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An Unlikely Cause of Possible Hormone-Dependent Abdominal Pain in Young FertileFemales: A Case SeriesBonnie Patek DOLehigh Valley Health Network, [email protected]
Hiral Shah MDLehigh Valley Health Network, [email protected]
Shashin Shah MDLehigh Valley Health Network, [email protected]
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Published In/Presented AtPatek, B., Shah, H., Shah, S. (2015, April 30). An Unlikely Cause of Possible Hormone-Dependent Abdominal Pain in Young Fertile Females:A Case Series. Poster presented at: 2015 POMA Clinical Assembly/Convention, Valley Forge, PA.
1Department of Medicine, 2Department of Gastroenterology, Lehigh Valley Health Network, Allentown, Pennsylvania
An Unlikely Cause of Possible Hormone-Dependent Abdominal Pain in Young Fertile Females: A Case Series
B. Patek, DO1, H. Shah, MD2 and S. Shah, MD2
Background Case Presentation•Solidpseudopapillaryneoplasm(SPN)isa
rarepancreaticexocrinetumor(Table1)withunknownetiology,firstdescribedbyDr.Frantzin1959.1-3
•SPNisabenignlesionwithlowgrademalignantpotential,oftencurativewithsurgery.1
•Hormonesmayplayintotheorigin/development,withastrongcorrelationwithfemalesandprogesteronereceptor(PR).
•SPNmimicspancreaticneoplasms,pseudocystsandpancreaticendocrineneoplasmsonimagingandcytomorphologicalfeatures2(Table2,Image1).
Discussion:
© 2015 Lehigh Valley Health Network
References:1. Kim,Mi-Jung,Se-JinJang,andEunsilYu.“OriginalContributionLossofE-cadherinandCytoplasmic-nuclearExpressionofBBB-cateninAretheMostUsefulImmunoprofilesinthe
DiagnosisofSolid-pseudopapillaryNeoplasmofthePancreas.”Human Pathology39(2008):251-58.2. Burford,H.,Z.Baloch,X.Liu,D.Jhala,G.P.Siegal,andN.Jhala.“E-Cadherin/-CateninandCD10:ALimitedImmunohistochemicalPaneltoDistinguishPancreaticEndocrineNeoplasm
FromSolidPseudopapillaryNeoplasmofthePancreasonEndoscopicUltrasound-GuidedFine-NeedleAspiratesofthePancreas.”American Journal of Clinical Pathology 132.6(2009):831-39.
3. Bardale,Ricardo,BarbaraCenteno,ShawnMallery,RebeccaLai,MarkPochapi,GeradoGuiter,andMichaelStanley.“EndoscopicUltrasound–GuidedFine-NeedleAspirationCytologyDiagnosisofSolid-PseudopapillaryTumorofthePancreas.”American Journal of Clinical Pathology121(2004):654-62.
4. Vassos,Nikolaos,andAbbasAgaimy.“Solid-pseudopapillaryNeoplasm(SPN)ofthePancreas:CaseSeriesandLiteratureReviewonanEnigmaticEntity.”International Journal of Clinical and Experimental Pathology 6.6(2013):1051-059.
5. Manfredi,Riccardo,andRobertoPozziMucelli.MagneticResonanceCholangiopancreatography(MRCP):Biliary and Pancreatic Ducts.6. Robles-DiazM,Guillermo,andAndresDuarte-Rojo.“Pancreas:ASexSteroid-dependentTissue.”Israel Medical Association Journal3.5(2001):364-68.7. Nieuwenhuizen,A.,G.Schuiling,S.Liem,H.Moes,T.Koiter,andJ.Uilenbroek.“ProgesteroneStimulatesPancreaticCellProliferationinVivo.”European Journal of Endocrinology 140.3
(1999):256-63.8. Yu,Peng-Feietal.“SolidPseudopapillaryTumorofthePancreas:AReviewof553CasesinChineseLiterature.”World Journal of Gastroenterology: WJG16.10(2010):1209–1214.PMC.6
Apr.2015.
Wepresenttwocasesofyoungfemaleswithepigastric pain, nausea/vomiting,withoutpruritus,jaundiceorsteatorrhea.
•BothcasesdemonstrateaclassicpresentationofSPN;ayoung,fertilefemalewithabdominalpainandpancreaticlesiononimaging.
•Lackofknownetiology/pathophysiology,specificIHCmarkers/patternstodistinguishSPNfromotherpancreaticmassesisachallenge.
–Etiology:ectopicovarianstromafromfemalegenitalbudorprimitivecentroacinarcellsthatarehypersensitivitytofemalesexhormonestimulationleadingtoproliferation.4-5
–PRarelocatedon75%α-cellsand5-20%β-cells.Progesteroneincreasesproliferationofacinarcellsmostinvivoandpromotesproliferationofdifferentiatedcells,notneogenesis.6-7
•SPNrequirescompletesurgicalresectiondespitelowmalignancyriskwith5yrsurvival~97%.1,4
–Metastaticdiseaseincludeliver,regionallymphnodes,mesentery,omentum,peritoneumwithlocalinvasiontoduodenum,stomach,spleenandmajorvessels.8
–Post-opcomplications:pancreaticfistula,pancreatitis,prolongedgastricemptying,bleeding,infection,diabetes.8
•PossibleIHCpatternforaccurateSPNdiagnosisis+CD10,+nuclearβ-cateninwithnegativemembranousE-cadherin.
–Notutilizeduniversallyleadingtomisdiagnosesorinappropriatetherapy.
Image 1. Characteristichyalineglobulesinclusters(arrows)(A).Solidsheetsofuniformcellswithpapillaryformationfromlackofadhesion(B).
Image 2. Case1-CTofabdomen/pelvisthatvisualizesthesolidpancreaticmass(arrow)locatedintheuncinateprocess.
Image 3. Case2-CTofabdomen/pelvisthatshowssolidpancreaticmass(arrow)locatedinthetailofthepancreas.
Table 1: Common Features of SPN1-3
Epidemiology Clinical Presentation
2nd-3rd decade of life Asymptomatic
Non-Caucasian Abdominal Pain/Mas
Female gender (10:1) Nausea/Vomiting
Rare (1-2% exocrine tumors) Weight loss
Benign (15% malignant) Jaundice
Table 2. Microscopic Features of SPN1-3
Size Distribution
Cytology
Round/oval eccentric nucleiUniform cells with fine granular chromatin No mitotic figures Extracellular hyaline globules
HistologyPseudopapillary, solid, cystic Thin walled vessels Bands of fibrous tissue
Immunohistochemistry (IHC)+ CD56, CD10, PR, β-catenin (nuclear)- E-cadherin membranous - Chromogranin/Synaptophysin (G/S)*
Table 3. Pathology and IHC Case Series Comparison
Case 1 Case 2
FNA
+ C56, Vimentin, CD10, AE1/AE3- C/S
+ CD10, Vimentin, PR, AE1/AE3, β-catenin-C/S
Oval nucleiNo necrosis/mitotic figures Salt/pepper chromatin
Monomorphic uniform cellsRound/oval nuclei with groovesFinely dispersed chromatin
Surgical Pathology
+ CD56, Vimentin, CD10, β-catenin (nuclear)+ weak C/SKi67 <2%22 benign lymph nodes
+ CD56, CD10, β-catenin (nuclear)+ C/S Ki67 25%25 benign lymph nodes
CASE 1:•A20-week pregnantfemalewithasolid
pancreatic lesionintheuncinate process (Image2)asincidentalfindingonimaging.Mirenawasinsertedpost-partumandrepeatimagingshowedanincreaseintumorsizeafter1year.
•Endoscopicultrasound-fineneedleaspiration(EUS-FNA)wasperformed(Table3),followedbyaWhipple procedure.
•Post-opcomplicationsincludedintra-abdominalabscess.
CASE 2:•Asolid mass inthepancreatic tailwasfoundon
CTimaging(Image3)withfollow-upMRIindicatingspontaneous lesion regression.Twoyearslaterandstartingoral contraceptive pills,repeatimagingshowedincrease tumor sizeandenhancing liver lesions.
•EUS-FNAwasperformed(Table3)andCTguidedliverbiopsyconsistentwithfocalnodularhyperplasia.Distal pancreatectomyandsplenectomywereperformed.
•Repeatimagingshowedincreasesizeinliverlesions,butPETscanwasnegative.