-aortic lymph nodes. A transbronchial lung biopsy reveals small-cell carcinoma.

What antigens would the tumor cells most likely express immunohistochemically?

A. S-100, HMB-45, Melan-A

B. Cytokeratin, neuron specific enolase, chromograin, synaptophysin

C. Calretinin, cytokeratin

D. Cytokeratin, CA-125, EMA

E. EMA, CA 19.9, CEA

F. LCA, CD-20, CD79a

G. CD-31, CD-34, Factor VIII associated Antigen

H. Vimentin, Desmin, Muscle specific actin

I. LCA, CD-3

A

B. Cytokeratin, neuron specific enolase, chromograin, synaptophysin

106
Q

A 45-year-old man presents with increase urinary frequency and dribbling. He is found to have a bladder mass. At biopsy, a tumor containing high-grade cytologically malignant epithelioid cells and spindle cells is found. Both epithelioid and spindle cells express cytokeratin (AE1/AE3, and CAM 5.2) by immunoperoxidase stains. Tumor cells do not express vimentin, calretinin, desmin, muscle specific actin, CEA, prostate specific antigen, TTF-1 or myoglobin by immunoperoxidase stains. Which one of the following is the most likely diagnosis?

A. Metastatic pulmonary carcinosarcoma

B. Primary spindle cell carcinoma of bladder

C. Malignant mesothelioma involving bladder

D. Metastatic prostatic carcinoma

E. Primary leiomyosarcoma of bladder

F. Primary rhabdomyosarcoma of bladder

G. Sarcoma, not further classified

A

B. Primary spindle cell carcinoma of bladder

107
Q

A 61-year-old woman presents with hypochromic microcytic anemia and jaundice. A CT scan reveals a large (8.5 cm) hepatic mass. A liver biopsy reveals high-grade cytologically malignant epithelioid cells forming nests separated by desmoplastic stroma. The tumor cells express cytokeratin (AE1/AE3), CEA and CDX-2 by immunohistochemical stains. Tumor cells do not express TTF-1, alpha-fetoprotein, CA19.9, CD117, CA125, muscle actin, desmin, or vimentin with immunoperoxidase stains. Which of the following is the most likely diagnosis?

A. Metastatic lung carcinoma

B. Metastatic leiomyosarcoma

C. Primary hepatocellular carcinoma

D. Metastatic pancreatic carcinoma

E. Metastatic colon carcinoma

F. Primary cholangiocarcinoma

G. Metastatic gastrointestinal stromal tumor (GIST)

H. Metastatic ovarian carcinoma

A

E. Metastatic colon carcinoma

108
Q

A 48-year-old alcoholic man with a 40 pack-year history of cigarette smoking presents with a 30 lb weight loss over the past 3 months and hemoptysis. Physical exam reveals leukoplakia of oral cavity with a 0.3 cm ulcer of the tongue. A CXR reveals a single right pulmonary-hilar mass. The lung mass is biopsied and reveals a high-grade “small blue cell neoplasm” with marked mitotic activity. Tumor cells express cytokeratin (AE1/AE3, and CAM5.2), TTF-1, neuron specific enolase (NSE), chromogranin, and synaptophysin by immunohistochemical stain. Tumor cells do not express S-100, HMB-45, LCA, muscle actin, myogenin and myoglobin with immunoperoxidase stains. Which one of the following is the most likely diagnosis of this patient?

A. Pulmonary carcinoid tumor

B. Pulmonary small cell carcinoma (neuroendocrine differentiation)

C. Lymphoma

D. Metastatic melanoma

E. Embryonal rhabdomyosarcoma

F. Squamous cell carcinoma

G. Metastic follicular carcinoma of thyroid

A

B. Pulmonary small cell carcinoma (neuroendocrine differentiation)

109
Q

A 33-year-old woman presents with a large soft tissue mass of her right thigh. The mass is biopsied producing a bloody field. The biopsy reveals cytologically malignant spindle cells creating cavernous spaces filled with erythrocytes. Some spindle cells bridge across the spaces. The spindle cells express vimentin, CD31, CD34, and Factor VIII associated antigen by immunoperoxidase stains. Tumor cells do not express cytokeratin, desmin, muscle specific actin, S-100, melan-A, HMB-45, or LCA (leukocyte common antigen) by immunohistochemical stain. Which one of the following is the most likely diagnosis?

A. Spindle cell carcinoma

B. Malignant melanoma

C. Malignant nerve sheath tumor (malignant Schwannoma)

D. B-cell lymphoma

E. Leiomyosarcoma

F. Rhabdomyosarcoma

G. Angiosarcoma

H. T-cell lymphoma

A

G. Angiosarcoma

110
Q

A 42-year-old woman presents with fatigue, 15 lb. weight loss over the previous 4 months, and a soft tissue mass of her left lower back. Physical exam reveals multiple small non-tender pea-size firm cervical and axillary lymph nodes. Present in the left lower back is a 4.5 cm firm non-tender soft tissue mass. A CBC reveals a Hgb of 9 gms and hematocrit of 27% with normal indices. Tear-drop shaped erythrocytes and a single nucleated RBC and single myelocyte are found in the peripheral smear. Platelets are present in adequate numbers with increased large forms. A biopsy of the left lower back mass reveals a malignant “small blue cell neoplasm”. Tumor cells express vimentin, LCA, CD20, and CD79a. Tumor cells do not express CD3, UCHL-1, synaptophysin, chromogranin, NSE, cytokeratin, epithelial membrane antigen (EMA), muscle actin, myoglobin, myogenin, S-100, CD31, or HMB-45. Which one of the following is the most likely diagnosis?

A. B-cell lymphoma

B. Small cell carcinoma

C. Malignant melanoma

D. Rhabdomyosarcoma

E. Angiosarcoma

F. Clear cell carcinoma

G. T-cell lymphoma

H. Large cell undifferentiated carcinoma

I. Sarcoma, not further classified

A

A. B-cell lymphoma

111
Q

Which of the following is the most likely diagnosis?

  1. Hairy cell leukemia
  2. CLL/SLL
  3. CML
  4. Acute leukemia
  5. Infectious mononucleosis
A

Which of the following is the most likely diagnosis?

  1. Hairy cell leukemia
  2. CLL/SLL
  3. CML
  4. Acute leukemia
  5. Infectious mononucleosis
112
Q

What do you expect to see on bone marrow aspirate?

A

Dry tap

113
Q

What is the most likely diagnosis?

A

CML (granulocytosis, basophilia, splenomegaly)

114
Q

What is the molecular/cytogenetic abnormality in these cells?

A

t(9;22)(q34;q11)

BCR-ABL1

115
Q

Which of the following is the most likely diagnosis?

  1. CML
  2. Acute leukemia
  3. Hairy cell leukemia
  4. CLL/SLL
  5. Infectious mononucleosis
A

Which of the following is the most likely diagnosis?

  1. CML
  2. Acute leukemia
  3. Hairy cell leukemia
  4. CLL/SLL (smudge cells shown above)
  5. Infectious mononucleosis
116
Q

Which of the following is a possible complication of this disorder?

  1. Serum hyperviscosity
  2. Blast crisis
  3. Bone marrow fibrosis
  4. Tumor lysis syndrome
  5. Development of autoimmune hemolytic anemia
A

Which of the following is a possible complication of this disorder?

  1. Serum hyperviscosity
  2. Blast crisis
  3. Bone marrow fibrosis
  4. Tumor lysis syndrome
  5. Development of autoimmune hemolytic anemia
117
Q

Which of the following is the most likely diagnosis?

  1. CLL
  2. Acute leukemia
  3. Hairy cell leukemia
  4. CLL/SLL
  5. Infectious mononucleosis
A

Which of the following is the most likely diagnosis?

  1. CLL
  2. Acute leukemia
  3. Hairy cell leukemia
  4. CLL/SLL
  5. Infectious mononucleosis
118
Q

This patient later presents with DIC and is found to have a balanced translocation involving chromosomes 15 and 17. Which of the following is an effective therapy for this disorder?

  1. 5-fluorouracil
  2. Prednisone
  3. All-trans retinoic acid
  4. Fludarabine
  5. Imatinib
A

This patient later presents with DIC and is found to have a balanced translocation involving chromosomes 15 and 17. Which of the following is an effective therapy for this disorder?

  1. 5-fluorouracil
  2. Prednisone
  3. All-trans retinoic acid
  4. Fludarabine
  5. Imatinib
119
Q

Which of the following is the most likely diagnosis?

  1. Myelodysplastic syndrome
  2. CLL/SLL
  3. CML
  4. Acute leukemia
  5. Hairy cell leukemia
A

Which of the following is the most likely diagnosis?

  1. Myelodysplastic syndrome (Pseudo Pelger Huet cells — ‘dumbbell’ neutrophils)
  2. CLL/SLL
  3. CML
  4. Acute leukemia
  5. Hairy cell leukemia
120
Q

What is the LEAST likely finding in this patient’s peripheral blood and/or bone marrow?

  1. Pancytopenia
  2. Leukocytosis
  3. Hypercellular bone marrow
  4. 7% blasts in the bone marrow
  5. Complex cytogenetic abnormalities
A

What is the LEAST likely finding in this patient’s peripheral blood and/or bone marrow?

  1. Pancytopenia
  2. Leukocytosis (not common in myelodysplastic syndrome)
  3. Hypercellular bone marrow
  4. 7% blasts in the bone marrow
  5. Complex cytogenetic abnormalities
121
Q
  1. Follicular lymphoma
  2. CLL/SLL
  3. AML
  4. ALL
  5. Infectious mononucleosis
A
  1. Follicular lymphoma
  2. CLL/SLL
  3. AML
  4. ALL
  5. Infectious mononucleosis
122
Q
A

B. (check for infectious mononucleosis FIRST — bone marrow biopsy is invasive/painful/expensive)

123
Q

A. Bernard-Soulier syndrome

B. Acute promyelocytic leukemia

C. von Willebrand disease

D. Hemophilia B

E. ITP

A
  1. Bernard-Soulier syndrome (the image shows giant platelets)
124
Q
A

D.

125
Q
A

D.

126
Q
A

B. (Auer rod in myeloblast)

127
Q
A

A. (follicular pattern)

128
Q

For a follicular lymphoma….

A

A.

129
Q

The CEA marker is most strongly associated with tumors from what location?

A

The GI tract

130
Q

True/False.

Iron deficiency is associated with adenocarcinoma.

A

True.

131
Q

While both are easily larger than a RBC, which is much larger?

Hairy cells in HCL

Blasts

A

Blasts

132
Q

True/False.

Alpha-fetoprotein is associated with certain pancreatic malignancies.

A

True.

133
Q

TTF-1 is a sensitive marker for what malignancies?

A

Pulmonary and thyroid adenocarcinomas

T1 - Phase 2 - Systems Modules (I) (27 decks)

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