Atypical and Rare Pancreatic Tumors

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 19/07/2015

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 Extremely aggressive and almost always unresectable

image Large, heterogeneous, moderately enhancing, exophytic mass with necrosis and cystic change
• Small cell carcinoma

image Highly aggressive, with hematologic and lymphatic metastases at time of diagnosis
image Large, homogeneous, mildly enhancing mass with confluent local and distant lymphadenopathy
image May be indistinguishable from lymphoma
• Giant cell carcinoma (pleomorphic or osteoclast)

image Resection often impossible due to large size
image Large, heterogeneous, cystic, low-density mass with frequent hemorrhage, septation, and calcification
• Acinar cell carcinoma

image Slightly better prognosis than adenocarcinoma
image Large, well-circumscribed mass with cystic degeneration, exophytic component, and enhancing capsule
image Usually no biliary/pancreatic duct dilatation
image Vascular occlusion uncommon (20%); may invade the portal vein/superior mesenteric vein
• Pancreatoblastoma

image Most often occurs in children (mean age 2.5 years), but very rarely affects adults (mean age 40 years)
image Poor prognosis: Worse outcomes in adults than children
image Large, heterogeneous mass with frequent internal calcifications and necrosis/hemorrhage
image No pancreatic or biliary duct obstruction
• Pancreatic plasmacytoma

image Consider in patients with known myeloma
image Homogeneous mass without pancreatic/biliary ductal obstruction or pancreatic atrophy
image Mimics lymphoma, but usually no lymphadenopathy
• Pancreatic Lipoma

image Benign fat-containing mass (-80 to -120 Hounsfield units) with surrounding capsule
image Most often occur in pancreatic head
• Pancreatic schwannoma

image Usually benign, with malignant transformation very rare
image Well-circumscribed mass ± cystic degeneration
image Can closely mimic neuroendocrine tumors (usually with less avid vascularity)

TOP DIFFERENTIAL DIAGNOSES

• Pancreatic adenocarcinoma, pancreatic neuroendocrine tumor, lymphoma, mucinous cystic neoplasm
image
(Left) Axial CECT demonstrates a well-circumscribed, encapsulated, enhancing mass image in the pancreatic head with solid and cystic components. This was found to be an acinar cell carcinoma at surgery. As in this case, these tumors often mimic the appearance of neuroendocrine tumors, albeit with less hyperenhancement.

image
(Right) Axial CECT demonstrates a lobulated, well-circumscribed enhancing mass image arising from the pancreatic tail. This mass was found to be an acinar cell carcinoma at surgery.
image
(Left) Axial CECT in a patient with known myeloma demonstrates a well-circumscribed, enhancing mass image in the pancreatic head.

image
(Right) Axial CECT in the same patient shows that the mass causes no pancreatic ductal obstruction or parenchymal atrophy, highly atypical for an adenocarcinoma. The mass was found to be a rare pancreatic plasmacytoma at biopsy.

TERMINOLOGY

Abbreviations

• Anaplastic carcinoma: Pleomorphic carcinoma, undifferentiated carcinoma, sarcomatoid carcinoma
• Small cell carcinoma: Poorly differentiated carcinoma
• Giant cell carcinoma: Pancreatic osteoclastoma
• Acinar cell carcinoma

Definitions

• Rare pancreatic malignancies that may mimic adenocarcinomas and neuroendocrine tumors

IMAGING

General Features

• Best diagnostic clue
• Anaplastic carcinoma

image Large, heterogeneous, moderately enhancing, exophytic mass with necrosis and cystic change
image Locally invasive with frequent lung and liver metastases
• Small cell carcinoma

image Large, homogeneous, mildly enhancing mass with confluent local and distant lymphadenopathy
image May be indistinguishable from lymphoma
image Pancreatic head; homogeneous, hypovascular
• Giant cell carcinoma (pleomorphic or osteoclast)

image Large, heterogeneous, cystic, low-density mass with frequent hemorrhage, septation, and calcification

– Tendency to arise in pancreatic body and tail
– Often grow to very large sizes (mean > 6 cm)
image Local invasion and distant mets (liver, lung) uncommon
• Acinar cell carcinoma

image Large, well-circumscribed mass with cystic and necrotic degeneration and frequent exophytic component

– Usually a well-defined enhancing capsule
– Hypodense on both arterial and venous phases
– Usually no biliary/pancreatic duct dilatation (even with large tumors)
– Vascular encasement or occlusion uncommon (20%), and may invade portal vein or SMV
image Commonly misdiagnosed as neuroendocrine tumor
image No predisposition for any specific location in pancreas
image Metastasizes to liver and local lymph nodes
• Pancreatoblastoma

image Can resemble either pancreatic adenocarcinoma or neuroendocrine tumors
image Associated with Beckwith-Wiedemann and familial adenomatous polyposis
image Large, heterogeneous mass with frequent internal calcifications and necrosis/hemorrhage

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