AP Flashcards
Carney Complex
Abnormal skin pigmentation
Atrial/cutaneous myxomas
endocrine abnormalities
asst w large cell calcifying sertoli cell tumor

Atrial myxoma
most common primary tumor of heart
may show heterologous elements/hematopoieisis
R atrial tumors (familial)
L atrrial tumors (sporadic)
+calretitin, CD34, CD31
PRKAR1 mx

idiopathic giant cell myocarditis
severe fatal disorder affecting young healthy adults
asociated with autoimmune diseases
presentation: congestive heart failure,necrosis, mixedinfiltrate, giant cells WITHOUT granulomas

cardiac sarcoidosis
necrosis uncommon
whites = blacks
hypertrophic cardiomyopathy
mx in genes encoding sarcomeric proteins
Beta myosin heacy chain

Dilated cardiomyopathy
ass’t with beta myosin heavy chain mx, and DMD mx (dystrophin)
Hereditary forms mostly AD
Chagas disease
trypanasoma cruzi
infection of myocytes
Reduviid bug (kissing bug)
C shaped organism with undulating membranes
Aschoff bodies
collections of plump macrophages and lymphocytes and plasma cells in Rheumatic heart disease
RHD:most common cause of mitral stenosis, caused by Strep pyogenes (GAS) beta hemolytic

Amyloid
Primary: plasma cell dyscrasia
Secondary: inflammation
age related systemic senile amyloid:transthyretin
Metachromatic w/crystalviolet. other stains:congored, toluidine blue, and thioflavine
Congo red sections should be thicc(10um)
vascular amyloid usually causeshemorrhage (notthrombosis)
most common types in lung: AL, AA, transthyretin
Papillary fibroelastoma
Benign
primary cardiac yumor affecting valves (aortic)
similar appearance to Lambl’s excrescenses
EVG highlighs elastic component

intramural cardiac myofibroma
hamartomatous. usually single. may have calcs/cysticdegeneration
myofibroblastic origin
See in GORLIN syndrome
PTC mx
+vimentin, SMA, - S100, desmin, myoD
Gorlin syndrome
nevoid basal cell carcinoma syndrome
low frequency of intramural cardiac myofibromas
PTC mx
9q22.3
Eosinophilic myocarditis
Ddx: hypersensitivity myocarditis (abx antiD, antiC)
Hypereosinophilic syndrome
allergy
Parasitic infection
hematologic makignancy
cardiac allograft vasculopathy
Main cause of death in long term transplant
itimal thickening
inflammatory infiltrate with or w/out myocyte damage
Quilty effect: dense endocardial lymphocytic infiltrate. NO ADVERSE prognostic
cardiac rhabdomyoma
multiple well circumscribed tumor nodules
large vacuolated cells
MOST common cardiac tumor in kids
50% of kids with tuberous sclerosis
non-invasive, non metastasizing, may regress
+desmin, vimentin,actin, myoglobin
tuberous sclerosis
mutations in TSC1 (hamartin)
and TSC2 (tuberin)
may have angiomyolipomas
hereditary hemochromatosis
HFE gene mx C282Y
Alkaptonuria
Blue/blackpigmentation in tissues/urine
due to increased homogentisic acid
stages of myovcadial infarction
<12 hours No microscopic change
24 hours; coagulative necrosis with wavu fibers (elongated/narrow). some neutrophils in spaces
3-4 days Dense neuts
7-10 days removal of dead myocytes by M0
10-14 days granulation tissue, hyperemic
>14 days, fibrous scar
HACEK organisms
Infective endocarditis
Haemophilus aphrophilus
Actinobacilus actinomycetemcomitans
Cadiobacterium hominis
Eikenella corrodens
Kingella kingii
Found in culture negative endocarditis
Although Staph epi most common on prosthetic valves
Mitral valve prolapse
myxomatous degeneration of mitral valve
Goodpasture’s disease
anti GBM disease
can also be seen in small vessel (ANCA) vasculitis
Histology: crescentic glomerulonephritis with linear IgG and C’ on basement mmb
Ab against proteinase 3 (cANCA) or myeloperoxidase (pANCA)
limited to ffecting glomerularand alveolar caps (not small arteries)
linearIgG can be seen in antiGBM, fibrillary glomerulonephropathy, and diabetic glomerulosclerosis

ANCA vasculitis
see livedo reticularis, net-line pattern of red-blue skin discoloration
pulmonary renal syndrome
Wegener’s granuomatosis
(granulomatosis with polyangitis if you aren’t a Nazi)
nose sinuses throat, lungs, kidneys
cough +blood in urine
granulomatous necrotizing inflammation of lungs/sinuses
target is cANCA
Giant cell arteritis
large and medium muscular arteries, granulomatous ANCA negative fibrinoid necrosis uncommmon
moprphologically identical to Takayasu (young Asian women)
antiphospholipid syndrome
affects veins and arteries throughtout the body
Takayasu arteritis
transmural granulomatous inflammation of vessel walls
Diabetic glomerulosclerosis
Kimmelsteil Wilson nodules
intramural hyaline deposits

Renal hypertensive changes
intimal fibroelastosis
paucity of inflammation
chemotherapeutic renal toxicity
Tacrolimus, cyclosporine
nodular hyaline deposits in arteriolar walls can lead to TTP/HUS thrombotic micoangiopathy
Pulmonary renal syndrome
most common cause: ANCA vasculitis
malignant HTN
Arteriolar fibrinoid necrosis
thrombotic microangopathies

fibromuscular dysplasia
most common cause of renovascular HTN in young people
polyarteritis nodosum
ANCA NEGATIVE
ANCA
+may be seen in autoimmune disorders
small vessel vasculitides
Wegeners: lungs, nasals, kidneys
Churgg Strauss: eosinophlia + asthma
microscopic polyangitis: lack of both
Diff on clinical features
Cryoglobulinemia inducedvasculitis
shows eosinophilic hyalinematerial/thrombi in vessel lumens
alveolar proteinosis
eosinophilic frother material with cholesterol clefts. patients asymptomatic with bilateral ground-glass, sharply demarcated opacities. tx with whol lung lavage, may spontaneously resolve
malignant mesothelioma
+calretinin, WT1, CK5/6
- B72.3 BEREP4, leuM (CD15). CEA
EM: long slender microvilli (short microvillicharacteristic ofadenoca)
primary pulmonary HTN
abnormal vessels with neolmen formation and compled intimal proliferation
Female>male
familial: BMPRII mx

pulmonary infarct
usually hemorrhagic
hemoptysis, pleuritis chest pain, SOB
may be due to PEs
centrilobular emphysema
smoking results in imbalance between peroteases and antiproteases. damage to bronchiole
panacinar emphysema
ass’t with alpha 1 antitripsin def
pulmonary squamous metaplasia
ass’t with bromnchiectasis
not usualy ass’t with PE b/c large airways do not receive majority of blood supply from pulmonary arteries
Kartagener’s syndrome
defects in ciliary structure
pulmonoary sarcoid
transbronchial biopsy yield fairly high
4-6 neg bx have strong NPV
granulomas-lymphatic routes
may have identical pattern to beryliosis and aluminum exposure
sclerosing hemangiom
benign lung tumor
misnomer (not an endothelial tumor)
papilary like projection lined by epithelioid cells with central proliferation of mesenchymal appearing cells
type II pneumocyte origin TTF1+

Giant cell interstitial PNA
bronchiocentric inflammatory pattern
aka hard metal pneumoconiosis
intraalveolar giantcells
cystic appearing lung
Ddx LCH
emphysema
fibrosis
lymphangioleiomyomatosis (see in TSC2 TS)
+SMA, HMB45
pulmonary andenovirus
see smudge cells
may also see HSV like inclusions
Asbstosis
Ferruginous bodies typical but not specific
fibrosis and pleural plaques
increased risk of bronchogenic carcinoma and malignant mesothelialioma
rounded atelectasis: see adhesions between visceral and parietal plaques

chronic eosinophilic PNA
fever, weight loss night sweats dyspnea
organizing PNA with eos
may be idiopathic or ass’t with CHurgg Strauss
DOES NOT result interstitial fibrosis
pulmonary meningothelial like nodule
+ EMA and vimentin, - CD34
round or spindle cells with whorls
discrete small lesion
pulmonary LCH
smokers M0 w Langerhans cells in alveolar spaces. strong link to smoking. nodular infiltrates with rnaging cellularity from very cellular to fibrotic areas with stellate shape and traction emphysema Birbeck granules are not lysosomal
+CD1a., langerin
-CD45

pulmonary adenocarcinoma in situ
lepidic growth pattern
may be multifocal and milateral
mucinous or nonmucinous
may be+ CK7/CK20
-TTF1
organizing PNA
intraluminal proliferation of fibroblasts and myofibroblasts in bronchiole, alveolar ducts, and sacs.
Asst with BOOP/COP
extrinsic allergic alveolitis
combination of lymphocyte bronchiolitis,mildinterstitial lymphocytic inflammation, scattered poorly formed non necrotizing granulomas
Hypersensitivity pneumonitis caused by exposure to inhaled substances
may lead to lung fibrosis if chronic
silo fillers disease
toxic exposure to nitrous oxide
may cause ARDS
not hypersensitivity pneumonitis
lymphocytic interstitial PNA
may be seen in AIDS
sjogren syndrome
autoimmune
meds
congenital cystic adenomatoid malformation
congenital pulmonary airway malformation
normal vasculature
pulmonary intralobular sequestration
most often acquired
lower lobe, may resemble normal lung
pulmonary extralobar sequestration
aberrant arterialvessel, lower lobe, congenital
malakoplakia
Michaelis guttman bodies
basophlicinclusions in foamy histiocytes
contain calcium and IRON
ass’t with AIDS
asst with rhodococcus equi infection
(bladder pic)

Disorders with lymphatic patterns in lung
sarcoid
lymphoma
Kaposi sarcoma
lymphangitis carcinoma
histolytic processes
legionella PNA
dx w Dieterle stain
short bacillary organism
neutrophils and M0 but not granulomas
tx with macrolides
atypical cacinoid
2-10 mits/HPF
mitotic activitynd necrosis predict LN mets and survival
pulmonary capillary hemangiomatosis
rare cause of pulm HTN
abnormal nodular capillary proliferations along alveolar walls that can compress veins
Diffuse panbronchiolitis
chronic bronchiolitis causing cough and sputum production
sinusitis:mostly japanese
interstitial foamy M0 in respiratory bronchioles
alveolar adenoma
likely fibroblast origin
nodular cystic proliferation with epithelial lining of cysts and mesencymal cell proliferations in intercystic areas
WD fetal type adenocarcinoma
females
young
glands look like secretory endometrium
congenital lobar emphysema
overinflation of lung d/t partialobstruction of the bronchus
architecture is normal but inflated
Giant bullous emphysema
residual lung parenchyma resembles chorionic villi
aka placental transmogrification

pediatric interstitial pulmonary emphysema
result of high pressure ventilation
giant cell reaction from air being forced into interstitium
silicosis
fibrotic lung disease
acute- proteinosis like reaction
predispose to real bad TB

hermansky-pudlack syndrome
AR
albunism
ceroid filled histiocytes.pulmonary fibrosis, coagulation defects
alveolar microlithiasis
alveolar spaces filled with lamellated calcium phosphate microliths

methrotrexate toxicity
NSIPpattern interstitial PNA
beryliosis
sarcoid likepattern ofgranulomatous injury
scleroderma
may show UIP or NSIP pattern of intersitialPNA
small cell carcinoma
may lack synaptophysin/chromogranin staining
ttf1+
staging 3 categories, limited and extensive
lymphomatoid granulomatosis
true granulomas not a feature
pneumocystis carinii
foamy intraalveolar exudated
silver stain

esophageal squamous cell carcinoma
risk factors: alcohol, tobacco, food with nitrosamines, burning bevs, Plumer vinson syndrome, achalasia
plummer vinson syndrome
dysphagia with esophageal webs, iron def, glossitis
pediatric eosinophillic esophagitis
usually have normal esophageal pH, abnormal allergen reactivity test
enterochromaffin like cel hyperplasia
ass’t with atrophic autoimmune gastritis limited togastric fundus/body.
achlorohydria b/c loss of PARIETAL cells. hypergastrinemia (G cells compensate for decreased HCl)
pernicious anemia
+ab against parietal cells and intrinsic factor
if >0.5 mm or invades submucosa, then classify as carcinoid

fundic gland polyp
not ass’t with atrophic gastritis
not asst with HP
ASST w/ PPIs
more common than adenomatous polyps
may seehysplasia in pts w FAP
inflammatory fibroid polyp
benign
most common in antrum/small intestine
bland spindle cells
small bloodvessels
inflammatory cells in edematous/myxoid background
+CD34,vimentin
-CD117
Peutz Jeghers
Dx criteria: at least 3 polyps, any polyps in pt w FH, prominent mucocutaneous pigmentation (freckle lips), any polyps with pigmentation
AD
polyps show distinctive arborizing pattern of smooth muscle
LKB1(STK11) gene mx
increased risk of adenocarcinoma, breast and pancreas ca, and sex cord stromal tumors
GIST
most common in stomahc
+CD117, DOG1, S100
if CD117 -, then PDGFR mx
-Desmin
sensitive to imatinib
Carney triad
GIST
pulmnonary chondroma
extra-adrenal paragangliioma
Glomus tumor
Benign
uniform cells with round nuclei
cytoplasmic eosinophlilia
delicate vasculature, lackof mitoses
+SMA
=Desmin, S100, CD117 chromogranin
may have bleeding

GI kaposi
+CD31, LANA (HHV8)
focal PAS+ hyaline bodiesin endothelial cells
bland spindle cell proliferation with slit like spaces with RBCs
HPgastritis
bacteria produce urease
urea–> ammonia and CO2
increase gastric/duodenal ulcers
increaseds gastric adenoca and extranodal marginal zone lymphoma
reactive gastropathy
foveolar hyperplasia (corkscrew glands)
fibromuscular hyperplasia or LP
vascularcongestion
minimal inflammation
asst w NSAIDs, alcohol, chemo, smoking, uremia, stress, bile reflux
celiac
ab to antigliadin, antireticulin, antiendomysial, anti tissue transglutaminase
(lasttwomost specific)
increased prevalence in DOWN and DIABETES
most common presentation iron def anemia
HLADQ2 or DQ8
Carcinoid
ileal more aggressive than duodenal
generally >1 cm
MEN1 ass’t w duodenal carcinoids
carcinoid sundrome inc flushing, diarrhea
rectal carcinoids usually small (>1 cm) found incidentally good prognosis
NOT asst w carcinoid syndrome
+NSE, PAP,
-PSA
cystic lymphangioma
seen in head/neck of children
can also be in GI tract
endothelial cells express CD31 and factor VIII related Ag
mucinous cystadenoma of appendix
need to sample thoroughly to exclude cystadenocarcinoma
may be asst w ovarian mets or pseudomyxoma peritoneii
pseudolipomatosis
complication of endoscopy
vacuolar spaces aretrapped air
pneumatosis intestinalis
NOT asst with barium enema
asst with infection amnd PUD
collagenous collitis
elderly woman eith watery diarrhea
asst w celiac/other autoimmune
collagenbandcontains tenascin
basallamina over allagen is normal
increased LP eos, trapped capillaries, increased intraep lymphs in supepithelial collagen layer

post inflammatory polyposis
diffuse post inflammatory polypsasst with IBD.
Elongated filiform and branching with core ofMM
FAP
mx in APC
negative regulatory of WNT pathway
Gardner syndrome
FAP, osteomas, epidermal cysts and fibromas
Turcot’s syndrome
coexistance of hereditary polypsos with CNS tumors
Hereditary nonpolyposis colorectal cancer
asst with microsatellite instability
HNPCC if MSI+ not typically BRAF
If BRAF in MSI+ then sporadic
has infiltrating lymphs
proximal poorly differentiated or mucinous adenocarcinomas
schwannoma
most common in stomach, can occur in colon
+S100
- Desmin, CD117, HMB45
oftem rimmedby lymphoidcuff
plexiformschwannoma asst w NF1/2

extramammary paget’s
presents in anogenital region as slowly spreading eczematoid plaque causing pruritis
may be asst w malignancy
+CK7 EMA CEA mucin
-S100
Ddx is Pagetoid Bowen and melanoma

solitary rectal ulcer syndrome
asst w mucosal prolapse. no increased risk of carcinoma. multiple polyp types. usually present w rectal bleeding
muscularization of LP, LP fibrosis, hyperplasia of MM
juvenile polyposis syndrome
usually solitary when not syndromic
multiplepolyps in GI tract
SMAD4/DPC4 gene mx
increased risk CRC, stomach ca, billiary tract ca,pancreatic ca
colonic acute GVHD
grade 1: seeapoptotic bodies
then crypt necrosis if more severe
lymphomatoid polyposis
asst with mantle cell lymphoma
usually affects colon at IC valve
aggressive lymphoma
cowden syndrome
lossof function in PTEN (tumor suppressor) leads to increased activity ofMTOR
microcephaly, igingival hyperplasia, intestinal hanartomas
benign skin tumors,
Lhermitte Duclosdisease: with dysplasticgangliocytoma of cerebellum
small intestine adenomas
usually occur in ampulla of vater
granular cell tumor
distal esophagus
+PAS S100
usually small
Meckel
most common congenital abnormality of GI tract
1-% of population
persistance ov proximal vitelline duct
50% have heterotopic elements
usually gastric, sometimes panc
menetrier’s disease
marked foveolar hyperplasia in gastric body w hypoproteinemia d/t protein losing enteropathy
endoscopic resection
considered adequate for polyp as long as NO:
HG
LVSI
extension to stalk margin
Intraductal papillary mucinous neoplasm
mean age 68
M>F
30% of invasive carcinoma

PANIN1-2
(low grade)
women(>50% of population over50)
pancreaticobililary carcinoma
+MUC1 and MUC5AC
most common source of carcinoma of unknown primary
pancreatitis/smoking are risj factors
increased CA19-9 and CA125
ass’t w BRCA
pancreatic mucinous cysticneoplasm
ovarian stroma, body or tail of pancreas
most occur in perimenopausal females
20-30% have carcinoma
serous cystadenoma of pancreas
benign. glycogen rich neoplasm originates from centroacinar cells. may be ass’t with VHL abnormalities
islet cell hyperplasia
ass’t with chronic pancreatitis
colloid pancreatic carcinoma
mucinous lakes with scanty carcinoma cells floating
large, betterprognosis thattypical ductal carcinoma
sugar tumor
type of perivascular epithelioid tumor (PEComa)
granular cytoplasm NO papillary architecture
nesidioblastosis
enlarged pancreatic islets with disproportionately higher amountsof B cells. can occur in male infants of diabetic mothers. ass’t with persistent hypoglycemia
islet amyloid deposition
Type 2 DM NOT type 1
hereditary pancreatitis
AD
ass’t w mutation in catiomic trypsinogen gene PR551
serine protease inhibitor Kanal type 1 SPINK1
liver acute rejection
T cell predominant immune infiltrate
cile duct damage endothelitis
portal>central
autoimmune hepatitis
periportal interface hepatitis with abundant plasma cells in clusters and regenerative rosettes. antinuclear (type1) antismooth muscle ab
Type 2: antiLKM
Type 3: antiSLA
Hepatic bile duct adenoma
medium sized regular bile duct structures embedded in well formed fibrous stroma aggregataed lymphocytes at interface within liver

von neyenburg complex
bile duct malformation
dilated, irregular bile duct structures (ductal plate malformation)
may contain intraluminal bile or mucinous secretion

bile ductual cholestasis
pathognomonic for sepsis in adults
usually caused by GNR like E coli
Focal nodular hyperplasia
central scar with arterial malformation and stelate radiating fibrous septa

chronic cholestasis
mallory bodies
accumulation of copper binding protein
pseudoxanthomatous change
PSC
pANCA
ass’t with UC
fibroobliterative lesions rounded scars appearing in portal areas at sites of former bile ducts

extrahepatic biliary atresia
need to see ductular reaction
mallory bodies
contain ubiquinated CK8/18
DO NOT contain organelles
post transplant CMV
may show intracellular inclusions and/or microabscesses
Primary biliary cholangitis
granuloma formation around bile ducts
antmitochondrial ab
middle aged females

budd chiari
sinusoidal dilation/congestion
perivascular fibrosis without significant portal change
macrovesicular steatosis
obesity, chronic HCV
steroids, manourishment
DM, EtOH, hyperlipidemia
biliary cystadenofibroma
resemble fibroadenomas ofthe breast
microvesicular steatosis
small vacuoles-reflects serious damage to mitochondria and B peroxidation of Fatty acids
acute fatty liver of pregnancy
Reyes syndrome
tetracyline toxicity
alcoholic foamy degeneration
valproic acid toxicity
Hemochromatosis
HFE
iron accumulation in Kupffer cells is late in disease
also portal fibrosis/cirrhosis
Hepatic sarcoid
portal/periportal fibrosis ass’t with portal based non caseating granulomas
liver cell adenoma
ass’t with oral contraceptives
thickened hepatocyte plates with interspersed venous and arterial channels
bile ducts absent

cholangiocarcinoma
KRAS
cmyc mx
sequalae of PSC
alagille syndrome
biliary tract disorders
ass’t w jagged 1 mx
fetal epithelial type hepatoblastoma
ass’t w extramedullary hematopoeisis
MOST COMMON liver tumor of childhood

HCC
polyclonal CEA surface staining
HepPAR 1/AFP cytoplasmic
precursor is small cellchange
subtypes: microtrabecular, adenoid, clear cell, and giant cell
LIver transplant ischemia repurfusion injury
centriolobular baloonning injury near cenrral veins withmild cholestasis
liver humoral allograft rejection
usually portal based
ferrous sulfate hepatic effect
periportal hepatic necrosis
paraquat toxicity
bileduct epithelial injury
acetaminophen toxicity
centrilobular necrosis
gn recurrent intrahepatic cholestasis
ass’t with mx in FIC-1 (ABCBII)
bile transport protein
portal tract eos ddx
PBC
acute rejection
drug toxicity
victoria blue orcein stains
demonstrate increased copper binding proteins such as biliary tract disease PSC/PBC or Wilsons
also stains HBV surface Ag
Bile ducts infarct
due to large bile duct obstruction periportal hepatocyte necrosis
absence of ductular reaction and portal edema
mild neurtophilic and lymphocytic portal infiltrate
Caroli’s disease
dilation ofintrahepatic bile ducts ass’t with polycystic kidney disease
Type I glycogen storage
plant like hepatocyte mmbs
diversion colitis
occurs in segments of colon surgically isolated fromfecal stream
colonic mucosa becomes susceptible to lack of nutrients (eg short chain fatty acids)
May resemble UC
Recovery with restoration of fecalflow
puchitis caused by bacterial overgrowth
hirschprung
most commonly involves rectum/sigmoid distention of segment proximal to agangionic part
male>female
RET gene/endothelin B receptor
IgG4 ass’t pancreatitis
ass’t with UC PSC Sjogrens
pancreatic acinar hyperplasia
+ trypsin
pancreatic ductal adenocarcinoma
may developacute DM
possible endocrine tumor? (this ddx would be more slow growing, not ass’t w jaundice or back pain)
KRAS mx in>90%
osteoclastic type giantcell carcinoma
sarcomatoid carcinoma withabundant osteockast type giant cells
+CD68 (not part of malignant clone)
asst with mucinous cystic tumor or ductal adenocarcinoma
pancreatic cyst fluid analysis
used to differentiate between pseudocyst (increased enzymes) and mucinous cystic neoplasm (Increased CEA/mucin)
serous microcystic cystadenoma
characteristic sponge like appearance well circumscirbed with central stellate scar
no mucin (even though shiny on gross)
benign but large
pancreatic solid pseudopapillary tumor
+ B catenin
most common in females, young
- CEA (r/o mucinous cystic tumor),chromogranin, keratins
degenerative cyst lining
may mets to liver or peritoneum but doesn’t affect prognosis
HISTO: cytologically bland cells with round oval nuclei. ependymoma like appearance nuclear grooves fomay M0 hynaline globules, cystic change, +vimentin CD56, PR, CD10, A1antitrypsin, NSE
insulinoma
small, usually detected early b/c symptomatic
glucagonoma
may be large/met at presentation
pancreatic endocrine tumors
grade dependent on mits/necrosis
acinar cell carcinoma
+trypsin
sheets of large round cells with granular cytoplasm and central prominent nucleolus
secretion of enzymes ass’t with polyarthralgia and subcutaneous fat necrosis
pancreatoblastoma
+ B catenin
sheets of round cells and scattered squamoid corpuscles
(meningothelial like whorls)
nuclear pseudoinclusions
early childhood/Asians
increased AFP
Beckwidth Wiedemann/AFP
Glomus tumor
benign cells with round nuclei surrounding bloodvessels
glomus cells derived from smooth musscle
glomus body: structure locatedat natural ateriovenous anastomoses
see in distal digits
stomach is most common extracutaenous site

fibrosarcoma
herringbone pattern
deeplower extremity
uniform spindled basophilic cells

osteochondroma
most common benign bone tumor
condrocytes frequently found incolums when approaching bone.
young people
surface lesion arising from bone continuous with medullary cavity
found in ditsal femur/proxhumerus/tubia
cartilage cap lined by perichondrium, continuous w mature bone
chondroblastoma
uniform cells with prominent borders and chicken wire calcifications
eosinophilic cytoplasm, nuclear grooves
almost always benign
epiphyseal plate, distal femur proximalhumerus
+s100
may have secondayr ABC

chondromyxoid fibroma
lobules ofchondroid and myxoid tissues separated by bands of connective tissue
periphery hasspindled-stellate cells with variablegiantcells
metaphysis of distal femur or proximal tibia

Dedifferentiated chondrosarcoma
can occurfrom dedifferentiation of other tumors such as enchondroma
older pts
proximal bones
malignant chondrocytes next to dediff sarcoma

conventional osteosarcoma
malignant spindle cells producing osteoid

giant cell tumor of bone
found around the knee
epiphiseal
numerous giant cells
uniform distribution of osteoclast like giat cells, stromal cells composed of m0 and mesenchymal cells
mets may happen with bland histo.
tumor in vessels near margins does not indicate mets

Hand Schuller Christian Disease
LCH multifocal unisystem
Letterer Siwe disease
multifocal mutisystem
atypical lipomatous tumor
aka WD liposarcoma
elongated ring chromosome 12
MDM2 amp
mature fat w atypicalcells in fibrous areas

Ollier’s disease
ass’t w enchondromas
Mafucci’s disease
ass’t with hemangiomas and spindle cell hemangioma
alveolar soft part sarcoma
noncohesive polygonal cells separated by fibrous/vascular septa
+PAS crystals in cyytoplasm
+TFE3
Deep ST head/neck
t(X;17) TFE/ASPL

Paget disease of bone
aka osteitis deformans
osteoclastic phase with bone resorption
osteoblastic phase with bone formation: increased woven bone with prominent cement lines
chalkstick fractures
increased alk phos, nlCa/Phosphorus

solitary fibrous tumor
aka hemangiopericytoma
patternless pattern with bands of thick collagen
+CD34 STAT6 CD99
invasion/attypical features upgrade to malignant SFT

clear cell sarcoma
nests of cells which may contain melanin
aka melanoma ofsoft parts
pleomorphic, mitotically active
rhabdoid morphology
+melanoma markers
T(12;22)(q13;12) ATF EWS

synovial sarcoma
may be monophasic, biphasic, or undifferentiated
(X;18)(p11q11)
SYT-SSX1 SSX2
+TLE1 CK7
- CD34
spindle and epithelioid cells
deep seated near knee

alveolar rhabdomyosarcoma
worst prognosis or all the rhabdomyosarcs
+MYO-D1
periorbital, kids
PAX7 mx t(1,13) better than PAX3 mx (t(2;13)

PARosteal oteosarcoma
LG neoplasm
found on back of knee (posterior distal femur)
immature trabeculae without osteoblastic rimming
collagenous background
may have cartilage cap, may look like fibrous dysplasia

pleomorphic/spindle cell lipoma
mature fat with atypical cells in fibrous areas
ropy collagen
head/neck/shoulders
old dudes

low grade osteosarcoma
rare
old ppl
goodprognosis
long bones
similar to parosteal osteosarcoma
NO osteoblasticrimming
spindled cells collagenous background

myxoid liposarcoma
t(12;16) DDIT CHOP FUS

liver transplantationsepsis
hepatocellular/bile canalicular cholestasis or bile ductular cholestasis
stellate cell
may transform into myofibroblast likecells producingcollagen and staining for SMA
overlap syndrome
overlap of PBC or pSC with autoimmune hepatitis
antinuclear/antismooth muscle ab in pt with PSC orPBC
alcoholic liver disease
see mega mitochondria
also seein in NASH
visualize with chromotrope aniline blue stain
schistosomiasis
ova in portal vein branches
get “pipestem” fibrosis

liver disease in pre-E
fibrin within protalvessels and periportal sinusoids
pregnancy related liver disease
acute fatty liver microvesicular intrahepatic cholestasis of pregnancy
HELLP nonspecific inflamation, glycogenated nuclei, periportal fibrosis, necrosis
fibrolamellarHCC
young pt without typical risk factors for cirrhosis

Qfever
coxiella burnetii
fibrin ring granulomas
central clear space or vacuolealso seen in Hep A infection, allopurinol hypersensitivity, hodgkin

liver GVHD from all HSCT
targets hepaticbile ducts
expressHLA II ag
endotheliitis is UNCOMMON (morecommon in acute rejection)
epithelioid hemangioendothelioma
sclerosis, signet ring type cells, predeliction for occlusion of vein branches
may be confusedwith steatohepatitis or desmoplastic adenocarcinomas
female>male
+CD31/CD34
-CK7/CK20
parenteral nutrition
see cholestasic ductular reaction, fibrosis
pleomorphic adenoma
aka Benign mixed tumor
solidcordsof cells
trabecular structures
tubules +/- small cysts, foci of stromal hyanlinization, myxoid chondroid areas
look for double cell layer in tubular structures

salivary gland myoepithelioma
+CK SMA
monomorphic neoplasm of myoepithelioid cells

basal cell adenoma
trabecular structures lined by prominent basal cell layer
overlaps withmonomorphic adenoma and PA
warthin tumor
male, >50, smoker, multifocal

polymorphouslow grsde adenocarcinoma
tumor cells uniform and bland
low mitotic activity
invasive glands with 2 cell layers should not be present

salivary gland adenoid cystic carcnoma
cribriform glands
spaces not lined by basal cell layer
most likely to show perineural invasion
painful

sal gland mucoepidermoid carcinoma
MOST COMMON malignant salivary gland tumor (overall including parotid)
goblet cells and tumor nests are large

acinic cell carcinoma
see PAS+ granules (helps differentiate from oncocytomas which don’t)

salivary duct carcninoma
uncommon
similar to duct carcinomas ofbreast
large nests are not in situ,they are invasive
+AR GCDFP-15 Her2-neu PSA PAP
-WT1

sal gland lymphoepithelial cysts
common in HIV
minor salivary gland neoplasms
most common site oral cavity and palate
major sal gland neoplasms
parotoid gland most common
then submandibular
then sublingual
forbenign and malignant
sal gland tumors
60-70% BENIGN
mostcommon is PA
canalicular adenomas
thought to arise from luminal duct cells
lack myoeps
+CK SMA
-p63
mixed malignant tumors/carcinoma ex pleomorphic adenoma
1-3 decades older than ppl w BMT(PA)
collapsing focal segmental glomerulosclerosis
may have focal IgM and C3 deposition by IF
loss of foot processes
but no immune complexes by EM
most common form in HIV glomerulonephropathy

lupus nephritis patterns
ass’t with subensothelial and mesangial deposits
Class II: mesangial proliferative LN
Class III: focal proliferative LN
ClassIV: global endocapillary proliferation
Class V: membranous LN
thrombotic microangiopathies
seemucoid intimal edema of small arteries
Ddx includes DIC HUS scleroderma antiphospholipid syndrome, pre-E
minimal change disease
most children present with fullnephrotic syndrome with preserved renal function, nbl serum compliment, NO hematuria
highly responsive to steroids
calcineurin inhibitor therapy toxicity
renal acute ab mediated rejection
C4d IF staining in peritubular capillaries
crescentic glomerulonephritis
granular/immune complex disease
eg SLE membranoproliferative glomerulosclerosis
post infectious GN IgA nephropathy
Goodpastures
90% +ANCA
ANCA mediated neutrophil degranulation into endothelial surfaces
membranous nephropathy
subepithelial depositis “spike and dome”
secondary forms dueto SLE RA drugs infections malignancy
NOT seen in Wegenegers
may have renal vein thrombosis as complication (bc hypercoagulation)
2nd most common cause of nephrotic syndorme
GRANULAR IgG and C3
myeloma cast nephropathy
most commonform of renal involvement in patients with myeloma
ARF usual presentation
ethylene glycol toxicity
oxalate crystals
light chain deposition disease
asst withnephrotic syndrome, renal insufficiency, and nodular glomerulosclerosis similar to amyloidosis
extracellular myxoid chondrosarcoma
small malignant cells in myxoid stroma
9;22 CHN EWS

myxoid liposarcoma
t(12;16) DDIT CHOP FUS

intramedullary chrodrosarcoma
most common type of chondroarcoma
chondroid matrix
middle age
deep fibromatosis
Beta catenin mx
IHC for B catenin stains NUCLEI
clear cell chondrosarcoma
clear cells and hyaline cartilage
may have MGCs

enchondroma
benign lobular cartilaginous tumorsfoundin SMALLbones
asst with Ollier’sdisease
Hypocellular, few binucleated cells, may be multifocal but does not infiltrate marrow
No myxoid change

non ossifying fibroma
spindled stromal cells in storiform pattern
fibrosis, foamy m0, giant cells
NO trabeculae (to distinguish from fibrous dysplasia)
If<3 cm and linmited to cortex, then call it a metaphyseal fibrous defect
benign lesion in children
No tx necessary

dermatofibroma
FXiiia+
-CD34,B catenin
acanthosis basal hyperpigmentation
peripheral collagenballs

DFSP
spindle cells invading ST
+CD34
- FXiiia
t (17;22) fusing collageb type 1 and PDGF B gene
Ddx dermatofibroma which is neg CD34, +FXiiia)
nodular fasciitis
reactive process
RAPID GROWTH
wavy collagen fibers
+SMA, calponin
may have numerous atypical mits
MYH9-USP6
tissue culture appearance, volar forearm, extravasated RBCs
pseudoepitheliomatous hyperplasia
asst with granular cell tumor
spitz nevi
blastomycosis
syphlis
TB
trauma
Pigmented vilonodular sinovitis
results from increased proliferation of synovium
+CD68
seehemosiderin stained MGCs
pigmented foam cells
highly vascular
rosai dorfman disease
sinus histiocytosis w massive LAD
histiocytes w emperipolesis (ingestion ofcells)
and mature lymphs
+s100 CD68
- CD1a

angiolipoma
mature adipocutes and prominent vascular network with fibrin thrombi

leiomyosarcoma
malignant spindle cells
+SMA
slower growing than nodular fasciitis
poor prognostic factors: age >60, HG, retro, deep, size>5cm, incomplete excision
Ewing sarcoma
SRBCT
+NSE synapto, CD99,PAS
t(11;22) Fli-1 EWS
second most common sarcoma of bone/ST inkids
diaphyseal, long bones/pelvix, necrosis common
onion layering, motheaten on rads

angiosarcoma
spindled to epithelioid cells with primitive vascular channels
+CD31 CD34 FViii Fli1 thrombomodulin
skin/ST tumor
osteoid osteoma/osteoblastoma
trabeculae composed of woven bone and osteoid RIMMED BY OSTEOBLASTS
vascular cellular stroma
OO: <1.5 cm oftenpainful but relievable
OB: >2.0cm: often painful, not relievable
epiphyseal gone tumors
giant cell tumor
chondroblastoma
clear cell chondrosarcoma
fibrous dysplasia
chinese letters traveculae
monostotic/polyostotic
craniofacial bones ribs metaphysis
McCune Albright (polyostotic, cafe au lait spots,endocrine disorders)

malignant peripheral nerve sheath tumor
MPNST fascicles of pleomorphic hyperchromatic spindle cells with increasednumbers of mitotic figures
asst w nerve
- S100, often w NF1, SMA
granular cell tumor
oral cavity,most commonextra oralsite is skin
+s100 NSE sudan black
asst w pseudoepitheliomatous hyperplasia

plexiform histiocytic tumor
+SMA cimentin CD68
young ppl
derm/subQ fat
female
plexiform or nodular proliferation of fibrocytic cells and giant cells in a background of chronic inflammatory cells separated by fibroustissue
tumors WITH osteoblastic rimming
osteoblastoma
osteoid osteoma
neuroblastoma
N myc
secretes catacholamines but isn’t symptomatic
tumors WITHOUT osteoblastic rimming
fibrous dysplasia
LG osteosarcoma
parosteal osteosarcoma
ganglioneuroma
arises along sympathetic chain
eg posterior mediastinum
+s100 NF GFAP synapto chromo
-CK
ST tumor mets
most sarcomas met hematogemnously
exceptions: rhabdo, epithelioid sarcoma, angiosarc, synovoal sarc clear cell sarc (LN)
conventional chondrosarcoma
more common in males30s
medulla of proximal bones like pelvis shoulder
NOT responsive to chemo/rads
multiple non reciprocal translocations and 1p rearrangements
mesenchymal chondrosarcoma
mandible pelvis ribs
small blue cells differentiated cartilage
biphasic immature mesenchymal cells and WD cartilage

periosteal osteosarcoma
intermediate HG tumor
proximal femur/tibia
spindled stroma
lobules of cartilage with central osteoid production
merkel cell carcinoma
CK20+ dot like pattern

juxta articular myxoma
well circomscribed adjacentto large joints esp knee
adenomatoid tumor
most common tumor of spermatic cord and epididymus
benign. won’t mets
+calretinin
inverted papiloma ofbladder
underlying stroma contains papillary structures with fibrovascular cores each w intact BM

spermatocytic tumor
3distinct cell populations
lacks fibrous septa
older doods
bilateral

sertoli cell tumor
tubules lined by epithelial cells
may also be difuse/trabecular
do notprodice hormones

nephrogenic adenoma
benign
papillae and tubules lined by benign cells withclear cytoplasm. frequently have hobnailing
+CK7, CK20, racemase, HMWCK, PSA
- p63

chromophobe RCC
vegetable like cell borders
sheet or alveolar pattern
+CK7 CD117 Hales coloidal iron
- vimentin

metanephric adenoma
benign cortical renal tumor
unencapsulated
tubules and papillae with benign cells and psmammoma bodies
+WT1 and CD57
- EMA CK7

angiomyolipoma
epithelioidto rhabdoid cells
thick walledvessels
scattered adipose
PEComa
TSC2 mx tuberous sclerosis
+SMA and melanocytic markers

desmoplastic small round cell tumor of ST
EWS-WT1 transloc
11;22 p13q12

Malignant fibrous histiocytoma
aka undifferentiated pleomorphic sarcoma
pleomorphic tumorcells and storiform pattern

medullary renal carcinoma
asst w sickle cell trait
arise distal collecting duct
fibromyxoid stroma
loss of SMARCB1/INI1

interstitial cystitis
middle aged females
dx of exclusion
reduced bladder capacity
increased mural mast cells
gonadoblastoma
most common tumor in pts with dysgenic gonads
80% phenotypic females
NOT malignant butcan give riseto malignancy
composed of germcells andsex cord cells

metanephric adenoma
may get paraneoplastic syndrome with polycythemia

nehrogenic adenoma
BENIGN
derives from displacement of renaltubules or metaplastic change

nephroblastoma
aka WILMs
aberrant WT1 and WT2
diffuse anaplasia= unfavorable histology
involved in Denys drash WAGR and Beckwith Wiedemann

Alport syndrome
Xlinked mx of collagen IV in the ear eye and kidney
can’t see can’t pee can’thear
megathrombocytopenia granulocyte abnormalities diffuse leiomyomatosis
thromboticmicroangiopathies
ddx DIC TTP HUS
scleroderma antiphospholipidab
see mucoid intimal edema of renal small arteries
minimal change disease
presents with full nephroticsyndrome with preserved renalfunction
nlserum compliment, no hematuria
hibernoma
benign non-metrastasizing brown fat tumor
cells with innumerable small vacuoles
11q rearrangements
fibromyxoid sarcoma
variantof fibrosarcoma
painless, deepST, large, capsule, curvilinear vessels
- MDM2 S100, SMA, desmin, keratin, CD34, B catenin
7;16 q 32-34 p11
FUS CREB3L2

urachal cyst
ass’t with carcinomas
mesoblastic nephroma
most commonrenalneoplasm of infancy
classic; fibromatosis appearance
cellular type: fibrosarcoma appearance

renal dysplasia
aberrant differentiation of metanephric blastema
results in abn kidney development get Pottersequence
angiomyolipoma
epithelioid to rhabdoidcells, thick walledvessels, scattered adipose tissue
PEComa
TSC2 mx, tuberous sclerosis
+ smooth muscle and melanocyticmarkers
cystitis cystica et glandularis
Brunns nests inLP with cystic spaces and other glands with cuboidal to columnar epi, intestinal type contains goblet cells

rapidly progressive glomerulonephritis
rapid decline of renal function
glomeruliform crescents
focal segmental glomerulsclerosis
MOST COMMON primary cause of nephroticsyndrome in adult population
scarring/colapse of somebut not all glomeruli
secondary asst w HIV heroin and loss of renal mass
papillary necrosis
asst with obstructive pyelonephritis, diabetic nephropathy, SCD, analgesics
HCV related kidney disease
membranoproliferative GN
cryoglobulinemic GN
membranousglomerulonephropathy
IgA nephropathy
most common glomerular disease
granular deposits

acute renal allograft rejection
interstitial inflammation
endovasculitis
post infectious glomerulonephritis
subepithelial HUMPS found on EM

choriocarcinoma
malignant cytotrophoblastic and syncitiotrophoblastic cells with hemorrhage and necrosis
elevated HCG
+ EMA

urothelial carcinoma in situ
+ CK20 entire thickness
+CD44 basal
p53 overexpression
clear cell renal carcinoma
derived from PROXIMAL convoluted tubule
+PAX2 PAX8 CD10 CAIX
- CD117 CK7
del 3p mx (VHL)
chromophobe carcinoma
tan on gross
vegetable like cellborders sheet or alveolar pattern
+CK7 CD117 Halescolloidal iron
- vimentin
derived fromintercalated cells of collectingducts
loss of chr 1 and y
asst w Birt Hogge Dube

papillary renal carcinoma
+ 7+ 17 -y
also x;1 TFE PRCC
MET gene
Better prognosis than clear cell RCC
If grade 1/2 and <1.5 cm, called papillary adenoma
derivedfrom DISTAL tubule

pheochromocytoma
Zellballen pattern
+ chromogranin and synapto
systentacular cells + for S100

Birt Hogge Dube
AD mx inFLCN folliculin
asst with hybriud chromophobe/oncocytoma
facial fibrofolliculomas
fibroadenomas
pulmonary cysts (invcreased risk fopneumothorax)
classical seminoma
fibrous septa with lympocytic infiltrate
+PLAP

AR PKD
asst with liver disease
PKHD1 mx
fibrocystin
oncocytoma
stellate central scar
nuclear change fromanoxia doesnotaffect prognosis
derived fromintercalated cells of collecting duct

leydig cell tumor
cells with prominent nucleoliabundant eosinophilic cytoplasm and well defined borders
may produce androgens
usually benign
Reinke’s crystalloids are pathognomonic
+inihbin, melanA celretinin vimentin
- B HCG
PLAP

embryonal carcinoma
poorly differentiated
+CD30 PLAP OCT 3/4 SOX2 SALL4
-EMA

follicular cystitis
lymphoid follicles with germinal centers
membranoproliferative glomerulonephritis
may cause nephrotic or nephritic syndrome
proloferation ofmesangial cells due to subendothelial andmesangial deposition of Ig
doubled upGBM

Fabry’s disease
Zebra bodies accumulating in myocardial endothelial and renal epthithelial cellsonEM
X linked
angiokeratomas hypohidrosis cornal opacities renal, cardiac and cerebrovascular disease with age

paragangliomas
+ chromogranin
- panK HMB45
sustentacular cells express B HCG
rhabdoid tumor
malignant aggressive tumor ofinfants
hypercalcemia
hSNFS/INI1 mx
yolk sac tumor
aka endodermal sinus tumor
retinular/sieve like pattern
schiller duval bodies
elevated AFP GATA 4
+ SALL4 PLAP Glyp3 CD117

CIN
arises in transformation zone of cervix
differentiated VIN
minimal basal atypia with brighteosinophilic cells
prominent nuclear bridges, prominent nucleoli, occasional keratin pearls
NOT HPV related
higher risk than HPV related
likely to show nuclear p53 staining (hyperplastic epi does not)
endometrial stromal neoplasms
+CD10
-h caldesmon
(smooh muscle tumors often h caldesmon +)
Grading of immature teratomas of children
based on amountof immature neuroepithelial tissue
Grade 1: <1 4x field on worst slide
grade 2: no more than 3 low power fields in worst slide
grade 3: >3 low poiwer fields on any slide
ovarian tumors of adolescence
most common mass: benign cyst (functional cyst)
most common neoplasm (GCT (mature cystic teratoma)
most common malignancy: dysgerminoma
least common: sex cordstromal tumors like sertoli leydig and granulosa
adenomatoid tumor female
benign mesothelioma of fallopian tube
not associated with increased risk for ectopic pregnancy
utrerine serous carcinoma
non estrogen dependent
more aggressive han uhterine endometrioid
cervical SCC microinvasion
1A1: <3mm deep, <7 mm wide
1A2: 3-5 mm deep, >7mm wide
condyloma latum
asst with syphilis

cervical sarcoma botyroides
less aggressive, occurs in adolescence
vaginal sarcoma botyroides
vaginal tumor
girls<5
polypoid grapelike massextrudig from vagina
form of embryonal rhabdomyosarcoma
dense accumulation of neoplastic cells under cambiumn layer
Krukenberg tumor
signet ring cell carcinomametastatic to ovary
primary usually GI or breast
endometrial dating
secretory:subnuclear vacuolization
don’t fate with chronicendometritis
Gartner’s (wolffian duct) cysts
anterolateral vaginal wall
simple columnar lining
Skene’s duct cyst
paraurethral
mucinous
Arias Stella reaction
benign mimic of cervical adenocarcinoma
hypersecretory endometrium withnuclear atypia in hobnail cells.
atypical mitoses must be ditinguishedfrom clear cell carcinoma which occursinolder not pregnant women.
+ Napsin A

microglandular hyperplasia
benign mimic of cervical adenocarcinoma
benign back to backglands with intraluminal neutrophils

tunnel clusters
benign mimic of cervical adenocarcinoma

CAH
loss of PTEN
pseudomyxoma peritonnei
results from ovarian mucinous neoplasms secondary to appendiceal neoplasm
leiomyosarcoma
coagulative necrosis
high mitotic activity
hemorrhage
vulvar paget
+ CK7 CEA GCDFP15
- uroplakin III HMB45
vulvar melanoma
2nd most common vulvar malignancy
Breslow thickness: top of nucleated epi to depth
fibrothecoma
mayproduce estrogen
usually unilateral

Meig syndrome
triad seen withovarian fibroma, ascites, and rightpleural effusion
papillary cystadenocarcinoma
elevated CA 125

mature cystic teratoma
SCC most common malignancy to arise
granulosa cell tumor
call exner bodies
gross: solid and cystic, hemorrhage and necrosis
+ inhibin
LG makignnacy BUT can secrete estrogen and lead toendometrial hyperplasia/carcinoma
In juvenile granulosa, nuclear groove absent

endonetrial carcinoma grading
Grade 1: 0-5% solid
grade 2: 6-50% solid
grade 3: >51% solid
nuclear atypia bumps up by 1
vulvar lichen planus
elderly caucasian women/children
small increased risk SCC

vaginal adenosis
subepithelial glandular tissue with squamous metaplasia
asst with DESexposure
atypical polypod adenomyoma
biphasic
stroma composed offasicles of smoothmuscle
numerous atypical epithelial glands with lost polarity but not pleomorphic
squamous met common

aggressive angiomyxoma
invasive deep soft tissue lesion most frequently in vulva
rarely in scrotum
>10cm non circumscirbed
+desmin, vimentin, ER, PR

endometrial stroma sarcoma
ovoid cells with scattered hyperchromatism
numerous mitoticfigures with atypicalmits
invasive, +LVSI
+CD10 to differentiate from leiomyosarcoma

dysgerminoma
aka ovarian seminoma
fibrous septa infiltrated by lymphocytes
tumor cells with clear cytoplasm
+OCT4 CD117
- EMA CK CD30

uterine clear cell adencarcinoma
largepleomorphic cells with clear cytoplasm
hobnailed and cuboidal
infrequent mitoses
papillary fibrous cores arehynalinized
VHL mx
ass’t with endometriosis

thecoma
lipid vacuolated spindle cells with hyanline plaques
canproduce estrogens and androgens
older women

hidradenoma papilliferum
benign apocrine lesion usually in labiamajora
well circumscribed dermalnodule with papillary projections and fibrous stroma
lined by double layered epi composed ofinner myoepis and outer columnar apocrine cells
decapitation secretion

gynecomastia
hyperthyroidism, cirrhosis, renalfailure, hormoneuse,drugs (digitalis,cimetidine, spironolactone)
microglandular adenosis
eosinophilicintraluminal secretions
absence of myoeps
+ S100
- ER/PR p53 Her 2 GCDFP15 EMA
surroundedby type IV collagen

tubular carcinoma
+ EMA
lacks type IV collagen
irregular angulated contours of glands (“teardrop-like”
better prognosis than IDC

cystic hypersecretory hyperplasia/carcinoma
colloid like secretions
micropapillary pattern

invasive micropapilary carcinoma
small clusters of glandular like structures in retracted spaces
mimic lymphangioinvasion
high rate of LN mets
+ER/PR Her2 overexpression

mucinous carcinoma
>50% mucin

intracystic papillary carcinoma
form of intraductal carcinoma
must be differebtiated from intraductal papilloma w myoep stains
ER/PR+
- Her 2
HG DCIS
p53overexpression
Her2 overexpression
high proliferative index
breast sentinel LN
must cut 2-3mm (not bissect)
view 3 levels H/E +IHC
male breast cancer
usually presents at higher stage but similar prognosis stagefor stage as female
BRCA2
multiple intraductal papillomas
arise in terminal duct lobular units and asst w 1.5 x2x RR cancer
allpapillomas contain luminal and myoep cells
breast cancers with better prognosis
mucinous
tubular
medullary
34betaER
LCIS and UDH
variableADH
Breast cancer with poor prognosis
micropapilary
tumors with central fibrotic focus
inflammatory carcinoma
basal like
Her 2 -
metaplastic
medullary breast carcinoma
>75% syncitial growth pattern
circumscription maderate-marked
lymphplasmacystic infiltrate
ER/PR/Her2neg
may be asst with BRCA 1 mx
good prognosis but oftenPD

lobular carcinoma
+ HMWK ER/PR
- Ecad, Her 2
increased risk bilat
nipple adenoma
aka florid papillomatosis of nipple
involves lactiferous ducts
my be associated with adenosis

Paget disease of breast
intraductal carcinima extendinginto epidermis fromnipple
may containmelanin
Toker cells (normal bland nipple cells with cleared cytoplasm) UseHer 2
Syringomatous adenoma of nipple
comma shaped gland
benign but infiltrative lesion

endometrial intraepithelial carcinoma
noninvasive +p53 strong ki67
precursor to serous carcinoma
relative risks for developing invasive breast carcinoma
UDH: 1.5-2x
ADH/ALH: 4-5x
cystic change: 1
DCIS/LCIS: 8-10x
juvenile papillomatosis of breast
localized area ofproliferative cystic change and intrauctal hyperplasia
10-15% develop cancer
Radial scar
DCIS, LCIS, tubular carcinoma
(absence of myoeps)
RR 1.8x for carcinoma
subareolar sclerosing duct hyperplasia
resembles radial scar and located in subareolar region
phyllodes tumor
Benign, borderline malignant, or HG malignant
Does not involve LN, mets to LUNG
polyclonal epi and clonal stroma
increased cellularity near ducts
stromal overgrowth:absence of epithelium in low power field

angiosarcoma
Stewart Treves syndrome (post mastectomy lymphangiosarcoma ofarm)
pot rads tumorsoften high grade

papillary endothelial hyperplasia
aka Massons vegetant intravascular hemangioendothelioma
circumscribed, intravascular lesion with thrombus
endometrioid vs endocervical adenoca IHC
Endonetrioid: +vimentin, ER, PR
Endocervical: +CEA, p16,p53
VIN
Two categories: Usual type (includes warty and basaloid)
and differentiated type
Differentiatedtype asst with vulvar dermatoses like lichen sclerosis HPV,p16 neg
Usual type asst with HPV, younger age, p16+
ZC3H7B-BCOR HG endometrial stromal sarconma
bland cytological features but aggressive clinical behavior. +cyclinD1 CD10
Endometrial stromal nodule
no atypical mitoses
no invasion into myometrirum or LVSI
complete mole
edematous vili with central cisterns and circumferential trophoblastic proliferation with atypia
Diploid
ONLY PATERNAL DNA
p57 NEGATIVE (maternal gene)
enlarged uterus, increased bHCG
Endometrial vs ovarian serous carcinoma IHC
endometrial +p16 PTEN p53 IMP3
+p16,k ER, WT1. IMP3 p53
osteosarcoma
distal femur/tibia (knee) evaluate for % necrosis want >90%
500x increased rish with 13q14 mx (Rb)
MDM2 amo
LOH @ 3, 7, 18
chordoma
notochord differentiation with cords or single cells with vacuolated cytoplasm
PHYSALIFEROUS cells pale multivacuolated cytoplasm and prominent nucleoli
sacrococcygeal region

ossifying fibroma
SLOW growing
cellular stroma withimmature trabaculae and calcific spherules
MANDIBLE
+osteoblastic rimming (as opposed to fibrous dysplasia andparosteal osteosarcoma)
juvenile version more aggresive

aneurysmal bone cyst
benign multiloculated hemorrhagic cyst separated by fibrous septa and new bone strands and MGCs in cyst walls.
Soap bubble on rads

giant cell reparative granuloma
nl calcium and phosphorus chemistry as opposed tobrown cell tumor
avascular necrosis
epiphyseal, longbones, post traumatic
Adamantinoma
rare LGmalignancy
biphasic

osteomyelitis
hematogenous spreadin children-
directpenetration inadults
S. aureus
Pott’s disease
TB in bone
Rheumatoid synovitis
villous formation ofsynovium, chronicinflam, fibrinoidnecrosis
lymphoid nodules with loose plasmacell infiltrates
giant cell tumor of tendomn sheath
aka localized nodular tenosinovitis
related to PVNS
well circ benign mass covered by collagenous mmb
small joints, fingers
synovial chondromatosis
multiple nodules of hyaline cartilage adjacent to synovium
metaplastic lesion
Li fraumeni
mx p53
500xincreasein osteosarcoma
von recklinghausen
primary hyperparathyroidism causing severe bone diasease
mazabraud syndrome
fibroud dysplasia and myxomasn of ST
Albus- Schonberg
deficiency of osteoclast formation
psedoangiomatous hyperplasia
neg for CK5,CD31, S100
+SMA

Flat epithelial atypia
shows 1-2 columnar cell layers within duct
mildatypia
APICAL SNOUTs
as’t with tubular and lobular carcinoma

intracystic papillary carcinoma
truefibrovascular cores in duct
- CK5/6
good prognosis
older pts

Breast adenoid cystic ca
ductal epi +CK,EMA
myoeps +SMA p63 s100
eosinophilic secretions
+PAS with diastase, ER/PR
-Her2

sclerosing adenosis
lobulated outline and myoep layer
stromal growth compressing glands

gynecomastia
ductal proliferation with prominent rim of edematous stroma

micropapilary ductal carcinoma in situ
myoepithelial layer surroundiung duct
malignant cells project into lumen
NO fibrovascular cores
projections narrow at base andexpand into lumen

fibroadenoma
proliferation of fibrous stroma that encloses and comrpresses glandular spaces
microglandular adenosis
small round glands wutheosinophilic secretions
can be infiltrative (may not have myoep layer)
+S100

Paget of nipple
+ CK7 LMCK Cam5.2
-HMWK CK903 CK5/6
Metaplastic breast cancer
squamous sarcomatous or chondromatous differentiation
poor prognosis
express nuclear p63

Breast node staging
pN(mol+): molecular methods only
pN0(i+) isolated tumor cells (<0.2mm or <200cells)
pN1mi: tumor cells >0.2mm <2mm
pN1a: 1-3LN<at>2mm</at>
secretory breast carcinoma
most common breast cancer in youngpeople
t(12;15)
triple negative
hyalinizedstroma with tubular or papillary growth

myoepithelial markers
SMA,p63
UDH
fills duct lumen
irregularly shaped
streaming appearance
CK5/6+
papillae wider at base

ADH
low grade DCIS but <2mm
- CK5/6
rigid bridges and polarization around lumen

LCIS
uniform, dyscohesive, round, fill the lumen
Ecad -
No calcs
more commonly multifocal
microinvasion criteria (for various tumors)
Breast: <1mm depth, 7 mm width
ovary: <3 mm dept, 10 mm area
cervix: <5 mm depth, 7 mm area (FIGO), <3 mm depth (SGO)
vulva: <1 mm inv from uppermost dermal papilla
salivary ca ex PA: <1.5 mm
pulmonary minimall invasive adenoca: <5 mm invasion into stroma, <3 mm size with predominantly lepidic growth
Her2
amplifiedin15-30% of breast tumors
amplification tx traztuzumab
complete intense circumferenial mmb staining
Her2/CEP17 ratio >/=2.0
amplification asst w worse prognosis
intraductal papilloma ofbreast
risk of malignant transformation is low
10% of benign breast lesions
most are asymptomatic but can have atypical features and do have mildly increased ridk for cancer
should excise for atypia
grover’s disease
focal acantholytic dermatosis with suprabasilar clefting
identical to darier’s disease
clinically: resembles athropod bite/drug eruption

molluscum contagiosum
Henderson Patterson bodies
chromomycosis
pigmented fungal elements = medlar bodies
like copper pennies

granuloma inguinale
calymmatobacterium granulomatis
donovan bodies (intracytoplasmic organisms)

lichen planus
hyperorthokeratosis
thickened granular layer
saw-toothed epidermal hyperplasia
necrotic keratinocytes in basallayer (civatte bodies)
bandlike lymphoidinfiltrate (T cells)
pruritic erythematous purple papules onflexor surfaces

mucha haberman disease
aka pityriasis lichenoides et varioliformis acuta
acute guttate psoriassis
wedge of lymphocytic lichenoid inflammation with extravasation into epidermis
porphyria cutanea tarda
subepidermal bullous disorder without signiifcant inflammation
dermal papillae protrudes into bulla with festooned pattern; roof of blister has eosinophilic, PAS+, and diastase resistant linear globules
sunburn histology
necrotic keratinocytes
malanoma
prognosis: thickness (granular layer to deepest level), presence of ulceration
in AAs, only mucus mmbs, palms, soles
trichelemoma
downward proliferation of pale keratinocytes
see in Cowden syndrome (PTEN mx)
bulbous profile, squamoproliferative lesion incontinuity with the epidermis

pilomatricoma
basaloid ghost cells
granulomatous inflammation
cheeks

trichoepithelioma
lace-like arrangement of basaloid cells with aggregates surrounded by onion skin pattern of spindle cells

eccrine spiradenoma
similar to cylindroma but basaloidcells are arranged in balls in circular pattern

cylindroma
sweat gland adenoma
glassy basement mmb like material
jigsaw puzzle configuration
scalp/forehead
+s100
CYLD gene mx

spindle cell lipoma
+CD34 s100
halo nevus
band like lymphoidinfiltrate obscuring nevus looks like white ring with flesh colored papule
desmoplastic melanoma
differentiate fromkeloid (won’t have hyalinized collagen ribbons)
survival rates are the same as conventional.
usually melan A negative
blue nevus
pigment (melanin)laden spindle cells

multicentric reticulohistiocytosis
ass’t with arthritis. large cels with ground glass appearance
little papules at base of nails

cutaneous lupus
hydropic alternationof basalepidermis thickening ofGBM, periadnexalinfiltrate,interstitial mucin formation
won’t see spongiosis

spitz nevus
maysee superficial mits
if mits deeper, think Spitzoidmelanoma
spindle and epithelioid melanocyticnevus
asst with maturation toward the base
Kamino bodies (eosinophilic globules) in epidermis

dermatitis herpetiformis
IgA depostion in dermal papillae
target is gliadin and reticulin
neutrophilic microabscesses
blistering disorder ast with celiac

pemphigus vulgaris
desmoglein 3 is target
often oral lesions
may get secondary staph infections
Intraepidermal blister just above basal layer due to acantholysis from IgG against desmosomes
INTERCELLULAR IgG and C3
tombstone pattern

bullous pemphigoid
IgG and C3 deposition in basement mmb
BPAg1 and 2 are targets on hemidesmosomes
tensebullae don’t rupture easily
Type 2 sensitivity

Hailey Hailey
aka benign chronic pemphigus,
painful genetic blistering rash, AD
intraepidermalacantholysis resultngin blister formation
NO Ig deposition
intertriginous areas
haploisufficiency of ATP2c1 gene
dilapidated brick wall

leukocystoclastic angiitis
neutrophils around/infiltrating vessels
fibrinoid necrosis

pyoderma gangrenosum
abscess like collection of PMNs in dermis
skin confitions with normal epidermis
granuloma anulare
kaposi
DFSP
acral lentiginous melanoma
increased single melanocytes in basallayer
soles palms mucusmmbs
subungual regions
morphea basal cell epithelioma
white scar-like patch orplaque on face
cords/strands of tumor cells infitrating dense collagen
syringoma
small discrete lesion confined to upper dermis withcuboidal cell lined ducts
lower eyelids
paisley/tadpole pattern

viral exanthum
usuallyshow sparse lymphoid perivascular infiltrate
psoriasis
loss of granular layer
parakeratisosi with neutrophils in stratum corneum
erythema multiforme
sparse perivascular superficial lymphoid infiltrate with necrotic keratinocytes
hypersensitivity reaction to infections
granular C3 and IgM in BM

arthropod bite reaction
superficial and deepperivascular lymphoid infiltrate of monos and eos
granuloma anulare
overlying epidermis isnormal
skin colored raised papules on distal extremities
Giant cells and interstitial histiocytes
angiosarcoma of skin
almost exclusively in head/neck of olderpatients
grenz zone
leprosy, tumor stage of MF, B cell lymphomas
narrow layer beneath epidermis ununvolvedby underlyingdermalpathology
BCC
may have retraction artifact from mucin deposition in tumor aggregates
necrobiosis lioidica
NO mucin deposition
hyaline connective tissue arranged parallel to epidermis with patchy plasma cell infiltrate
similar to granuloma annularebut thicker cells and + PCs
mucin stains negative
syringocystadenoma papilliferum
derived from apocrine gland
arises from nevus sebaceus ofJadassohn

keratoacanthoma
asst with intraepidermal neutrophilic microabcesses

erythema nodosum
septal panniculitiswithout dermal involvement

fibroepithelioma of Pinkus type BCC
anastomosing pattern of basaloid proliferation entrapping dermis

solar keratosis
alternating parakeratosis and absence of dysplasia in intraepidermal component
aka actinic keratosis
sweet’s disease
papillary dermal edema and dense interstitial neutrophilic infiltrate inupper 2/3 of reticular dermis

juvenile xanthogranuloma
infants, lymphohistiocytic proliferation CD68 and vimentin
Muir Torre syndrome
MLH1 andMSH2 Lynch syndrome + skin tumors like keratoacanthoma
pemphigus foleaceous
similar to BP but ab to desmoglein 1
lichen simplex chronicus
elongated rete
cicatrical pemphigoid
similar to BP .
use salt split test
IgG bottom
(IgG on top in BP)
Menke disease
aka kinky hair disease
Xlinked
ATP7A gene mx
Xq13.3
nevus sebaceous of Jadasohn
congenital head/neck yellow greasy lesion w cobblestone appearance
rheumatoid nodule
densely eosinophilic fibrin
atypical fibroxanthoma
benign rapidly growing lesion found on sun exposed areas inelderly
+CD10 CD117 CD68