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Cystic pancreatic lesions
 

Cystic pancreatic lesions

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  • Renal US & DNA analysis for ADPKD were performed in 319 patients who were at risk. PKD1: short arm of chromosome 16 – Account for 85 – 90 % of population with ADPKD.PKD2: Long arm of chromosome 4 – Account for 10 – 15% of population with ADPKD.In some other families, no linkage to either PKD1 or PKD2 has been reported.

Cystic pancreatic lesions Cystic pancreatic lesions Presentation Transcript

  • Cystic pancreatic lesions Practice guidelines for diagnosis & management Samir Haffar M.D. Assistant Professor of Gastroenterology
  • Cystic masses of the pancreas *IPMN: Intraductal Papillary Mucinous Neoplasm Sahani DV et al. RadioGraphics 2005 ; 25 : 1471 – 1484. True epithelial cysts Autosomal-dominant polycystic kidney disease von Hippel–Lindau disease Cystic fibrosis Pseudocyst Cystic pancreatic neoplasm Common Serous cystadenoma Mucinous cystic neoplasm IPMN Rare Solid pseudo-papillary tumor Acinar cell cystadenocarcinoma Lymphangioma Hemangioma Paraganglioma Solid pancreatic neoplasm with cystic degeneration Pancreatic adenocarcinoma Cystic islet cell tumor (insulinoma, gastrinoma,.) Metastasis Cystic teratoma Sarcoma
  • True simple cyst in pancreas Probably congenital cystic lesion • Thin walled lesion, no intra-luminal excrescence or solid compound, large amounts of clear fluid, no connection with PD • Mostly occur in infants & younger patient • Polycystic disease, fibrocystic disease, or von Hippel-Lindau • Essentially benign & mostly remain asymptomatic • Final diagnosis made by histological study Heindryckx E et al. Eur Radiol 1998 ; 8 : 1627 – 1629.
  • True simple cyst of pancreas Heindryckx E et al. Eur Radiol 1998 ; 8 : 1627 – 1629. Uniloculate cyst in pancreatic head CT scan Pancreatic cyst lined by mono-layer of cylindrical/cuboidal epithelium Histological specimen
  • Screening of ADPKD Renal US & DNA analysis in 319 patients at risk Ravine D et all. Lancet 1994 ; 343 : 824 – 827. Nicolau C et al. Radiology 1999 ; 213 : 273 – 276. Person at risk & younger than 30 years Two cysts in one kidney or one cyst in each kidney Person at risk & aged 30 – 59 years Two cysts in each kidney Person at risk & aged 60 years or older Four cysts in each kidney Sen: 95% (ADPKD-1) – 65% (ADPKD-2) (Sen: 100%) (Sen: 100%)
  • Autosomal dominant polycystic kidney disease Small cyst in body of pancreas Enlarged polycystic kidneys & small hepatic cysts 41-year-old man with ADPKD Demos TC et al Am J Roentgenol 2002 ; 179 : 1375 – 1388.
  • von Hippel-Lindau disease Rare disease (prevalence 1/ 35.000 – 40.000) • Autosomal dominant disease with high penetrance • Development of variety of benign & malignant tumors • Broad clinical manifestations: 40 lesions in 14 organs • Diagnostic criteria More than one CNS hemangioblastoma One CNS hemangioblastoma & visceral manifestations Any manifestation & familial history of VHL disease
  • Manifestations of VHL Disease 40 different lesions in 14 different organs Leung RS et al. RadioGraphics 2008 ; 28 : 65 – 79. Manifestations Prevalence Pancreatic cysts Cerebellar hemangioblastoma Renal cysts Retinal hemangioblastoma Renal cell carcinoma Spinal cord hemangioblastoma Pheochromocytoma Neuroendocrine tumor of pancreas Serous cystadenoma of pancreas Medullary hemangioblastoma Papillary cystadenoma of epididymis 50 – 91% 44 – 72% 59 – 63% 45 – 59% 24 – 45% 13 – 59% 0 – 60% 5 – 17% 12 % 5 % 10 – 60%
  • Von Hippel-Lindau disease Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388. Small pancreatic cysts Multiple renal cysts & renal cell carcinoma 37-year-old man
  • Multiple unilocular cysts scattered throughout an otherwise healthy-looking pancreas Sahani DV et al. RadioGraphics 2005 ; 25 : 1471 – 1484. Von Hippel-Lindau disease Contrast-enhanced CT scan
  • Retinal hemangioblastoma Retinal angioma Leung RS et al. RadioGraphics 2008 ; 28 : 65 – 79. Well defined orange-red mass Prominent feeding artery Prominent draining vein Ophthalmoscopic image Fluorescein angiogram Retinal angioma with its hyperfluorescence
  • Cystic masses of the pancreas *IPMN: Intraductal Papillary Mucinous Neoplasm Sahani DV et al. RadioGraphics 2005 ; 25 : 1471 – 1484. True epithelial cysts* Autosomal-dominant polycystic kidney disease von Hippel–Lindau disease Cystic fibrosis Pseudocyst Cystic pancreatic neoplasm Common Serous cystadenoma Mucinous cystic neoplasm IPMN Rare Solid pseudo-papillary tumor Acinar cell cystadenocarcinoma Lymphangioma Hemangioma Paraganglioma Solid pancreatic neoplasm with cystic degeneration Pancreatic adenocarcinoma Cystic islet cell tumor (insulinoma, gastrinoma,.) Metastasis Cystic teratoma Sarcoma
  • Initial evaluation of a pancreatic cyst should be directed toward exclusion of a pseudocyst Patients with pseudocyst generally have a history of acute or chronic pancreatitis, whereas those with cystic tumors most often lack such a history
  • Pancreatic pseudocyst EUS of pancreatic pseudocyst with dependent layering debris Fasanella KE et al. Best Pract Res Clin Gastroenterol 2009 ; 23 : 35 – 48.
  • Calcified pseudocyst Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388. 44-year-old man with chronic pancreatitis Pseudocyst with calcified wall in head of pancreas When pseudocysts are chronic, the wall can calcify
  • Pancreatic abscess 30-year-old man with a history of pancreatitis Rim enhancing fluid collection with multiple foci of internal air Axial CECT Molvar C et al. Curr Probl Diagn Radiol 2011 ; 40 : 141 – 148. In absence of gas, differentiation of abscess from necrosis or simple fluid is not possible with imaging
  • Gas in pancreatic bed • Pancreatic abscess • Pancreatic-enteric fistula • Previous internal pseudo-cyst drainage • Previous drainage of pancreas by pancreatico-jejunostomy Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388. Pancreatic gas is not pathognomonic of an abscess
  • Pancreatic-enteric fistula Thin-walled collection with air-fluid level in pancreatic tail Asymptomatic 58-year-old man Several weeks after episode of acute pancreatitis Fistula between colon & pancreas as source of gas Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
  • Pancreatic hydatid cyst Rare: < 1% of cases Acute pancreatitis due to pancreatic hydatid cyst: 9 reported cases Pancreatic cystic mass of 10 cm Calcified wall & daughter cysts Left pancreatectomy with splenectomy Wall cyst & daughter cysts Makni et al. World J Emergency Surg 2012 ; 7 : 7 - 10. 38-year-old man presented with acute pancreatitis
  • Pancreatic tuberculosis Complex solid/cystic mass encasing PV & CBD CT scan Heterogeneous green predominant pattern Tissue elastographyEUS Large hypoechoic LN With central necrosis EUS-FNA: Granuloma – Positive AFB culture Chatterjee S et al. J Gastrointestin Liver Dis 2012 ; 21 : 105 – 107.
  • Cystic masses of the pancreas *IPMN: Intraductal Papillary Mucinous Neoplasm Sahani DV et al. RadioGraphics 2005 ; 25 : 1471 – 1484. True epithelial cysts* Autosomal-dominant polycystic kidney disease von Hippel–Lindau disease Cystic fibrosis Pseudocyst Cystic pancreatic neoplasm Common Serous cystadenoma Mucinous cystic neoplasm IPMN Rare Solid pseudo-papillary tumor Acinar cell cystadenocarcinoma Lymphangioma Hemangioma Paraganglioma Solid pancreatic neoplasm with cystic degeneration Pancreatic adenocarcinoma Cystic islet cell tumor (insulinoma, gastrinoma,.) Metastasis Cystic teratoma Sarcoma
  • WHO classification of neoplastic pancreatic cysts Serous cystic tumor Serous cystadenoma Serous cystadenocarcinoma Mucinous cystic tumor Mucinous cystadenoma Mucinous cystadenoma with mod dysplasia Mucinous cystadenocarcinoma Non-invasive Invasive IPMT IPM adenoma IPMT with moderate dysplasia IPM carcinoma Non-invasive Invasive Solid pseudopapillary tumor Solid pseudopapillary neoplasm Solid pseudopapillary carcinoma Aaltonen LA, Hamilton SR. WHO classification of tumours. Pathology & genetics of tumours of the digestive system. Lyon: IARC Press; 2000.
  • Serous cystadenoma/Microcystic adenoma Khalid A et al. Am J Gastroenterol 2007 ; 102 : 2339 – 2349. ‘Honeycombed’ microcystic appearance of serous cystadenoma EUS image
  • Serous cystic neoplasm/Macrocystic-type Lim LG et al. J Gastroenterol Hepatol 2011 ; 261 : 702 – 1708. EUS
  • Serous cystadenoma Microcytic lesion with central scar Diagnostic of benign serous cystadenoma T2-weighted MRI Sahani DV et al. J Am Coll Radiol 2009 ; 6 : 376 – 380.
  • Serous cystadenocarcinoma • 26 published cases of serous cystadenocarcinoma • Mean age at diagnosis 68 2 years (range: 52 to 81) • Women affected more commonly: 2:1 King JC et al. J Gastrointest Surg 2009 ; 13 : 1864 – 1868. Small but finite risk of malignancy for serous cystic neoplasms of pancreas
  • Mucinous cystic neoplasm Sahani DV et al. J Am Coll Radiol 2009 ; 6 : 376 – 380. Large septated macrocyst in pancreatic tail of middle-age women Typical of mucinous cystic neoplasm Contrast-enhanced multi-detector CT
  • Mucinous cystic neoplasm Khalid A et al. Am J Gastroenterol 2007 ; 102 : 2339 – 2349. Hypodense lesion in body of pancreas Internal septations & wall calcifications suggesting MCN Contrast-enhanced CT scan
  • Mucinous cystic neoplasm Fasanella KE et al. Best Pract Res Clin Gastroenterol 2009 ; 23 : 35 – 48. Unilocular cystic lesion in pancreatic tail CT scan EUS Small septation/‘daughter cyst’ Posterior cyst enhancement
  • Morphologic classification of IPMN Branch pancreatic duct Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269. Diffuse main pancreatic duct Segmental main pancreatic duct Mixed (main & branch ducts)
  • Prevalence of cancer in IPMNs • Main duct IPMN 60 – 90% • Branch duct IPMN 5 – 45% Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269.
  • Intraductal papillary mucinous neoplasm Endoscopic view Fish mouth deformity Secondary to mucin overproduction & extrusion Pathognomonic for IPMN Talley NJ , Kane SV, & Wallace MB. Practical Gastroenterology & Hepatology: Small & Large Intestine & Pancreas. Blackwell Publishing, 1st edition, 2010.
  • Main-duct IPMN Multiple filling defects secondary to mucinous globules Sarr MG et al. J Gastrointest Surg 2003 ; 7 : 417 – 28. ERCP
  • Main duct IPMN 79-year-old man with prostate cancer & rising PSA underwent CECT Total pancreatectomy → High risk of associated adenocarcinoma Zaheer A et al. Abdom Imaging 2012 in press. Markedly dilated main pancreatic duct Dilation of main duct ≥ 1 cm strongly suggests IPMN
  • Main duct IPMN Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269. EUS of 66-year-old man with non-specific symptoms Dilated main pancreatic duct with mural nodule
  • Pre-surgical follow-up according to IPMN size Sahani DV et al. Clin Gastroenterol Hepatol 2009; 7 : 259 – 269. EUS 1 cm: 1 year 1 – 2 cm: 6 – 12 months 2 – 3 cm: 6 months Malignant Resection Benign Follow-up Resection Suspicious features Increased size > 3 cm > 1 cm growth per year Mural nodules Main duct dilatation Solid component Thick wall/septations
  • Major features of cystic pancreatic neoplasms Pseudocyst SCN MCN IPMN Age Variable Middle age Middle age Elderly Sex M > F F > M F M > F Alcohol abuse Yes No No No History of pancreatitis Yes No No Frequent Malignant potential None Very rare Moderate to high Low to high Barresi L et al. World J Gastrointest Endosc 2012 ; 4 : 247 – 259.
  • EUS/FNA of cystic pancreatic neoplasms Brugge WR. Gastrointest Endosc 2009 ; 69 (suppl): 203 – 209. Pseudocyst SCN MCN IPMN Location Evenly Evenly Tail Head Cytology Pigmented histiocytes Bland PAS + Mucinous Mucinous Viscocity Low Low Increased High Cystic amylase High Low Low High Cystic CEA < 200 ng/mL < 0.5 ng/mL > 200 ng/mL > 200 ng/mL K-RAS mutations Negative Negative Positive Positive
  • Traditional therapeutic approach to management of cystic lesions Pseudocyst Serous Mucinous Malignant Head Drain Monitor Monitor Resect Body Drain Monitor Resect Resect Tail Resect Resect Resect Resect Brugge WR. Gastrointest Endosc 2009 ; 69 (suppl): 203 – 209.
  • EUS evaluation of cystic lesions of pancreas • Morphologic analysis Microcystic Macrocystic Associated mass • FNA Complete evacuation if possible Antibiotic prophylaxis • Cyst fluid analysis Amylase CEA Cytology Brugge WR. Gastrointest Endosc 2004 ; 59 : 698 – 707. Interpretation in conjunction with history & CT scanning
  • Solid pseudo-papillary tumor • Infrequently-encountered tumor • Typically affects young women without significant symptoms • Its behavior relatively indolent & largely benign • Patients may survive long time after radial resection • If possible, surgery justified for local invasion or metastasis • Prognosis even with unresectable metastasis is good • Role of chemo & radiotherapy remains to be studied Yu PF et all. World J Gastroenterol 2010 ; 16(10): 1209 – 1214.
  • Solid pseudo-papillary tumor Contrast-enhanced CT scan Well encapsulated heterogeneous mass in tail of pancreas Typical appearence Choi JY et al. AJR 2006 ; 187 : W178 – W186.
  • Solid pseudo-papillary tumor Atypical appearance • Metastasis • Ductal obstruction • Parenchymal and extracapsular invasion • Simulation of islet cell tumor • Intratumoral calcification • Occurrence in a male patient Choi JY et al. AJR 2006 ; 187 : W178 – W186.
  • Cystic masses of the pancreas *IPMN: Intraductal Papillary Mucinous Neoplasm Sahani DV et al. RadioGraphics 2005 ; 25 : 1471 – 1484. True epithelial cysts* Autosomal-dominant polycystic kidney disease von Hippel–Lindau disease Cystic fibrosis Pseudocyst Cystic pancreatic neoplasm Common Serous cystadenoma Mucinous cystic neoplasm IPMN Rare Solid pseudo-papillary tumor Acinar cell cystadenocarcinoma Lymphangioma Hemangioma Paraganglioma Solid pancreatic neoplasm with cystic degeneration Pancreatic adenocarcinoma Cystic islet cell tumor (insulinoma, gastrinoma,.) Metastasis Cystic teratoma Sarcoma
  • Pancreatic adenocarcinoma with cystic degeneration Infiltrative lesion in head of pancreas involving SMA & SMV Obstructed–dilated pancreatic duct Axial CECT image Khan A et al. Am J Roentgenol 2011; 196 : W668 – W677.
  • Cystic insulinoma Small cystic masses in body & tail of pancreas 36-year-old woman with MEN-1 Patient presented with primary hyperparathyroidism Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
  • Cystic glucagonoma 34-year-old woman presented with diabetes & necrolytic migratory erythema Large cystic mass in tail of pancreas Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388.
  • Cystic nonfunctioning neuro-endocrine neoplasm Demos TC et al. Am J Roentgenol 2002 ; 179 : 1375 – 1388. 65-year-old man Cystic elements in neoplasm extending from tail of pancreas
  • Metastatic renal cell carcinoma Demos TC et al. AJR 2002 ; 179 : 1375 – 1388. Large cystic mass with irregularly thickened wall in body & tail Note similarity to RCC of right kidney 70-year-old woman with RCC
  • Pancreatic schwannoma Mummadi RR et al. Gastrointest Endoscopy 2009 ; 69 : 341. 7-cm multiloculated cystic pancreatic body mass Thick septations with many solid areas More consistent with a cystic neoplasm EUS-FNA not performed & surgical resection performed Linear EUS
  • Pancreatic arteriovenous malformation Yamamoto T et al. J Clin Ultrasound 2000 ; 28 : 365 – 367. Pulsatile color signals with low resistance Color & pulsed Doppler US Cystic pancretic lesions Gray-scale US Racemose hypervascular network Celiac arteriogram Early filling of PV
  • Conclusion Discriminate benign lesions from those that require surgery • Clinical history is essential to suggest diagnosis • CT gives critical information: size, septations, ductal dilatation, calcifications, mural nodules, etc. • MRI/MRCP recommended as next imaging study in: Equivocal differentiation between cystic & solid lesions Evaluation of subtle enhancement Communication with pancreatic duct • EUS morphology, fluid analysis & cytology aid in diagnosis Zaheer A et al. Abdom Imaging 2012 in press. Barresi L et al. World J Gastrointest Endosc 2012 ; 4 : 247 – 259.
  • Conclusion • No single test accurate enough to make sure diagnosis • Diagnosis of cystic pancreatic lesion is a puzzle Bits of information deriving from demography, clinical history, radiology, EUS & intracystic fluid analyses Barresi L et al. World J Gastrointest Endosc 2012 ; 4 : 247 – 259.
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