Pancreatic Cystic Neoplasm.

MARCO CASTILLO

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.

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

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

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)

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.

Serous Cystic Tumors

Treatment: No Symptoms:

Observation 6-12 months.

Consider resection if: > 4 cm. Symptomatic. No definite diagnosis. Rapid growth.

Serous Cystic Tumors:

Serous Cystic Tumors:

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

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.

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:

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

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.

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.

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.

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.

Treatment: Pancreatodudenectomy Distal Pancreatectomy.

Prognosis: Favorable even in advance

disease. Survival after 5 years > 95%.

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.

Pseudocyst. Localized fluid collection. Presented after pancreatitis. Male > Female. Fibrous wall and granulation tissue. Presentation:

Abdominal pain. Jaundice. Palpable mass. GI obstruction. Infection. Peritonitis.

Pseudocyst. Fluid content:

Normal CEA. Low viscosity. Increased amylase. Negative cytology.

CT: Large cavity. Thick wall. Irregular. Calcifications. Pseudo aneurysm of splenic artery.

Pseudocyst. Treatment: None unless.

Complications. Infection. Bleeding. Peritonitis. GI obstruction.

Symptoms. Concerns about malignancy.

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