Atypical and Rare Pancreatic Tumors

 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
– May be either well circumscribed or infiltrative

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Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Atypical and Rare Pancreatic Tumors

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