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Acinar cell carcinoma
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Acinar cell carcinoma

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Contributors: Abhijeet Waghray MD, Khaled Bittar MD, Nishant H. Patel MD, Desiree Rivera-Nieves MD, Paritosh Prasad MD
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Synopsis

Acinar cell carcinoma is a rare malignant exocrine tumor of the pancreas resembling cells of the pancreatic acini. It comprises less than 1% of all pancreatic neoplasms and is usually a solid tumor. Its gross appearance is a pink to tan homogeneous growth. Tumors may grow to over 10 cm in diameter. Although seen most frequently in males of Northern European ancestry who are middle-aged or older, acinar cell carcinoma can occur at any age.

Findings include abdominal pain, a pancreatic mass, nausea, fatigue, weakness, elevated lipase, and weight loss. Some patients present with Schmid's triad (subcutaneous fat necrosis, polyarthritis, and eosinophilia) caused by elevated lipase secreted by the tumor.

Acinar cell carcinomas can easily be differentiated from adenocarcinoma and pancreatic neuroendocrine tumors based on immunohistochemical stains. Overall prognosis depends on tumor size, lymph node involvement, and presence of metastasis at diagnosis.    

Treatment includes surgical resection with potential chemotherapy or radiation.

Related topic: Pancreatic carcinoma

Codes

ICD10CM:
C80.1 – Malignant (primary) neoplasm, unspecified

SNOMEDCT:
45410002 – Acinar cell carcinoma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Pancreatic endocrine tumor
  • Solid pseudopapillary tumor
  • Ductal adenocarcinoma
  • Medullary carcinoma
  • Acinar cell cystadenoma
  • Serous microcystic adenoma
  • Serous oligocystic adenoma
  • Pancreatoblastoma (see pancreatic carcinoma)

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 03/02/2018
Last Updated: 03/02/2018
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Acinar cell carcinoma
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Acinar cell carcinoma : Nausea, Vomiting, Diffuse abdominal pain, Glucagon elevated, Pancreatic mass, Weight loss
Imaging Studies image of Acinar cell carcinoma
Axial post-contrast MRI demonstrates two arterially enhancing lesions in the left hepatic lobe, which demonstrated washout in the portal venous phase (not shown). Findings were consistent with hepatocellular carcinoma.
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