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2024 Spring ITE Flashcards

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1
Q

List five subtypes of myeloproliferative neoplasms according to the 5th edition of WHO

A
  1. Chronic myeloid leukemia (CML)
  2. Polycythemia vera (PV)
  3. Essential thrombocythemia (ET)
  4. Primary myelofibrosis (PMF)
  5. Chronic neutrophilic leukemia (CNL)
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1
Q

List the three broad, major categories of myeloid neoplasms.

A
  1. Myeloproliferative neoplasms
  2. Myelodysplastic neoplasms
  3. Myelodysplastic/myeloproliferative neoplasms
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2
Q

List one genetic abnormality seen in acute myeloid leukemia with defining genetic abnormalities.

A

AML with RUNX1::RUNX1T1 fusion
AML with BCR::ABL1 fusion

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3
Q

List two most common anatomical sites involved by extramedullary hematopoiesis in primary myelofibrosis

A

Spleen
Liver

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4
Q

List two clinicopathologic variants of Langerhans cell histiocytosis (LCH).

A

Single-system LCH
Multisystem LCH

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5
Q

Subtypes of LCH

A

Eosinophilic granuloma
Letterer-Siwe disease
Hand-Schuller-Christian disease

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6
Q

Most common genetic alteration seen in LCH

A

BRAF V600E mutation

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7
Q

Other entities with BRAF V600E mutation

A

Melanoma
Papillary thyroid carcinoma
Anaplastic thyroid carcinoma
Papillary craniopharyngioma
Sessile serrated lesion
Borderline serous ovarian tumor
Hairy cell leukemia
Erdheim-Chester disease
Ameloblastoma
Metanephric adenoma

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8
Q

List three specific immunostains used for diagnosis of LCH

A

S100
CD1a
Langerin (CD207)

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9
Q

List one specific finding of LCH on electron microscopy

A

Birbeck granules

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10
Q

What is the characteristic clinical finding in IgA nephropathy?

A

Recurrent hematuria

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11
Q

What is the specific finding on immunofluorescence in IgA nephropathy?

A

Mesangial deposition of IgA antibodies within the glomeruli

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12
Q

List three histologic findings that can be seen in IgA nephropathy

A

Mesangial hypercellularity
Focal segmental endocapillary hypercellularity
Crescents
Tubular atrophy/interstitial fibrosis
Segmental sclerosis

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13
Q

What related (to IgA nephropathy) 1) clinical syndrome can occur in the pediatric population, and what 2) three extrarenal symptoms can be seen in these patients?

A

Henoch Schonlein Purpura (IgA vasculitis)

Palpable purpura
lower extremity arthralgia
abdominal pain/GI bleeding

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14
Q

List four risk factors for development of urothelial carcinoma

A

Genetic - Lynch syndrome, Costello syndrome
Aristolochic acid based herbal medicine
Radiation exposure
Schistosoma infection
Smoking
Medications: Cyclophosphamide, phenacetin

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15
Q

3 histologic variants of urothelial carcinomas

A

Micropapillary, nested, large nested, tubular, microcystic, plasmacytoid, sarcomatoid, lipid-rich, clear cell, giant cell, lymphoepithelioma-like, poorly differentiated

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16
Q

3 types of divergent differentiation of urothelial carcinomas

A

Squamous differentiation, glandular differentiation, trophoblastic differentiation, Mullerian differentiation

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17
Q

List three urothelial carcinoma variants associated with worse prognosis than conventional urothelial carcinoma

A

Micropapillary
Sarcomatoid
Clear cell
Poorly-differentiated

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18
Q

List two histologic findings on bladder biopsy/TURB that are associated with higher risk of progression in otherwise T1 urothelial carcinoma

A

High grade, pT1 stage:
1. tumor size > 30 mm
2. multifocality
3. association with CIS
4. LVI
5. variant histology (those with worse prognosis)

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19
Q

List five clinical manifestations seen in Birt-Hogg-Dube syndrome

A

Fibrofolliculomas
Trichodiscomas
Acrochordon
Pulmonary cysts
Oncocytic RCC
Chromophobe RCC

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20
Q

What gene is involved in the development of Birt-Hogg-Dube syndrome?

A

FLCN gene encoding for folliculin

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21
Q

List 1) one immunostain and 1) one histochemical stain most helpful in differentiating oncocytoma from chromophobe renal cell
carcinoma.

A

CK7 - positive in chromophobe RCC but not oncocytoma

Colloidal iron stain - diffuse/granular staining in chromophobe RCC

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22
Q

List two immunostains most helpful in differentiating oncocytoma from low-grade oncocytic tumor.

A

CK7: Low-grade oncocytic tumors typically show strong and diffuse positivity for CK7. In contrast, oncocytomas usually exhibit weak or focal staining for CK7 or may be negative.

CD117 (C-kit): Low-grade oncocytic tumors often demonstrate positive staining for CD117, whereas oncocytomas are typically negative for CD117.

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23
Q

List the three major parameters used in prognostication of gastrointestinal stromal tumors (GISTs).

A

Tumor size

Mitotic rate

Tumor location

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24
List three patterns of KIT immunoexpression that can be seen in GISTs.
1. Dot-like perinuclear staining 2. Membranous pattern 3. Cytoplasmic
25
What are the three most common molecular alterations found in GISTs?
KIT PDGFRA Wildtype GIST (SDH-deficient GIST) BRAF mutation
26
List three syndromes associated with GISTs
Neurofibromatosis type 1 (NF1) Familial GIST syndrome Carney-Stratakis syndrome Carney triad
27
List five subtypes of lung adenocarcinoma according to the fifth edition of WHO
Invasive non-mucinous adenocarcinoma of the lung Invasive mucinous adenocarcinoma of the lung Colloid adenocarcinoma of the lung Fetal adenocarcinoma of the lung Enteric-type adenocarcinoma of the lung
28
What are the two recognized precursor glandular lesions?
Atypical adenomatous hyperplasia of the lung Adenocarcinoma in situ of the lung
29
List the three genes that should be tested in ALL advanced lung adenocarcinomas as per the updated molecular testing guidelines provided by College of American Pathologists, the International Association for the Study of Lung Cancer, and the Association for Molecular Pathology
EGFR ALK ROS1
30
Provide the diagnostic criteria for diagnosis of minimally invasive lung adenocarcinoma
A small (≤ 30 mm) lepidic-predominant adenocarcinoma with an invasive component ≤ 5 mm, solitary on a resection specimen that is completely sampled
31
List the five groups constituting the WHO classification of pulmonary hypertension
Group 1: Pulmonary arterial hypertension (PAH) Group 2: PH due to left heart disease Group 3: PH due to lung diseases and/or hypoxia Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH) Group 5: PH with unclear and/or multifactorial mechanisms
32
List three clinical signs or symptoms that can be found in patients with advanced idiopathic pulmonary hypertension
Dyspnea Fatigue Syncope Chest pain Cyanosis Right ventricular hypertrophy
33
What is the most common gene mutated in familial pulmonary arterial hypertension?
Bone Morphogenetic Protein Receptor Type 2 (BMPR2 gene)
34
Name 1) one macroscopic and 2) one microscopic finding that can be seen in pulmonary hypertension
Macroscopic - Atherosclerotic plaques in larger pulmonary arteries, Right ventricular hypertrophy, pulmonary emboli Microscopic - Pulmonary arteries may have medial smooth muscle hypertrophy Intimal ("onion skin") fibrosis ± adventitial thickening Plexiform lesions
35
List five causes of myocarditis.
Viral - coxsackie virus Bacterial - Strep, Staph, Borrelia Parasitic infections Autoimmune - SLE Toxic substances - cocaine Allergic reactions Sarcoidosis Giant cell myocarditis Connective tissue disease
36
List the two histologic criteria required for diagnosis of myocarditis
Inflammatory infiltrate Myocyte necrosis/degeneration
37
Which form of myocarditis is associated with particularly bad prognosis?
Giant cell myocarditis
38
Which form of cardiomyopathy can develop as a consequence of myocarditis?
Dilated cardiomyopathy
39
Define quality assurance in pathology.
System/program that ensures the final result reported is as correct and accurate as possible and standard is met
40
Define quality control in pathology.
Tools included in every test to help detect and correct defects in the system and control the quality of the service being output
41
List three examples of quality control in pathology
Daily assessment of H&E staining External control tissue in IHC slides Verifying more than 2 patient identifiers in specimens Periodic calibration of digital slide scanners Daily checking of temperature and pH value of staining solution
42
Define proficiency testing in pathology.
Process of evaluating the results of unknown specimens to a standard test
43
List 1) the most common primary tumor of the adult heart and 2) one associated syndrome
1. Cardiac myxoma 2. Carney syndrome, McCune-Albright
44
List 1) the most common primary tumor of the pediatric heart and 2) one associated syndrome
1. Cardiac rhabdomyoma 2. Carney complex, Gorlin syndrome, tuberous sclerosis
45
Two genes associated with tuberous sclerosis
TSC1 TSC2
46
List three most common causes of metastatic tumors to the heart.
Bronchogenic carcinoma Hematologic malignancies Breast cancer Renal cell carcinoma
47
Define amyloidosis.
Inherited inflammatory disorder characterized by the deposition of insoluble protein fibrils in various tissues of the body and causing damage.
48
List two special stains that can highlight amyloid in tissue and specify the expected positive staining for each.
1. Congo red - apple green birefringence under polarized light 2. Crystal violet - metachromatic staining (purple/blue) 3. Sirius red - rose red
49
Give 1) one example of disease associated with systemic amyloidosis and 2) one example of disease associated with localized amyloidosis.
Systemic: Immunoglobulin light chain (AL) amyloidosis, multiple myeloma, monoclonal plasma cell proliferations, chronic inflammatory disorders, chronic kidney disease Localized: Primary localized cutaneous amyloidosis - Macular, Nodular, anosacral amyloidosis, Alzheimer's disease, type 2 diabetes mellitus
50
What is 1) the major fibril protein in Alzheimer disease and 2) its chemically related precursor protein?
1. beta-amyloid 2. amyloid precursor protein (APP)
51
List three indications for intraoperative evaluation of lymph nodes.
Triage for lymphoma protocol Exclusion of metastatic malignancy Exclusion of infectious process
52
List five additional techniques that can be performed on fresh lymph node tissue during intraoperative consultation
B+ fixative (different fixative) Imprints for cytogenetics RPMI Snap freeze for molecular analysis Microbiology cultures
53
List four main causes of error during intraoperative consultation
Sampling error Insufficient/inappropriate clinical history Technical errors Interpretative errors
54
Which among these is the most common cause of error during intraoperative consultation?
Sampling error Interpretive error
55
List three extramammary locations that can be involved by myofibroblastoma
inguinal/groin area, including in the vulva/vagina, perineum, and scrotum trunk/axilla extremities abdominal cavity retroperitoneum viscera
56
List the two most useful immunostains for diagnosis of myofibroblastoma.
Desmin CD34
57
List the two immunostains that can overlap between myofibroblastoma and invasive lobular carcinoma of the breast.
ER PR
58
List four entities to consider in the morphologic differential diagnosis of myofibroblastoma in the breast
Spindle cell lipoma Pseudoangiomatous stromal hyperplasia Solitary fibrous tumor Nodular fasciitis Invasive lobular carcinoma Fibromatosis
59
Define oral leukoplakia
white plaque/patch that cannot be scraped off and cannot clinically be characterized as any other disease
60
How does erythroplakia differ from leukoplakia?
Erythroplakia is a thin slightly depressed red patch on the oral mucosa
61
What is the most common predisposing factor to leukoplakia?
Tobacco
62
List three possible histologic correlates of leukoplakia
Verrucous hyperplasia Proliferative verrucous leukoplakia Squanous hyperplasia Hyperkeratosis Squamous hyerplasia / dysplasia
63
List two commonly used scoring systems for assessing PD-L1 immunohistochemical expression and the corresponding formula
Tumor proportion score = (# PDL1 positive tumor cells/total # of viable tumor cells) x 100 Combined positive score = (# of PDL1 positive tumor cells and tumor associated immune cells/total # viable tumor cells) x 100
64
For each of the two scoring systems for PDL1, list one organ system where the specific scoring system is used
TPS - NSCLC CPS - ENT (head and neck SCC), GU (urothelial), GI (gastric and esophageal ca), Gyne (cervical ca)
65
List the two normal tissues that can be used as controls for PD-L1 immunohistochemistry
Tonsil Placenta
66
What is the minimum number of cases that would be required for validation of a new PD-L1 antibody within a laboratory?
40 cases
67
Outline the seven categories in the classification of the epithelial tumors of the ovary according to 5th edition WHO.
Serous tumors Mucinous tumors Seromucinous tumors Endometroid tumors Clear cell tumors Brenner tumor Other carcinomas
68
List three precursor lesions of mucinous carcinoma of the ovary.
Mucinous borderline tumors Mature cystic teratoma Brenner tumor
69
List four pathologic features that favor ovarian involvement by metastatic carcinoma rather than a primary mucinous carcinoma of the ovary.
Bilaterality (metastatic are usually bilateral while primary are unilateral) Smaller size Surface involvement / hilar involvement Infiltrative growth pattern High grade cytology Dissecting mucin
70
List five immunostains that can be used to distinguish ovarian involvement by metastatic carcinoma versus a primary mucinous carcinoma of the ovary.
Things in the differential of mucinous neoplasm: breast, GI, ovary PAX8 CK7 CK20 CDX2 SATB2 WT1 ER PR SMAD4 GATA3
71
List the four most important points to include in a pathology report on salivary gland carcinoma.
Tumor histologic type and pattern Anatomic site of origin / location, side Tumor size Tumor histologic grade (for mucoepidermoid carcinoma, adenocarcinoma, NOS, malignant mixed tumor, adenoid cystic carcinoma) Tumor extension (intra or extraglandular), to adjacent structures Status of resection margins Vascular invasion Perineural invasion Lymph node involvement
72
Name two specific salivary gland carcinomas for which histologic grading is pathologically relevant.
Mucoepidermoid carcinoma Adenoid cystic carcinoma Carcinoma exPA Adenocarcinoma NOS
73
List two risk factors for the development of salivary gland carcinoma.
Age Sex - more common in males Radiation Tobacco chewing NOT smoking EBV Genetic predisposition Nickel exposure Chromium exposure
74
In general, what is the single most important pathologic prognostic factor for carcinoma of the head and neck?
Lymph node status
75
List three criteria of malignancy in conventional adrenocortical neoplasms in adult patients.
Weiss criteria: ≥ 3 for adrenal cortical carcinoma 1. Nuclear grade III or IV (Fuhrman) 2. > 5 mitotic figures/50 high power fields* 3. Atypical mitotic figures* 4. Clear or vacuolated cells ≤ 25% tumor 5. Diffuse architecture (> 33% of tumor) 6. Necrosis 7. Venous invasion* (of smooth muscle walled vessels) 8. Sinusoidal invasion 9. Capsular invasion *= major criteria; 1 is required to diagnose adrenal cortical carcinoma
76
List two immunostains useful in distinguishing adrenocortical adenocarcinoma from pheochromocytoma
SF1, α-inhibin - negative in pheochromocytoma Melan A - negative in pheochromocytoma Chromogranin - positive in pheochromocytoma GATA3 - positive in pheochromocytoma
77
List one adverse prognosis factor in adrenocortical adenocarcinomas
Angioinvasion Advanced tumor stage Lack of completeness of the tumor excision Tumor functionality High-mitotic tumor grade High Ki67 proliferation index
78
List five causes of adrenal insufficiency.
Primary Adrenal Insufficiency: caused by 1. Autoimmune diseases 2. Amyloidosis 3. Congenital Adrenal Hyperplasia 4. Adrenal bleeding due to trauma 5. Fungal infections Secondary adrenal insufficiency: 1. Pituitary dysfunction 2. Long-term use of high-dose corticosteroids
79
What is the definition of sudden unexpected death in epilepsy (SUDEP)?
Sudden unexpected death in epilepsy (SUDEP) is sudden, unexpected, witnessed or unwitnessed, non-traumatic and non-drowning death, occurring in benign circumstances, in individual with epilepsy, ± evidence for seizure and excluding documented status epilepticus, in which postmortem examination does not reveal cause of death
80
List five causes of death that can be seen in patients with epilepsy
SUDEP Status epilepticus Seizure-related injuries (head injury) Suicide Arrhythmia Trauma Drowning Postictal asphyxia
81
List two macroscopic findings in epileptic death.
foam cone tongue biting muscle rigidity urinary incontinence gastric aspiration
82
What is the most common histologic finding in brain in epileptic death?
normal brain

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