2024 Spring ITE Flashcards
List five subtypes of myeloproliferative neoplasms according to the 5th edition of WHO
- Chronic myeloid leukemia (CML)
- Polycythemia vera (PV)
- Essential thrombocythemia (ET)
- Primary myelofibrosis (PMF)
- Chronic neutrophilic leukemia (CNL)
List the three broad, major categories of myeloid neoplasms.
- Myeloproliferative neoplasms
- Myelodysplastic neoplasms
- Myelodysplastic/myeloproliferative neoplasms
List one genetic abnormality seen in acute myeloid leukemia with defining genetic abnormalities.
AML with RUNX1::RUNX1T1 fusion
AML with BCR::ABL1 fusion
List two most common anatomical sites involved by extramedullary hematopoiesis in primary myelofibrosis
Spleen
Liver
List two clinicopathologic variants of Langerhans cell histiocytosis (LCH).
Single-system LCH
Multisystem LCH
Subtypes of LCH
Eosinophilic granuloma
Letterer-Siwe disease
Hand-Schuller-Christian disease
Most common genetic alteration seen in LCH
BRAF V600E mutation
Other entities with BRAF V600E mutation
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
List three specific immunostains used for diagnosis of LCH
S100
CD1a
Langerin (CD207)
List one specific finding of LCH on electron microscopy
Birbeck granules
What is the characteristic clinical finding in IgA nephropathy?
Recurrent hematuria
What is the specific finding on immunofluorescence in IgA nephropathy?
Mesangial deposition of IgA antibodies within the glomeruli
List three histologic findings that can be seen in IgA nephropathy
Mesangial hypercellularity
Focal segmental endocapillary hypercellularity
Crescents
Tubular atrophy/interstitial fibrosis
Segmental sclerosis
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?
Henoch Schonlein Purpura (IgA vasculitis)
Palpable purpura
lower extremity arthralgia
abdominal pain/GI bleeding
List four risk factors for development of urothelial carcinoma
Genetic - Lynch syndrome, Costello syndrome
Aristolochic acid based herbal medicine
Radiation exposure
Schistosoma infection
Smoking
Medications: Cyclophosphamide, phenacetin
3 histologic variants of urothelial carcinomas
Micropapillary, nested, large nested, tubular, microcystic, plasmacytoid, sarcomatoid, lipid-rich, clear cell, giant cell, lymphoepithelioma-like, poorly differentiated
3 types of divergent differentiation of urothelial carcinomas
Squamous differentiation, glandular differentiation, trophoblastic differentiation, Mullerian differentiation
List three urothelial carcinoma variants associated with worse prognosis than conventional urothelial carcinoma
Micropapillary
Sarcomatoid
Clear cell
Poorly-differentiated
List two histologic findings on bladder biopsy/TURB that are associated with higher risk of progression in otherwise T1 urothelial carcinoma
High grade, pT1 stage:
1. tumor size > 30 mm
2. multifocality
3. association with CIS
4. LVI
5. variant histology (those with worse prognosis)
List five clinical manifestations seen in Birt-Hogg-Dube syndrome
Fibrofolliculomas
Trichodiscomas
Acrochordon
Pulmonary cysts
Oncocytic RCC
Chromophobe RCC
What gene is involved in the development of Birt-Hogg-Dube syndrome?
FLCN gene encoding for folliculin
List 1) one immunostain and 1) one histochemical stain most helpful in differentiating oncocytoma from chromophobe renal cell
carcinoma.
CK7 - positive in chromophobe RCC but not oncocytoma
Colloidal iron stain - diffuse/granular staining in chromophobe RCC
List two immunostains most helpful in differentiating oncocytoma from low-grade oncocytic tumor.
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.
List the three major parameters used in prognostication of gastrointestinal stromal tumors (GISTs).
Tumor size
Mitotic rate
Tumor location
List three patterns of KIT immunoexpression that can be seen in GISTs.
- Dot-like perinuclear staining
- Membranous pattern
- Cytoplasmic
What are the three most common molecular alterations found in GISTs?
KIT
PDGFRA
Wildtype GIST (SDH-deficient GIST)
BRAF mutation
List three syndromes associated with GISTs
Neurofibromatosis type 1 (NF1)
Familial GIST syndrome
Carney-Stratakis syndrome
Carney triad
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
What are the two recognized precursor glandular lesions?
Atypical adenomatous hyperplasia of the lung
Adenocarcinoma in situ of the lung
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
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
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
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