GIT Pathology Flashcards

Question

Answer 1

* portal hypertension due to cirrhosis or HCC * Ddx w Mallory-Weiss (assoc w pain); **esophageal varices -\> no pain** * Overlying mucosa normal, inflamed or ulcerated * Usually asymptomatic until rupture * Cause of death in 50% of patients with advanced cirrhosis

Answer 2

Meckel's diverticulum * Incomplete of involution/persistence of vitelline duct aka omphalomesenteric duct; true diverticulum, * involves **all 3 layers** of the bowel wall. * common in anti-mesenteric border * Rule of 2’s: · 2% of population · 2 feet (proximal to ileocecal valve) · 2 inches in length · 2 types of common ectopic tissue: gastric and pancreatic · 2 years is the most common age at clinical presentation · 2:1 male:female ratio * Sx: painless rectal bleeding (most common) & feces from vitelline duct * Complications * Hemorrhage (most common) and peptic ulcer * Intestinal obstruction * Diverticulitis * Perforation * Fistula

Answer 3

Celiac Sprue * young white kids * Changes more marked in proximal part of intestine: * Marked atrophy and loss of villi in duodenum (reduced area for absorption) -\> malabsorption * jejunum and ileum spared * Increased intraepithelial lymphocytes * Elongated and hyperplastic crypts * Increased number of lymphocytes, macrophages and plasma cells in lamina propria * Reversal of changes after gluten free diet * **Sx Children**: Ab distention, diarrhea, and failure to thrive * **Sx Adults**: chronic diarrhea & bloating * **Dx:** * Documentation of malabsorption * Iron deficiency anemia * Small intestine biopsy showing flattening of villi and hyperplasia of crypts, and incr intraepithelial lymphocytes * Reversal of changes and signs and symptoms after gluten free diet * Serologic tests * Anti **gliadin** and **anti endomysial** antibodies * Anti-tissue Transglutaminase **(tTG**) antibodies – Is a specific endomysial antigen. – Sensitivity of 95% and specificity of 94% in untreated patients – HLA genotype: **HLA DQ2 (majority**) and **DQ8** * C&C: long-term risk of intestinal T- cell lymphoma, small bowel carcinoma, **dermatitis herptiform due to deposition of IgA-mediated destruction of dermal papillae**

Answer 4

* Tropics & Caribbean * Symptoms appear months or even years after visit * Pathogenesis related to bacterial infection * superimposed on pre-existing small intestine injury * All parts of small intestine equally involved * Small intestine may appear near normal * Difficult to differentiate from celiac disease * **damage is more in jejunum and ileum (Pathoma)** * **Folic acid and B12 deficiencies** * Responds to antibiotics

Answer 5

Whipple Disease * Systemic disease * Gram+ actinomycete Tropheryma whippelii * More common in males (10:1) * Mucosa laden with distended macrophages in * lamina propria-contain PAS positive granules and foamy macrophages * May have granulomatous inflammation **Sx** * Signs & sx of malabsorption: weight loss, diarrhea due to effects on SI * Signs & sx of infection: fever, lymphadenopathy, polyarthritis, skin hyperpigmentation **Dx** * Rod shaped bacilli can be seen on EM * Treated with antibiotics

Answer 6

Intramural * Congenital atresias * Inflammatory conditions Extramural * hernia * volvulus * intussusception

Answer 7

* Dilation of lumen due to mucinous secretion * Lining epithelium may be adenomatous (mucinous cystadenoma) or carcinomatous (cystadenocarcinoma) * Rupture may lead to diffuse peritoneal mucin (clinically pseudomyxoma peritonei)

Answer 8

* Peritoneal studding by mucinous implants * Range • Entirely mucinous – No epithelium • Abundant mucin – Scant low grade neoplastic epithelium • Abundant malignant epithelial cells – Signet ring cells – Infiltrating columnar epithelium

Answer 9

* Most common cause of congenital intestinal obstruction * Incidence is 1 in 5000 to 1 in 8000 * M:F is 4:1 * **In 10% patients of Down Syndrome** * Absence of ganglion cells in Meissner and Auerbach’s plexus in submucosa * Rectum is always affected * Dilatation and hypertrophy proximal to aganglionic segment (congenital megacolon) **Sx:** * Delayed passage of meconium * Constipation * Abdominal distension * Diagnosed by rectal biopsy **C&C:** * Enterocolitis * Perforation and peritonitis * risk of rupture

Answer 10

Diverticular disease * Includes diverticulosis and diverticulitis * pseduo-diverticulum involving only 2 layers (mucosal & submucosal) * Common in western world * 50% in older than 60 years * Flask like structures ( 95% sigmoid colon) of mucosal outpouchings from lumen through muscular layer * more common in mesenteric border **Etiopath:** * Lack of **dietary fiber** leads to sustained bowel * contractions and increased **intraluminal pressure** -\> **Herniation of colonic wall at sites of focal defects** * Not true diverticula **Sx:** * Usually asymptomatic but some times painless bleeding * Diverticulitis – Lower abdominal pain (pain in L. iliac fossa) – Constipation, diarrhea, flatulence -\> massive hematochezia * – Fever **C&C:** * fistula & perforation * rectal bleeding (hematochezia) * iron deficiency anemia due to chronic bleeding

Answer 11

Ulcerative colitis * young white people * Extends in continuous fashion * **Starts in rectum** and then extends proximally to involve whole of the colon **(no SI involvement) -\> left lower quad pain -\> hematochezia** * Associated with Primary Sclerosing Cholangitis (**PSC**) and **P-ANCA** * HLA-DRB1 association * 10% cases back wash ileitis * Broad based ulcers -\> diffuse * Isolated islands of intervening regenerating mucosa bulge create pseudopolyps * No Fat/vitamin malabsorption **Morphology (Pathoma)** * Gross: pseudopolyps, loss of haustra, toxic megacolon * Microscopic **Left image: red friable mucosa & submucosa layers flaking off, no granuloma formation, Serosa is usually normal** * Microscopic Right image: crypt abscess (exudate; hallmark of UC), loss of goblet cells in in crypts **Sx:** stringy mucoid material of bloody diarrhea, pain due to inflammatory **Dx**: leadpipe appearance on X-Ray **C&C:** * Toxic megacolon * Back wash ileitis: mild inflammation of distal ileum * colon carcinoma

Answer 12

Crohn's Disease aka Terminal ileitis, regional ileitis and granulomatous colitis * adolescents and young adult women * More common in Jews * Associated with HLA-DR7 and DQ4 * Microscopic: T-cell mediated autoimmune disease; Dense lymphocytic infiltration (blue), deep fissure into thickened muscle, shallow ulcer (creates fistulas and strictures) * Characterized by – Sharply delimited & transmural involvement by an inflammatory process; **mouth to anus w skip lesions; terminal ileum most common; rectum least.** – Presence of non caseating granulomas – **Mucosal** fissuring with formation of fistulas-\> **knife-like fissures** * Crohn's disease involves both SI and colon **Morphology** * Granulomas & **skip lesions** * Image below right: **Mesenteric fat wraps around bowel serosa (creeping fat)** * Mesentery thickened and fibrotic * Full Wall thickened (edema, hypertrophy, fibrosis and inflammation) * Long narrow thickened segments of small intestine (unlike tuberculosis strictures which are short in length): * **radiographically- String sign (severe narrowing of loop of bowel) -\> stricture** * Sx: General to IBD * Relapsing disorder * Attacks of bloody mucoid diarrhea rare * Right Lower Quad pain, abdominal cramps, Tenesmus * Flare ups with physical and mental stress * Fever and weight loss in severe cases * **Features of malabsorption**: Loss of surface epithelium -\> loss of absorptive fx * Small intestine - features of subacute intestinal obstruction **C&C:** * Perianal fistula * increase risk of colon adenocarcinoma (higher risk in UC) **Assoc (Pathoma)** * ankylosing spondylitis * sacroileitis * polyarthritis * erythema nodosum * uveitis

Answer 13

* Seen in both CD and UC (though more common with Ulcerative colitis) * Can develop even before the onset of GI sign symptoms – Migratory polyarthritis – Sacroileitis – Ankylosing spondylitis – Erythema nodosum – Clubbing of finger tips – Sclerosing cholangitis * Increased risk of developing malignancy (CD Less than UC)

Answer 14

Pseudomembrane colitis * Associated with broad spectrum antibiotic use * Caused by Clostridium difficile * Toxin mediated damage * Toxin A = enterotoxin --\> fluid accumulation in bowel Toxin B = cytotoxin --\> decreases cellular protein synthesis & disrupts microfilament system of the cells --\> destruction of cellular cytoskeleton * Colon, particularly rectosigmoid, has raised yellow plaques * Fibrinopurulent-necrotic debris (pseudomembranes) – Surface epithelium denuded – Superficially damaged crypts distended by mucopurulent exudate which erupts to form a mushrooming cloud – Coalescence of these clouds leads to pseudo membrane formation * Sx: Watery diarrhea, Tenesmus (painful defecation), Occult blood in stool may be present --\> iron def. anemia

Answer 15

Ischemic bowel disease * Venous vs. Arterial Insufficiency * Occlusive vs. Non-occlusive Occlusive – Arterial thrombosis in SMA - systemic atherosclerosis, systemic vasculitis, hypercoagulable states, angiographic procedures – Arterial embolism in SMA- cardiac vegetations, athero- thromboembolism and angiographic procedures – Venous thrombosis- hypercoagulable states, Oral contraceptives, intraperitoneal, sepsis, post operative etc. * Non occlusive ischemia- cardiac failure, shock, dehydration * Miscellaneous- radiation, volvulus, herniation Chronic ischemic colitis * Insidious * Chronic inflammation and fibrosis * Stricture formation * Common at water shed areas – **Splenic flexure (most common)** * Red infarct due to ischemia -\> loss of blood supply -\> reperfusion * Common in elderly * Intermittent attacks of pain – **Intestinal angina (**pain due to eating & no blood circulation) & weight loss * Can mimic Inflammatory bowel disease

Answer 16

* Ulcerative colitis – Intermittent rectal bleeding, bloody diarrhea and abdominal pain – Typically continuous colonic involvement * Crohn disease – More variable symptoms than UC that include abdominal pain, fatigue, weight loss and fever – Typically patchy transmural involvement • Infectious colitis – Bloody diarrhea; nausea, vomiting, fever – Lamina propria hemorrhage, erosion and neutrophils infiltrate lamina propria and glands.

Answer 17

Infectious colitis * Amebic Colitis – Gradual dysentery, C&C: amebeic abscesses – Flask shaped ulcer with organisms engulfing red cells (organisms burrow laterally) • Typhoid – Fever, headache, abdominal pain, rash and diarrhea – Longitudinal ulcers, typically over Peyer patches • Tuberculosis – Chronic abdominal pain – Multifocal involvement, jejunum to ileum, TI most common * – Annular circular or oval ulcers, lying transversely – Single ulcers large, multiple ulcers smaller – Granulomatous inflammation, often necrotizing

Answer 18

Benign * Adenomas * Leiomyoma * Lipoma * Hemangioma * Neuroma Malignant * Adenocarcinoma * Endocrine (Carcinoid) tumor -\> chromagranin marker * carcinoid syndrome only manifests when metastasis to liver occurs (where 5HT can be pumped into Hepatic v.) * Lymphomas * GIST and other sarcomas

Answer 19

Benign * hyperplastic polyps -\> serrated appearance * adenomas (tubular, tulovillous, villous) * GIST * Lipoma * Neuroma * Angioma Malignant: **adenoma-carcinoma sequene APC -\> K-RAS -\> p53 assoc w incr COX** * adenocarcinoma * carcinoid * GIST * Kaposi * Sarcoma

Answer 20

Aka Retention polyp * Common in children \< 5 years; also seen in adults * **Rectum is most common site; hamartomatous growth** * Rarely juvenile polyposis syndrome (AD) * Usually 1-3 cm, lobulated with stalk * Lamina propria forms the bulk and encloses abundant cystically dilated glands ± inflammatory cells * Sporadic single polyps: NO MALIGNANT POTENTIAL * Juvenile polyposis syndrome: Increased risk of * malignancy * Also seen in Cowden and Bannayan-Ruvacalba-Riley syndromes (PTEN mutations)

Answer 21

Sporadic or Syndromic forms P.J. syndrome – Autosomal dominant – Multiple polyps in whole GIT – Melanotic pigmentation in mucocutaneous areas, lips, perioral areas, face, genitalia and palms * Arborizing network of smooth muscle extending into the polyp and surrounds glands * Glands are lined by non-dysplastic epithelium rich in goblet cells * No malignant potential in colon * BUT such patients are at risk of developing carcinomas of * pancreas, breast, lung, ovary and uterus

Answer 22

Neoplastic GIT polyps * Left: Raspberry appearance of polyp (single); Malignant \> 2 cm commonly from adenomatous polyp; * Pedunculated w stalk * Most common polyp is hyperplastic * Right: sessile (no stalk) -\> more flattened

Answer 23

Adenoma: * can be classified as tubular, tubulovillous, villous * pre-malignant –Left: tubular: nuclear polychromasia -\> dysplastic –Right: villous -\> complication: hypokalemia * "villous is the villain"

Answer 24

FAP * genetic defect in APC gene (5q21) * Usually tubular type * Sometimes in SI and stomach * Typically 500-2500 mucosal adenomas (minimum number for diagnosis is 100) * Carcinoma occurs in young individuals * Prophylactic colectomy is done **Sx**: asx for a long time, Osteomas & Turcot syndrome **C&C**: progression to adenocarcinoma & intussusception **Gardner Syndrome-Tubular adenomas with multiple osteomas, desmoid tumors and epidermal cysts** **Turcot Syndrome-Adenomas and CNS (gliomas)**

Answer 25

Colorectal carcinoma * Occurs in elderly individuals * Occurs in young individuals in setting of ulcerative colitis and polyposis syndromes * Risk factors: Obesity, physical inactivity, Diet low in indigestible fiber and rich in animal fat * Antioxidants protective * Usually solitary • Multiple cancers * Ulcerative colitis, polyposis syndromes and HNPCC * Proximal colon- exophytic polypoidal lesions and obstruction is uncommon * Distal colon- annular, encircling **napkin ring constrictions** **Etiopath:** * 2 major pathways: 1. Chromosomal instability pathway (85 to 90% cases) APC gene But also K-RAS, p53, DCC, hMSH2 Arise from adenoma-carcinoma sequence 2. MSI pathways (10 to 15% cases) May arise from adenomas or other lesions (SSA or HP) Defect in DNA mismatch repair genes ex. MLH1, MSH2, MSH6, PMS Sporadic (85%) or Familial (15%;) -\> HNPCC Lymphatic spread **Sx:** * Asymptomatic RIGHT SIDED- fatigue, weakness & iron deficiency anemia (Bulky & bleed easily) LEFT SIDED- altered bowel habits **Dx:** * Colonoscopy and fecal occult blood testing * Tumor marker - CEA used for checking for tumour regrowth. **C&C:** * Iron deficiency anemia in elderly man is due to GI malignancy unless proved otherwise. * incr risk for Strep bovis endocarditis

Answer 26

Aka Warthin-Lynch Syndrome * Defective DNA repair genes (AD)-MLH1 and MSH2 genes * Microsatellite instability (MSI) * Less polyps than AP * Tumors; – May be multiple; Precursor – none, sessile serrated adenoma, large hyperplastic polyps – More common on right side (proximal colon) – Mucinous histology – Increased number of tumor infiltrating lymphocytes Associated with carcinomas of other sites – Endometrium, ovaries, stomach, small intestine, biliary tract

Answer 27

Carcinoid tumour Small intestine and appendix – Most common site in GI tract; mucosal layer – Often small, occult primary tumors; salt and pepper chromatin – Can metastasize widely leading to carcinoid syndrome when liver involved • Carcinoid syndrome – Wheezing, diarrhea and flushing • Rectum – Often small and benign

Answer 28

Lymphoma presenting with the main bulk of disease in the gastrointestinal tract +/- contiguous lymph nodes – No liver, spleen or bone marrow involvement at diagnosis – Gut most common site of extranodal lymphomas • Sites – Stomach (50%)\> small intestine (37%)\> colon and rectum **B-CELL** **– Mucosa associated lymphoid tissue (MALT)-type is common • Low grade and indolent** **– High grade (DLBCL) also common** – Mantle cell (lymphomatous polyposis) – Burkitt and Burkitt-like lymphoma – Follicular lymphomas and other nodal types of lymphoma – Immunodeficiency related • Post-transplant; acquired (AIDS); congenital immunodeficiency syndrome * IBD asst. * Methotrexate therapy asst. **T-CELL** **– Enteropathy associated (EATL) including ulcerative** **jejunitis** - celiac disease background = T cell lymphoma – Other types not associated with enteropathy – Gamma delta hepatosplenic T-cell lymphoma – Angioimmunoblastic-type T cell lymphoma – Extranasal NK-cell and NK-cell like T-cell lymphoma Anaplastic large cell (Ki-1) lymphoma True histiocytic lymphoma (sarcoma) Hodgkin’s disease Leukemic infiltration of GI tract

Answer 29

* Age: 27-84 (Mean 60); M:F=1.5:1 * Symptoms – Non-specific symptoms: Dyspepsia: 80%; Pain abdomen, Nausea vomiting, Weight loss: 45% * Examination – Abdominal mass: 25% – Blood loss: acute 20%, chronic 10%

Answer 30

Appendicitis * Underlying obstruction of the lumen in 50-80% cases: lypmhoid hyperplasia (children) or fecalith (adults) * Pathogenesis unclear in non obstructive cases * inflammation of appendicoeal wallMild fever, Leukocytosis (Neutrophilia ) **C&C:** – Perforation – Peritonitis – Periappendiceal abscess – Liver abscess – Bacteremia

Answer 31

**Sx** - Right upper quadrant pain – Anorexia – Yellowish discoloration of skin (Jaundice) – Pruritus – Fever – Mental confusion – Easy bruising **Acute Signs** Icterus (Jaundice) Hepatomegaly Tenderness in right hyochondrium Peripheral edema **Chronic Signs** * Splenomegaly * Palmar erythema * Spider angiomas * Gynecomastia and testicular atrophy * Dupuytren contracture * Parotid enlargement * Clubbing * White nails (leukonychia) * Muscle wasting

Answer 32

Degeneration - Ballooning: irregularly clumped cytoplasm, assoc w hepatitis – Feathery: fine foamy cytoplasm because of detergent action of bile salts • Steatosis Macrovesicular * Single fat vacuole displaces nucleus to periphery * Alcohol * NASH/NAFLD * Malnutrition Microvesicular * multiple vacuoles, nucleus is central * Acute fatty liver of pregnancy * Reye's syndrome * Drugs Necrosis: focal, bridging, massiveor sub-massive Apoptotic hepatocyte: Councilman's bodies/Acidophilic bodies Inflammation-commonlycausedbyTcells Regeneration – thickening of hepatocyte cords, mitosis and some disorganization Fibrosis

Answer 33

Liver cirrhosis Defined by 3 characteristics (Trichrome stained image before) – Bridging fibrous septa – Parenchymal nodules created by regeneration – Architectural disruption * **Etiology:** * Alcoholic liver Disease (most common) * Viral hepatitis * NASH * Biliary tract diseases * Hereditary hemochromatosis: liver damage via free radicals -\> incr risk of HCC * Wilson disease * α1 antitrypsin deficiency * Cryptogenic cirrhosis **Pathogenesis:** * Diffuse liver process with fibrosis and conversion of normal architecture into structurally abnormal nodules -\> representing I**to cells (stellate cells)** secreting **TGF-Beta** to stimulate **regeneration & fibrosis.** * Stage in evolution of many chronic liver diseases with consequences unrelated to the primary etiology * Based on nodule sized, often classified as micronodular (\<3mm) or macronodular (\>3mm) * hepatocytes die -\> ECM deposition -\> vascular fibrosis **Sx:** May be silent * Non specific signs and symptoms * Usually features of Portal hypertension and Liver failure **Signs of chronic liver disease: jaundice, ascites, parotid swelling, palmar erythemia, clubbing, Depuytren’s contracture, spider angioma & gynecomastia (estrogen), ascites, portal hypertension (cirrhosis specific)** **Dx:** Prothrombin time (PT) [not PTT] is used to assess the coagulopathy due to liver disease. This is because prothrombin has a short half life and is first to disappear upon onset of liver disease **C&C:** * Significantly increased risk of hepatocellular carcinoma (HCC) * **Portal hypertension** * hepato-renal syndrome * congestive splenomegaly * decr protein synthesis leading to hypoalbuminemia & coagulaopathy * Decreased detox liver fx * Asterixis: flapping tremor, or liver flap * Decr estrogen metabolism leading to gynecomastia, spider angioma, palmar erythema * Jaundice

Answer 34

Common in females Prominent interface plasma cell hepatitis High titers of IgG Anti nuclear and anti smooth muscle antibodies Anti LKM (Liver kidney microsomal type) antibodies Respond to immunosuppressive therapy

Answer 35

* May occur even with moderate intake * Patterns of liver disease – Hepatic steatosis – Alcoholic hepatitis (Inflammation and steatohepatitis) – - - Variable amount of fibrosis to cirrhosis * Mallory hyaline/ bodies- * **Tangled skeins of intermediate filaments -\> damaged IF** * Seen as eosinophilic cytoplasmic inclusions * Initially enlarged yellow fatty liver which progresses to fibrotic, fatty and shrunken * Mainly micronodular, macronodules may be formed at a later stage * **Dx:** AST \> ALT * AST is mitochondrial enzyme and preferentially incr when alcohol causes damage.

Answer 36

Primary Biliary Cirrhosis Disease of middle aged women **Etiopath:** * **auto**Immune mediated: **Anti mitochondrial antibodies in 90%** * Chronic, progressive and can progress to cirrhosis * Non suppurative, granulomatous destruction of medium sized bile ducts * Death usually occurs because of liver failure Gross image: Green indicates bile obstruction Microscopic image: Portoportoseptal fibrosis * Left: Regenerating bile ducts with Fibrosis in bw Right: Destruction of epithelium (long thin arrow), granuloma formation (curved arrow) **Sx:** hyperbilirubinemia, pruritus, jaundice **Dx:** * Detection of anti-mitochondrial Ab * Elevated serum **ALP** (alkaline phosphatase) - signifies **obstructive** liver disease **C&C**: hepatic decompensation, Sjorgren syndrome, scleroderma, thyroiditis, rheumatoid arthritis, Raynaud phenomenon & celiac disease.

Answer 37

Due to partial or total bile duct obstruction Tumors, strictures, gallstones implicated

Answer 38

* Inflammation, concentric fibrosis **(onion-skinning)** and dilatation of intra and extra hepatic ducts * uninvolved regions are dilated resulting in "beaded" apperance on imaging. * ERCP- beading of the biliary tree * **P-ANCA** in about 80% cases * Associated with **chronic ulcerative colitis ** **Sx:** * obstructive jaundice **C&C** * cirrhosis is a late complication * incr risk for colangiocarcinoma

Answer 39

Wilson disease * Autosomal recessive disorder (Gene on chromosome 13) * Presentation is rare before 6 years of age * Characterized by accumulation of toxic levels of Cu in Liver, Brain and Eyes * BRAIN: * Deposited in basal ganglia -\> neuro manifestations * EYES: * Deposits of copper in Descemet membrane of corneal limbu aka. Kayser Fleischer ring * In some sun flower cataracts * BLOOD: hemolysis * In liver variable morphology – Fatty change – Acute hepatitis – Chronic hepatitis-Ultimately cirrhosis – Rarely massive necrosis Investigations – Decreased serum ceruloplasmin levels – Increased hepatic Cu (\>250μgm/gm dry weight of liver is diagnostic) – Increased urinary copper excretion

Answer 40

Associated hepatic syndromes are variable – Neonatal hepatitis with or without cholestasis – Smoldering chronic inflammation – Cirrhosis at early age Hepatocellular carcinoma

Answer 41

Changes like ALD in non drinkers Steatosis with or without hepatitis Risk factors – Obesity – Insulin resistance – Hyperlipidemias Usually asymptomatic – Mild elevation of serum transferases – May lead to cirrhosis

Answer 42

Commonly associated with Oral Contraceptive Pill & anabolic steroids May spontaneously hemorrhage and may undergo malignant transformation May be fatal during pregnancy Clinical presentation – Acute (about 50%, Mortality 20%) – Pain abdomen – Shock and hemorrhage » Hemorrhage into tumor or peritoneal cavity – Episodic pain or discomfort Normal serum AFP * Rx: Stop OCP or steroids and/or resect

Answer 43

No relation to OCP More common in women Most often small, may be very large Central scar on CT, MRI Normal liver spleen scan Resect if symptomatic or uncertain diagnosis

Answer 44

* Hepatocellular carcinoma (hepatoma) – Variants include fibrolamellar * Cholangiocarcinoma * Hepatoblastoma * Sarcomas * Lymphoma • Metastasis – Most commonly colonic

Answer 45

**Most common liver tumor in neonates and children** at ~ 18 months (birth to 40 mths) Morphology (2 types): 1. Epithelial & 2. Combined epithelial-mesenchymal components 1. Epithelial – Fetal * Small uniform cells in cords – Embryonal and fetal * Fetal components with sheets of round, fusiform cells * Arranged in rosettes, cords and ribbons 2. Combined epithelial-mesenchymal components – Epithelial with mesenchymal elements • Fibrous tissue • Osteoid or cartilaginous differentiation Sx: Hepatomegaly or abdominal mass, jaundice rare Associated with Beckwith-Wiedemann syndrome, Down syndrome, familial polyposis coli, hemihypertrophy, renal malformation and various cytogenetic abnormalities AFP elevated in 80-90% Rapid growth, poor prognosis, spreads to lungs, LN and peritoneum Transplantation considered if no metastasis

Answer 46

HCC aka Hepatoma * Majority cirrhosis associated * Global distribution strongly related to prevalence of HBV * Highest frequencies in Taiwan, Mozambique and China * Male preponderance * Usually in adults * Children with metabolic liver disease **Morphology** * Are usually paler than surrounding hepatic parenchyma * Strong propensity for invading vascular channels (portal vein, IVC) Histology – Well differentiated to poorly differentiated – Trabecular, sinusoidal or pseudoacinar pattern – Bile production by tumor cells, cytoplasmic inclusions – Usually cirrhosis in adjacent liver parenchyma **Etiopath**: * **High incidence** in **Asia & Japan;** most common primary malignant tumour of liver * **Cirrhosis** * **Hepatitis**: integration of viral DNA into genome * **Alcohol** * **Aflatoxin: p53 transversion** **Sx** 1. Usually masked by underlying liver disease 2. Ill defined upper abdominal pain, fatigue, cachexia, asterixis **Dx** * Raised **AFP** levels in 60-75% (markedly increased) * Diagnosis-FNAC, biopsy * Fibrolamellar variant has better prognosis * Majority are no surgical candidates – Multicentricity – Parenchymal dysfunction **C&C** * Budd-Chiari syndrome: thrombosis of hepatic v. * tendency for hematogenous spread

Answer 47

M=F Usually single hard tumor with fibrous bands traversing through it Well differentiated polygonal cells in cords or nests, separated by fibrous septa Not associated w alcoholism Has a better prognosis

Answer 48

Carcinoma of bile duct origin Normal serum AFP Majority have no well known risk factors Remainder: – Sclerosing cholangitis – Cystic dilatations/ malformation of biliary system – Gallstones – Chemicals: benzidene, nitrosamines – Parasitic infections: Clonorchis sinensis & Opisthorchis viverini * Intrahepatic * Extrahepatic – Most commonly at the hilum of the liver – Upper third 60% – Middle third 20% – Lower third 15% – Diffuse 5% Adenocarcinoma with extensive fibrosis (markedly desmoplastic) Cut surfaces - Firm and Gritty **Sx** Malaise Weight loss Jaundice Cholangitis – Charcot triad: Jaundice, fever, chills – Raynaud pentad: Charcot triad + hypotension and mental status changes -\> Poor prognostic sign Clinically silent for long periods More likely to spread beyond liver than HCC Prognosis is poor: usually death within 6 months

Answer 49

Risk factors * Cholesterol stones: **Female, fat, forty, fertile;** radiolucent * Westernized world * Elderly * Female (OCP & pregnancy) * obesity * rapid wt reduction * GB stasis * Inborn error of Bile salt metabolism * Hyperlipidemia syndromes * Pigment stones: radio-opaque * Asians * Rural \> urban * **Chronic hemolytic syndromes** * **Biliary infections** * GI disorders: **Crohn's disease** or **ileal resection** or bypass, CF of pancreas **Etiopath** * GB hypomotility * Cholesterol nucleation * mucus hypersecretion traps crystals **Dx:** **Murphy’s test (palpate -\> pain with inspiration)** **C&C** - Empyema – Perforation – Fistulas – Cholangitis – Pancreatitis – Gallstone ileus – Increased risk of carcinoma

Answer 50

Etiopath * Empyema * Perforation * Fistulas * Cholangitis * Pancreatitis * Gallstone ileus * Increased risk of carcinoma Sx: may be surgical emergency * Pain in R. hypochondrium or epigastrium * Assoc w fever, nausea and vomiting

Answer 51

* Can be sequel of repeated bouts of acute cholecystitis * BUT more commonly no antecedent attacks of acute cholecystitis * Role of gall stones not clear * Microorganisms can be cultured from bile in 1⁄3 * of patients * Rarely extensive **dystrophic calcification** aka **PORCELAIN GALL BLADDER** -\> incr risk of GB carcinoma * outpouchings called Rokatansky-Aschkoff sinuses

Answer 52

GB carcinoma Most common in seventh decade of life Slightly more common in females Gallstones in 60-90% patients (Chronic cholecystitis w dystrophic calcifications) Other risk factors: Pyogenic and parasitic infections of the biliary tract Carcinogenic derivatives of bile may play a role Mostly adenocarcinoma Most have invaded or spread to liver at diagnosis

Answer 53

Multisystem disease – Pancreas affected in 85-90% – Pulmonary changes most serious – Superimposed infections **Dx: **Must meet 2 criteria – 2 disease causing mutations in CFTR – Abnormal nasal potential difference Gene sequencing is gold standard

Answer 54

Acute pancreatitis **Morphology** Focal fat necrosis in pancreas and peripancreatic tissues and abdominal cavity Ca++ deposition in these areas – Appear radiopaque on radiographs Severe cases – Necrosis of pancreatic tissue (acini, ducts and islets) – Hemorrhage (Hemorrhagic pancreatitis) **Etiopath** 80% cases associated with GALLSTONES and ALCOHOLISM Other causes: I GET SMASHED -\> activated enzymes -\> Acute onset of abdominal pain due to enzymatic necrosis and inflammation of pancreas **Sx** * Pain can vary from mild to very severe * Usually severe epigastric pain with nausea and vomiting * Constant, intense and referred to upper back **Dx** * Grey-Turner sign: subq bruises on side of abdomen due to hemorrhage * Cullen’s sign: peri-umbilical bruising & edema * Lipase is most specific marker **C&C** * Leucocytosis * DIC * Hemolysis * Peripheral vascular collapse * Shock with ATN, ARDS * Hypocalcemia, tetany * Raised amylase levels in first 24 hours * Followed by lipase with in 72-96 hours * Complications * – ARDS * – ATN * – Pancreatic abscess

Answer 55

Chronic pancreatitis Repeated bouts of mild to moderate pancreatic inflammation with loss of pancreatic parenchyma and its replacement by fibrous tissue **Morphology** * Gross: shrunken pancreas due to fibrosis * Micoscopic: lymphocytic inflammation; squamous metaplasia Etiology: acute pancreatitis, alcohol, pancreas divisum Other causes very rare – Tropical pancreatitis, Hereditary pancreatitis, CFTR (mutation associated) **Sx** Repeated attacks of moderately severe pain or persistent abdominal and back pain Possible progression to pancreatic insufficiency and diabetes – Features of malabsorption, corrected by pancreatic enzyme supplements **Dx:** X-ray for calcifications and C&C: diabetes, cancer, duct stones, pseudocysts

Answer 56

Carcinoma of the head of the pancreas Fifth most common cause of death in U.S. Only convincing association is with smoking Proposed risk factors like alcohol, diet rich in fats inconsistent Strong association with familial relapsing pancreatitis (very rare itself Location – Head 60-70% – Body 5-10% – Tail 10-15% Majority ductal-type adenocarcinomas – Dense stromal fibrosis (desmoplasia) – Propensity for perineural invasion **Sx** * Carcinoma of head of pancreas/ampulla obstructs bile flow – Obstructive jaundice (painless jaundice) * Majority silent until till late * Steatorrhea * Pain is usually first symptom (because of invasion posterior abdominal wall and nerves * Trousseau’s sign (migratory thrombophlebitis) in 10: Release of platelet activating factors and procoagulantsfrom tumor **Dx** Tumour marker for screening: CA19-9 C&C: •perineural invasion -\> pain •Trousseau’s

Answer 57

Usually benign, solitary Arise from ß cells – Signs and symptoms because of hypoglycemia – Hypoglycemia accentuated by fasting and relieved with intake of glucose – Low blood glucose levels Clinically may manifest as Whipple triad – Documentation of low blood sugar – Presence of symptoms – Reversal of symptoms when the blood sugar normalized Insulin levels are increased

Answer 58

Gastrin producing endocrine tumors Can also arise in duodenum (with similar frequency as in pancreas) and peripancreatic tissues Hypergastrenemia Multiple ulcers- esophagus, stomach, duodenum & jejunum Ulcers are refractory to conventional treatment Result in Zollinger Ellison Syndrome – Multiple duodenal peptic ulcers – Prominent gastric rugal folds due to incroxyntic gland mass – Steatorrhea Many locally invasive or metastatic at diagnosis Associated with MEN-I **Dx** +Secretin test (secretin normally released due to increased gastric juices)

Answer 59

Almost exclusively in women Mostly in tail Not connected to the ductal system Tall columnar mucin secreting cells Cytology-Benign-Borderline-Malignant + invasion Characteristic feature is ovarian type stroma