Dr. Ketul V. Shah
1st year MCh G.I.Surgery resident
        V.S. Hospital, Ahmedabad

  Discussion; Dr Sanjay Nagral,
 Consultant GI surgeon,Mumbai.
Cystic lesion in Pancreas
           Task ahead
I. Is the lesion from pancreas?
II. Lesion is solid or cystic?
III. Neoplastic v/s non-neoplastic?
IV. SCA v/s MCN v/s IPMN?
V. Management?
CASE HISTORY
 45y/F
 c/o recurrent vomiting and loss of appetite - 6 mths.
    Vomitus contained food and occurred ½ to 1 hour after
     meals & was non-bilious, non-projectile
 h/o diffuse abdominal pain
 significant weight loss ++
 Anorexia ++
 There was no preceding history of any severe
 abdominal pain or jaundice

 No h/o lump in abdomen, abdominal distension


 Pt is not a k/c/o diabetes and no other positive medical
 or surgical history
ON EXAMINATION
 pallor +, No LNpathy


 P/A- soft, non tender, no palpable lump, no
 organomegaly. No ascitis.

 other systems normal.
INVESTIGATIONS DONE
 LFT – wnl
 S. Amylase and Lipase were normal
 USG - A cystic lesion in the pancreatic head and
  neck region of about 7x5cm
 CECT - 73x60 mm cystic lesion in pancreatic head
  & neck region; cystic wall-3mm; MPD- normal; no
  peri-pancreatic LN or fluid.
 S. CA 19-9 – wnl
 S. CEA - wnl
Cystic lesions of the pancreas
While approaching a cystic lesions
we need to know….
 Broad differential diagnosis
 Epidemiology of common lesions
 Clinical presentation
 Blood tests
 Imaging
 Histology
Broad D/D’s of Cystic Pancreatic
            Lesions
1) Pseudocyst (75-80%)

2) Common cystic pancreatic neoplasms
       Mucinous cystic neoplasm (10-45%)
       Serous cystic neoplasm (32-39%)
       IPMN (21-33%)

3) Rare cystic pancreatic neoplasms
       Solid pseudopapillary tumor (<10%)
       Acinar cell cystadenocarcinoma (<!%)
       Lymphangioma
       Hemangioma
       Paraganglioma
4) Solid pancreatic lesions with cystic degeneration
      Pancreatic adenocarcinoma (<1%)
      Cystic islet cell tumor
       (insulinoma, glucagonoma, gastrinoma) (<10%)
      Metastasis
      Cystic teratoma
      Sarcoma
5) Hydatid cyst
6) Lymphatic cyst
7) True epithelial cysts a/w-
    von Hippel–Lindau disease
    autosomal -dominant polycystic kidney disease
Imaging in Cystic lesions….

 As  against Solid lesions, Imaging may be
 diagnostic in many cystic lesions, obviating further
 investigations
Imaging in cystic lesions of
        pancreas;
How does it help???
Four Morphologic Types of Cystic
     Lesions of the Pancreas
a) Unilocular Cyst
 Pseudocyst- most common


Other causes-
 IPMN occasionally
 Unilocular serous cystadenoma
 Lymphoepithelial cyst
 Multiple
       von Hippel-Lindau
       Pseudocysts
b) Microcystic Lesions

 Serous cystadenoma

(Only lesion included in this category)
c) Macrocystic Lesions
 Mucinous cystic neoplasms


 Intraductal Papillary Mucinous Neoplasm (IPMN)
d) Cysts with a solid component
- Unilocular or multilocular

 True cystic tumors or solid pancreatic neoplasms with
  cystic component/degeneration

    Solid pseudopapillary tumor (SPEN)
    Mucinous cystic neoplasms
    IPMNs
    Islet cell tumor
    Adenocarcinoma
    Metastasis
Let us look at the possibilities in
our patient……
Could this patient have
        PANCREATIC PSEUDOCYST?
 Symptoms
       Abdominal pain (80 – 90%)
       Lump in abdomen
       Nausea / vomiting ( due to gastric or duodenal compression)
       Early satiety
       Bloating, indigestion
       Jaundice ( due to compression of bile duct)
       Hemorrhage


 Signs
       Tenderness
       Abdominal fullness
       Palpable mass
Blood tests in suspected
pseudocyst
 Amylase/Lipase
Imaging in Pseudocyst…..
 Ultrasonography
    Most practical & Sensitivity 75 – 90%
    limited by patient habitus, operator experience and air in stomach


 CT scan
    Gold standard for initial assessment and follow-up
    Sensitivity  90- 100%


 MRI
    Better detail of content of cyst


 MRCP
    Establish the relationship of the pseudocyst to the pancreatic ducts


 Endoscopic Ultrasonography (EUS +/- FNA)
    Distinguishing pancreatic cystic lesions, helps in FNA
So, if you have a cystic lesions
with…..
 Sudden onset of pain consistent with pancreatitis pain
 Imaging features of associated pancreatitis
 Unilocular cyst; and
 Elevated amylase/lipase



 You may not investigate any further…… It must be
 a pseudocyst
Common neoplastic lesions
  and their features….
Cystic neoplasm of pancreas
MUCINOUS CYSTIC NEOPLASMS
 Most common - 10% to 45% (MCA -67%, MCAC -
 33%)
 > 95% in women ( Mean ~ 50 yrs)


 Typically involve the body and tail of the pancreas


 Never multifocal, occurring only in one location
 within the pancreas.
 Asymptomatic in 75% cases

       If symptoms, usually due to mass effect

       Adominal pain

       Palpable mass
 CT or MRI of the abdomen

   Complex macrocystic mass with internal septations


   MRCP no communication between duct and the cyst


   Contrast enhanced scans show enhancement of the cyst wall and
    accentuate any septations and mural nodules

   Distal to the tumor, the pancreas may show changes of CP


   Presence of mural nodule and septal calcification s/o –
    malignancy
 EUS can identify septations and cyst wall nodules in
  more detail than MRI or CT

 Allows cyst wall biopsy and cyst fluid aspiration for
  analysis

 Cyst fluid analysis generally reveals
    thick and mucoid material and low fluid amylase
    elevated tumor markers (CEA)
    mucinous epithelial cells by cytology
Mucinous Cystadenocarcinoma
 Complex macrocystic lesion with internal septations
 Peripheral and septal calcification indicative of malignancy
  (arrowheads)
SEROUS CYSTADENOMAS
 Second MC Cystic tumor of the pancreas
   formerly known as microcystic adenomas



 Occurring mostly in women (75%) with a mean 62 years


 Most (50% to 70%) occur in the body or tail of the
  pancreas

 An association with von Hippel-Lindau disease
 Mostly asymptomatic
   being detected during evaluation for other unrelated conditions




 Can present with a palpable mass - size (10 to 25 cm)
Serous Cystadenoma


                     Lesion with numerous
                     microcysts giving a
                     “honey-comb”
                     appearance
                     Lobulated outline
                     Central stellate scar
Serous cystadenoma
 Pathognomonic image by CT scan is that of a spongy mass
  with a central “sunburst” calcification - only 10% of patients

 Visualization of four of the following five CT and MRI features
  aid in making the diagnosis

    location in the pancreatic body and tail
    wall thickness < 2 mm
    lobulated contour
    lack of communication with the pancreatic duct
    minimal wall enhancement
IPMN
(Intra-ductal Papillary Mucinous Neoplasm)

 Types - depend on involvement of duct
   main pancreatic duct, isolated side branches, or a combination of
    both


 Benign (adenoma), borderline, or malignant


 Malignant neoplasms account for 60% of IPMNs
IPMN FEATURES
 Equal frequency in men and women


 Median age at diagnosis - about 65 years


 75% of patients are symptomatic
   Abdominal pain and weight loss – MC complaints
   Recurrent pancreatitis or
   Acute pancreatitis
 Patients with malignant neoplasms are more likely to be
   older and more likely to present with jaundice or new-onset
    diabetes
DIAGNOSIS
 Differentiation of IPMN from other cystic pancreatic
  masses may be difficult at CT

 Most reliable findings for the diagnosis
    Presence of a communication between the cystic lesion and the
     main pancreatic duct


 Presence of mural nodules projecting into the main
  pancreatic duct or cystic lesions
DIAGNOSIS
 Diffusely distended pancreatic duct with mucinous filling
  defects and grape-like, cystic, space-occupying lesions

 Sensitivity in diagnosing an IPMN
   highest for MRI with MRCP (88%),followed by ERCP (68%) and
    CT (42%)
 Pathognomonic for IPMN in ERCP
   A wide and gaping papilla with secretion of mucin and filling
    defects in the dilated pancreatic duct –FISH MOUTH AMPULLA
Cystic lesions of pancreas;
     will blood tests help ?
 Amylase and/or Lipase??
 CEA? Ca 19-9 ??



Not diagnostic of any of the cystic pancreatic tumors
Only provide corroborative evidence
Serum amylase or lipase levels
            Increased - pseudocyst, IPMN
Serum CA 19-9 & CEA
            normal - benign cystic pancreatic tumors
            modestly elevated - MCNs and IPMNs,
                   particularly patients with malignancies
            Markedly elevated -retention cyst secondary
                   to obstruction of the main pancreatic
                   duct by an adenocarcinoma.
Cystic lesions of Pancreas; will
aspiration and analysis of fluid
             help?
Cyst Fluid Analysis
                                     Viscosity          Amylase   Cytology
 Pseudocyst                             Low              High     Inflamm.
 SCA                                    Low              Low         5% +
 MCA                                   High              Low        40% +
 MCAC                                  High              Low        67% +

                                         CEA            CA 15-3   CA 72-4
 Pseudocyst                              Low             Low       Low
 SCA                                     Low             Low       Low
 MCA                                     High            High      Low
 MCAC                                    High            High      High


[1] Lewandrowski KB, et al. Ann Surg 1993, 217:41-7.
[2] Brugge WR, et al. N Engl J Med 2004, 351:1218-26.
Coming back to our case…
Our patient has….
 No clear cut diagnosis on history
 Serum markers were non-informative
 Imaging not diagnostic



 Therefore EUS guided FNA was
 done..……Adenocarcinoma with cystic degeneration
DR. KETUL SHAH

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Cystic lesions of the pancreas

  • 1. Dr. Ketul V. Shah 1st year MCh G.I.Surgery resident V.S. Hospital, Ahmedabad Discussion; Dr Sanjay Nagral, Consultant GI surgeon,Mumbai.
  • 2. Cystic lesion in Pancreas Task ahead I. Is the lesion from pancreas? II. Lesion is solid or cystic? III. Neoplastic v/s non-neoplastic? IV. SCA v/s MCN v/s IPMN? V. Management?
  • 3. CASE HISTORY  45y/F  c/o recurrent vomiting and loss of appetite - 6 mths.  Vomitus contained food and occurred ½ to 1 hour after meals & was non-bilious, non-projectile  h/o diffuse abdominal pain  significant weight loss ++  Anorexia ++
  • 4.  There was no preceding history of any severe abdominal pain or jaundice  No h/o lump in abdomen, abdominal distension  Pt is not a k/c/o diabetes and no other positive medical or surgical history
  • 5. ON EXAMINATION  pallor +, No LNpathy  P/A- soft, non tender, no palpable lump, no organomegaly. No ascitis.  other systems normal.
  • 6. INVESTIGATIONS DONE  LFT – wnl  S. Amylase and Lipase were normal  USG - A cystic lesion in the pancreatic head and neck region of about 7x5cm  CECT - 73x60 mm cystic lesion in pancreatic head & neck region; cystic wall-3mm; MPD- normal; no peri-pancreatic LN or fluid.  S. CA 19-9 – wnl  S. CEA - wnl
  • 8. While approaching a cystic lesions we need to know….  Broad differential diagnosis  Epidemiology of common lesions  Clinical presentation  Blood tests  Imaging  Histology
  • 9. Broad D/D’s of Cystic Pancreatic Lesions 1) Pseudocyst (75-80%) 2) Common cystic pancreatic neoplasms  Mucinous cystic neoplasm (10-45%)  Serous cystic neoplasm (32-39%)  IPMN (21-33%) 3) Rare cystic pancreatic neoplasms  Solid pseudopapillary tumor (<10%)  Acinar cell cystadenocarcinoma (<!%)  Lymphangioma  Hemangioma  Paraganglioma
  • 10. 4) Solid pancreatic lesions with cystic degeneration  Pancreatic adenocarcinoma (<1%)  Cystic islet cell tumor (insulinoma, glucagonoma, gastrinoma) (<10%)  Metastasis  Cystic teratoma  Sarcoma 5) Hydatid cyst 6) Lymphatic cyst 7) True epithelial cysts a/w-  von Hippel–Lindau disease  autosomal -dominant polycystic kidney disease
  • 11. Imaging in Cystic lesions….  As against Solid lesions, Imaging may be diagnostic in many cystic lesions, obviating further investigations
  • 12. Imaging in cystic lesions of pancreas; How does it help???
  • 13. Four Morphologic Types of Cystic Lesions of the Pancreas
  • 14. a) Unilocular Cyst  Pseudocyst- most common Other causes-  IPMN occasionally  Unilocular serous cystadenoma  Lymphoepithelial cyst  Multiple  von Hippel-Lindau  Pseudocysts
  • 15. b) Microcystic Lesions  Serous cystadenoma (Only lesion included in this category)
  • 16. c) Macrocystic Lesions  Mucinous cystic neoplasms  Intraductal Papillary Mucinous Neoplasm (IPMN)
  • 17. d) Cysts with a solid component - Unilocular or multilocular  True cystic tumors or solid pancreatic neoplasms with cystic component/degeneration  Solid pseudopapillary tumor (SPEN)  Mucinous cystic neoplasms  IPMNs  Islet cell tumor  Adenocarcinoma  Metastasis
  • 18. Let us look at the possibilities in our patient……
  • 19. Could this patient have PANCREATIC PSEUDOCYST?  Symptoms  Abdominal pain (80 – 90%)  Lump in abdomen  Nausea / vomiting ( due to gastric or duodenal compression)  Early satiety  Bloating, indigestion  Jaundice ( due to compression of bile duct)  Hemorrhage  Signs  Tenderness  Abdominal fullness  Palpable mass
  • 20. Blood tests in suspected pseudocyst  Amylase/Lipase
  • 21. Imaging in Pseudocyst…..  Ultrasonography  Most practical & Sensitivity 75 – 90%  limited by patient habitus, operator experience and air in stomach  CT scan  Gold standard for initial assessment and follow-up  Sensitivity  90- 100%  MRI  Better detail of content of cyst  MRCP  Establish the relationship of the pseudocyst to the pancreatic ducts  Endoscopic Ultrasonography (EUS +/- FNA)  Distinguishing pancreatic cystic lesions, helps in FNA
  • 22. So, if you have a cystic lesions with…..  Sudden onset of pain consistent with pancreatitis pain  Imaging features of associated pancreatitis  Unilocular cyst; and  Elevated amylase/lipase You may not investigate any further…… It must be a pseudocyst
  • 23. Common neoplastic lesions and their features….
  • 24. Cystic neoplasm of pancreas
  • 25. MUCINOUS CYSTIC NEOPLASMS  Most common - 10% to 45% (MCA -67%, MCAC - 33%)  > 95% in women ( Mean ~ 50 yrs)  Typically involve the body and tail of the pancreas  Never multifocal, occurring only in one location within the pancreas.
  • 26.  Asymptomatic in 75% cases  If symptoms, usually due to mass effect  Adominal pain  Palpable mass
  • 27.  CT or MRI of the abdomen  Complex macrocystic mass with internal septations  MRCP no communication between duct and the cyst  Contrast enhanced scans show enhancement of the cyst wall and accentuate any septations and mural nodules  Distal to the tumor, the pancreas may show changes of CP  Presence of mural nodule and septal calcification s/o – malignancy
  • 28.  EUS can identify septations and cyst wall nodules in more detail than MRI or CT  Allows cyst wall biopsy and cyst fluid aspiration for analysis  Cyst fluid analysis generally reveals  thick and mucoid material and low fluid amylase  elevated tumor markers (CEA)  mucinous epithelial cells by cytology
  • 29. Mucinous Cystadenocarcinoma  Complex macrocystic lesion with internal septations  Peripheral and septal calcification indicative of malignancy (arrowheads)
  • 30. SEROUS CYSTADENOMAS  Second MC Cystic tumor of the pancreas  formerly known as microcystic adenomas  Occurring mostly in women (75%) with a mean 62 years  Most (50% to 70%) occur in the body or tail of the pancreas  An association with von Hippel-Lindau disease
  • 31.  Mostly asymptomatic  being detected during evaluation for other unrelated conditions  Can present with a palpable mass - size (10 to 25 cm)
  • 32. Serous Cystadenoma Lesion with numerous microcysts giving a “honey-comb” appearance Lobulated outline Central stellate scar
  • 33. Serous cystadenoma  Pathognomonic image by CT scan is that of a spongy mass with a central “sunburst” calcification - only 10% of patients  Visualization of four of the following five CT and MRI features aid in making the diagnosis  location in the pancreatic body and tail  wall thickness < 2 mm  lobulated contour  lack of communication with the pancreatic duct  minimal wall enhancement
  • 34. IPMN (Intra-ductal Papillary Mucinous Neoplasm)  Types - depend on involvement of duct  main pancreatic duct, isolated side branches, or a combination of both  Benign (adenoma), borderline, or malignant  Malignant neoplasms account for 60% of IPMNs
  • 35. IPMN FEATURES  Equal frequency in men and women  Median age at diagnosis - about 65 years  75% of patients are symptomatic  Abdominal pain and weight loss – MC complaints  Recurrent pancreatitis or  Acute pancreatitis  Patients with malignant neoplasms are more likely to be  older and more likely to present with jaundice or new-onset diabetes
  • 36. DIAGNOSIS  Differentiation of IPMN from other cystic pancreatic masses may be difficult at CT  Most reliable findings for the diagnosis  Presence of a communication between the cystic lesion and the main pancreatic duct  Presence of mural nodules projecting into the main pancreatic duct or cystic lesions
  • 37. DIAGNOSIS  Diffusely distended pancreatic duct with mucinous filling defects and grape-like, cystic, space-occupying lesions  Sensitivity in diagnosing an IPMN  highest for MRI with MRCP (88%),followed by ERCP (68%) and CT (42%)
  • 38.  Pathognomonic for IPMN in ERCP  A wide and gaping papilla with secretion of mucin and filling defects in the dilated pancreatic duct –FISH MOUTH AMPULLA
  • 39. Cystic lesions of pancreas; will blood tests help ?  Amylase and/or Lipase??  CEA? Ca 19-9 ?? Not diagnostic of any of the cystic pancreatic tumors Only provide corroborative evidence
  • 40. Serum amylase or lipase levels Increased - pseudocyst, IPMN Serum CA 19-9 & CEA normal - benign cystic pancreatic tumors modestly elevated - MCNs and IPMNs, particularly patients with malignancies Markedly elevated -retention cyst secondary to obstruction of the main pancreatic duct by an adenocarcinoma.
  • 41. Cystic lesions of Pancreas; will aspiration and analysis of fluid help?
  • 42. Cyst Fluid Analysis Viscosity Amylase Cytology Pseudocyst Low High Inflamm. SCA Low Low 5% + MCA High Low 40% + MCAC High Low 67% + CEA CA 15-3 CA 72-4 Pseudocyst Low Low Low SCA Low Low Low MCA High High Low MCAC High High High [1] Lewandrowski KB, et al. Ann Surg 1993, 217:41-7. [2] Brugge WR, et al. N Engl J Med 2004, 351:1218-26.
  • 43. Coming back to our case…
  • 44. Our patient has….  No clear cut diagnosis on history  Serum markers were non-informative  Imaging not diagnostic  Therefore EUS guided FNA was done..……Adenocarcinoma with cystic degeneration