Diseases of the liver and pancreas



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PICTURES HAVE BEEN DELETED, EXCEPT CHARTS AND DIAGRAMS!!!
Credits go towards: Rashmi, Doug, Kathy, Denham. Without their help, these review would not have been possible.

Pathology Exam Review II
Kathy review

Female Genital Tract Diseases

Candida is curdy and common with diabetes

Trichomoniasis is watery (the water trickles down the stream). Flagellate protozoa and fiery red vagina and cervix (strawberry cervix)

Gardnerella is fishy/musty (fish guts go in the garden)

PID caused by infections of gonococcus, Chlamydia and enteric bacteria, can cause infertility

Bartholin cyst from obstruction of Bartholin gland

Hidradenoma is a benign sharply circumscribed nodule

Condyloma latum comes from syphyllis

Condyloma Acuminatum are genital warts from HPV 6,11,20, show koiliocytosis

Carcinoma VIN – women over 60, also called Bowen’s disease. Associated with HPV and dystrophies characterized by epithelial thickening resembling leukoplakia.

Squamous Cell Carcinoma – causes keratin pearl, may metastasis to inquinal, pelvic, and iliac nodes, lungs, liver.

Extramammary Pagets disease – pruritic, red, crusted sharply demarcated, large, clear tumor cells. Poor prognosis.

Malignant melanoma – occurs in women 60-70. Stains with S100 to differentiate from Pagets.

Adenocarcinoma – increased in women whose mothers took DES. Ages 15-20, glycogen filled clear cells.

Embryonal Phabdomyosarcoma – also called sarcoma botyroides due to grapelike appearance of tumors. Usually seen in infants and children under 5.

Chronic cervicitis – glands blocked by inflammation and scarring.

Servical cancer – also called cervical intraepithelial neoplasms. Associated with E6,7 proteins. Rb and p53, HPV 16,18, 31,33. Risk factors include early intercourse, multiple partners, or partner with previous multiple partners. Pap smear for early detection. Graded by C1 (dysplasia) through CIII (carcinoma in situ). Usually found in the transformation zone. Staging 0 (CIN 1) through IV (metastasis). Irregular vaginal bleeding, leucorrhea, bleeding, pain with coitus.

Adenomyosis – endometrial glands extend into myometrium. Menorrhagia and pelvic pain.

Endometriosis – usually appears 30-40. Most often in ovaries, then ovarian ligaments. Red/blue to yellow/brown nodules, may cause adhesions, chocolate cysts. Dysuria, infertility.

Endometrial hyperplasia – abnormal bleeding, occurs around menopause. Related to endometrial cancer.

Endometrial polyps – over 40. Vaginal bleeding, may harbor adenocarcinoma.

Leiomyoma – benign smooth muscle uterine tumors. Most common tumor in women. More common in blacks.

Leiomyosarcoma – does not come from leiomyomas. More common in blacks, over age 50. tumor necrosis and hemorrhage.

Salpingitis – inflammation of the fallopian tubes associated with ovaran inflammation (PID). Most commonly caused by Chlamydia trachomatis, N. gonorrhea, violin string adhesions (Fitz-Hugh-Curtis syndrome). Caused by ascending infections after sex, IUDs, postpartum endometrial infection.

Ectopic pregnancy – 95% caused by PID. Cause hematosalpinx. Abrupt abdominal pain, uterine bleeding. May rupture.

Fallopian tube tumors – Adenomatoid most common benign tumor.

*Pregnancy is the most common reason for pelvic mass, or if menstruating, then ovarian cyst.

Follicular (ovarian) cysts – mature follicles rupture then seal, filled with serous fluid, glistening membrane.

Polycystic Ovarian Disease – bilateral. Multifollicular cysts due to lack of FSH, causing follicular atresia. Women are obese, acanthosis nigricans, and hirsute. Absence of corpus lutea. Oligo/amenorrhea, infertility, viralization (Stein Levonthal syndrome). Incereased risk for endometrial cancer. Treated with oral contraceptives.

Ovarian tumors – 80-90% are benign. Most common is serous cystadenoma, second is Brenner’s tumor. Appear mostly in 20-45 year olds. High mortality due to late clinical presentation. Risk factors include family history and nulliparity. Most arise from surface epithelium. (94%) The tumor marker is Ca125.

Cystadenoma – 20% bilateral. 75% benign or borderline. 20-50 years. Unilocular or multilocular with lining epithelim similar to fallopian tube

Serous Cystadenoma – 40% of all ovarian cancers and most common primary ovarian tumor. 66% are bilateral. Psammoma bodies can be seen on XRay. (Dead cancer cells that have calcified). Tumors have solid areas and cyst lining has three layers.

Mucinous Cystadenoma – lining of cysts resembles endocervical mucosa. Single cell layer. Cysts filled with sticky, gelatinous fluid. 20% bilateral. Solid areas, no connective tissue (over 4 layers) CEA is the tumor marker. Pseudomyxoma peritonei – can cause bowel obstruction. Ascites.

Endometrioid Tumors – 20% malignant, 40% bilateral. Composed of glands resembling endometrial glands.

Clear Cell Adnocarcinoma – can occur with endometriosis or endometrioid CA. Solid or cystic, large cells with clear cytoplasm. Cells lining are called hobmail cells.

Brenner Tumor of Ovary – adenofibroma composed of nests of epithelial cells resembling transitional cells of urinary bladder (Walthard’s rests). Benign and 90% unilateral.

Teratoma mature) – benign. Also called dermoid cysts, orginate from single germ cell. Usually in women 20-40. Contains ecto/endo/mesoderm. 10-15% bilateral. Often calcified bone or tooth formation.

Immature malignant teratomas - resemble fetal tissue, occurs around age 18, solid tumors, tumor grading based on amount of tumor tissue that is immature neuroepithelium.

Struma ovarii – unilateral, mature thyroid tissue in teratoma. May cause hyperthyroidism.

Carcinoid tumor – unilateral. May produce carcinoid syndrome. Aggressive.

Dysgerminoma – solid tan tumors with nests of germ cells and lymphocytes in stroma.

Yolk Sac Tumor – alpha fetoprotein is tumor marker. Glomerular like structures called Schiller-Duval bodies. Children or young women under 20, present with rapidly expanding abdominal mass. Resemble primitive lung tissue.

Embryonal Carcinoma – unilateral, young patients with abdominal mass, positive pregnancy test.

Choriocarcinoma – primary disease is rare, malignant

Granulosa-theca cell tumor – hormone producing, marker called Call-Exner body (resembles graffian follicle) bean shaped nucleus.

Thecoma – fibroma – 90% unilateral. Fibroblasts with plump spindle cells filled with lipid. Fibromas do not produce estrogen but thecomas do. Usually postmenopausal, with pain and pelvic mass. Meigs syndrome – hydrothorax (rt side) + ovarian tumor + ascites. Associated with basal cell nevus syndrome.

Sertoli-Leydig Cell Tumors (Androblastoma or arrhenoblastoma) – usually 20-30, virilizing tumors producing excess androgens. Benign. Contain large, lipid filled cells and rod like crystals called Reinke Crystalloids.

Sex cord tumor with annular tubules (SCTAT) 33% associated with Peutz-Jeghers syndrome. Somewhere between ranulose and Sertoli cell tumor.

Gonadoblastoma – combination of germ cell and sex-cord stromal tumors, occurs with gonadal dysgenesis, usually Turner’s with Y chromosome.

**Tumors that metastasis to ovary – Breast, stomach cancer (signet ring type), Krukenberg tumors, colon cancer, Burkitts lymphoma.

Spontaneous abortion – pregnancy terminating before 22 weeks. Usually trisomys, hydatidform mole or neural tube defects. Present with vaginal bleeding and lower abdominal pain.

Placenta accrete – attachment of placenta directly to myometrium due to defect in ranulos. Causes significant bleeding during delivery. Most common sign is bleeding third trimester. 15% cause uterine rupture. Caused by scars (Ashermanns syndrome)

Placenta Previa – implantation over to close to cervical opening. Presents with painless bleeding. Most cause of antepartum hemorrhage. Risk factors include maternal age, parity, uterine abnormalities and previous uterine surgery.

Abruptio Placenta – complication of pre-eclampsia. Premature separatin of placenta, Presents with painful vaginal bleeding, can result in fetal death. Associated with maternal hypertension.

Chorioamnionitis – infection of fetal membranes from ascending infection or premature rupture of membranes longer than 18 hours. Usually S. agalactiae. May cause funisitis (infection of umbilical cord).

Villitis – infection of chorionic villi from endometriosis or organisms with placenta, usually maternal bacterial sepsis from T, pallidum, M tuberculosis, Mycoplasma, Chlamydia, rubella, CMV, toxoplasma, candida

Pre-eclampsia – sudden onset of maternal hypertension, edema and proteinuria (ranulose syndrome) usually after 32 weeks in primigravidas.

Eclampsia - all of the symptoms of pre-eclampsia plus convulsions. Caused from lack of protective prostaglandin E with neutralizes vasoconstrictive effect of angiotension II. Risk factors include first pregnancy, poly hydraminos, DM or pre-existing HTN, Hydatidiform mole.

Hydatidiform mole – associated with alpha-thalassemia and high incidence in Asian countries, chorionic villi resemble grapes, ultrasound shows “snowstorm” appearance, painless vaginal bleeding, uterus too large for gestational age, and high beta-HCG. No embryo in complete mole, but in partial mole.

Invasive moles – invades myometrium and metastasize, though benign. Common in patients over 40.

Choriocarcinoma – 50% from complete cydatidiform moles. Makes beta HCG and human placental lactogen. Trophoblastic tissue only no villi. Metastasize to lungs (cannon-vall) . Treated with actinomycin D and methotrexate. Central core of cytotrophoblasts surrounded by covering of syncytiotrophoblasts.
Diseases of the Breast

Acute mastitis – unilateral. Caused by staph (multiple abcesses) or Strep (diffuse). Extensive necrosis.

Mammary Duct Ectasia – occurs 50-60 in multiparous women. Obstruction of ducts due to inpissation of breast secretions (thick, cheesy) . Can be mistaken for carcinoma.

Fat necrosis – necrosis of fat followed by inflammatory reaction. Isolated in one breast. Can be confused with tumor.

Fibrocystic Disease – cyst formation, epithelial hyperplasia, and sclerosing adenosis. Palpable lump. Most common disorder of breast. Usually ages 20-40. Hormona imbalances. Oral contraceptives to balance excess estrogen. Usually mutlifocal and bilateral. Blue domed cysts.

Epithelial hyperplasia – can accompany fibrosis, and can develop into carcinoma. Increase in duct-lining epithelium. May grow into lumen of ducts (florid papillomatosis)

Fibroadenoma – most common benign tumor of breast. From intralobular stroma. Usually before age 30. Usually solitary, discrete, freely movable nodule in breast (mouse in breast)

Cystosarcoma phyllodes – from intralobular stroma. Can be benign or malignant. May become massive.

Breast carcinoma – rare before age 25 unless family history, peaks after menopause. Five times more common in US than Asia. Risk factors include early menarche and late menopause, nulliparity, older maternal age with first pregnancy, obesity, contraceptives. More common left breast in upper, outer quadrant. Can be invasive or non-invasive Indtraductal 20-30%.

Paget’s disease of the nipple – form of ductal carcinoma. Nipple is fissured, oozing and ulcerated with huperemia, edema and possible underlying mass.

Axirrhous carcinoma – stony hard mass in breast

Medllary carcinoma – large, fleshy tumors masses

Colloid or mucinous carcinoma – occurs in older women, very soft like pale gray-blue gelatin.

Liver and Pancreas

Bilirubin end product of heme degradation (old red blood cells) Formed outside liver and bound to albumin for transport to liver. In liver, uptake at sinusoidal membrane, intracellular binding (ligandin), delivery to ER, conjugation with glucuronic acid by bilirubin UDP-glucoronosyltransferase (UGT), excretion into water soluble fofrm into bile. 20% recycled.

Bile – bilirubin and bile acies (taurine and glycine conjugates of cholic and chenocholic acid). Provides large pool of bile acides for digestion and excretion.

Jaundice – serum bilirubin above 2.0-2.5 (normal is 1.2).

Kernicterus – toxic damage to neonatal brain from excess, unbound bilirubin.

Types of Jaundice


    1. Unconjugated (hemolytic disease) – fasting blood – bilirubin will be increased

      1. Excessive production of bilirubin from hemorrhage or ineffective erythropoiesis

      2. Reduced hepatic intake due to drugs or Gilbert’s syndrome

      3. Impaired conjugation such as physiologic jaundice of newborn, breast milk jaundice, or genetic deficiency of UGT (Crigler-Najjar syndrome types I and II, Gilbert’s syndrome, or diffuse hepatocelluar disease.




  1. Conjugated

    1. Decreased hepatic excretion of glucuronides (Dubin-Johnson,Rotors syndrome)

Hepatocelluar damage or toxicity from virus, drugs, TPN, infection

Drug impairment

Intrahepatic bile duct disease, cirrhosis, cholangitis, GVH, liver transplantation


    1. Extrahepatic bile duct obstruction

Gallstones, carcinomas head of pancreas, extrahepatic biliary atresia, fluke infestation
Cholestasis – bile secretory failure accompanied by accumulation in blood.
Hereditary Hyperbilirubinemias (unconjugated)

  • Crigler-Najjar type I (AR) – absent bilirubin UGT, fatal in newborn

  • Crigler-Najjar type II (AD) – decreased bilirubin UGT, mild, occasional hemicterus

  • Gilbert’s syndrome (AD) – decreased UGT and uptake – very mild

Conjugated hereditary hyperbilirubinemias

  • Dubin-Johnson syndrome (AD) – impaired biliary secretion, membrane carrier defect, pigmented liver

  • Rotors syndrome – (AR) decreased uptake and storage – very mild

Hepatic Failure – must exceed 80-90%. Disorders causing failure are ultrastructural lesions such as Reye’s syndrome, fatty liver of pregnancy or tetracycline toxicity, or chronic liver disease such as hepatitis, cirrhosis, or inherited metabolic disorders, or massive hepatic necrosis from viral hepatitis, massive toxic damage fro drugs, industrial agents and mushroom poisoning.

Hepatic Encephalopathy – associated with hepatic failure. Distrubances in consciousness, limb rigidity, hyperreflexia, asterixsis (rapid flexion/extension of arms)

Hepatorenal syndrome – appearance of renal failure in pts with severe liver disease when there is no problem with kidneys. Function will improve if hepatic failure reversed.

Cirrhosis – end stage liver disease characterized by fibrosis, paenchymal nodules and disruption of liver architecture. 60-70% from alcoholism. Clinical presentation of anorexia, weight loss, weakness and debilitation.

Causes of Portal Hypertension


  1. Prehepatic

    1. Obstructive thrombosis

    2. Massive splenomegaly

  2. Intrahepatic

    1. Cirrhosis

    2. Schistosomiasis

    3. Veno-occlusive disease

    4. Massive fatty change

    5. Diffuse fibrosing granulatomous disease

  3. Posthepatic

    1. Severe right-sided heart failure

    2. Constrictive pericarditis

    3. Hepatic vein outflow obstruction

Portal hypertension causes ascites, shunts, congestive splenomegaly, and encephalopathy. Shunts to rectumn (hemorrhoids), cardioesophageal junction (esophageal varicies), retroperitoneum (ascites), and falciform ligament of liver (caput medusa)


HEPATITIS

Name

Hepatitis A

Hepatitis B

Hepatitis C

Hepatitis D

Hepititis E

Common name

Infectious

Hepatitis



Serum

Hepatitis



Post-transfusion

Delta

Enteric

Virus

Picornavirus

Naked RNA



Hepadenavirus

Envelope DNA



Flavivirus

Envelope RNA



Defective

Envelope


Circular RNA

Calcivirus

Naked RNA



Transmission

Fecal-oral

Parenteral, sexual

Parenteral, sexual

Parenteral,

Sexual


Fecal-oral

Onset

Abrupt

Insidious

Insidious

Abrupt

Abrupt

Severity

Mild

Occ severe

Subclinical

Co-infection w/ B, severe

Superinfect



Mild but severe if pregnant

Mortality

Very low

Low

Very low

High to very high

Higher in pregnant pts

Carrier state

No

Yes

Yes

Yes

No

Hepatitis Serology



Abbreviation

Name and Description

HBV

Hep B virus, hepadnavirus, enveloped, partial double strand DNA. Dane particle = infectious

HbsAg

Surface antigen on HBV,positive during acute stage, continues in carrier state

HbsAb

MhsAg antibody, immunity to Hep B

HbcAb

HbcAg antibody, positive during window phase, IgM MhcAb indicator of recent disease

HbcAg

Core antigen HBV

HbeAg

Second core antigen HBV, indicator of transmissibility

HbeAb

E antigen antibody; indicates low transmissibility

Delta agent

Small Rna virs with HbsAg envelope. Defective

90-95% cases of vertical transmissions become carriers (vs 10% or less of adults)

Carriers have “ground glass” appearance of hepatocytes (finely granular, eosinophilic cytoplasm laden with HbsAg)
Stages of Hepatitis


  1. incubation – peak infectivity during last days of asymptomatic period

  2. symptomatic preicteric- non-specific symptoms, elevated serum aminotransferase

  3. symptomatic icteric – conjugated hyperbilirubinemia, itching, prolonged prothrombin time

  4. convalescence

Councilman bodies – fragmented, eosinophilic bodies due to acute viral hepatitis

Chronic hepatitis – initially limited to inflammatory portal tracts but progresses to piecemeal necrosis (into adjacent parenchyma), can lead t cirrhosis

Autoimmune hepatitis – mostly females, elevated IgG but no other viral markers, elevated ANA, SMA, AMA, LKM bodies, increased frequency in those with HLA B8 or DRw3

Fulminant hepatitis – onset to hepatic insufficiency within 2-3 weeks
Drugs that can cause hepatic injury or tosicity – CCl4 (centrilobular necrosis) , Halothane (necrosis), oral contraceptives (cholestasis or adenoma), Vinyl chloride, aflatoxin (HCC)
Alcoholic Liver Disease

Most common cause of liver disease, fatty liver reversible until cirrhosis develops. Neutrophils accumulate around degenerating liver cells, accumulation of hemosiderin in hepatocytes and Kupffer cells . Marker for alcoholic hepatitis is gamma-glutamite transferase

Mallory bodies – scattered hepatocytes containing eosinophilic cytoplasmic inclusions

Laennec’s cirrhoisis – protruding nodules from surface of cirrhotic liver


Hereditary hemochromatosis – more common in males in ages 50-60. Micronodular cirrhrosis, DM, and skin pigmentation as iron accumulate in tissues, predisposing to HCC, liver is slightly enlarged, dense and chocolate brown due to hemosiderin deposits in acinar and islet cells, causes heart enlargement, synovitis, abdominal pain, cardiac dysfunction
Wilson’s disease – AD disorder of copper metabolism, accumulation in liver, brain and eye, linked to chromosome 13 with ranulos D locus, changes usually evident by 5 years of age, progressively leads to cirrhosis, red-brown, granular material in hepatocytes, also causes cavitations in putamen, Kayser-Fleischer rings in the cornea.
Alpha-1-Antitrypsin Deficiency – AR disorder, abnormally low levels of major protease inhibitor, leads to pulmonary disease, hepatic disease, on chromosome 14, can cause childhood cirrhosis, red hyaline globules, needing liver transplant
Biliary cirrhosis – primarily a disease of middle-aged women, peak ages 40-50, asymptomatic for years, first symptom usually itching, then jaundice, hepatomegaly, xanthomsa, xanthelasmas, hyperbilirubinemia, 90% have antimitochondrial bodies (M2), also Sjodogren’s symdrome, cclerodrma, thyroiditis, RA, glomerulonephritis, and celiac disease
Primary sclerosing cholangitis – mostly males in 50s, 70% have ulcerative colitis, inflammation, obliterative fibrosis, and segmental dilatation of intrahepatic and extrahepatic bile ducts, causes “onionskin” fibrosis, elevated serum alkaline phosphatase
Neonatal hepatitis – prolonged conjugated hyperbilirubinemia in neonate

Liver infarction – rare, but can occur due to thrombosis or compression of intrahepatic branch of artery by embolism, neoplasia, polyarteritis nodosa or sepsis.


Banti’s sundrome – also called noncirrhotic or idiopathic portal hypertension, with splenomegaly, hypersplenism, portal hypertension, arises following subclinical occlusion of portal vein.
Nutmeg liver – acute, passive congestion of liver due to right-sided heart failure

Peliosis hepatitis – rare, dilatation of sinusoids impedes blood efflux, due to anabolic steroids, oral contraceptives and danazol. Mottled, blotchy areas on liver.


Budd-Chiari syndrome – hepatic vein thrombosis, usually fatal, associated with polycythemia vera, pregnancy, postpartum state, oral contraceptives, paroxysmal noturnal hemoglobunuria, intra-abdominal cancers, esp HCC
Cavernous hemangioma of liver- most common benign lesion, red-blue, soft nodules under 2cm beneath capsule, can hemorrhage if biopsied.
Adenomas – benign neoplasms of hepatocytes usually in women taking oral contraceptives, or those on steroids. May be mistaken for HCC, can rupture causing severe hemorrhage, esp due to estrogen, usually pale, yellow-tan, beneath capsule, up to 30cm, sheets and cords of cells, portal tracts absent
Hepatoblastoma – tumors of young children, have epithelial type composed of small, compact fetal cells, form acini, tubules or papillary structures, or mixed types, containing epithelial as well of primitive mesenchymes, osteiod, cartilage or striated muscle. Fatal within a few years unless treated.
Angiosarcoma – related to exposure to vinyl chloride, arsenic or Thorotrast, aggressive, metastasize widely, death within a year.
Hepatocellular carcinoma – highest in Taiwan, Mozambique and SE China, higher in blacks and males, linked to HBV infection, higher risk for infants infected with HBV at birth, in West usually occurs after age 60, may be uni/multi-focal, with liver enlargement at 2-3000 gm, long snake-like tumor masses in portal vein, inferior vena cava. Elevated serum alpha protein.
Fibro-lamellar carcinoma – variant of HCC seen in young people 20-40, better prognosis, no association with HBV or cirrhosis, well-diff polygonal cells in nests or cords separated by parallet lamellae
Cholangiocarcinomas – rare, firm and gritty, resemble adenocarcinomas, vascular invasion less than HCC, mucin production but no bile production
Metatstatic liver tumors – usually from breast, lung or colon, multiple lesions
Cholelithiasis (gallstones) – Two types of stones, (1) crystalline cholesterol monohydrate, (2) pigment stones, mostly of bilirubin calcium salts (radiolucent) , risk factors, female, fat, forty, fertile. Brown stones found in infected hepatic ducts (radiolucent)
Cholecystitis – inflammation of the gallbladder, usually due to obstruction, primary complication of gallstones, and most common reason for cholecystectomy. RUQ or epigastric pain, N/V, fever, anorexia, sweating,
Biliary atresia – major contributor to neonatal cholestasis. Most common cause of death from childhood liver disease, cause of 50-60% of childhood transplants. Due to massive inflammation of biliary tree after birth, cause unknown but likely Reovirus, CMV, rubella, genetics
Choledochal cysts – occurs in children under 10, congential dilatation of common bile duct, jaundice, non-specific abdominal pain, affects females 4:1, predispose to stone formation and elevated risk of bile duct cancer
Adenocarcinoma of the gallbladder – more common in women over 70, gallstones present in Pmajority of cases, can be infiltrating and fungating, usually seeds peritoneum, GI tract, and lungs.
Cancer of extrahepatic bile ducts – usually due to pancreatic carcinoma, adenoma of ampullary orifice, slightly more often in males, usually age 70, firm gray nodules, may or may not be mucin secreting, one type called Klatskin tumors Ifrom common bile duct between cystic duct junction and right/left hepatic duct), jaundice, palpable gallbladder.

PANCREAS


Pancreatitis

  1. Acute – usually from biliary tract disease or alcoholism, abdominal pain due to enzymatic necrosis and inflammation of pancreas, can become hemorrhagic, pain localizes in epigastic area with radiation to back, elevated plasma amylase and lipase, can also cause ARDS and shock with renal tubular necrosis. Serum amylase will be marked first 24 hours, then serum kipase with 72-96 hours

  2. Chronic – usually in the middle-aged, male alcoholic, reduced number and size of acini with fibrosis. Pseudocyst formation common

Von-Hippel-Lindau disease – characterized by pancreatic cysts, angiomas of retina and cerebellum or brain stem, cysts in liver and kidneys. Cysts lined with glistening membrane


Papillary-cystic tumor – mostly in women under 35, large, rounded with solid and cystic zones, causing abdominal pain
Cancer of the pancreas – arises in the exocrine portion on the gland, adenocarcinomas of the ductal epithelium, strong association with smoking, most in the head of the pancreas (60%), can cause biliary obstruction. Clinically: weight loss, abd and back pain, anorexia, N/V, jaundice and trousseau’s syndrome (migratory thrombophlebitis). Elevated CEA and CA19-9 antigen
Endocrine Pancreas disorders

Cell types



  1. Beta cells – about 70%, produce insulin

  2. Alpha cells – about 20% secrete ranulose which induces hyperglycemia by glycogenolytic action

  3. Delta cells – about 5-10%, contain somatostatin which suppresses insulin and glucagons release

  4. PP cells (pancreatic polypeptide) – GI effects including release of GI enzymes

  5. D1 cells – elaborate vasoactive intestional polypeptide (VIP), which induces glycogenolysis and hyperglycemia, stimulates DI secretion, and causes secretory diarrhea.

  6. Enterochromaffin cells - synthesize serotonin and source of pancreatic tumors that induce carcinoid syndrome.


Diabetes Mellitus

Two types of DM:



  1. IDDM or Type I – juvenile onset, ketosis, decreased blood insulin, islet cell antibodies, HLA-D linked, autoimmunity, beta cell depletion and marked atrophy of beta cells

  2. NIDDM or Type II – onset after age 30, usually obese, normal or increased blood insulin, no antibodies, no HLA association, mild beta cell depletion, focal atrophy

Complications/Morphology of Diabetes

Islet changes, diffuse thickening of basement membranes, atherosclerosis, MI, stroke, gangrene of lower extremities, diabetic neuropathies including arteriolosclerosis and glomerulosclerosis (either diffuse or nodular aka Kimmelstiel-Wilson Disease), retinopathy, cataract formation, glaucoma, peripheral neuropathy, sexual impotence, and bowel/bladder dysfunction.
Islet Cell Tumors


  1. Insulinomas – most common, causing attacks of hypoglycemia, CNS symptoms such as confusion, stupor, loss of consciousness. Firm, encapsulated yellow-brown nodules composed of cords and nests of well-diff B cells. Can vary in size up to huge masses

  2. Zollinger-Ellison Syndrome (gastinoma) – triad of recalcitrant peptic ulcer disease, gastric hypersecretion, pancreatic islet cell tumor, more than half malignant. Produces intractable scars, extreme diarrhea, electrolyte problems and malabsorption syndromes

DISEASES OF THE LOWER URINARY TRACT
Oliguria – less than 400cc urine per day

Anuria – less than 100 cc urine per day, usually due to obstruction

Azotemia – increased creatinine and BUN

Uremia – increased creatinine and BUN with symptoms

Diverticula – outpouching of ureteral wall. Can be congenital or acquired.

Hydroureter – dilatation, elongation, and tortousity of the ureters from neurogenic defect of musculature. Can be congenital or acquired.

Megalourereter – massive englargement of ureter due to functional defect of muscle

Urinary bladder diverticula – eversion or bladder wall, congential or acquired, due to persisten urethral obstruction, prostatic enlargement, causes urinary stasis, infection, bladder calculi, and reflux.

Acute cystitis – secondary to bacterial infection, usually E.coli (also Proteus, Klebsiella, S. saprophyticus, and Enerobacter). More common in women due to shorter urethra. Causes frequency of urination, dysuria, urgency, suprapubic discomfort and fever. May see pyuria and hematuria. Positive dipstick for nitrite will diff E. coli and S. saprophyticus. WBC casts mean acute pyelonephritis

Malakoplakia – chronic, inflammatory cystitis char by yellow plaques, nodules or polyps in bladder mucosa, related to chronic bacterial infection, usually E. coli. Most common in bladder but can be in renal pelvis, ureter, prostate, epididymus, colon, and lungs. Accumulation of macrophages filled with round, laminated concretions know as Michaelis-Gutman bodies.

Urothelial (Transitional) cell tumors – half are high grade tumors, mostly arise from posterior/lateral wall of base of bladder, can be papillary, flat, noninvasive or invasive.

Grade I tumors are atypical, well differentiated but resemble normal transitional cells, mitoses rare, with increase in layers. Grade II tumors have increased number of layers (more than 10), increased mitoses, loss of polarity, though still recognizable as transitional in origin.

Grade III tumors barely recognizable, with dissarry.

Squamous cell bladder carcinomas – less than 10% of bladder cancers, arise in areas or squamous metaplaisa. (Adenocarcinomas rare). Affects males usually between 50-80, in industrialized nations. Risk factors are smoking, industrial exposure to arylamines, especially 2-naphthylamine, schistomsoma hematobium, and long term phenacetin use. Painless hematuria with frequency, urgency, and dysuria. Tend to recur.

Mesenchymal tumors – benign tumor of bladder. Most common is leiomyoma. Malignant mesenchymal tumor (sarcoma) is rare, but produce large vesicles (10-15 cm), large fleshy tumors.

Secondary tumors – usually from nearby organs such as cervis, uterus, prostate, and rectum

Urinary bladder obstruction – causes thickening of the bladder wall due to hypertrophy of the smooth muscle.

Urethritis – usually from gonococcus, E. coli, or Chlamydia. Also a component of Reiter’s syndrome along with arthritis and conjunctivitis. Causes local pain, itching and frequency.

Urethral caruncle – inflammatory lesion that is small, red and friable. Usually found at external urethral meatus in females. Consists of young, fibroblastic tissue, ulcerates and bleeds easily.
DISEASES OF THE RENAL SYSTEM

Normal GFR is 120-125 cc

Horseshoe kidneys – fused at lower poles and located lower due to root of inferior mesenteric artery. Seen often in Turner’s syndrome

Polycystic disease – an AD disorder in adults (chromosome 16) , usually in 40s with HTN and hematuria. In adults it is bilateral but unilateral in children. Causes renal HTN in adults. Causes oligohydramnios due to decreased urine flow in an affected fetus. (also most common abd mass in newborns). Is AR is newborns. Will cause lungs to be hypoplastic and the kidneys enlarged massively. Cysts will fill most of parenchyma and glomeruli will be hard to find.

Potter’s facies – a characteristic appearance of a fetus affected with renal agenesis, bilateral renal dysplasia or infantile polycystic kidney disease. The fetus presents with low set ears, parrot beak-like nose, eggshell skull, and receding chin

Medullary sponge kidney – common but innocuous structural change. Multiple cystic dilatations of collecting ducts in medulla, usually normal renal function.

Nephronophthisis – also called Ureic medullary cystic disease (UMCD). Onset in childhood, variable number of cysts in medulla and significant cortical atrophy, interstitial fibrosis and eventual insufficiency. Causes Na wasting and tubular acidosis

Simple retention cysts – most common renal cystic disease, usually over age 50, large, solitary cysts that might be confused with carcinoma. Can also be seen in pts treated with dialysis or transplantation.


Nephritic vs Nephrotic syndrome

Nephritic syndrome

Nephrotic syndrome

Hematuria (RBC casts)

Severe proteinuria (more than 3.5 g/day)

Hypertension

Hypoalbuminemia (less than 3g/dl)

Azotemia

Generalized edema

Oliguria

Hyperlipidemia

Proteinuria (less than 3.5g/day)

Lipiduria

Post-Strepococcal Glomerulonephritis – occurs in children 2-4 wks after Strep (b Hemolytic grp A) causing smoky urine, elevated ASO titers, granular deposits in glomeruli highlighted by IgG, IgM antibodies. Most recover well with corticosteroid treatment.

Membranous Glomerulonephritis – most common adult ranulose syndrome. Causes include drugs, hepatitis, SLE, DM, or idiopathic. Causes thickening of basement membrane and spiking of membrane on silver stain. Granular pattern and subendothelial deposits

Goodpasture’s Syndrome – also known as anti-GBM disease, autoantibodies against type IV collagen of basement membrane, also causes lung and kidney damage, usually in males ages 20-40. First symptom usually hemoptysis. Linear pattern with immunofluorescence.

Minimal Change Disease – also known as Lipoid Nephrosis or Nil’s Disease, most common in children 2-6 when other syndromes excluded. Thin basement membrane and absence of proliferation. Treatment with steroids. Excellent prognosis.

Rapidly Progressing Glomerulonephritis – also called crescentic glomerulonephritis. Rapid progression to renal failure. Can follow Goodpasture’s, vasculitis (wegener’s), or be idiopathic

Focal Segmental Glomerulosclerosis – causes segmental sclerosis and hyaline mass and lipid in sclerotic areas.

IgA Nephropathy (Beurger’s Disease) – most common in world, usually young male adults in France, Japan, and Italy. Gross hematuria. Assoc with resp infection and IgA diseases like celiac sprud, and Henoch-Schonlein ranulo. Will see meseangial deposition of IgA on IF

SECONDARY DISEASE

Kimmelstein-Wilson Disease, due to DM



Membrano-proliferative Glomerulonephritis – two types (I and II), type II has antibody (C3) lobulated glomeruli, splitting of basement membrane

SECONDARY DISEASE

Amyloidosis – due to DM. Congo Red Stain.


Chronic Glomerulonephritis – end stage renal disease with uremia, anemia, HTN, and azotemia. Small kidneys, hyalinization of glomeruli, requiring dialysis or transplant.


Acute Renal failure – can be ischemic or nephrotoxic

  1. Organic vascular obstruction

    1. Polyarteritis nodosa

    2. Malignant HTN

    3. Hemolytic-uremic syndrome

  2. Severe glomerular disease – rapidly progressing

  3. Acute tubulointerstitial nephritis – hypersensitivity to drugs

  4. Pyelonephritis – especially when accompanied by papillary necrosis

  5. Disseminated intravascular renal coagulation

  6. Urinary obstruction

    1. Tumors

    2. Prostatic hypertrophy

    3. Blood clots

  7. Acute tubular necrosis

    1. Ischemic – focal necrosis at multiple points in nephron with large skip areas, rupture of basement membranes, occlusion of lumen by casts, usually in straight portion of proximal tubule or ascending thick limb in medulla. Casts usually eosinophilic hyaline or pigmented granular consisting of Tamm-Horsfall protein, hemoglobin, myoglobin and other plasma proteins. Divided into initiating stage (about 36 hours) with slight decline in urine output and rise in BUN, then maintenance with continued decrease in output, salt and water overload, manifestation of uremia, then recovery. Hypokalemia is a problem.

    2. Toxic – acute injury in proximal tubules, usually due to mercuric chloride, CCl4, and ethylene glycol.

Acute Tubulointerstitial Nephritis (TIN) – interstitial edema, leukocytic infiltration, focal tubular necrosis. Can be caused by infections, drugs, heavy metal toxins, metabolic diseases neoplasms, radiation, immune reactions, vascular diseases. Differentiated from glomerular diseases by inability to concentrate urine, salt wasting, and metabolic acidosis.l

Chronic Tubulointerstitial Nephritis (CIN) – infiltration with mononuclear cells, prominent interstitial fibrosis and tubular atrophy.

Pyelonephritis – affects tubules, interstitium and renal pelvis. One of the most common kidney diseases. Can be acute (usually bacterial), or chronic (due to bacteria with reflux or obstruction)

Urinary Tract Infections – extremely rare, Most cases are gram negative bacilli (E. coli). Occurs with obstruction, debilitation, immunosuppressive therapy.

Acute pyelonephritis – acute suppurative inflammation of kidney caused by bacterial infection. Will be patchy and discrete, focal abscesses one or both kidneys, Large, wedge-shaped area of doalescent suppuration. Complications include papillary necrosis (diabetics) bilaterally, pyonephrosis with total or almost complete destruction when pus unable to drain or perinephric abscess. In infants affects males more, but in 40s affects females more, elderly again males. Risks include DM, reflux, catheterization, BPH, neurogenic bladder, immune deficiency. Will have sudden onset of pain at costovertebral angle, fever, malaise, dysuria, frequency and urgency, pus casts. Diagnosis with urine culture.

Chronic Pyelonephritis (CPN) – chronic inflammation and scarring causes damage and is cause of end-stage renal disease. Can be obstructive or reflus-associated.

Acute Drug-Induced Intersitial Nephritis – adverse drug reaction most often with penicillins, synthetic antibiotics, ciuretics, NSAIDs, phenindione, cimetidine. Begins about 15 days after exposure to drug with fever, eosinophilia, skin rash, hematuria, proteinuria, leukocyturia and rising creatinine level

Analgesic Abuse Nephropathy – chronic renal disease from excessive intake of analgesic mixtures. Cortical atrophy, patchy papillary necrosis and entire area with progressions. Affects women more than men, mostly those with recurrent headaches.

Benign Nephrosclerosis – sclerosis of renal artieroles and arteries causing focal ischemia of renal parenchyma. Kidneys may be reduced in size, slightly decreased GFR. Mild proteinuria.

Malignant nephrosclerosis – associated with malignant or accelerated HTN. Affects younger people, usually males. More common in blacks. Kidney has “flea-bitten” appearance. Can also develop diastolic pressures over 130, papilledema retinopathy, encephalopathy, CV abnormalities, and rental failure. Marked proteinuria, hematuria. Medical emergency.

Renal artery stenosis – rare but curable cause of HTN. Occurs more in males with advancing age ad DM due to occlusion by plaque or thickening of lumen. Causes ischemia of kidney. Elevated . plasma or renal-vein ranul.

Hydronephrosis – marked dilation of renal pelvis and calyces, thinning of parenchymal and atrophy due to chronic obstruction. May have slight to massive enlargement. Cortical tubular atrophy with interstitial fibrosis. Earliest signs are polyuria and nocturia. HTN is common.

Urolithiasis (renal calculi) – most arise in kidney, peak ages 20-30, familial and hereditary disposition, gout, cystinuria, primary hyperoxaluria are causes. Four main types of stones (1) calcium oxalate, associated with hypercalcemia and hypercalciuria, increased uric acid, (2) triple or struvite stones (make staghorn stones), usually follow infections by urea-splitting bacteria such as Proteus and Staph, (3) uric acid stones, which are common in those with gout and leukemias and are radiolucent , and (4) cystine stones, which are genetically determined. Low urine volume will favor supersaturation. Stones are unilateral in 80% Usually formed in renal calyces and pelves and in the bladder. Can obstruct urinary flow, cause renal colic while passing down ureters and cause hematuria.

Renal cell carcinoma – 85-90% renal cancers in adults, usually 60-70s. Affects males 3:1. Also called hypernephromas and arise from tubular epitheliam. Associated with smoking. Occurs in nearly 2/3 of patients with von Hippel-Lindau syndrome, which also includes hemangioblastomas of the CNS and retina. Usually affects upper poles and tends to invade renal veins and grow as a solid column of cells up to the heart. Most common cell type is clear cell tumor then granular. Will cause costovertebral pain, palpable mass and hematuria (90%). Tends to produce diverse symptoms that are hard to classify. Can metastasize widely before symptoms develop, usually lungs, then bones. The affected kidney is usually removed.

Wilm’sTumor – embryonal renal neoplasm and second most common solid tumor in children, usually under age 5. Associated with chromosome 11p13, gene WT2-1. It is associated with WAGR syndrome (also has lack of iris, GU abnormalities, MR and deletion of chromosome, and Beckwith-Weidemann Syndrome which is a rearrangement of chromosome 11p15 and includes hemihypertrophy, Drash syndrome, which includes GU abnormalities and nephropathy, and Periman Maliformation Syndrome. Blasternal, epithelial and/or stromal elements. Basic fibroblast growth marker (bFGF) is a marker for more advanced neoplasm. Treated with actinomycin-D with vincristine and/or Doxorubicin.


OVARIAN TUMORS

Two highest risk factors are nulliparity and family history (BCRA1) on chromosome 17q21. Half of ovarian carcinomas express mutations in the tumor suppressor p53 oncogene.



Primary Ovarian Tumors

  1. Epithelial

    1. Surface cells of ovary

      1. Serous – 30% of tumors, approx 75% benign, ages 20-50, if malignant tend to occur later in life. 10-40cm,usually bilateral, increased malignancy has increased papillary projections. Contain psammoma bodies.

      2. Mucinous – occur middle life, 80% benign or borderline, more cysts of various size, up to 25 kg. Multiloculated with sticky, gelatinous fluids. May result in pseudomyxoma peritonei in which extensive mucinous fluid fills peritoneum.

      3. Endometrioid – mostly carcinomas, diff from above due to tubular glands resembling endometrium, usually co-exist with endometriosis. Solid and cystic areas, 40% bilateral.

      4. Clear cell – uncommon, solid or cystic, sheets or tubules, abundant clear cytoplasm

      5. Brenner’s tumor – uncommon, nests of transitional cells, solid or cystic, usually unilateral, vary in size from tiny to 30 cm, mostly benign, cells have coffee-bean shaped nuclei in dense, fibrous stroma.

      6. Cystadenofibroma

Clnical manifestations are the same, with lower abdominal pain and abdominal enlargement, causing GI complaints, pelvic pressure, dysuria and urinary frequency.


    1. Most common form of ovarian cancer

    2. Primarily in adults



  1. Germ cell – primarily benign cystic teratomas

    1. Arise from egg producing cells

    2. Primarily in children and teens

    3. Rare

    4. Types

      1. Dysgerminoma – ovarian counterpart of seminoma, lg vesicular cells, 75% ages 20-30, few have elevated HCG, extremely radiosensitive, lg solid with smooth surface

      2. Mature teratoma – shows differentiation towards embryonal tissue, may be solid or cystic, uncommon, always unilateral, usually ages 20-30, filled with sebaceous material and hair and possibly teeth

      3. Immature teratoma – partially of immature or fetal tissues, usually pre-pubertal to age 20s, bulky, unilateral tumors

      4. Monodermal teratomas – rare, unilateral, assoc with struma ovarii and carcinoid

      5. Yolk sac carcinoma – highly aggressive, young women under 30, large, solid, showing necrosis and hemorrhage, abdominal pain, Schiller-Duval body, AFP marker.

      6. Choriocarcinoma – placental in origin, unilateral, solid and hemorrhagic, marker beta – HCG

  2. Sex cord stromal

    1. Rare

    2. Often produce steroid hormones

    3. Types

      1. Granulose-theca cell – unilateral, estrogen secreting,juvenile form 20s and under or adult form in postmenopausal women, islands of ranulose cells with coffee bean nuclei and punched out areas called Call-Exner bodies

      2. Fibroma – most common stromal tumor, non-functioning, occurs around menopause, unilateral, fibrous, with hard, gray/white whorled surface, calcified

      3. Thecoma – rare, produce estrogen in postmenopausal women causing breast enlargement and menstrual abnormalities. Vary in size, usually rubbery, solid tumors 5-10 cm yellow to orange depending on lipid content

      4. Sertoli-Leydig Cell tumorw (Androblastoma) – extremely rare, unilateral, usually in mid 20s, androgenic, cause viralization, mimic cells of testes

Metastatic Ovarian Tumrs – most common sites are breast, large intestine, stomach and other GU organs.


DISEASES OF THE MALE GENITAL TRACT

Hypospadias – urethral opening on the ventral (underside) surface of the penis

Epispadias – urethral opening on the dorsal surface of the penis.

Both are associated with failure of testes to descend and GU abnormalities. Can cause cystitis or possible sterility due to obstruction.

Phimosis – foreskin is too tight to pull back over glans of penis. Can be congenital or due to scarring. Makes hygiene problematic and can cause infection and/or cancer.

Balanoposthitis – infection of the penis due to phimosis, or various organisms such as staph, coliforms, fungi, Chlamydia or mycoplasmas

Condyloma acuminatum – caused by HBV 6 and 11, shows koiliocytosis

Bowen’s Disease – usually affects shaft of penis and scrotum. Usually solitary, white, opaque plaque with ulceration and crusting. Sharply demarcated border and dysplasia which can progress to carcinoma. Usually in people over age 35.

Erythroplasia of Queyrat – appears on glans as single or multiple shiny or velvety plaques.

Bowenoid papulosis – indistinguishable from Bowen’s disease. Occurs in sexually active young adults. Causes multiple reddish brown lesions.

Carcinoma of the penis – rare especially among Muslims and Jews, likely due to circumcision. Usually occurs between age 40-70. Begins near glans sulcus and creates papule then progresses to ulceration, and if not treated, destruction of penis. Slow growing and usually not discovered immediately. Can metastasize to inguinal or iliac lymph nodes.

Cryptoorchidism – failure of testes to descend into scrotum. MIF controls descent to pelvis. Descent through inguinal canal to scrotum is androgen dependent. Undescended testes atrophy becoming fibrotic and can become cancerous.

Atrophy – can occur from age, female hormones, cachexia

Torsion – twisting of the spermatic cord which can cut off the blood supply. Usually due to trauma cut can occur due to absence of scrotal ligaments, abnormal attachment of the testis to the epididymis. Must be repaired in under 4 hours. MRI will show “donut sign”.

Seminoma – most common germ cell tumor, peak incidence in 40s. A small portion have cell similar to syncytiotrophoblast and will secrete HCG. Fried egg appearance. Can be anaplastic but the prognosis is usually the same.

Spermatocytic seminoma – usually occurs after age 65, rare, bilateral more often than classic form, poorly demarcated, soft, gelatinous or mucoid appearance. Small cells resemble lymphocytes, large or giant cells. Mitotic figures abundant.

Emryonal Carcinoma – most primitive form of germ cell tumor, accounts for 15-35% of tumors, very pleomorphic, may form glands, tubules and even primitive embryo-like structures.

Yolk sac tumor – contains Schiller-Duval bodies, produces large amounts of AFP

Teratomas – both mature and immature, similar to those found in females.

Choriocarcinoma – highly malignant, widely disseminated and frequently fatal, presents in adolescence and young adults, usually as small, primary, painless lesion. My be hemorrhagic and necrotic. Hemoptysis due to pulmonary involvement is common


Kathy’s review finishes



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