rsna 2004 everything you need to know about solid and papillary epithelial neoplasms of the pancreas...

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Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 RSNA 2004 Department of Radiology/Surgery+/Pathology++ Department of Radiology/Surgery+/Pathology++ University of Miami School of Medicine/Jackson University of Miami School of Medicine/Jackson Memorial Hospital Memorial Hospital Click Here to Begin O Qureshi MD O Qureshi MD VJ Casillas VJ Casillas MD MD L Rivas MD L Rivas MD JU Levi MD + JU Levi MD + M Jorda MD ++ M Jorda MD ++ C Solozarno C Solozarno MD + MD +

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Page 1: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

Everything You Need to Know About Solid and Papillary Epithelial

Neoplasms of the Pancreas

RSNA 2004RSNA 2004

Department of Radiology/Surgery+/Pathology++Department of Radiology/Surgery+/Pathology++ University of Miami School of Medicine/Jackson Memorial University of Miami School of Medicine/Jackson Memorial

HospitalHospital

Click Here to Begin

O Qureshi MDO Qureshi MD VJ Casillas MDVJ Casillas MD

L Rivas MDL Rivas MD JU Levi MD +JU Levi MD +

M Jorda MD +M Jorda MD +++

C Solozarno C Solozarno MD +MD +

Page 2: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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History of Solid and Papillary History of Solid and Papillary Epithelial Neoplasms of the Epithelial Neoplasms of the

Pancreas (SPEN)Pancreas (SPEN)

1959: Described by 1959: Described by FrantzFrantz as “papillary tumor of as “papillary tumor of pancreas, benign or malignant”pancreas, benign or malignant”

1970: Pathology first described by Hamoudi1970: Pathology first described by Hamoudi 1981: Became a well-known clinical entity after 1981: Became a well-known clinical entity after

publication of cases by Klöppelpublication of cases by Klöppel 1996: Renamed by the World Health 1996: Renamed by the World Health

Organization as solid-pseudopapillary tumor Organization as solid-pseudopapillary tumor (SPT)(SPT)

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Page 3: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Also Known As…Also Known As… Solid pseudopapillary tumor (SPT)Solid pseudopapillary tumor (SPT) Frantz’s tumorFrantz’s tumor Papillary cystic neoplasm of the pancreasPapillary cystic neoplasm of the pancreas Solid cystic papillary tumorSolid cystic papillary tumor Solid and cystic acinar cell tumorSolid and cystic acinar cell tumor Papillary tumor of the pancreasPapillary tumor of the pancreas Papillary epithelial neoplasmPapillary epithelial neoplasm

Page 4: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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EtiologyEtiology

Pluripotential pancreatic embryonic Pluripotential pancreatic embryonic stem cellsstem cells

Cells capable of endocrine or Cells capable of endocrine or exocrine differentiationexocrine differentiation Variety of markers from various Variety of markers from various

pancreatic cell typespancreatic cell types

Page 5: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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EtiologyEtiology

Alternative hypothesis that SPEN Alternative hypothesis that SPEN originates from genital ridge-originates from genital ridge-related cells incorporated into related cells incorporated into pancreas during organogenesispancreas during organogenesis

Prevalence in women suggests Prevalence in women suggests hormonal influencehormonal influence Case report of increased tumor growth Case report of increased tumor growth

during pregnancyduring pregnancy

Page 6: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Genetics Alterations in Alterations in APCAPC/ß-/ß-catenincatenin pathway pathway

Also identified in pancreatoblastomas and acinar Also identified in pancreatoblastomas and acinar cell carcinomascell carcinomas

Nuclear and cytoplasmic accumulation of ß-Nuclear and cytoplasmic accumulation of ß-catenin catenin protein in 95% cases (study of 20 protein in 95% cases (study of 20 patients)patients)

Activating ß-Activating ß-catenin catenin oncogene mutations in oncogene mutations in 90%90%

Over expression of cyclin D1 protein in 74%Over expression of cyclin D1 protein in 74% Predilection for young females not understood Predilection for young females not understood

Page 7: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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EpidemiologyEpidemiology Prevalence: 0.13 – 2.7% of all Prevalence: 0.13 – 2.7% of all

pancreatic tumorspancreatic tumors 82-93% cases in 82-93% cases in womenwomen 70% tumors occur under age of 3070% tumors occur under age of 30 Average age at presentation: 21-27Average age at presentation: 21-27 Men present with disease at an age Men present with disease at an age

10 years older than women 10 years older than women

Page 8: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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EpidemiologyEpidemiology

Racial predilectionRacial predilection Blacks Blacks andand East Asians East Asians 50% of reported cases in the United 50% of reported cases in the United

States amongst African-AmericansStates amongst African-Americans SPEN in children show less female SPEN in children show less female

preponderance than in adultspreponderance than in adults

Page 9: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Gross PathologyGross Pathology Specimens range from 2 to 25 cmSpecimens range from 2 to 25 cm May occur throughout pancreas, more May occur throughout pancreas, more

common in head and tail; Exophytic growth common in head and tail; Exophytic growth patternpattern

Well-circumscribedWell-circumscribed with fibrous capsule with fibrous capsule Solid, cystic, and papillary regionsSolid, cystic, and papillary regions Variable degrees of internal hemorrhageVariable degrees of internal hemorrhage Necrotic and thrombotic contentsNecrotic and thrombotic contents Fluid-debris levels in cystic cavitiesFluid-debris levels in cystic cavities

Page 10: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CysticCystic SolidSolid Mixture of componentsMixture of components Hemorrhage and fluid levelsHemorrhage and fluid levels Peripheral calcificationsPeripheral calcifications

Page 11: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CytologyCytology

Aspirates are highly cellularAspirates are highly cellular FNA: 72% diagnostic or suggestive of SPENFNA: 72% diagnostic or suggestive of SPEN Solid areas: Necrosis, foamy macrophages, Solid areas: Necrosis, foamy macrophages,

cholesterol granulomas, and occasionally cholesterol granulomas, and occasionally calcificationscalcifications

Papillary configurations: Papillary configurations: Fibrovascular stalkFibrovascular stalk surrounded by several layers of epithelial cellssurrounded by several layers of epithelial cells

Frequently arranged around tiny vessels as Frequently arranged around tiny vessels as “pseudorosettes”“pseudorosettes”

Poorly supported blood vessels that result in Poorly supported blood vessels that result in numerous and extensive hemorrhagenumerous and extensive hemorrhage

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Tumor cells arranged around a hyalinized fibrovascular stalk

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CytologyCytology Absence of pleomorphism, Absence of pleomorphism,

hyperchromasia, or mitotic activityhyperchromasia, or mitotic activity Bland, oval to round nuclei that may Bland, oval to round nuclei that may

contain small nucleoli and grooves or foldscontain small nucleoli and grooves or folds Eosinophilic granular cytoplasmEosinophilic granular cytoplasm HyalineHyaline cytoplasmic globules, cytoplasmic globules,

multinucleated giant cellsmultinucleated giant cells Infiltrative growth pattern into adjacent Infiltrative growth pattern into adjacent

pancreas despite gross circumscriptionpancreas despite gross circumscription

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ImmunohistochemistryImmunohistochemistry Commonly seen immunoreactivityCommonly seen immunoreactivity

CD 10 and CD 56CD 10 and CD 56 Found in all cases in a study of 19 patientsFound in all cases in a study of 19 patients

αα-1 antitrypsin-1 antitrypsin Neuron-specific enolaseNeuron-specific enolase VimentinVimentin Progesterone receptorsProgesterone receptors

Stains negative for:Stains negative for: chromogranin Achromogranin A

Page 15: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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ImmunohistochemistryImmunohistochemistry

Neuron-specific enolase positive Chromogranin A negative

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ImmunohistochemistryImmunohistochemistry

Occasionally stains positive for:Occasionally stains positive for: KeratinKeratin αα-1 antichymotrypsin-1 antichymotrypsin SynapthophysinSynapthophysin S-100 proteinS-100 protein

Neurosecretory granules Neurosecretory granules occasionally seenoccasionally seen

Page 17: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Clinical PresentationClinical Presentation Vague symptomsVague symptoms Abdominal fullnessAbdominal fullness or discomfort or discomfort Epigastric or LUQ abdominal painEpigastric or LUQ abdominal pain Early satietyEarly satiety Asymptomatic: 9%Asymptomatic: 9% Duration of symptoms: acute to 5 yearsDuration of symptoms: acute to 5 years Nontender, palpable mass in LUQ or RUQNontender, palpable mass in LUQ or RUQ

Page 18: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Clinical PresentationClinical Presentation

Symptoms also seen: Jaundice, Symptoms also seen: Jaundice, polyarthralgia, dyspepsia, weight loss, polyarthralgia, dyspepsia, weight loss, nausea, anorexianausea, anorexia

Laboratory values are non-diagnosticLaboratory values are non-diagnostic Rare cases exhibit mildly elevated CA 19-9 Rare cases exhibit mildly elevated CA 19-9

values, eosinophiliavalues, eosinophilia NonspecificNonspecific symptomology often leads to a symptomology often leads to a

delay in diagnosisdelay in diagnosis Diagnosis is often incidentalDiagnosis is often incidental

Page 19: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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University of Miami Case University of Miami Case SeriesSeries

Cases obtained from the institutional Cases obtained from the institutional hospital of the University of Miami, hospital of the University of Miami, Jackson Memorial HospitalJackson Memorial Hospital

15 cases of surgically resected and 15 cases of surgically resected and pathology proven SPEN collected for pathology proven SPEN collected for retrospective reviewretrospective review

Page 20: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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University of Miami Case University of Miami Case SeriesSeries

GenderGender # of # of CasesCases Avg. AgeAvg. Age RangeRange

FemaleFemale 1313 21.521.5 9-379-37

MaleMale 22 58.558.5 56-6156-61

Page 21: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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University of Miami Case University of Miami Case SeriesSeries

87% of cases in females, consistent 87% of cases in females, consistent with expected prevalencewith expected prevalence

Average age at presentation of 21.5 Average age at presentation of 21.5 within expected normal for SPENwithin expected normal for SPEN

Male age of presentation > female, Male age of presentation > female, but also much higher than normally but also much higher than normally observedobserved

Page 22: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Ultrasound FindingsUltrasound Findings

Well-encapsulated masses with variable Well-encapsulated masses with variable echotextureechotexture

Combined Combined cystic and solidcystic and solid portions portions May demonstrate septations and internal echoesMay demonstrate septations and internal echoes Solid masses with good through-transmission Solid masses with good through-transmission

correlate to friable neoplastic tissue with massive correlate to friable neoplastic tissue with massive hemorrhagic necrosishemorrhagic necrosis

Masses of low echogenicity correspond to Masses of low echogenicity correspond to neoplastic tissue with focal cystic degenerationneoplastic tissue with focal cystic degeneration

Echogenic tumor capsulesEchogenic tumor capsules

Page 23: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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US : Round complex mass predominantly solid with small cystic components in head of the pancreas

20 yr old white Latin female with RUQ pain, nausea, fatty food intolerance and elevated liver function tests

Page 24: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Transverse scan

US: Ovoid cystic mass in the neck of the pancreas

20 yr old female with RUQ abdominal pain

Page 25: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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US: Complex mass in the pancreatic head mostly cystic with small solid components

28 yr old female with abdominal pain

Page 26: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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US : Well-circumscribed hypoechoic mass (m) in the body of the pancreas with minimal posterior enhancement (arrows)

Sagittal midline

m

17 yr old female with epigastric pain and early satiety

Page 27: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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US: Large hyperechoic solid mass, body and pancreatic tail

13 yr old Black female with abdominal fullness

Page 28: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT FindingsCT Findings Well-defined, round or lobulated massesWell-defined, round or lobulated masses HeterogeneousHeterogeneous with variable ratio of cystic with variable ratio of cystic

and solid componentsand solid components Regions of hyperdensity correspond to Regions of hyperdensity correspond to

hemorrhagehemorrhage Improved definition of mass with IV Improved definition of mass with IV

contrast administration, with slight contrast administration, with slight peripheral enhancementperipheral enhancement

Peripheral calcifications in ~ 1/3Peripheral calcifications in ~ 1/3rdrd patients patients Mass effectMass effect on local structures on local structures

Page 29: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Pancreatic mass mostly cystic with septations and small solid components

21 yr old Hispanic female with vague abdominal symptoms

Page 30: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT demonstrates large ovoid complex mass with cystic and solid components involving the body and tail of the pancreas. Biopsy showed that this was SPEN, and not metastasis.

55 yr old Hispanic male with known transitional cell carcinoma of the bladder

Page 31: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Well circumscribed solid mass in the pancreatic tail

26 yr old African-American female with abdominal discomfort

Page 32: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Large complex mass involving the body and tail of the pancreas

Page 33: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT : Pancreatic mass of low attenuation with thick walls

17 yr old female with epigastric pain and early satiety

Page 34: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Complex pancreatic masses with fluid levels

9 yr old Latin female 22 year old Latin female

Page 35: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Large cystic pancreatic mass with mural nodule

19 yr old Black female with progressive LUQ abdominal fullness for three years

Page 36: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Pancreatic mass with mostly cystic, thin walls

28 yr old female with left upper quadrant abdominal pain

Page 37: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT: Pancreatic mass mostly cystic with peripheral calcifications

37 yr old female with early satiety and abdominal discomfort

Page 38: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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MRI FindingsMRI Findings

Improved visualization of capsule and Improved visualization of capsule and internal hemorrhageinternal hemorrhage, hence more , hence more specificspecific

Well-defined encapsulated lesionWell-defined encapsulated lesion T1: Heterogeneous hypointense or T1: Heterogeneous hypointense or

hyperintense signal relative to adjacent hyperintense signal relative to adjacent pancreatic parenchymapancreatic parenchyma

T2: Heterogeneously hyperintense signalT2: Heterogeneously hyperintense signal

Page 39: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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MRI FindingsMRI Findings

Hematocrit effect: Hematocrit effect: Fluid-fluidFluid-fluid or fluid- or fluid-debris levelsdebris levels

T1 and T2: Peripheral hypointense rimT1 and T2: Peripheral hypointense rim T1 Post-GadoliniumT1 Post-Gadolinium

Arterial phase: Heterogeneous Arterial phase: Heterogeneous peripheral enhancementperipheral enhancement

Portal and delayed phase: Portal and delayed phase: Progressive heterogeneous fill-in Progressive heterogeneous fill-in (incomplete)(incomplete)

Page 40: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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MRI: Low signal intensity mass in the neck of the pancreas on T1WI and high signal intensity on T2WI with fat saturation

20 yr old female with RUQ abdominal pain

T1 T2 Fat Sat

Page 41: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Classifying SPEN by MRIClassifying SPEN by MRI Influences surgical strategyInfluences surgical strategy Type 1: High signal on T1 and T2 – subacute Type 1: High signal on T1 and T2 – subacute Type 2: Low signal on T1 and high signal on Type 2: Low signal on T1 and high signal on

T2 – chronic phase after bleedingT2 – chronic phase after bleeding Type 3: Low signal on T1 and homogeneous Type 3: Low signal on T1 and homogeneous

intermediate signal on T2 -- no bleedingintermediate signal on T2 -- no bleeding Type 1 and 2 lesions had peripheral rims Type 1 and 2 lesions had peripheral rims

corresponding to fibrous capsulecorresponding to fibrous capsule Type 3 had only partial capsules indicative Type 3 had only partial capsules indicative

of invasive disease, requiring extensive of invasive disease, requiring extensive operationsoperations

Page 42: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Other ImagingOther Imaging Endoscopic ultrasonographyEndoscopic ultrasonography

Useful in diagnosis tumors that measure less Useful in diagnosis tumors that measure less than 2 cm; Limited utility as most SPEN > 4cm than 2 cm; Limited utility as most SPEN > 4cm

Provides guidance for FNAProvides guidance for FNA Arteriography:Arteriography:

Avascular or Avascular or hypovascularhypovascular mass mass Useful in differentiating from islet cell tumors Useful in differentiating from islet cell tumors

which are typically hypervascularwhich are typically hypervascular CalcificationsCalcifications

Case report of detection by bone scintigraphyCase report of detection by bone scintigraphy Rarely seen on abdominal plain film x-raysRarely seen on abdominal plain film x-rays

Page 43: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Arteriography : Large hypovascular mass body and tail of the pancreas. Note the displacement of the SMA to the right

13 yr old Haitian female with abdominal fullness

Page 44: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Differential DiagnosisDifferential Diagnosis Cystic islet cell tumorCystic islet cell tumor Serous microcystic adenomaSerous microcystic adenoma Mucinous cystic neoplasmMucinous cystic neoplasm Intraductal papillary mucinous tumorIntraductal papillary mucinous tumor Acinar cell carcinomaAcinar cell carcinoma Papillary cystadenocarcinomaPapillary cystadenocarcinoma PancreatoblastomaPancreatoblastoma Vascular tumors: Hemangioma, lymphangioma, Vascular tumors: Hemangioma, lymphangioma,

angiosarcomaangiosarcoma Calcified hemorrhagic pseudocystCalcified hemorrhagic pseudocyst Inflammatory pseudocystInflammatory pseudocyst Dysgenetic cyst, as seen in von Hippel-Lindau and Dysgenetic cyst, as seen in von Hippel-Lindau and

polycystic kidney diseasepolycystic kidney disease Retention cyst, as seen in cystic fibrosisRetention cyst, as seen in cystic fibrosis

Page 45: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Differential DiagnosisDifferential Diagnosis Islet cell tumorsIslet cell tumors

SPEN commonly misdiagnosed as SPEN commonly misdiagnosed as non-non-functioning islet cell tumorsfunctioning islet cell tumors

Islet cell tumors are hypervascular with different Islet cell tumors are hypervascular with different CT/MRI enhancement patternsCT/MRI enhancement patterns

Cystic components have moderately elevated Cystic components have moderately elevated signal intensity on T1 and increased signal on T2signal intensity on T1 and increased signal on T2

PancreatoblastomaPancreatoblastoma Childhood malignant neoplasm with poor Childhood malignant neoplasm with poor

prognosisprognosis Male predominanceMale predominance No intratumoral hemorrhageNo intratumoral hemorrhage

Page 46: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Differential DiagnosisDifferential Diagnosis Acinar cell carcinomaAcinar cell carcinoma

Always malignantAlways malignant Affects both genders in 6Affects both genders in 6thth or 7 or 7thth decades decades

Pancreatic Pancreatic pseudocystpseudocyst Thin wallsThin walls Totally cystic lesion without any solid component Totally cystic lesion without any solid component History of pancreatitisHistory of pancreatitis

Intraductal papillary mucinous tumorIntraductal papillary mucinous tumor Dilatation of the main pancreatic ductDilatation of the main pancreatic duct Soft villous tumor associate with Wirsung’s ductSoft villous tumor associate with Wirsung’s duct

Page 47: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Differential DiagnosisDifferential Diagnosis Microcystic adenomaMicrocystic adenoma

Female predominance presenting in 6Female predominance presenting in 6thth decade decade CT reveals low-attenuation with marked CT reveals low-attenuation with marked

enhancement with “honeycomb pattern”enhancement with “honeycomb pattern” Echogenic central stellate scarEchogenic central stellate scar No peripheral or capsular enhancement on MRINo peripheral or capsular enhancement on MRI

Mucinous cystic tumorsMucinous cystic tumors Female predominance presenting in 5Female predominance presenting in 5thth - 6 - 6thth

decadesdecades Large mucin-secreting cystsLarge mucin-secreting cysts Multilocularity with thin septationsMultilocularity with thin septations

Page 48: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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TreatmentTreatment

Definitive treatment is Definitive treatment is surgical surgical Partial pancreatectomy (48%)Partial pancreatectomy (48%) Whipple procedure (29%)Whipple procedure (29%) Local excision (17%)Local excision (17%) Pancreatectomy (6%)Pancreatectomy (6%)

No known role for chemotherapy or No known role for chemotherapy or radiation therapyradiation therapy Past cases have resulted in recurrencePast cases have resulted in recurrence

Requires lengthy follow-up because of Requires lengthy follow-up because of inability to determine aggressive behaviorinability to determine aggressive behavior

Page 49: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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PrognosisPrognosis

Surgical resection is often Surgical resection is often curablecurable Long-term survival is the rule despite Long-term survival is the rule despite

local invasivenesslocal invasiveness Not related to pathologyNot related to pathology

Microscopic positive margins not Microscopic positive margins not significant predictors of survivalsignificant predictors of survival

Page 50: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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ComplicationsComplications MetastaticMetastatic disease (6-15%) disease (6-15%)

Predilection for males and older patientsPredilection for males and older patients Predominantly to the liver, less commonly Predominantly to the liver, less commonly

to lymph nodes to lymph nodes Also described in spleen, colon, Also described in spleen, colon,

mesentery, skin, lung, generalized mesentery, skin, lung, generalized carcinomatosiscarcinomatosis

May be microscopic and undetectable by May be microscopic and undetectable by imagingimaging

Long-term survival in 10-15% patientsLong-term survival in 10-15% patients

Page 51: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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ComplicationsComplications

Pseudocyst formationPseudocyst formation Death (4%)Death (4%)

Hemorrhagic coagulopathyHemorrhagic coagulopathy CholangitisCholangitis Septic shockSeptic shock

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Solid Pseudopapillary Solid Pseudopapillary CarcinomaCarcinoma

SPEN/SPT with clear SPEN/SPT with clear malignantmalignant criteria criteria Vascular and nerve sheath invasionVascular and nerve sheath invasion Metastasis to lymph node or liverMetastasis to lymph node or liver

Morphologically identical to SPENMorphologically identical to SPEN Average age at presentation: 30Average age at presentation: 30

Slightly older than that of SPENSlightly older than that of SPEN Uncertain whether SPEN becomes Uncertain whether SPEN becomes

malignant with tumor growthmalignant with tumor growth

Page 53: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Interesting CaseInteresting Case

History: 24 year old black female History: 24 year old black female presents to the ER with acute onset presents to the ER with acute onset of abdominal pain. Pain is epigastric, of abdominal pain. Pain is epigastric, sharp, and constant. Denies fever, sharp, and constant. Denies fever, nausea, vomiting, constipation, or nausea, vomiting, constipation, or diarrheadiarrhea

Labs: Elevated LFT’sLabs: Elevated LFT’s

Page 54: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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CT:CT: 2 x 2 cm mass located in the porta 2 x 2 cm mass located in the porta hepatis. CT-FNA was nondiagnostichepatis. CT-FNA was nondiagnostic

Page 55: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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MRI:MRI: T1W T1W images demonstrate hypointense images demonstrate hypointense mass in porta hepatis mass in porta hepatis

T1 T1

Page 56: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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MRI: Note the mass has relatively high Note the mass has relatively high signal onsignal on T2W T2W imageimage

T2

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MRCP:MRCP: Obstruction of the biliary system at the Obstruction of the biliary system at the biliary bifurcation and proximal CHD (mass effect)biliary bifurcation and proximal CHD (mass effect)

Surgical pathologySurgical pathology reveals solid mass engulfing reveals solid mass engulfing bifurcation of CBD with extension to cystic and bifurcation of CBD with extension to cystic and hepatic ductshepatic ducts

Cut sectionCut section: Solid and cystic, filled with necrotic debris: Solid and cystic, filled with necrotic debris

Page 58: RSNA 2004 Everything You Need to Know About Solid and Papillary Epithelial Neoplasms of the Pancreas RSNA 2004 Department of Radiology/Surgery+/Pathology++

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Interesting CaseInteresting Case

Histology: Fibrous capsule with Histology: Fibrous capsule with lobules containing papillary pattern, lobules containing papillary pattern, consisting of epithelial cells around consisting of epithelial cells around hyalinized fibrovascular stalkshyalinized fibrovascular stalks

RBC’s in spaces between papillary RBC’s in spaces between papillary structuresstructures

Extensive perineural invasion and focal Extensive perineural invasion and focal lymphovascular space involvementlymphovascular space involvement

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Interesting CaseInteresting Case

Diagnosis: Solid and papillary Diagnosis: Solid and papillary epithelial neoplasm of the epithelial neoplasm of the extrahepatic bile ductsextrahepatic bile ducts

Majority of extrapancreatic SPEN (a Majority of extrapancreatic SPEN (a very rare entity) affiliated with very rare entity) affiliated with heterotopic pancreatic tissueheterotopic pancreatic tissue

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Review QuizReview Quiz

Who is most likely to be affected by Who is most likely to be affected by SPEN?SPEN?

A.A. 70 year old black male70 year old black male

B.B. 6 year old white female6 year old white female

C.C. 21 year old Asian female21 year old Asian female

D.D. 45 year old white male45 year old white male

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Review QuizReview Quiz

The answer is The answer is CC,, 21 year old Asian 21 year old Asian female.female.

SPEN has a racial predilection for SPEN has a racial predilection for young females, predominantly in the young females, predominantly in the black and Asian population.black and Asian population.

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Review QuizReview QuizWhich is uncharacteristic of SPEN?Which is uncharacteristic of SPEN?

A.A. Internal hemorrhageInternal hemorrhage

B.B. Cystic mucinous secretionsCystic mucinous secretions

C.C. Fluid-debris levelsFluid-debris levels

D.D. Fibrous capsuleFibrous capsule

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Review QuizReview Quiz

The answer is The answer is BB. .

Cysts are seen in almost every case of Cysts are seen in almost every case of SPEN. However, mucin secretion is NOT SPEN. However, mucin secretion is NOT characteristic of this neoplasm. Such characteristic of this neoplasm. Such secretions are seen in mucinous cystic secretions are seen in mucinous cystic tumors of the pancreas, which is tumors of the pancreas, which is included in the differential diagnosis of included in the differential diagnosis of SPEN.SPEN.

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Review QuizReview Quiz

MRI imaging offers which benefit?MRI imaging offers which benefit?

A.A. Improved detection of intratumoral Improved detection of intratumoral bloodblood

B.B. Better visualization of the capsuleBetter visualization of the capsule

C.C. Specific enhancement patternSpecific enhancement pattern

D.D. All of the aboveAll of the above

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The answer is The answer is DD, all of the above., all of the above.

MRI offers all of the mentioned MRI offers all of the mentioned benefits, making it a more specific benefits, making it a more specific test than CT or ultrasound in the test than CT or ultrasound in the diagnosis of SPEN.diagnosis of SPEN.

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Review QuizReview Quiz

Which of the following statements about Which of the following statements about SPEN is false?SPEN is false?

A.A. Clinical presentation is classicClinical presentation is classic

B.B. Stains negative for chromogranin AStains negative for chromogranin A

C.C. Fibrovascular stalks are seen in cytologyFibrovascular stalks are seen in cytology

D.D. Pseudocyst is in the differential Pseudocyst is in the differential diagnosisdiagnosis

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Review QuizReview Quiz

The answer is The answer is AA. .

SPEN presents with vague abdominal SPEN presents with vague abdominal symptoms, including fullness, pain, symptoms, including fullness, pain, and early satiety. However, and early satiety. However, presentation is anything but classic.presentation is anything but classic.

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What percent of SPEN metastasize?What percent of SPEN metastasize?

A.A. Almost neverAlmost never

B.B. 5-15%5-15%

C.C. 50%50%

D.D. Greater than 80%Greater than 80%

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The answer is The answer is BB, 5-15%., 5-15%.

SPEN has low malignant potential. Hence it SPEN has low malignant potential. Hence it is mandatory to establish early diagnosis, is mandatory to establish early diagnosis, as surgical removal of tumor offers an as surgical removal of tumor offers an excellent prognosis. Even in cases of local excellent prognosis. Even in cases of local invasiveness or metastasis, the outcome invasiveness or metastasis, the outcome can be promising if properly treated.can be promising if properly treated.

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Buetow PC, Buck JL, Pantongrag-Brown, et al. Solid and papillary epithelial neoplasm of the pancreas: imaging-pathologic Buetow PC, Buck JL, Pantongrag-Brown, et al. Solid and papillary epithelial neoplasm of the pancreas: imaging-pathologic correlation in 56 cases. Radiology 1996; 199:707-711.correlation in 56 cases. Radiology 1996; 199:707-711.

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ReferencesReferences

Hurley ME, Corbally M, McDermott M. Solid pseudopapillary tumour of the pancreas. 1 Apr 2003.Hurley ME, Corbally M, McDermott M. Solid pseudopapillary tumour of the pancreas. 1 Apr 2003.

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Jung SE, Kim DY, Park KW, et al. Solid and papillary epithelial neoplasm of the pancreas in children. World J Surg 1999; 3: Jung SE, Kim DY, Park KW, et al. Solid and papillary epithelial neoplasm of the pancreas in children. World J Surg 1999; 3: 233-236.233-236.

Koizumi J. Solid and papillary epithelial neoplasms of the pancreas: classification based on MR imaging. 2000.Koizumi J. Solid and papillary epithelial neoplasms of the pancreas: classification based on MR imaging. 2000.

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Lee DH, Yi BH, Joo WL, Ko YT. Sonographic findings of solid and papillary neoplasms of the pancreas. J Ultrasound Med Lee DH, Yi BH, Joo WL, Ko YT. Sonographic findings of solid and papillary neoplasms of the pancreas. J Ultrasound Med 2001; 20: 1229-1232.2001; 20: 1229-1232.

Martin RCG, Klimstra DS, Brennan MF, Conlon KC. Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Annals Martin RCG, Klimstra DS, Brennan MF, Conlon KC. Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Annals of Surgical Oncology 2002; 9: 35-40.of Surgical Oncology 2002; 9: 35-40.

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Shimizu M, Matsumoto T, Hirokawa M, et al. Solid-pseudopapillary carcinoma of the pancreas. Pathology International 1999; Shimizu M, Matsumoto T, Hirokawa M, et al. Solid-pseudopapillary carcinoma of the pancreas. Pathology International 1999; 49: 231-234.49: 231-234.

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Zeytunlu M, Firat O, Nart D, et al. Solid and cystic papillary neoplasms of the pancreas: report of four cases. Turkish J of Zeytunlu M, Firat O, Nart D, et al. Solid and cystic papillary neoplasms of the pancreas: report of four cases. Turkish J of Gastroenterolgoy 2004; 15: 178-182.Gastroenterolgoy 2004; 15: 178-182.