PATHOLOGY: DISEASES OF THE SMALL AND LARGE INTESTINE DR.

CAGAMPAN
I. CONGENITAL ANOMALIES MECKEL’S DIVERTICULUM remnants of vitelline duct (b/w umbilicus and top of bladder) – antimesenteric portion of bowel located 30 cm. from ileocecal valve small pouch or blind segment containing layers of small bowel may contain heterotopic rests of gastric/ pancreatic tissue, may ulcerate/bleed “true diverticulum” (with 3 layers of normal bowel wall)

HIRSCHSPRUNG’S DISEASE (Congenital Aganglionic Megacolon) - arrest of migrating neural crest cells (ganglion cells) along the bowel - lack of both Meissner’s submucosal and Auerbach’s myenteric plexus - usually affect the rectum and proximal segments (rectosigmoid) - 1 in 5,000 to 8,000 LB, 4:1(M:F ratio) - manifest early after birth by failure to pass meconium followed by obstructive constipation and abdominal distention - may be seen in 10% of cases of Down’s syndrome - secondary enterocolitis, F & E imbalance, perforation - fxnal type of obstruction (distal portion= contracted; proximal portion= dilated/ganglionic) - Sxs: irritability, failure to suck, failure to pass meconium, abdominal distention - Tx: temporary colostomy, resection

ATRESIA, STENOSIS, IMPERFORATE ANUS Atresia is a condition in which a body orifice or passage in the body is abnormally closed or absent.  Duodenal atresia- most common Stenosis is an abnormal narrowing in a blood vessel or other tubular organ or structure. Imperforate anus is a defect that is present from birth (congenital) in which the opening to the anus is missing or blocked.

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II. VASCULAR DISORDERS a. ISCHEMIC BOWEL DISEASE i. PREDISPOSING FACTORS 1. Arterial thrombosis: AS, Systemic Vasculitis, Angiographic procedures 2. Arterial embolism: cardiac vegetations TRANSMURAL INFARCTION MURAL AND MUCOSAL INFARCTION involve all visceral layers (entire - may be multifocal or bowel wall) continuous follows total occlusion of a - hemorrhagic, thickened mesenteric artery (sup/inf) mucosa and submucosa, occurs at splenic flexure and superficial ulcerations, mesenteric/hypogastric watershed absence of inflammation areas  Mural- mucosa and intensely congested and dusky submucosa purple, hemorrhagic lumen contain  Mucosal- mucosa only bloody mucus may undergo gangrene and perforation Hx: (+) architecture, (-) cell detail

CHRONIC ISCHEMIA in chronic vascular insufficiency, segmental and patchy Mucosal inflammation and ulceration, fibrosis stricture Ssx: acute abdomen, rapid onset, absent bowel sounds, bloody diarrhea, N.V. 50-75% mortality rate Tx: resection

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3. Venous thrombosis: hypercoagulable states 4. Nonocclusive ischemia: cardiac failure, shock 5. Miscellaneous: radiation, volvulus, strictures, hernias ANGIODYSPLASIA HEMORRHOIDS tortous dilatations of - most common cause of submucosal and mucosal lower GI bleeding vessels - Variceal dilatations within mostly in cecum or right submucosa of anal and th colon, 6 decade of life perianal plexuses (elderly) - 5% of general population, 20% of lower GI bleeding pregnancy, chronic hemorrhage may be chronic, constipation intermittent, acute and - Internal (superior H plexus); massive external (inferior H plexus)

III. ENTEROCOLITIS  DIARRHEA- Increase in stool mass, frequency or fluidity MAJOR CAUSES OF DIARRHEAL ILLNESSES Secretory Diarrhea 1. Infectious: viral damage to mucosal epithelium - Rotavirus - Caliciviruses - Enteric Adenoviruses - Astroviruses 2. Infectious: enterotoxin mediated - Vibrio cholera - Escherichia coli - Bacillus cereus - Clostridum perfringens 3. Neoplastic - Tumor elaboration of peptides, serotonin, prostaglandins - Villous adenoma in distal colon (nonhormone mediated) 4. Excessive Laxative Use Osmotic Diarrhea - Disaccharidase (Lactase) deficiencies - Lactulose therapy (for hepatic encephalopathy) - Prescribed gut lavage for diagnostic procedures - Antacids ( MgSO4 and other magnesium salts) - Primary bile acid malabsorption Exudative Diseases 1. Infectious: bacterial damage to mucosal epithelium - Shigella DISEASE DESCRIPTION -

DYSENTERY- low volume, painful, bloody diarrhea - Salmonella - Campylobacter - Entamoeba histolytica Idiopathic Inflammatory Bowel Disease Typhlitis (neutropenic colitis in the immunosuppresed) - Defective intraluminal digestion - Primary mucosal cell abnormalities - Reduced small intestinal surface area - Lymphatic obstruction - Infectious: impaired mucosal cell absorption o Giardia lamblia infection Decreased intestinal transit time - Surgical reduction of gut length - Neural dysfunction, including irritable bowel syndrome - Hyperthyroidism - Diabetic neuropathy - Carcinoid Syndrome Decreased motility (increased intestinal transit time) - Small intestinal diverticula - Surgical creation of a “blind” intestinal loop - Bacterial overgrowth in the small intestine MISCELLANEOUS INTESTINAL INFLAMMATORY DISORDERS - HIV enteropathy - Post bone marrow transplant, graft vs. Host disease

2. 3. Malabsorption

Deranged motility

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MORPHOLOGY S/Sxs COMPLICATIONS

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INFECTIOUS ENTEROCOLITIS NECROTIZING ENTEROCOLITIS (NEC) PSEUDOMEMBRANOUS COLITIS (Antibiotic-Associated Colitis) acute necrotizing inflammation of small - Acute colitis with formation of “pseudomembrane” in and large intestine injured mucosa in neonates, premature or LBW - Caused by toxins of C. difficile in first 3 months(2-4 day-old), formula fed - Other causes: Salmonella, C. perfringens type A and S. result from combination of ischemic injury, aureus rd pathogenic colonization, excess protein - Follow course of broad spectrum antibiotic therapy (3 substrate in intestinal lumen or functional generation cephalosporins) immaturity of neonatal gut Edema, hemorrhage and necrosis of - superficial erosion of mucosa with “mushroom” cloud of terminal ileum and ascending colon adherent pseudomembrane of fibrinopurulent exudate Abdominal distention, diarrhea, - acute and chronic diarrhea, confirmed by detection of C. tenderness, ileus or frank hemorrhage difficile cytotoxin in stool Gangrene, perforation, sepsis, shock

IV. MALABSORPTION SYNDROMES - suboptimal absorption of fats, vitamins, proteins, CHO, electrolytes, mineral and water - passage of abnormally bulky, frothy, greasy, yellow or gray stools; with weight loss, abdominal distention, borborygmi and muscle wasting - multi-system effect DISEASE CELIAC SPRUE/NON-TROPICAL SPRUE TROPICAL SPRUE (POSTINFECTIOUS SPRUE) DESCRIPTION - mucosal lesions of SI and impaired absorption which - Common in Carribean, Central and South improves on withdrawal of wheat gliadins from diet Africa and America, Southeast Asia, India - wheat, oat, barley, rye - Endemic or epidemic outbreak - genetic susceptibility and immune mediated intestinal - Implicate bacterial overgrowth of injury enterotoxigenic organisms (E. coli and - 90-95% express DOW2 Haemophilus sp.) - Increase risk of development of intestinal lymphomas MORPHOLOGY - diffuse enteritis with marked atrophy of villi and chronic - variable intestinal changes, normal to severe inflammation of lamina propria, duodenum and proximal enteritis jejunum mostly affected - affect all levels of SI, megaloblastic, changes - Increased intraepithelial CD8 T-lymphocytes in epithelial cells -

WHIPPLES DISEASE rare systemic condition which may involve the intestine, CNS and joints in whites; 4th to 5th decade; M10:F1

S/Sxs

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diarrhea, flatulence, weight loss

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diarrhea, malabsorption

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distended macrophages in lamina propria of SI with PAS positive granules and rod-shaped bacilli by EM; Tropheryma whippelii – Gm+ Actinomycete; cannot culture involvement of LN with lymphatic dilatation, in synovial membranes, brain, cardiac valves no inflammation diarrhea, malabsorption and weight loss for years polyarthritis, CNS complaints, etc.

Dx

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Malabsorption Intestinal lesion by SI biopsy Improvement in signs and symptoms and SI biopsy after gluten-free diet Antibiotics antibiotics

Tx IRRITABLE BOWEL SYNDROME (IBS) - Chronic relapsing abdominal pain, bloating, changes in bowel habits - PATHOLOGY: psychologic stressors, diet, abnormal GI motility - CLINICAL FINDING: 20 – 40 y.o. - PATHOLOGIC FINDING: normal GIT V. IDIOPATHIC INFLAMMATORY BOWEL DISEASES ETIOLOGY AND PATHOGENESIS: 1. Genetic Disposition o NOD2 – susceptibility gene in crohn’s o 50% concordance rate in monozygotic twins (CD) o 16% concordance rate in monozygotic twins (UC)

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DIAGNOSTIC CLINICAL CRITERIA: o abdominal pain or discomfort at least 3 days per month over 3 months o improvement with defecation o change in stool frequency and form

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Mucosal Immune Responses o polarization of helper T-cells to the TH1 type (CD) o TH2-mediated disease in UC Epithelial defects- epithelial tight junction-barrier function Microbiota

DISEASE DESCRIPTION

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CROHN’S DISEASE (REGIONAL ENTERITIS) May affect any portion (common: ileum and colon) of the bowel (“skip lesions”) 1-3 per 100,000, any age (more in 2-3 decade and 6th decade) More in females creeping fat string sign in X-ray(narrowed lumen due to fibrosis) skip lesions (with intervening normal bowel) cobblestone appearance of mucosa linear serpentine ulcers thick, rubbery, fibrosed intestinal wall fissures and fistula formation sharply demarcated transmural bowel involvement Mucosal inflammation; crypt abscess, ulceration Chronic mucosal damage: villus blunting, crypt destruction, pyloric or paneth cell metaplasia (diagnostic factor) in colon Transmural inflammation with lymphoid aggregates Non-caseating granulomas in 50% of cases (sarcoid-like) attacks of diarrhea, fever, abdominal pain alternating with asymptomatic period of many months attacks precipitated by emotional stress fibrosing strictures, fistulas, malabsorption of protein, vit.B12 and bile salts extraintestinal manufestations: ankylosing spondylitis, polyarthritis, sacroiliitis 5-6x increased risk for development of CA

GROSS

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ULCERATIVE COLITIS Ulceroinflammatory disease limited to colon only Affects only mucosa and submucosa Continuous affectation of colon starting at rectum going proximally 4-6/100,000; F>M; 20-25 yrs onset of disease Continuous lesions, pancolitis if severe, broad-based ulcers, pseudopolyps, toxic megacolon

MICROSCOPIC

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Mucosal inflammation of mononuclear infiltrates in continuous manner, may show dysplastic changes Healed lesions show fibrosis

S/Sxs

COMPLICATIONS

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Relapsing attacks of bloody, mucoid diarrhea With interspersed asymptomatic period over months/years Attacks and flare-ups precipitated by stress Increased risk of malignancy (marked increase risk compared to Crohn’s)

*Additional table of comparison from doc’s lecture ppt Feature CROHN DISEASE MACROSCOPIC Bowel region Distribution Stricture Wall appearance MICROSCOPIC Inflammation Pseudopolyps Ulcers Transmural Moderate Deep, knife-like Ileum ± colon Skip lesions Yes Thick

ULCERATIVE COLITIS

Lymphoid reaction Fibrosis Serositis Granulomas Fistulae/sinuses CLINICAL Perianal fistula Fat/vitamin malabsorption Malignant potential Recurrence after surgery Toxic megacolon

Marked Marked Marked Yes (∼35%) Yes Yes (in colonic disease) Yes With colonic involvement Common No

Moderate Mild to none Mild to none No No No No Yes No Yes

Colon only Diffuse Rare Thin Limited to mucosa Marked Superficial, broad-based

INDETERMINATE COLITIS - extensive pathologic and clinical overlap between CD and UC - diagnosis not possible in 10% - Perinuclear anti-neutrophil cytoplasmic antibodies – (+) in 75% of patients with UC, but only 11% in CD - Antibodies to Saccharomyces cereveciae often positive in CD COLITIS-ASSOCIATED NEOPLASIA - long term complication of UC and CD - Risk increases sharply 8 – 10 years after onset - Pancolitis > left-sided colitis - Greater frequency and severity of active inflammation VI. SIGMOID/ COLONIC DIVERTICULOSIS A blind pouch protruding from GIT lined by mucosa that communicates with the lumen of the gut Congenital (all 3 layers) or acquired (lack of muscularis propria) In >60 y/o, are multiple “false diverticulitis” PATHOGENESIS: o 2 FACTORS: 1. Focal weakness in colonic wall 2. Increased intraluminal pressure MORPHOLOGY: o Small, flask-like outpouchings, 0.5-1.0 cm in diameter, in sigmoid alongside taenia coli o Thin wall with flattened or atrophic mucosa, compressed submucosa and attenuated or absent muscularis propia SIGNS AND SYMTPOMS: o mostly asymptomatic o 20% develop cramping, abdominal discomfort, constipation, diarrhea, distention COMPLICATIONS: o Diverticulitis o may perforate  pericolic abscess, sinus tract formation, peritonitis

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VII. BOWEL OBSTRUCTION (mechanical obstructions) HERNIAS ADHESIONS - Weakness or defect in wall of - Most common mech obs peritoneal cavity with protrusion of a - results from infections, previous pouch-like, serosa-lined sac of surgical procedures, endometriosis peritoneum (peritonitishealsadhesions) - Inguinal and femoral canals, umbilicus - Fibrous bridges create loops and in surgical scars herniations strangulation - Visceral segments may protrude and obstruction herniateincarceration and strangulation

INTUSSUSCEPTION - telescoping of 1 segment into another  obstruction  infarction - in infants – usually normal GIT - in adults – usually due to an intraluminal mass (suspect malignancy)

VOLVULUS - complete twisting of a loop of bowel about its mesenteric base obstruction infarction

VIII. TUMORS - 70% of cases – AdenoCA nd ranks 2 to lung CA A. NON-NEOPLASTIC POLYPS - Result from abnormal mucosal maturation, inflammation or architecture - No malignant potential - Represent 90% of epithelial polyp - 60 y/o or older

1. INFLAMMATORY POLYPS - e.g. solitary rectal ulcer syndrome - Triad of rectal bleeding, mucus discharge and inflammatory lesion of rectal wall - Cause: impaired relaxation of anorectal sphincter that creates a sharp angle at the anteior rectal shell leading to abrasion and ulceration of mucosa polyp

2. HYPERPLASTIC POLYP - Small (<5 mm in diameter), nipple-like protrusion from mucosa - 6-7th decade; single or multiple; at rectosigmoid - Composed of well-formed glands and crypts lined by non-neoplastic epithelial cells with a serrated epithelial profile

B. NEOPLASTIC EPITHELIAL LESIONS (ADENOMAS) - Small pedunculated or large sessile polyp - Prevalence rises with age; M=F - Encounter all degrees of dysplasia in adenomas - Malignant risk correlated with: o Polyp size o Architecture o Severity of dysplasia

C. FAMILIAL SYNDROMES

1. TUBULAR ADENOMA - 75% show tubular architecture; most common - Mostly found in colon - Smooth contour to coarsely lobulated; pedunculated or sessile - 1-2.5 cm, branching glands lined by tall columnar epithelium with fibromuscular and vascular stalk devoid of neoplastic covering epithelium - May see all degrees of dysplasia - CA in situ, intramuscular CA or frank invasive CA (stalk invasion) 1. FAMILIAL ADENOMATOUS POLYPOSIS

2. VILLOUS ADENOMA - 50% villous architecture; 1% of adenomas - Rectum and rectosigmoid - Usually large and sessile (up to 10 cm in diameter) - Velvety or cauliflower-like masses projecting above normal mucosa - Finger-like papillae lined by dysplastic columnar epithelium with scant lamina propria - If >4 cm risk of CA increases(40%) - Causes mucoid diarrhea d/t vasopeptides 2. GARDNER’S SYNDROME - FAP + multiple osteomas,

3. HAMARTOMATOUS POLYPS A. JUVENILE/RETENTION POLYP - are hamartomatous - In children <5 y/o; 80% occur in rectum - Large; 1-3 cm, rounded, smooth, with a stalk - Cystically dilated glands in abundant lamina propria with inflammatory cells/ulceration * Choristoma- benign looking normal tissues found in abnormal locations B. PEUTZ-JEGHERS POLYPS (Syndrome) - are hamartomatous, large and pedunculated with a firm lobulated contour, connective tissue and smooth muscle surround abundant glands - multiple poyps, melanotic mucosal and cutaneous pigmentation around the lips, oral mucosa, face, genitalia and hands - polyps do not become malignant but patient with syndrome have increased risks of development of CA of pancreas, breast, lung, ovary and uterus C. COWDEN SYNDROME AND BANNAYAN-RUVALCABA-RILEY SYNDROME D. CRONKHITE-CANADA SYNDROME 3. TUBULOVILLOUS ADENOMA - Contain 25-30% villous architecture - 5-10% of adenomas - Intermediate between tubular and villous adenomas - SIGNS AND SYMPTOMS: rectal bleeding, anemia

3. TURCOT’S SYNDROME - FAP + CNS tumor

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develop 500-2,500 colonic adenomas that carpet the mucosa minimum of 100 polyps for diagnosis of FAP Mostly tubular type 100% chance of progression to CA autosomal dominant; onset nd rd at 2 -3 decade Prophylactic colectomy (develop CA in 10-15 yrs)

epidermal cysts, fibromatosis, dental abnormalities, duodenal and thyroid CA

D. SMALL INTESTINE NEOPLASMS - Mostly benign: leiomyomas, adenomas, lipomas - Malignant lesions are rare : AdenoCA and Carcinoids - Adenocarcinomas presents as fungating or napkin-ring like constrictions o Most arise in duodenum, presenting as obstructive jaundice E. COLORECTAL CARCINOMA GROSS - 98% are adenoCA, arise from adenomatous polyps; 60-70 y/o LEFT SIDED COLONIC CA RIGHT SIDED COLONIC CA - More common in affluent countries - annular, encircling lesions; - polypoid, fungating masses - PREDISPOSING DIETARY FACTORS: napkin-ring constrictions that extend along one wall of o Low fiber diet - S/Sxs: occult bleeding, changes in the cecum or ascending colon o High content of refined sugar bowel habit or crampy, left - S/Sxs: fatigue, weakness, Fe o High fat content abdominal pain deficiency anemia o Decreased intake of protective micronutrients - MORPHOLOGY: o 25% - cecum or ascending colon (right side) o 25% - rectum or ascending sigmoid o 25% - descending colon and proximal sigmoid - Two important prognostic factors: o Depth of invasion of tumor o Presence or absence of lymph node metastasis - Liver: most common site of metastasis due to portal drainage of the colon F. CARCINOID TUMORS MORPHOLOGY: - From neuroendocrine cells along GIT - most common site: APPENDIX - Secrete bioactive amines - Present as bulbous swelling at tip, usually solitary - 6th decade; potentially malignant - Stomach and ileum – are multicentric - <2% of colorectal malignancies - Intramural or submucosal masses with overlying intact or ulcerated - Appendiceal/rectal – infrequently metastasize mucosa; may permeate bowel wall - Colonic, gastric, ileal - metastasize - Solid, yellow tan grossly - Neoplastic cells in discrete glands, trabeculae, sheets of monotonous similar cells with round nucleus and scant granular cytoplasm; rare mitosis

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MICROSCOPIC both show well to poorly differentiated lesions Desmoplastic stromal reaction; may produce mucin(poorer prognosis)

SIGNS AND SYMPTOMS: - Usually asymptomatic - Carcinoid syndrome – if hepatic metastasis is present; elaboration of serotonin in blood - 5HIAA present in urine - 90% overall 5-year survival

G. GIT LYMPHOMAS MORPHOLOGY: - 1-2% of GIT malignancies; Large cell type (most common) - varied; plaque-like, polypoid or fungating - Usual site of extranodal lymphoma (40% of lymphomas) - layers infiltrated by monotonous malignant lymphoid cells of small, cleaved and non-cleaved lymphocytes and - Occur in patients with: immunoblasts o Chronic sprue-like malabsorption syndromes o Natives of mediterranean region o Congenital immunodeficiency states o HIV infection o Following organ transplantation with immunosuppression H. MESENCHYMAL TUMORS - Lipomas, GIT stromal tumors - MORPHOLOGY: o Well-demarcated, firm, <4.0 cm arise in submucosa or muscle layer o Large bulky masses with hemorrhage and necrosis  malignant - SIGNS AND SYMPTOMS: o Usually asymptomatic but symptoms of bleeding or obstruction may be seen STAGING SYSTEMS: T1 T2 T3 T3a T3b T3c T3d T4 T4a T4b Tumor invades submucosa Tumor invades into, but not through, muscularis propria Tumor invades through muscularis propria Invasion <0.1 cm beyond muscularis propria Invasion 0.1 to 0.5 cm beyond muscularis propria Invasion >0.5 to 1.5 cm beyond muscularis propria Invasion >1.5 cm beyond muscularis propria Tumor invades adjacent organs or visceral peritoneum Invasion into other organs or structures Invasion into visceral peritoneum

Regional Lymph Nodes NX Lymph nodes cannot be assessed N0 N1 N2 No regional lymph node metastasis Metastasis in one to three regional lymph nodes Metastasis in four or more regional lymph nodes

Distant Metastasis MX Distant metastasis cannot be assessed TNM CLASSIFICATION Tumor Tis In situ dysplasia or intramucosal carcinoma M0 M1 No distant metastasis Distant metastasis or seeding of abdominal organs

IX. APPENDIX ACUTE APPENDICITIS Most common acute abdominal condition Associated with obstruction by fecalith, gallstone, tumor or ball of worms in 50-80% of cases; in young adults SIGNS AND SYMPTOMS: o Periumbilical pain later localizing at RLQ, fever, tenderness at Mcburney’s point, increased WBC count MORPHOLOGY: neutrophilic infiltration of muscularis; dull, granular, congested (early acute appendicitis) Fibrinopurulent exudate over serosa, abscess (suppurative appendicitis) Gangrene (gangrenous appendicitis) RUPTURE MUCOCOELE AND PSEUDOMYXOMA PERITONEI MUCOCOELE: dilated appendix filled with mucin - Caused by: o Epithelial hyperplasia o Mucinous cystadenoma (most common)- Associated with appendiceal perforation in 20% o Mucinous cystadenocarcinoma- PSEUDOMYXOMA PERITONEI (islands of mucus in the peritoneal cavity; complication of appendiceal lesion)

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X. PERITONEUM PERITONITIS - bacterial or chemical - most common in sterile peritonitis Bile, pancreatic enzymes, foreign substance, blood, tumor products (teratoma) A. PERITONEAL INFECTION B. SCLEROSING C. MESENTERIC CYSTS RETROPERITONITIS - Found within the 1. Bacterial - 2º extension of - Obscure conditions; dense mesenteries in the abdl infection from a hollow fibromatous overgrowth of cavity or attached to the viscus or ruptured viscus retroperitoneal tissues peritoneal lining of the 2. Spontaneous – absence of abdominal wall obvious source of contamination MORPHOLOGY: - membranes initially gray and dull with serous fluid later becoming frankly suppurative (localized or generalized) TUMORS PRIMARY TUMORS - Mesotheliomas; are rare; associated with asbestos exposure in 80% of cases SECONDARY TUMORS - More common; from ovarian and pacreatic CA or intra-abdominal malignancy

Katemendoza2014 ^_^ Please read your book for more info.. Sources: doc cagampan’s lecture, old cor tranx (B2011), plus my additional notes 

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