pancreatic cystic neoplasm: definition, classification, diagnosis and treatment
TRANSCRIPT
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Pancreatic Cystic Neoplasm.
MARCO CASTILLO
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Pancreatic cystic neoplasm. Definition. Risk of malignancy. Differential diagnosis. Classification.
Serous cystadenoma (SCA). Mucinous cystic neoplasm (MCN). Intraductal papillary mucinous neoplasm
(IPMN). Solid pseudopapillary tumor (SPPT). Lymphoepithelial cyst. Pseudocyst.
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Pancreatic cystic neoplasms. Heterogeneous group of cystic lesions.
Histopathologically different. Diverse natural history.
Incidental finding in 2% of abdominal image.
Risk of Malignancy:At diagnosis 0.1%.Cyst > 3 cm.Solid componentPancreatic duct dilation.Histological type of the cyst.
Differential Diagnosis: Congenital cysts. Parasitic cysts. Pseudocyst. Systemic diseases:
Von Hippel-Lindau diseaseCystic fibrosis.Neuroendocrine tumor
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Classification Cystic variants of solid
neoplasmsCystic ductal
adenocarcinomaCystic neuroendocrine tumorCystic acinar cell carcinoma
Non-neoplastic Retention cyst. Inflammatory cyst. Mucinous non-neoplastic cyst
Neoplastic Serous
Serous cystadenomaSerous
cystadenocarcinoma Mucinous
Mucinous cystic neoplasmIntraductal papillary
mucinous neoplasm Solid pseudopapillary tumor Lymphoepithelial cyst
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Serous cystic neoplasm: Cystadenoma 1% of exocrine pancreas tumors. 30% of cystic neoplasm. Women 50 – 70 years old. No preferred location. Low risk of malignancy. Clinical presentation: > 4 cm.
50% Incidental. Epigastric pain Abdominal fullness. Weight loss.
Glycogen rich, PAS positive, cuboidal epithelium.
Polycystic (microcystic) or Oligocystic (macrocystic)
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Serous Cystic Tumors Characteristics: CT:
Polycystic / Microcystic. Stellate scar or sunburst calcifications. Internal septate. Honey comb appearance.
MRCP: Endoscopic ultrasonography with FNA
Cytology: Scant cellularity/Bloody Biochemistry:
Low CEA. Low amylase. Low CA 19-9.
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Serous Cystic Tumors
Treatment: No Symptoms:
Observation 6-12 months.
Consider resection if: > 4 cm. Symptomatic. No definite diagnosis. Rapid growth.
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Serous Cystic Tumors:
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Serous Cystic Tumors:
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Female – middle age 30 – 50 years. Body or tail of the pancreas 90%. Presentation:
Abdominal discomfort. Recurrent Pancreatitis. Gastric outlet obstruction
Classification: Adenomas 75%. Borderline tumors Carcinoma.
Mucinous Cystic Neoplasm
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Mucinous Cystic Neoplasm
Characteristics: Round thick walled cysts, septate. There is no communication with
the pancreatic ducts. Subepithelial ovarian-like stroma.
Spindle cells with scant cytoplasm
Small clusters of leutinized cells.
There is association with KRAS mutation.
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Characteristics: CT/MRI:
Thick cyst wall Smooth sharp boundaries. Do NOT communicate with pancreatic
ductal system. Pancreatic duct dilation. Eggshell calcification
Endoscopic ultrasonography with FNA: Viscous fluid. CEA
Adenoma > 200 ng/mL Mucinous cystadenocarcinoma >6000
ng/mL PET-Scan.
Mucinous Cystic Neoplasm:
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Surgical resection if any of the following: > 3 cm. Main duct dilation Mural nodule. Candidates.
Observation if no candidate with small tumors.
Prognosis: Poor if Invasive disease.
Follow up: Non invasive: Annually the first years. Invasive:
Every 4 month the first 2 years. Biannually until year 5.
Mucinous Cystic Neoplasm: Treatment
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25% of the pancreatic cystic Neoplasm.
Male = Female 50 – 70 years. Head > Body Presentation:
Abdominal pain. Pancreatitis. Weight loss. Jaundice. New onset diabetes.
Characteristics: Main duct dilation. Papillary projections Excessive mucin production.
Intraductal Papillary Mucinous Neoplasm.
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Genetics: Mutations in K-Ras, CDKN-2a, RNF 43,
Types: According to the affected duct:
Main duct type. Invasion 57 – 92 %.
Branch duct type. Invasion 6 – 46%
According to the dysplasia: Adenoma. Borderline. Carcinoma in situ. Frankly invasive.
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Contrast enhanced CT characteristics: Main pancreatic or duct dilation. Involvement of any part of the
pancreas or the whole pancreas. Continuity of cyst with ductal system. Irregular and poorly demarcated.
Surgical Treatment: Imaging suggesting malignancy. Partial Pancreatectomy with Frozen
section analysis.
Follow up: Annually.
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Female 20 - 30 years. Rare tumor. Characteristics:
Large, encapsulated. Solid and cystic area. Hemorrhage. Cystic degeneration. Calcification.
Differential diagnosis: Neuroendocrine tumor.
Accumulation of β-Catenin protein. Vimentin+ and CD10 + Negative Chromogranin
Solid Pseudopapillary tumor.
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Treatment: Pancreatodudenectomy Distal Pancreatectomy.
Prognosis: Favorable even in advance
disease. Survival after 5 years > 95%.
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Female 50 – 70 years old. Asymptomatic. Evenly distributed over the pancreas. Characteristics:
Lining of stratified squamous epithelium.
Surrounding of lymphoid tissue. Filled by Keratin and cholesterol.
CT: Multi or unilocular cyst. Encapsulated
Lymphoepithelial Cyst MRI:
Hyperintense signal in T1. Hypointense in T2.
FNA: Cytology:
Squamous cells and keratin debris.
Cholesterol crystals. Treatment:
Observation. Surgery only in equivocal
diagnose.
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Pseudocyst. Localized fluid collection. Presented after pancreatitis. Male > Female. Fibrous wall and granulation tissue. Presentation:
Abdominal pain. Jaundice. Palpable mass. GI obstruction. Infection. Peritonitis.
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Pseudocyst. Fluid content:
Normal CEA. Low viscosity. Increased amylase. Negative cytology.
CT: Large cavity. Thick wall. Irregular. Calcifications. Pseudo aneurysm of splenic artery.
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Pseudocyst. Treatment: None unless.
Complications. Infection. Bleeding. Peritonitis. GI obstruction.
Symptoms. Concerns about malignancy.
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References: Katz MH, Mortesson MM, Wang H, et al. Diagnosis and Management of Cystic
Neoplasms of the Pancreas: An Evidence-Based Approach. J Am Coll Surg. 2008 Jul;207(1):106-20.
UpToDate: Classification of pancreatic cysts. Pancreatic cystic neoplasms: Clinical manifestations, diagnosis, and
management. Intraductal papillary mucinous neoplasm of the pancreas (IPMN):
Pathophysiology and clinical manifestations. Imaging in Mucinous Cystic Neoplasms of the Pancreas.
http://emedicine.medscape.com/article/371197-overview Pancreatic Pseudocyst Imaging
http://emedicine.medscape.com/article/373117-overview#a1