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www.downstatesurgery.orgPANCREATIC CYSTICNEOPLASMSKings County HospitalFebruary 2009Joelle Pierre

www.downstatesurgery.orgCASE PRESENTATION33 yy/o female ppresented with an incidentallyyfound pancreatic mass on CT scan Had vague abdominal pain x several months Palpable mass on exam PMHx: none PsurgHx:PH none Meds: OCP

www.downstatesurgery.orgCT SCANLargeg mass with necrotic center arisingg from thehead of the pancreas measuring 10.0 cm x 7.7 cmx 11 cm no llymphadenopathyh dth No liver masses Clear fascial plane between the mass and thesuperior mesenteric artery

www.downstatesurgery.orgFNA OF THE MASS EGDy Endoscopic transmural pancreatic biopsyPathologyySolid Pseudopapillary tumor

www.downstatesurgery.orgOPERATIVE my OperativeOtipathologyth l 8x5x5 cm tumory Pseudopapillaryp py tumorof the pancreasy

www.downstatesurgery.orgCYSTIC NEOPLASMS OF THEPANCREAS

www.downstatesurgery.orgCYSTIC NEOPLASMS OF THE PANCREASWILLIAM R. BRUGGE, M.D., GREGORY Y. LAUWERS, M.D., DUSHYANTSAHANI, M.D.,CARLOS FERNANDEZ-DEL CASTILLO, M.D.,M D AND ANDREW L.L WARSHAW, M.D.MDNENGL J MED 351;12 WWW.NEJM.ORGSEPTEMBER 16, 2004Cyst c neoplasmsCysticeop as s - 10%0% ofo pancreaticpa c eat c neoplasmseop as s benign, malignant, and borderline neoplasms that either are primarily cystic or resultfrom the cystic degeneration of solid tumors

www.downstatesurgery.orgCYSTIC NEOPLASMSSolidpseudopapillaryserous Typesyp of cysticyneoplasms includeserous cystadenomas (32to 39 %),%)y mucinous cysticneoplasms (10 to 45 %)y papillary mucinousneoplasms (21 to 33 %)y Solid pseudopapillarytumors ( 3-5%)ymucinouspseudocyst

www.downstatesurgery.orgPRESENTATIONCan be asymptomaticy p Recurrent pain, jaundice or pancreatitis y Involving or connected to the pancreatic ductAdvancedPain, weight loss, jaundicey Can present with pseudocystsy Pain, and even early satiety if compressing the stomach orsmall bowel; jaundice secondary to compression of thecommon bile duct

www.downstatesurgery.orgDIAGNOSIS CTiinitialiti l detectiond t ti off a llesioniy visualization of calcifications, septa, mural nodules,pancreatitisy MRI/MRCPbetter characterization of the morphologic features of a cysty showing a communication between the cyst and thepancreatic duct.yTransabdominal ultrasonography The use of PET is not firmly established

www.downstatesurgery.orgCYTOLOGICAL INVESTIGATION Cytologicyg examination of cysty fluidy analysis the aspirated fluid for a variety ofbiochemical markers and tumor cellscytologict l i analysisl i off cystt flfluidid hhas ididentifiedtifi d cellsllto confirm of malignant disease mucinous cysticlesion in perhaps only half the aspirates obtained

www.downstatesurgery.orgCHARACTERISTICS TO BE EXAMINEDSignsg and symptomsy p Histology Location in the pancreas Diagnostic features Surgical treatment Malignant potential Prognosis

www.downstatesurgery.orgDIAGNOSIS AND MANAGEMENT OF CYSTICNEOPLASMS OF THE PANCREAS: ANEVIDENCE-BASED APPROACH,MATTHEW H G KATZ, MD MELINDA M MORTENSON, MD,HUAMINWANG, MD, PHD, ROSA HWANG, MD,ERIC P TAMM,MD GREGG STAERKEL, MD,MD,MD JEFFREY H LEE, MD,MD DOUGLAS BEVANS, MD, FACS, JASON B FLEMING, MD, FACSJOURNAL OF AMERICAN COLLEGE OF SURGEONSVOL. 207, NO. 1, JULY 2008

www.downstatesurgery.orgSEROUS CYSTADENOMAS 30 % of cysticyppancreatic neoplasmsp Women in their 7th decade Mainly in body and tail but can be anywhere inthe pancreas Usually asymptomatic and found incidentally WhenWh symptomatictti - epigastricit i pain,i abdominalbd i lfullness and weight loss; rarely jaundice – evenin the pancreatic head.

www.downstatesurgery.orgHISTOLOGY Associated withchromosomal alterations ofthe gene for von Hippel–Lindau disease located onchromosome 3p2570 % serous neoplasmslarepolycystic (microcystic) ,multiple tiny cysts vsmacrocystic with largerfewer cystsThe cysts contain serousfluid that are PAS ,glycogen rich cuboidalepitheliumSolid and oligocysticvariants of serous adenomashave also been reported.A)Microcysts lined by cuboidalepithelium with clear cytoplasmB) PAS staint i ddemonstratest tcharacteristic intracytoplasmicglycogen granules

www.downstatesurgery.orgOTHER DIAGNOSTIC FEATURES RadiologicalgLobulated contour,absence of wallenhancement, localein pancreatic head.y They may appear solidor show a singled idominantt cyststy The presence of acentral/stellate scarvisualized withseptated honey combappearance, sunburstcalcification.y

www.downstatesurgery.orgSEROUS CYSTADENOMAS Cyst fluid - 20 percent of serous cystadenomasLow CEAy Low CA19-9y Low amylasey low potential for malignant disease.diseaseObserved no significant increase in diameter of thetumor after 69 monthsy GrowthGh off 0.120 2 cm per year forf tumors 4 cmy Larger tumors 1.98 cm per yeary

www.downstatesurgery.orgSEROUS CYSTADENOMASTreatment – “our bias is that that operations are applied tooearly and too often for patients with this disease.” yThere is some data that tumors 4 cm can beobservedAdmittedly the number four is arbitrary. Does not apply for tumors of undetermined pathology Operative procedure depends of the locale in the pancreas – Pancreaticoduodectomy , central or partialpancreatectomy, distal pancreatectomy

www.downstatesurgery.orgSEROUS CYSTADENOMA PROGNOSIS In a series byy Bassi and colleagues’g50 patients with SCA were treated with definitivesurgical resection.y At a median followup of 43 monthsmonths, all patients werealive and free of disease except one, who died of othercauses.y I an earlierInli seriesi PykeP k andd colleaguesllyreported a 5-year survival of approximately 81% in 36patients who underwent resection.

www.downstatesurgery.orgSEROUS CYSTADENOCARCINOMA AggressiveggbehaviorLymphovascular invasiony Microscopic infiltrationy SynchronousShor metachronoust hextrapancreaticttimetastasisy

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN) Mucin producing cystic neoplasmyIncludes intraductal mucinous neoplasmsIPMN MCN yPre-malignant yCan progress to invasive cancerCan be determined from cyst aspirate

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN)45% of all resected ppancreatic neoplasmsp Almost all are female Middle aged 90% located in the body and tail Symptoms can include discomfort, nausea,ddyspepsiai Jaundice is uncommon

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN)Typicallyypy round,, thick walled and septated.p No communication with the pancreatic ductalsystem Histopathological features of mucinous cysticneoplasms include a dense mesenchymal ovarianovarian-likelike stroma,stromay requisite feature of mucinous cystic neoplasms.y

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN)CT – thick cysty wall. Macrocysticylesion that canbe multiloculated. Peripheral eggshell calcification on CT uncommony specific to a mucinous cystic neoplasmy highlyg yppredictivee c ve oof cacancercey The use of PET is not firmly established

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN) EUSyEvaluate the viscosity of the fluid on aspiration y“string sign”Tumor markers can be used to evaluate the cyst fluidCEA 800 ng/ml is 98% specific although 48% sensitive CEA 6000 is strongly suggestive of mucinousadenocarcinoma CA 19-9 is less accurate. FNAHigh rates of sampling errory Also can not take into account malignanttransformationy

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN)PROGNOSISSurvival after surgicalgresection correlates withthe presence or absence of invasive disease. Surgical resection is curative for non invasivedidiseasewherehas therethhavehbbeen cases offrecurrence with invasive disease.

www.downstatesurgery.orgMUCINOUS CYSTIC NEOPLASM (MCN) In a series of 56 patients who underwent surgical resectionoff MCN,MCN neitheri h tumor recurrence or tumor-relatedl dddeathhwas observed in 34 patients with adenomas or borderlineMCN during a median follow-up period of 42 months and69 months, respectively.Six patients with noninvasive carcinoma (carcinoma insitu) were also all alive without recurrence at a medianfollowup of 76 months.In contrast, 8 (50%) of16 patients with invasive MCN diedwithin 45 months.Reddy and colleagues found that none of the 52 patientswith noninvasive MCN had recurrence of disease afterresection,Goh and colleagues identified only 4 (2%) recurrencesamong 189 resectedt d patientsti t withith noninvasiveii MCN ini apooled analysis of 344 previously reported patients

www.downstatesurgery.orgINTRADUCTAL PAPILLARYMUCINOUS NEOPLASM (IPMN)25% of ppancreatic cysticyneoplasmsp 20% of pancreatic resections for malignancy Disease of the elderly Higher prevalence of invasive adenocarcinomawith advanced age EqualEl didistributiont ib ti amongstt ththe gendersd Salient feature – connection to pancreatic duct Clinical symptoms are generally non specific Can also include : pancreatitisy Diabetes, weight loss or jaundice is generallycorrelatedl d withi h invasiveii diseasediy

www.downstatesurgery.orgADDITIONAL FEATURES OF IPMN Histologicallygy–yCystic pancreatic ductdilation withassociated papillaryprojections and mucinproductionCharacteristic microscopicp features ofintraductal papillarymucinous neoplasm with well-formed,finger-like papillae, andan absence of ovarian-type stroma

www.downstatesurgery.org CT – polycystic massassociated with dilation ofpancreatic ductthe main por its side branches.Involvement of the mainduct is associated with ahighergdegreegofinvasivenessy Head of pancreas- 50% ofpatientsy Can be found anywhereyand even throughout theentire pancreasy MRI may be better able todemonstrate thecommunication with theductsA) Large, cystic mass (arrow) consistent with main ductintraductal papillary mucinous neoplasm (IPMN), identified on CTscan in a 6565-year-oldyear old man who presented with abdominal pain andjaundice. Pancreaticoduodenectomy revealed IPMN without dysplasiaor invasive adenocarcinoma. (B) Incidentally identified 2.5-cmcyst in the uncinate process

www.downstatesurgery.orgIPMNERCP mayy show mucin pproduction from anenlarged papilla IPMN can be associated with a focus of ductalcarcinomaielsewherelhini theth pancreas Current recommendations Resection should be offered to all pts with main ductIPMNsy Tumors with side branch involvement that aresymptomatic or greater than 3 cm in size should bebe resectedy 3cm can be observedy

www.downstatesurgery.orgIPMN PROGNOSISPostoperativepsurvival of ppatients is relatedprimarily to the presence of invasiveadenocarcinoma. Five-yearFipostoperativetti survivali l off patientsti t withithnoninvasive disease is between 77% and 100% In contrast, the prognosis for patients withinvasive IPMN is similar to that of patients withinvasive ductal adenocarcinoma, with the mostoptimistic 5-year5 year survival rates no better thanapproximately 36%. Noninvasive IPMN carries a recurrence rate afterresection of 10%.

www.downstatesurgery.orgSOLID PSEUDOPAPILLARY TUMORRare Predominates in women Median age around 30 years Can occur anywhere in the pancreas Symptoms are usually vague and associated withsizei off ththe ttumor

www.downstatesurgery.orgTypically largeencapsulated lesionswith solid and cysticco po e s.components. Have pseudopapillarypatterns on histologyCT Well – encapuslatedsolid masses withthickened capsules andvariable amount ofinternal hemorrhage,cystic degeneration andcalcificationA) specimen demonstrating focal hemorrhage and cystic degeneration consistent (B) A 4.5-cm solid andcystic lesion with coarse internal calcification,((arrow),), found incidentallyy on a CT scan pperformed for workuppof nephrolithiasis.

www.downstatesurgery.orgSOLID PSEUDOPAPILLARY TUMOR Operationpis offered for this low ggrade tumorPrevent local tumor growth and metsy Palliate symptomsy CanC leadl d tot favorablefbl survivali l even withith locall l ttumorextension or metsy Usually requires distal pancreatectomy or apancreaticoduodenectomy because of its large sizey

www.downstatesurgery.orgSOLID PSEUDOPAPILLARY TUMORPROGNOSIS A single center report by Tipton and colleaguesyyy described 14 patients with a median tumor diameter of 7cm.13 of the patients in whom curative resection waspperformed12 were alive after longterm followup.In a similar series by Martin and colleaguesyyyyy18 patients who underwent resection for localized SPPT100% recurrence-free survivalOf these, four patients presented with synchronous livermetastasis underwent combined pancreatectomy andymetastasectomyled to survival of 6 years and 11 years in 2 of the 4patients.Overall, aggressive surgical resection is associated with a5-year5year survival of 95%.95%

www.downstatesurgery.orgLYMPHOEPITHELIAL CYSTSThe rarest : 70 ppts in the literature More common in men 5th to 7th decade Usually asymptomatic and discoveredincidentally UsuU didistributedt ib t d throughoutthh t ththe pancreas Histology lined by a layer of stratified squamous epitheliumsurrounded by a characteristic layer of lymphoidtissue.y Cysts are filled with a dense materialmaterial, composedmainly of debris, keratin, and cholesterol crystals.y

www.downstatesurgery.orgLYMPHOEPITHELIAL CYSTSThere are no ppathognomonicgfeatures on crosssectional imaging, but several radiographicfeatures are highly suggestive of this diagnosis. CT can demonstrate either a multimulti- or aunilocular cyst, which is well-encapsulated by anenhancing thin wall protruding from the body ofthe gland.gland A cystic component of low attenuation is typical,but a solid component of variable magnitude canalso exist.exist MRI can be useful; ythe high-keratin content of cyst fluid often produces ahyperintense signal on T1- and a hypointense signalon T2-weighted images

www.downstatesurgery.orgLYMPHOEPITHELIAL CYSTSFew reportspexist of FNA biopsyp y and analysisyofcyst fluid for cytology and biochemical