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 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
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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 +
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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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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
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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
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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
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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
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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
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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
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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
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CysticCystic SolidSolid Mixture of componentsMixture of components Hemorrhage and fluid levelsHemorrhage and fluid levels Peripheral calcificationsPeripheral calcifications
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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
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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
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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
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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
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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
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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
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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
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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
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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
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Transverse scan
US: Ovoid cystic mass in the neck of the pancreas
20 yr old female with RUQ abdominal pain
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US: Complex mass in the pancreatic head mostly cystic with small solid components
28 yr old female with abdominal pain
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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
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US: Large hyperechoic solid mass, body and pancreatic tail
13 yr old Black female with abdominal fullness
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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
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CT: Pancreatic mass mostly cystic with septations and small solid components
21 yr old Hispanic female with vague abdominal symptoms
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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
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CT: Well circumscribed solid mass in the pancreatic tail
26 yr old African-American female with abdominal discomfort
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CT: Large complex mass involving the body and tail of the pancreas
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CT : Pancreatic mass of low attenuation with thick walls
17 yr old female with epigastric pain and early satiety
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CT: Complex pancreatic masses with fluid levels
9 yr old Latin female 22 year old Latin female
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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
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CT: Pancreatic mass with mostly cystic, thin walls
28 yr old female with left upper quadrant abdominal pain
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CT: Pancreatic mass mostly cystic with peripheral calcifications
37 yr old female with early satiety and abdominal discomfort
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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
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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)
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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MRI:MRI: T1W T1W images demonstrate hypointense images demonstrate hypointense mass in porta hepatis mass in porta hepatis
T1 T1
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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
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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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Review QuizReview Quiz
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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Review QuizReview Quiz
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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Review QuizReview Quiz
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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ReferencesReferencesAbraham SC, Klimstra DS, Wilentz RE, et al. Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic Abraham SC, Klimstra DS, Wilentz RE, et al. Solid-pseudopapillary tumors of the pancreas are genetically distinct from pancreatic
ductal adenocarcinomas and almost always harbor ductal adenocarcinomas and almost always harbor ß-ß-catenincatenin mutations. American Journal of Pathology 2002; 160: 1361- mutations. American Journal of Pathology 2002; 160: 1361-1369.1369.
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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.
http://http://www.eurorad.org/case.cfm?UIDwww.eurorad.org/case.cfm?UID=2154=2154
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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ReferencesReferences
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