C.P.C.C.P.C.

RUQ Cystic Mass and FeverRUQ Cystic Mass and Fever

ByBy

William E. Stevens M.D.William E. Stevens M.D.

CPC Case HighlightsCPC Case Highlights

Young, Latin-American, Male, HomosexualYoung, Latin-American, Male, Homosexual2 weeks fever, chills, night-sweats2 weeks fever, chills, night-sweats1 week RUQ pain1 week RUQ pain5-10 pound weight loss5-10 pound weight lossExam: temp 102.5; RUQ tendernessExam: temp 102.5; RUQ tendernessLabs: normal WBC 5.7, mildly abnormal LFT’s Labs: normal WBC 5.7, mildly abnormal LFT’s (hepatitis risk factors); elevated total protein to (hepatitis risk factors); elevated total protein to albumin ratio; elevated CRP and ESRalbumin ratio; elevated CRP and ESRCT scan: 8.5 cm cystic / solid mass in RUQ, CT scan: 8.5 cm cystic / solid mass in RUQ, heterogeneous, non-enhancing walls, IVC heterogeneous, non-enhancing walls, IVC thrombosis, ?pushing or invading into liverthrombosis, ?pushing or invading into liver

RUQ Cystic Mass and FeverRUQ Cystic Mass and FeverDifferential ConsiderationsDifferential Considerations

Primary infectionPrimary infectionBacterial abscessBacterial abscessAmebic abscessAmebic abscessEchinococcosisEchinococcosis

MalignancyMalignancyLiverLiverAdrenalAdrenalRenalRenalPancreaticPancreaticLymphoma, otherLymphoma, other

Benign Mass with secondary infectionBenign Mass with secondary infectionHepatic cyst with infection or focal cholangitisHepatic cyst with infection or focal cholangitisPancreatic pseudocyst with infection Pancreatic pseudocyst with infection Choledochal cystCholedochal cystRenal, adrenal, mesenteric and duplication cystsRenal, adrenal, mesenteric and duplication cysts

Multiple problemsMultiple problemsChronic viral hepatitis with a cystic massChronic viral hepatitis with a cystic massHIV infection with a cystic massHIV infection with a cystic mass

Bacterial AbscessBacterial Abscess

Pyogenic liver abscessPyogenic liver abscessSymptoms: Fever 90%; pain 55%; weight loss 40%; Symptoms: Fever 90%; pain 55%; weight loss 40%; jaundice 50%jaundice 50%Usually multiple 50%; right lobe involved 60%Usually multiple 50%; right lobe involved 60%Etiology: biliary 55%, cryptogenic 15%, other: appendicitis, Etiology: biliary 55%, cryptogenic 15%, other: appendicitis, diverticulitis, etc.diverticulitis, etc.

Other abdominal abscessesOther abdominal abscessesDiverticular abscessDiverticular abscessPerinephric abscessPerinephric abscessCholecystitis with abscessCholecystitis with abscessPerforated ulcer with abscessPerforated ulcer with abscessPerforated appendix with abscessPerforated appendix with abscess

TreatmentTreatmentDrainage, antibiotics, surgeryDrainage, antibiotics, surgery

Amebic AbscessAmebic Abscess

Entamoeba histolyticaEntamoeba histolyticaFecal-oral transmissionFecal-oral transmission

5-10% in U.S. are asymptomatic cyst passers5-10% in U.S. are asymptomatic cyst passers

PathophysiologyPathophysiologyIngested cyst is resistant to gastric acidIngested cyst is resistant to gastric acid

Ameba becomes active in small intestine and passes into Ameba becomes active in small intestine and passes into coloncolon

Ameba invade colonic mucosa causing acute and chronic Ameba invade colonic mucosa causing acute and chronic colitiscolitis

Ameba invade into mesenteric veins thus reaching the liverAmeba invade into mesenteric veins thus reaching the liver

Cystic necrosis of hepatocytesCystic necrosis of hepatocytes

Entamoeba histolyticaEntamoeba histolytica

Amebic AbscessAmebic AbscessClinical PresentationClinical Presentation

More common in young, male, Latin-American, HIV+More common in young, male, Latin-American, HIV+

60% have history of travel to endemic country60% have history of travel to endemic country

Duration of illness is usually < 2 weeksDuration of illness is usually < 2 weeks

20% have history of dysentery, 10% have dysentery20% have history of dysentery, 10% have dysentery

90% have RUQ pain or epigastric, chest or right shoulder 90% have RUQ pain or epigastric, chest or right shoulder painpain

75% have fever75% have fever

Right hepatic lobe involved in 80-95%Right hepatic lobe involved in 80-95%

LFT’s mildly, nonspecifically elevated; < 10% jaundiceLFT’s mildly, nonspecifically elevated; < 10% jaundice

Leukocytosis and anemia are commonLeukocytosis and anemia are common

Amebic AbscessAmebic AbscessDiagnosisDiagnosis

Ultrasound and CTUltrasound and CTUsually solitary, round, peripheral, right lobeUsually solitary, round, peripheral, right lobeThrough transmission, hypoechogenicityThrough transmission, hypoechogenicityWall usually enhances with IV contrastWall usually enhances with IV contrastOccasionally will have nodular border or internal septationsOccasionally will have nodular border or internal septations

Amebic SerologyAmebic SerologyELISA has > 90% sensitivityELISA has > 90% sensitivityCan’t differentiate past from current infectionCan’t differentiate past from current infection

Liver AspirationLiver AspirationNot usually necessaryNot usually necessaryPus is reddish brown “anchovy paste”, usually sterilePus is reddish brown “anchovy paste”, usually sterileAspirate if diagnosis is uncertain, large abscess, “impending Aspirate if diagnosis is uncertain, large abscess, “impending rupture”, left lobe involvement, non-response to treatmentrupture”, left lobe involvement, non-response to treatment

Amebic AbscessAmebic Abscess

ComplicationsComplicationsPleuropulmonary rupturePleuropulmonary ruptureHepatic rupture, amebic peritonitisHepatic rupture, amebic peritonitis

– Usually sudden severe abdominal painUsually sudden severe abdominal pain– Occasionally slow leak with walled off abscessOccasionally slow leak with walled off abscess

Rupture into pericardium, pericarditisRupture into pericardium, pericarditisHemobiliaHemobiliaRupture into bowelRupture into bowel

TreatmentTreatmentAspiration if neededAspiration if neededFlagyl 750 mg TID for 10 daysFlagyl 750 mg TID for 10 daysIodoquinol 650 mg TID for 20 daysIodoquinol 650 mg TID for 20 days

EchinococcosisEchinococcosis

Echinococcus granulosaEchinococcus granulosa and and multilocularismultilocularisSmall 3-6 mm tape wormsSmall 3-6 mm tape wormsDefinitive host: Dog, othersDefinitive host: Dog, others

Ingests infected visceraIngests infected visceraPasses eggs into stoolPasses eggs into stool

Intermediate host: Human, sheep, cattle, othersIntermediate host: Human, sheep, cattle, othersIngests food contaminated with egg laden fecesIngests food contaminated with egg laden fecesEgg is digested in small bowel releasing embryoEgg is digested in small bowel releasing embryoEmbryo invades mucosa entering mesenteric veinsEmbryo invades mucosa entering mesenteric veinsEmbryo become trapped in liver (70%), lung (20%), spleen, kidney, Embryo become trapped in liver (70%), lung (20%), spleen, kidney, bone, CNS, etc.bone, CNS, etc.

Endemic in areas where dogs are used to help raise Endemic in areas where dogs are used to help raise livestocklivestock

Echinococcus granulosaEchinococcus granulosa

Echinococcus Life CycleEchinococcus Life Cycle

EchinococcosisEchinococcosis

Symptoms occur years after acute infectionSymptoms occur years after acute infectionCyst produces mass effect enlarging ~1 cm/yearCyst produces mass effect enlarging ~1 cm/year

– RUQ painRUQ pain– CBD compression, obstructive jaundiceCBD compression, obstructive jaundice– Portal vein compression, portal hypertensionPortal vein compression, portal hypertension

Cyst may rupture; contents are highly antigenicCyst may rupture; contents are highly antigenic– Anaphylactic shockAnaphylactic shock– Free rupture into peritoneumFree rupture into peritoneum– Rupture into biliary tract: obstruction, pancreatitis, cholangitisRupture into biliary tract: obstruction, pancreatitis, cholangitis– Rupture into pleura, pericardium, colon, duodenum, kidneyRupture into pleura, pericardium, colon, duodenum, kidney

Cysts may become secondarily infectedCysts may become secondarily infected

LabsLabsMild leukocytosis, 40% have eosinophiliaMild leukocytosis, 40% have eosinophiliaELISA IgG is 97% sensitiveELISA IgG is 97% sensitive

RadiologyRadiologyCystic mass; walls occasionally calcified; rim often enhances with IV contrastCystic mass; walls occasionally calcified; rim often enhances with IV contrastDaughter cystsDaughter cysts

EchinococcosisEchinococcosisCT AppearanceCT Appearance

EchinococcosisEchinococcosis

DiagnosisDiagnosisTypical radiological appearance Typical radiological appearance

– MRI provides more data than CTMRI provides more data than CTELISA IgG is 97% sensitiveELISA IgG is 97% sensitiveAspiration is relatively contraindicated Aspiration is relatively contraindicated due to risk of cyst due to risk of cyst leakage and anaphylaxisleakage and anaphylaxis

TreatmentTreatmentSurgery: remove all cysts if possibleSurgery: remove all cysts if possible

Avoid spillage of cyst contentsAvoid spillage of cyst contentsLarge cysts may be injected with hypertonic saline and Large cysts may be injected with hypertonic saline and aspirated in OR before removalaspirated in OR before removal10-30% recur10-30% recur

Albendazole 400 mg BID for 28 daysAlbendazole 400 mg BID for 28 daysNon-surgical injection and aspiration only in unresectable Non-surgical injection and aspiration only in unresectable cysts or patients who are non-operable cysts or patients who are non-operable

Neoplastic Hepatic CystsNeoplastic Hepatic Cysts

Any primary or metastatic cancer can appear cystic due to central Any primary or metastatic cancer can appear cystic due to central necrosis or hemorrhagenecrosis or hemorrhage

Choriocarcinoma, ovarian carcinoma, sarcoma, islet cell tumors, lymphomaChoriocarcinoma, ovarian carcinoma, sarcoma, islet cell tumors, lymphoma

Biliary cystadenoma / cystadenocarcinomaBiliary cystadenoma / cystadenocarcinomaMost common in women over age 40Most common in women over age 40Cystic, septated masses, irregular wall margins, more often in right lobeCystic, septated masses, irregular wall margins, more often in right lobeMucous secreting cuboidal epithelium with ovarian type stromaMucous secreting cuboidal epithelium with ovarian type stromaWalls and septations are more vascular by pulse dopplerWalls and septations are more vascular by pulse doppler

Cavernous hemangiomaCavernous hemangiomaMost common hepatic tumor; usually asymptomaticMost common hepatic tumor; usually asymptomaticMen = women, but estrogens enhance growthMen = women, but estrogens enhance growthOften appear cystic, occasionally pedunculatedOften appear cystic, occasionally pedunculatedRarely will rupture, usually due to traumaRarely will rupture, usually due to trauma

TeratomaTeratomaMesenchymal hamartomaMesenchymal hamartoma

Benign Noninfectious Benign Noninfectious Hepatic CystsHepatic Cysts

SolitarySolitarySimple CystsSimple Cysts

– 3.6% population; F:M = 4:13.6% population; F:M = 4:1– Almost always asymptomaticAlmost always asymptomatic– Usually <5 cm, usually right lobeUsually <5 cm, usually right lobe– Thin walls; more than one cyst may be presentThin walls; more than one cyst may be present

Post-traumaticPost-traumaticPost-hepatic infarctionPost-hepatic infarctionRarely can cause pain, hemorrhage, become infectedRarely can cause pain, hemorrhage, become infected

PolycysticPolycysticAutosomal dominant adult polycystic kidney diseaseAutosomal dominant adult polycystic kidney diseaseAutosomal recessive polycystic kidney disease and Congenital hepatic Autosomal recessive polycystic kidney disease and Congenital hepatic fibrosisfibrosisCaroli’s DiseaseCaroli’s DiseaseVon Meyenburg complexesVon Meyenburg complexesOther congenital syndromesOther congenital syndromesPeliosis hepatisPeliosis hepatis

Other RUQ Cystic MassesOther RUQ Cystic Masses

Pancreatic CystsPancreatic CystsSimple pancreatic cystsSimple pancreatic cystsPancreatic pseudocystsPancreatic pseudocysts

– Almost always have a history of pancreatitis or traumaAlmost always have a history of pancreatitis or trauma– Complicates 10% cases of acute pancreatitisComplicates 10% cases of acute pancreatitis– More common in ETOH pancreatitisMore common in ETOH pancreatitis– Usually lack septae; may be loculated; may have calcium; cysts may travel to Usually lack septae; may be loculated; may have calcium; cysts may travel to

unexpected areasunexpected areas– Cyst fluid has a very high amylaseCyst fluid has a very high amylase

Pancreatic cystic neoplasmPancreatic cystic neoplasm– 80% are women; usually > 55 years old; usually involve body and tail pancreas80% are women; usually > 55 years old; usually involve body and tail pancreas– Mucinous cystic neoplasm (45%); intraductal papillary mucinous neoplasm Mucinous cystic neoplasm (45%); intraductal papillary mucinous neoplasm

(30%); serous cyst adenoma (15%)(30%); serous cyst adenoma (15%)– EUS is best diagnostic testEUS is best diagnostic test

Choledochal CystsCholedochal CystsCongenital cystic dilation of the biliiary tractCongenital cystic dilation of the biliiary tract60% present before age 1060% present before age 10Recurrent RUQ pain, fever, abnormal LFT’sRecurrent RUQ pain, fever, abnormal LFT’s

More RUQ Cystic MassesMore RUQ Cystic Masses

Adrenal CystsAdrenal Cysts15% of adrenal cysts are malignant15% of adrenal cysts are malignant

– Adrenal cortical carcinoma and adenomaAdrenal cortical carcinoma and adenoma– PheochromocytomaPheochromocytoma– Cystic lymphangioma and neuroblastomaCystic lymphangioma and neuroblastoma

85% are benign85% are benign– Hemorrhagic adrenal pseudocystHemorrhagic adrenal pseudocyst– Hydatid cystHydatid cyst

Renal CystsRenal CystsSolitary cysts and Polycystic kidney diseaseSolitary cysts and Polycystic kidney diseaseRenal cell carcinomaRenal cell carcinoma

– male > female; usually age > 50; increased risk IVC thrombosis; 60% have male > female; usually age > 50; increased risk IVC thrombosis; 60% have hematuriahematuria

Mesenteric CystsMesenteric CystsVery rare; 3% are malignantVery rare; 3% are malignant60% have localized pain, occasionally fever, chills60% have localized pain, occasionally fever, chills

Duplication CystsDuplication CystsRare; congenital; attached to gut; lined by G.I. mucosaRare; congenital; attached to gut; lined by G.I. mucosa

HIV Related Liver DiseasesHIV Related Liver Diseases

Hepatitis B and CHepatitis B and CIncreased incidence in HIV+ patientsIncreased incidence in HIV+ patientsWhen compared to HIV negative patients:When compared to HIV negative patients:

– Higher viral loadHigher viral load– Lower ALTLower ALT– More severe histologyMore severe histology– Less responsive to medical therapyLess responsive to medical therapy– Mortality from liver disease much higherMortality from liver disease much higher

Increased risk of Hepatoma and Lymphoma with chronic hepatitisIncreased risk of Hepatoma and Lymphoma with chronic hepatitis

Peliosis hepatisPeliosis hepatisFever, RUQ pain, hepatic cystic masses usually < 3cm in sizeFever, RUQ pain, hepatic cystic masses usually < 3cm in sizeBlood filled cavitiesBlood filled cavitiesRelated to Related to BartonellaBartonella infection; CD-4 count < 200 infection; CD-4 count < 200

Amebic abscessAmebic abscess60% of patients 60% of patients without a travel historywithout a travel history are immunosuppresed or HIV+ are immunosuppresed or HIV+

Many others: fungal abscess, Kaposi’s sarcoma, LymphomaMany others: fungal abscess, Kaposi’s sarcoma, Lymphoma

RUQ Cystic Mass and FeverRUQ Cystic Mass and FeverDiagnostic StudiesDiagnostic Studies

Recommend:Recommend:– Send serologies for ameba and EchinococcusSend serologies for ameba and Echinococcus– Send serologies for HIV, Hepatitis B and CSend serologies for HIV, Hepatitis B and C– Aspirate and Biopsy cystic mass if ameba and Aspirate and Biopsy cystic mass if ameba and

Echinococcus serologies are negativeEchinococcus serologies are negative