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245 Cards in this Set
- Front
- Back
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disease process associated with renal medullary carcinoma
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sickle cell trait (mostly) but also sickle cell disease
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ihc for renal medullary carcinoma
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CD10 focal, Cd7+, negative for 34 beta12 and loss of INI
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does collecting duct ca show loss of INI1?
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no
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ihc for collecting duct ca
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ck7, hmck, vimentin, ulex
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ihc for urothelial ca
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ck7+ ck20+ hmck+, p63, uroplakin ii, thrombomodulin, ck5/6 ini1 and ulex
negative for: RCC, vimentin and cd10 |
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population in which one finds rhabdoid tumors
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patients less than 1 years old
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ihc for rhabdoid tumors
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+ for keratin, ema, vimentin
- INI1, smooth muscle actin and desmin |
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what is castle?
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ca with thymus-like elements
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how does CASTLE stain with ihc
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+Ae1/ae3; negative for ck7 and ck20
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in Viral associated hemophagocytic syndrome, name a blood test that is part of the diagnostic criteria for the disease
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ferritin is high
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Another name for Rosai-Dorfman disease
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Sinus histiocytosis with massive lymphadenopathy (note: true emperipolesis in this dz)
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IHC for Rosai-Dorfman disease histiocytes
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CD1a and S100
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histologic fx of low grade intraosseous osteosarcoma
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subtle cytologic atypia, can have thick trabeculae but that fx can vary down to minute foci of osteoid; fibrous stroma that is low-moderate cellularity and collagen production
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reproducible genetic abnormality for aneurysmal bone cyst
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TRE17/USP6
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common locations for a desmoplastic fibroma
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mandible and occasionally long bones
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how to distinguish between fibrous dysplasia and a low grade intraosseous osteosarcoma
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fibrous dysplasia does not usually show cortical destruction or cytologic atypia; fibrous dysplasia is most commonly jaw, head, femur, tibia and ribs (vs. long bones for LGIO).
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fibrous dysplasia - describe histology
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curved trabeculae of woven bone (naked boen spicules) with bland fibrous component (bland spindle cells)
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low grade fibrosarcomas are genetically related to what tumor
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many are thought to be monophasic variants of synovial sarcomas
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histologic desc of low grade fibrosarcoma
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herringbone pattern with variable collagen production. no osteoid
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histo desc of glomus tumor
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monotonous cells wtih central nucleus, lightly eosinophilic cytoplasm, HPC-like vascular pattern (note: some refer to those with prominent hpc vessels as glomangiopericytoma vs glomangioma with prominent dilated veins)
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ihc for glomus tumors
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since they are modified smooth muscle cells, they are SMA+ and vimentin + (interestingly, desmin -)
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how to distinguish glomus tumor from a solitary fibrous tumor (which has HPC like vessels)
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usually the SFT show areas of hypocellular and cellularity and have spindle-shaped cells. by IHC, SFTs will be CD34+ and CD99+
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how to distinguish myopericytoma from glomus tumor
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myopericytoma has more spindled cells without distinct cell membranes, ; but be aware HPC like vessels will be present and stain for SMA and MSA.
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how to distinguish between a cellular hidradenoma from a glomus tumor
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duct-like structures usually found in hidradenoma and hidradenomas will stain for CAM5.2, EMA and CEA
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what IHC markers would you use for an angiosarcoma
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CD31 and CD34
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name 4 neoplasms of the peritoneum of mesothelial origin
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1. malignant mesothelioma, 2. multicystic mesothelioma, 3.well-differentiated papillary mesothelioma; 4. adenomatoid tumor
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name 2 neoplasms of the peritoneum of epithelial origin
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primary peritoneal serous carcinoma and primary peritoneal serous borderline tumor
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clarification of borderline serous tumor vs. benign cystadenoma
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in borderline tumors, papillary projections gradually lose their stroma support as they branch; they will ultimately be stroma-free clusters of neoplastic cells detached (hierarchical pattern of branching); cells start to lose their cytoplasm (occasionally find those with abundant eosinophilic cystoplasma- these can predominate at a site of microinvasion); nuclear atypia, mits
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how to distinguish between borderline serous tumor of ovary vs. serous carcinoma
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disorderly disposition of neoplastic glands in stroma with stromal response (usually desmoplasia)
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what histologic pattern of serous borderline tumors displays a higher frequency of bilaterality, surface involvement and invasive peritoneal implants
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micropapillary/cribriform
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how often will you expect to find microinvasion in a serous borderline tumor
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10%
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what are the criteria for calling microinvasion in a serous borderline tumor
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1. one or more invasive foci (<or= 3mm); single neoplastic cells or nests with nuclear atypia and eosinophilic cytoplasm in the stroma without endothelial lining (i.e. not LVI)
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histologic description of a high-grade serous carcinoma (thought to arise de novo, not from borderline tumors)
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obvious stromal invasion; disorderly papillae with little to no stromal support
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histologic description of a low-grade serous carcinoma
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psammoma bodies, irrgeular infiltration of small nests of tumor cells with desmoplastic/hyaline stroma
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what is a psammocarcinoma
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can arise out of ovary or peritoneum, four criteria: invasion, only mild atypia, fewer than 15 cells and psammoma bodies in at least 75% of papillae
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name 1 smooth muscle tumor of peritoneum
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leiomyomatosis peritonealis disseminata
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name at least 2 neoplastic processes of peritoneum with cells of unclear origin
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desmoplastic small round cell tumor and solitary fibrous tumor
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describe desmoplastic small round cell tumor and cytogenic alteration
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"sarcoma" with nests of small primitive cells embedded in a desmoplastic stroma. The nuclei of the primitive cells are round to oval with stippled chromatin. Cytoplasm is scant.
associated with t(11;22)(p13;q12), which generates (WT1-EWS) fusion gene |
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name five ihc markers that should stain a malignant mesothelioma
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calretinin, wt1, d240, ck7 and ck5/6
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name two useful ihc markers for endosalpingiosis
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CK7 and ER
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name two histologic features that can help distinguish between late phase of pseudomembranous colitis (caused by C. difficle) vs. late stage ischemic colitis
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in ischemic colitis, you will see fibrosis of the lamina propria and hemosiderin-laden macrophages
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buzzword for histologic appearance of amoebic colitis
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flask -shaped; amoebic trophozoities can be seen in the debris (abundant cytoplasm with eccentric nucleus)
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three histologic features of a complete mole with two additional more subtle findings for earlier cases
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1. concentric cytotrophoblastic/syncytiotrophoblastic proliferation "medusa-head", 2. hydropic villi with central cisterns; 3. increased cytologic atypia of intermediate trophoblasts within the decidua plus 1. karyorrhexis of villous mesenchymal cells and 2. cauliflower-like bulbous projections off of villi
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genetics of complete mole
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46 xx (all male; empty egg fertilized by sperm with genome duplication)
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what ihc is helpful for a complete mole and why
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p57; normally this is exclusively expressed by mom (paternally imprinted) and thus gone when only paternal copies. REMEMBER this is only in the cytotrophoblastic ring around villus and the villus mesenchymal cells (the maternal synctioblasts will be positive)
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Persistent trophoblastic disease develops in what percent of those with a complete mole and what forms can this take
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20%; persistent mole, invasive mole and choriocarcinoma
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is there a fetus in a partial mole
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can usually find some evidence of fetal life (including nrbcs)
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what does p57 look like and why
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all positive (as represents one egg plus two sperm)
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what percent of partial moles develop persistent trophoblastic disease
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really low (like <3%)
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three defining histologic features of chronic biliary diseases
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ductopenia, ductular proliferation and cholestasis
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what is caroli disease
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sporadic; mostly in adults; biliary sludge with impaired bile flow (stone formation; cholangitis)
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what is caroli syndrome
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caroli disease + congenital hepatic fibrosis; inherited
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most common heritable liver disease in children
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alpha-1 antitrypsin deficiency
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defining histologic feature of PBC
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destructive granulomatous cholangitis (florid duct lesion)
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Defining immunological abnormality in classic PBC
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95% with anti-mitochondrial antibodies
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what is INI1 and where is its loss most commonly associated with
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INI-1 (integrase interactor 1) is a tumor suppressor gene[1] that is constitutively expressed in most normal cells in the body. It is mutated resulting in loss of expression in malignant rhabdoid tumors and epithelioid sarcomas.
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what is glut1 and where is it found positive
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erythrocyte glucose transporter; infantile hemangioma
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what is kaposiform hemangioendothelioma (clinically)
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rare vascular proliferation (with features of a malformation and a neoplasm), usually a mass; upper extremity and retroperitoneum is most common; children under 2 years; treatment is surgery+/- steroids
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will infantile hemangioma regress or no
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will regress
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explain the difference between congenital hemangiomas and infantile hemangioma and describe the subtypes of congenital hemangiomas
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Congenital hemangiomas are FULLY DEVELOPED at birth, whereas the infantile hemangiomas will increase and then later regress; the congenital hemangiomas can be divided into the RICH and NICH (rapidly involuting vs. noninvoluting) groups; Glut1 negative; D240negative
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besides a mass effect, what's the big deal for a Kaposiform hemangioendothelioma
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life-threatening platelet trapping/thrombocytopenia
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name two ihc markers that might be useful in confirming rhabdomyosarcoma
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myogenin or myoD1
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name the characteristic cytogenetic finding for congenital infantile fibrosarcoma
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t(12;15) - ETV6-NTRK fusion product
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three main subtypes of malignant mesothelioma
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epithelioid, sarcomatoid and biphasic (rare variants include: lymphohistiocytoid, dediuoid and anaplastic)
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name three histochemical stains that can stain the hyaluronic acid in a mesothelioma (and reason to know this)
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musicarmine, alcian blue and PAS-D (the first two in particular can be confused with mucin from an adenoca)
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name three other cancers which can mimic the gross pattern "rind" of a malignant mesothelioma
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rarely adenoca; epithelioid vascular lesion (epithelioid hemangioendothelioma and epithelioid antiosarcoma)
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name 6 ihc markers that are usu positive in malignant mesotheliomas but negative in adenocarcinomas
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CK5/6, WT1, calretinin, thrombomodulin, D240 and HBME-1
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name 5 ihc markers that are usu postive in adenocarcinomas but negative in malignant mesotheliomas
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CEA, LEU-M1 (CD15), MOC31, BER-EP4, B72.3
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name two metastatic cancers that might be confused with mesotheliomas based on ihc
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1. ovarian serous carcinomas (WT1 is positive; MM can be CA125 positive)
2. RCC (usually negative for most adenoca markers; MM may be positive for CD10 and RCCMa) |
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name three surgically correctable causes of HTN
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pheo, renal artery stenosis and aldosterone secreting tumors
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name 5 diseases that can have pheochromocytomas
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1. MEN2a and 2. MEN2b; 3. von Recklinghausen (NF1); 4. Sturge-Weber, 5. VHL (von Hippel Lindau)
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what are the findings in Sturge-Weber patients
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portwine stain of trigeminal (CN V) nervce; pheochromocytomas
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what are the findings of MEN 1
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3 p's and a c: pituitary tumor, parathyroid (hyperplasia or adenoma), pancreatic endocrine tumors, carcinoids: BIG ONE is parathyroid
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what are the findings of MEN2a
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BIG ONE is medullary thyroid (nearly 100%), pheochromocytomas (~40%), parathyroid hyperplasia (10-15%)
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mode of inheritance for MEN2a and MEN2b
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autosomal dominant with high penetrance for 2a; either AD or sporadic for 2b
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what mutation results in MEN2a or MEN2b
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mutation in RET proto-oncogene (RET is surface receptor that is part of the TGFbeta signaling system)
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what are the findings in MEN2b
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same as MEN2a medullary thyroid (nearly 100%), pheochromocytomas (~40%), parathyroid hyperplasia (10-15%) except also (BIG ONE) mucosal neuromas (mouth, eyes and submucosa of organs) and ganglioneuromas; the parathyroid hyperplasia may be absent
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Which MEN is the most severe
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MEN2b - tends to occur early and the medullary thyroid ca can be very aggressive - onset before 10 years of age and tall lanky with blubbery lips
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what do paragangliomas secrete
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location dependent - if in adrenal medulla - mostly epi; if in paravertebral/aortic sites mostly norepi; if in head and neck, little catechol as tend to be more PNS-derived - be aware that they can secrete some other hormones (like ACTH to lead to Cushings, VIP or parathormone-like substance)
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what are the "10%"'s of pheos
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10% tumor, 10% bilateral, 10% extra-adrenal, 10% malignant, 10% in children and 10% hereditary
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name 4 specific mutations that are thought to lead to pheos
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RET (MEN2), VHL (von hippel lindau), NF1 (NF1), and SDHB (mitochondrial enzyme succinate dehydrogenase; heriditary paraganglioma syndrome)
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name 1 non-hereditary extra-adrenal paraganglioma associated disease syndrome
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Carney Triad (pulmonary chondroma, gastric malignant GIST and extra-adrenal paraganglioma)
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if pigment is seen in a pheo, what is it
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neuromelanin - waste product of catechols
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name three ihc that will stain pheos
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s/c and CD56 (NCAM)
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are malignant pheos more common or less common in the inheritable forms
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less common in heretidary forms
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what are the three highest risk indicators for malignant pheo
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large size, extra-adrenal location and SDHB mutation
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where is the most common site that paragangliomas metastasize to
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bone
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how do you distinguish between a pheo and a metastatic medullary thyroid ca
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metastatic thyroid should stain for CEA, CT and ck and lack sustentacular cells (S100+)
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how to distinguish between an adult granulosa cell tumor and a juvenile granulosa cell tumor
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juvenile GCT will have frequent luteinized cells, rare Call-Exner bodies, diffuse and nodular pattern with follicular spaces, NO GROOVES, and irregular nuclear contours
adult GCT will have GROOVES, CALL-EXNER BODIES and not usually so much follicles but rather a variety of low power patterns |
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name 8 IHC for juvenile granulosa cell tumor
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inhibin, calretinin, vimentin, keratin, CD56, WT1 (nuclear) and S100, SMA positive - note: desmin is negative, can see rare EMA in these, CEA is negative
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most important prognostic indicator in juvenile granulosa cell tumor
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staging
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what would be a stain one could use to distinguish between thecomas and juvenile granulosa cell tumors
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reticulin
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why could small cell carcinoma, hypercalcemic type of the the ovary be confused with a juvenile Granulosa cell tumor and how would you distinguish
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so the small cell carcinoma can have eosinophilic secretions, follicle-like spaces but usually is ck positive and inhibin negative and lacks thecal cells
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describe a synovial sarcoma histologically
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typically biphasic with spindle cells (sarcomatous like) and epithelioid differentiation but can be monophasic with just the spindle cells; then expect to see staghorn vessels and mast cells
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what IHC would you expect for a synovial sarcoma
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positive for EMA, CK7, CD99, BCL2 (anti-apoptosis) vimentin, TLE1 and S100
negative for CKD19, SMA, CD10 and CD34 |
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what is the characteristic cytogenetic finding for synovial sarcoma
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t(X;18) SYT and SSX (x1, x2 or x4) with x2 showing best prognosis
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which mutation is most associated with the monophasic variant of synovial sarcoma
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t(x18) with the SYT-SSX2 mutation
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describe arrangement of fasicles in leiomyosarcomas
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characteristically the fasicles intersect at right angles
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name some tumor in which CD99 is positive
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granulosa cell tumors, synovial sarcomas, meningiomas, Ewing's sarcoma, thymic tumors and hemangiopericytomas, SFT (note: hemangiopericytoma-like vessels in a lot of these)
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what IHC can be used to distinguish between and a MPNST and a synovial sarcoma
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virtually all Synovial sarcomas express CK7, CK19 or both; whereas MPNSTs lack these proteins - also CD10 positivity favors a MPNST and CD99 favors a synovial sarcoma
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what ihc can be used to distinguish between an SFT and a synovial carcoma
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SFT is CD34 + and negative for epithelial markers; synovial sarcomas are negative for CD34 and positive for epithelial markers. Both share bcl2 and CD99
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what is TLE1 and what utility is it?
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part of Wnt/Beta-catenin pathway and in correct setting, useful (positive) for identifying synovial sarcomas
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describe a borderline Brenner tumor
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Cystic appearance with papillary projections into lumen resembling a low grade papillary urothelial ca (note a malignant brenner will look like high grade ca; if there is no benign or borerline component, better termed urothelial ca)
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ihc for brenner tumors
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positive for keratins, EMA, CEA and CK7, special stains for glycogen are positive; reactivity for uroplakin II, thrombomodulin and CK20; negative for p16, p53
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pathognomonic czomal alteration for extraskeletal myxoid chondrosarcoma
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9q22 rearrangement resulting in a NR4A3 fusion gene product
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if you get a MPNST, what syndrome should you think of
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NF1
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ihc for a MPNST
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positive for S100, cd57, myelin basic protein adn p53 (BUT S100 SHOULD BE ONLY FOCAL - if more than focal, think cellular schwannoma, which usu lacks atypia and mit act seen in MPNST)
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cytogenetics for clear cell sarcoma
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t(12;22)(q13;q12) resulting in EWS/ATF1 gene fusion
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what are warthin-finkeldey giant cells
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cells of T-cell origin, giant cells with often >50 nuclei (syncticial), can be seen in kimura lymphadenopathy (rich in eos), measles
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what is angiolymphoid hyperplasia with eosinophilia and who does it affect
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mostly Caucasians, females, seen in soft tissues adn superficial dermis forming clusters of eosinophilic cells; cutaneous papules (not usu masses), nonnodal collections
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what pancreatic cyst is associated with von Hippel Lindau
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microcystic serous cystadenoma
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where are mucinous cystic neoplasms usu found within pancreas
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tail
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name three tumors where you might find thrombomodulin
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urothelial, thyroid and mesothelioma
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alternate name for NF1
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von Recklinghausen's disease
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most common sx of NF1 (4)
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cafe au lait spots, Lisch nodules (colored nodules on iris), freckles in unusual places and neurofibromas
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what percentage of MPNST are s100+
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50%
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ihc for clear cell sarcoma
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melanocytic differentiation so S100+, HMB45+
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cytogenetic alteration in clear cell sarcoma
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t(12;22)(q13;q12) for EW/ATF1 gene fusion
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clinical picture for patient with clear cell sarcoma
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rare tumor that affects extremitites in young adults; often associated with tendons or aponeuroses and presetns as slow growing mass
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IHC for synovial sarcoma
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cytokeratins (AE1, CK7 and CK19), EMA, CD99. bcl-2
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cytogenetic hallmark of synovial sarcoma
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t(X;18)(p11;q11) resulting in SS18/SSX gene fusion
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In a serous cystadenoma of the pancreass what histochemical stain would be useful
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PAS but recall that it would be staining glycogen so thus be diastase sensitive
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featurse of a lymphoepithelial cyst
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squamous lined and surrounded by dense lymphoid tissue
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describe clinical and histologic features of mucinous cystic neoplasms
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almost all females, don't communicate with duct system, cysts lined by mucinous cells that have no true papillae, involve tail and surrounded by a dense cellular ovarian type stroma
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describe histologic features of a chordoid meningioma
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epithelioid (polygonal with abundant cytoplasm and vacuoles) and spindle cells forming trabeculae and cords with a background of mucinous stroma. can have lymphoplasmacytic infiltrate (can mimic chordoid glioma but that will be GFAP+)
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ihc of chordoid meningioma (3)
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EMA, vimentin and D240
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If a sarcoma of the uterus displays benign glands, what do you call it
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Mullerian adenosarcoma (often glands are in phyllodes pattern)
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Malignant cells of a mullerian adenosarcoma demonstrate what IHC pattern (5)
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myogenin, MyoD1, SMA, desmin and HHF35 (note: mullerian adenosarcomas can frequently have a rhabdomyosarcoma appearance; hence the positive myogenin and myoD1)
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how would myoD1, myogenin, SMA and desmin stain in a leiomyosarcoma
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the more skeletal muscle markers (myoD1, myogenin) would be negative, the more smooth muscle markers SMA and desmin would be positive
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what is the clinical features and course of angiomyofibroblastoma
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slow-growing, subcutaneous mass of vulva/vagina of reproductive age women. upon excision, it's cured
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histologic fx of angiomyofibroblastoma
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alternating hypercellular and hypocellular stroma (with wavy collagen fibers) with a prominent vascular pattern. Spindle shaped cells with bipolar eosinophilic processes, tending to cluster around blood vessels.
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ihc of angiomyofibroblastoma (2 pos; four neg)
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stromal cells are vimentin and desmin + (negative for MSA (HHF35), S100, CD34 and CK)
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compare histologic features of angiomyofibroblastoma to an aggressive angiomyxoma
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an aggressive angiomyxoma is less cellular, fewer vessels than an angiomyofibroblastoma and a more prominent myxoid stroma. The vessels are different too - the aggressive angiomyxoma tends to have thickwalled vessels with prominent hyalinization
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ihc for an aggressive angiomyxoma (2)
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positve for MSA +/- desmin
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histologic features of cellular angiofibroma (and critical ihc for distinguishing from angiomyofibroblastoma)
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bland spindle-shaped cells with medium to small blood vessels (desmin negative)
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what are the primary features that distinguish a low grade phyllodes tumor from a high grade phylloides tumor
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high grade phyllodes tumors tend to have stromal overgrowth (with areas where epithelium can be hard to find); marked nuclear atypia and significant mitotic activity (just be aware that heterologous differentiation - ex bone, fat - can be found in both low and high grade PTs) - four major featurs are: tumor size, mitotic activity, stromal nuclear atypia and marginal behavior (circum or not)
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what syndrome is myxoid fibroadenomas associated with
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Carney's syndrome
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lactoferrin increase in GI tract is suggestive of what
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IBD
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serologic finding in Gaucher's disease
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elevated serum acid phosphatase
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gold standard assay for identifying Gaucher's disease
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detection of insufficent beta-glucosidase activity (celaving glucose from ceramide) in wbcs or fibroblasts
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what enzymatic deficiency is seen in Niemann-Pick disease
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deficiency in phingomyelinase (increased sphingomyelin in macrophage cytoplasm)
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what is the enzyme deficiency in Tay-Sachs
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hexosaminidase A with accumulation of GM2 ganglioside in hear, liver, CNS and spleen
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at what age do patients with Tay-sach usually die
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fatal by 2-3 years
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enzyme deficiency in Pompe disease
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acid maltase deficiency with glycogen accumulation in liver, muscle (skeletal and heart)
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useful histochemical stain for detecting Gaucher cells
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Prussion blue iron stain
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category under which sclerosing stromal tumors of the ovary should be placed
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sex cord stromal tumor
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age at which sclerosing stromal tumors of the ovary occurs
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young woman
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microscopic description for sclerosing stromal tumors of the ovary
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pseudolobulated low power view (high cellularity mixed with low cellularity areas); collagenous-type bands, prominent vascular network (gaping irregular nucli); tumor cells with scanty, eosinophilic cytoplasm, spindled to round with occasional vacuolated cells (lipid)
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ihc for sclerosing stromal tumors of the ovary (4)
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calretinin, SMA, vimentin, ER, PR; note: inhibin is variable and epithelial markers are negative
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age range for Brenner tumors
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older (more like 60's or higher)
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cell of origin in medullary carcinoma of the thyroid
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neural creast derived C-cells
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hormone produced by medullary carcinoma of the thyroid
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calcitonin
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patients with familial medullary carcinoma of the thyroid have what gene mutation
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ret (czome 10)
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besides familial medullary carcinoma of the thyroid, what syndromes are medullary carcinoma of the thyroid associated with
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MEN2B (infant, young child) and MEN2A (presents in adolescences)
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three key ihc for a paraganglioma (1 pos, 2 neg)
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Positive for synaptophysin, negative CT and TTF1
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ihc for medullary ca of thyroid
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CT, CEA, C/s, TTF1 and low molecular weight ck
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what is the most common malignant liver tumor in kids
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hepatoblastoma
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name three syndromes with association with hepatoblastoma
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beckwith-wiedemann, FAP and Down
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serology associated with hepatoblastoma (1)
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AFP
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if there is a mesenchymal component to hepatoblastoma (ie. a mixed epithelial-mesenchyrmal pattern), what does form does it take histologically
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immature fibrous tissue, osteoid and/or cartilage; can more rarely have teratoid features with a wide range of tissue types
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ihc for hepatoblastoma (3 plus 3 other categories)
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glypican-3, glutamine synthease expression (in epithelial component)
nuclear beta-catenin (embryonal mostly) hepatocellular markers (AFP, Hep Par1 and pCEA) hepatic keratins (8 and 18) and biliary keratins (7 and 19) can be variably present focal neuroendocrine |
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what subtype of hepatoblastoma has the best prognosis
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pure fetal
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factors assocaited with a bad outcome for hepatoblastoma
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age < 1yr, lg tumor size, involvement of vital structures and certain histologic subtypes (small cell, macrotrabecular variants)
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ihc for undifferentiated (embryonal) sarcoma (2)
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vimentin and bcl-2 (also focal for keratin, desmin and alpha1antitrypsin)
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what histologic subtypes of pulmonary adenocarcinomas can not be considered lepidic growth patterns (4)
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acinar, papillary, micropapillary and solid
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name three sites in which one would find a colloid ca
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colon, breast, lugn
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ihc for a pure dysgerminoma (3 positive, 1 neg)
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PLAP, OCT4 and KIT, negative for pan-CK
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typical age of diagnosis for a dysgerminoma
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adolescent or young women
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what is the male counterpart to a dysgerminoma and thus what is the common czomal alteration
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seminoma; czome 12p abnormalities
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name four serum markers that may be elevated in a dysgerminomas
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bHCG, LDH, CA125, NSE
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name two germ cell tumors in ovary in which one would find OCT4 expression
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dysgerminoma and embryonal carcinoma
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main czomal alteration in dysgerminoma and a secondary pathway in which this can evolve
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czome 12p and presence of Y czomal material leading to a gonadoblastoma pathway that leads to dysgerminoma
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what serum marker is almost always associated with yolk sac tumors
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AFP
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name two IHC markers for yolk sac tumors
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AFP and glypican-3
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what is the primary ihc marker that can distinguish an embryonal ca from a dysgerminoma; name 4 that can be expressed in both
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embryonal ca are CK+, dysgerminomas are CK negative;
OCT4, CD30, PLAP and CD117 (last is less so) can be expressed in both |
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is a dysgerminoma more often pure or mixed
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more likely pure
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what is the most common set of sx for juvenile granulosa cell tumors
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menstrual irregularities or precocious puberty
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what does the ihc marker s100 stain
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a family of ca+binding proteins important to intracellualr ca metabolism
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poor prognostic factors for malignant melanoma include (13)
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increased age, male, non-Caucasian, increasing Breslow thickness, ulceration, mits, volume of tumor, satellite deposits, LVI, advanced clinical stage, occult mets, local recurrence
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three ihc positive in Merkel cell
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c, s and CD56
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ihc association with Merkel cell
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perinuclear dot-like positivity with CK20
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typical location for an atypical fibroxanthoma
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head and neck of elderly
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what population does immunoproliferative small intestinal disease (IPSID) occur in
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unique form of extranodal marginal zone lymphoma (MALT) in young adult males of Middle East and Mediterranean descent, presenting with chronic diarrhea/weight loss
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histologic features of acetaminophen hepatotoxicity
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necrosis (most prominently zone 3), little inflammatory response, congestion
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three geographic locations in which gastric carcinoma is higher
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Japan, South America and Eastern Europe
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germline mutation in gastric ca (hereditary diffuse gastric carcinoma)
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CDH1 gene; autosomal dominant
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name four syndromes with an increased risk of gastric ca
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1. hereditary diffuse gastric ca
2. Peutz-Jeghers 3. HNPCC 4. JP syndrome NOTE: NOT FAP |
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alternate name for serous borderline tumors
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Serous neoplasm of low malignant potential
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do areas of microinvasion in serous neoplasms of low malignant potential bump it up to serous carcinoma
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Not necessarily, if the areas are less than 3mm, no.
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ihc for serous neoplasms of low malignant potential
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CK7, EMA, WT1 (nuclear), ER and often Ca125
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criteria for low grade serous carcinoma of the ovary
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destructive stromal invasion: mild to moderate atypia, up to 12 mits/10 hpg
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criteria for high grade serous carcinoma of the ovary
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destructive stromal invasion; pleomorphic nuclei, >12 mits/10hpf
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retiform Sertoli-Leydig cell tumors are so named b/c they look like what
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rete testis
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name two Ihc markers positive in Sertoli-Leydig tumors
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WT1 positive (similar in that respect to serous neoplasm of low malignant potential) and inhibin positive (unlike serous neoplasms)
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useful markers in gyn: wt1 (2 tumors)
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most serous ca positive; small cell carcinoma of hypercalcemic-type are also positive
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useful markers in gyn: p53 (3)
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most high grade serous positive; clear cell can be positive too and so can high grade endometroid
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useful markers in gyn: p16 (1)
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positive in most high grade serous ca
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useful markers in gyn: hnf1beta (1)
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clear cell ca
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useful markers in gyn: ER (2-3)
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most endometroid are positive, most clear cell are negative; serous can be positive
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useful markers in gyn: main CK7/20 profile for gyn
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CK7+, CK20-
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useful markers in gyn: CD56
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sensitive (not specific) marker for sex-cord stromal tumors
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useful markers in gyn: ST1
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positive in ovarian sex cord stromal tumors
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three ihc markers great for sex-cord stromal tumors
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calretinin, inhibin, CD56 (NCAM; last is also found in neuroendocrine tumors)
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why would we order an EMA in a sex-cord stromal tumor
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usually negative (so to rule out); only one that can have positivity is the juvenile granulosa cell tumor
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useful markers in gyn: panel for comparing endometrioid vs. endocervical (4)
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ER (positive in EM), vimentin (EM), p16 (endocervical), mCEA (endocervical)
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useful markers in gyn: panel for comparing endometrioid to serous
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ER (EM), p53 (favors serous), p16 (serous) - but IHC is more problematic in higher grade tumors
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how to categorize endometrioid ca based off of risk factors, prognosis, etc
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Type 1 - majority, estrogen dependent, low grade, more often sporadic/tamoxifen, though some HNPCC
Type 2 - higher grades, more syndromic: HNPCC, PTEN, MSI, p53, bad histologic types (serous and clear) |
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spectrum of neuroblastic tumors (3)
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most mature to least mature: ganglioneuroma, ganglioneuroblastoma (intermixed and nodular types), neuroblastoma
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important mutation that can be found in neuroblastic tumors (prognostic and treatment stratification)
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MYCN amplification (2p) - unfavorable prognosis
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unfavorable prognostic factors
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MYCH amplification (2p), 1p, 14q, 11q deletions or 17q gain
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three cell types in neurofibroma
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neural cells, fibrocytes and collagen
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spindle cells of a spindle cell thymoma stain for what ihc (2)
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AE1/AE3, CK19
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what three clinical conditions can be associated with thymomas
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MG, hypogammaglobulinemia, erythroid hypoplasia
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most critical prognostic indicator in a thymoma
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sign of invasion (so look hard for capsular invasion)
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name 5 ihc markers for synovial sarcoma
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keratin, EMA, Vimentin, bcl-2, calponin
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characteristic translocation in synovial sarcoma
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t(X;18)
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what mitotic rate is important in a leiomyoma vs. a leiomyosarcoma
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0-5 mits/50 hpf - considered better for leiomyoma
1-4/10 hpf probably better considered malignant |
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two ihc markers for GISTs
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ckit, CD34 (60-70%)
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site in which fibrosarcomas are found
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extremities
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ihc for fibrosarcomas (3)
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vimentin, focally and weakly positive for MSA and SMA (neg for ck)
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ihc for synovial sarcoma (4)
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Ck, EMA, CD99 (60-70%), bcl2
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what syndrome does one see MPNSTs
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NF1
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where do MPNSTs occur
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in association with major nerve trunks: sciatic, brachial and sacral
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ihc (1) for MPNST
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S100 (other neural markers vary)
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which tumor group has a better prognosis and why between Type 1 and 2 papillary renal cell carcinoma
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Type 1 tend to be smaller, lower stage and lower nuclear grade than 2
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what czomal alterations would one find in a renal cortical adenoma and what is thsi similar to
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trisomy 7, 17 and loss of Y, similar to papillary RCC
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what are the criteria for diagnosing a renal cortical adenoma (3)
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Less than 5 mm, papillary or tubal architecture adn low grade nuclear fx
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what single ihc can help delineate papillary rcc from a convential clear cell rcc that may have papillary or tubulopapillary architecture
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CK7 is positive in papillary and negative in conventional rcc
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what histochemical stain is helpful for distinguishing between chromophobe RCC and papillary RCC
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Hale's colloidal iron is positive in chromophobe and negative in papillary RCC
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what three ihc markers can help distinguish between papillary u/e ca vs. papillary rcc
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papillary rcc is RCC+, CD10+CK20-; u/e is RCC-, CD10-, CK20+
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what four markers can help distinguish papillary RCC from collecting duct carcinoma
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CD10 and RCC are negative in collecting duct ca but positive in papillary RCC; peanut lectin and ulex are positive in collecting duct but negative in papillary rcc.
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rank order the prognosis of papillary rcc, conventional rcc and chromophobe rcc
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conventional rcc<papillary rcc=chrombophobe rcc
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is tumor necrosis associated with a worse prognosis in papillary rcc
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no - should be the same
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what does HAM56 stain
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macrophages/microglial cells
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what is the characterisitic cytogenetic finding in myxoid liposarcomas
|
t(12;16)(q13;p11), fusion of CHOP on 12 with FUS on 16. (less common 12:22 or ins(12:16))
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what syndrome are myxomas associated with
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McCune-Albright syndrome
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what are the clinical fx of McCune-Albright syndrome
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polyostotic fibrous dysplasia, endocrinopathies, precocious puberty, cafe au lait spots and multiple myxomas
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what three histochemical stains can be positive in a myxoid lipoma
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Oil red O (positive in lipomatous areas)
PAS-A and alcian blue for mucoid areas |
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clinical setting for a myxoid chondrosarcoma
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M>F; 5th and 6th decades, deep seated muscles of extremities
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composition of myxoid matrix in a myxoid chondrosarcoma
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condroitin sulfate
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only IHC consistently positive in a myxoid chondrosarcoma
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vimentin
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cytogenetic abnormality in a myxoid liposarcoma
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t(9;22)(EWS and NOR1)
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clinical setting for mixed epithelial and stromal tumor of kidney
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Essentially ONLY perimenopausal women with history of prolonged estrogen use (note: ovarian-like stroma present invariably)
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ihc markers for mixed epithelial and stromal tumor of kidney
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stromal cells are: SMA, desmin, ER, PR and CD10
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what is thought to be on the same morphologic spectrum as a mixed epithelial and stromal tumor of kidney
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cystic nephroma
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