Acinar cell carcinoma of the pancreas

Acinar cell carcinoma of the pancreas

Micrograph of an acinar cell carcinoma of the pancreas. H&E stain.
Classification and external resources

Acinar cell carcinoma of the pancreas, also acinar cell carcinoma, is a rare malignant exocrine tumour of the pancreas. It represents 5% of all exocrine tumours of the pancreas, making it the second most common type of pancreatic cancer.[1] It is abbreviated ACC. It typically has a guarded prognosis.

Symptoms

CT scan in a patient with acinar cell carcinoma

The disease is more common in men than women and the average age at diagnosis is about 60.[2] Symptoms are often non-specific and include weight loss. A classic presentation, found in around 15% of cases includes subcutaneous nodules (due to fat necrosis) and arthralgias, caused by release of lipase.[3]

Pathology

ACC are associated with increased serum lipase and manifest in the classic presentation as the Schmid triad (subcutaneous fat necrosis, polyarthritis, eosinophilia).[4]

ACC are typically large, up to 10cm, and soft compared to pancreatic adenocarcinoma, lacking its dense stroma. They can arise in any part of the pancreas.[5]

Histomorphologically, the tumour resembles the cells of the pancreatic acini and, typically, have moderate granular cytoplasm that stain with both PAS and PASD.[6]

Treatment

ACC can be treated with a Whipple procedure.

See also

References

  1. Tobias Jeffrey S., Hochhauser, Daniel, Cancer and its Management, p. 276, 2010 (6th edn), ISBN 1118713257, 9781118713259
  2. Von Hoff, 23
  3. Von Hoff, 23
  4. Jang, SH.; Choi, SY.; Min, JH.; Kim, TW.; Lee, JA.; Byun, SJ.; Lee, JW. (Feb 2010). "[A case of acinar cell carcinoma of pancreas, manifested by subcutaneous nodule as initial clinical symptom].". Korean J Gastroenterol 55 (2): 139–43. doi:10.4166/kjg.2010.55.2.139. PMID 20168061.
  5. Von Hoff, 23
  6. Klimstra, DS.; Heffess, CS.; Oertel, JE.; Rosai, J. (Sep 1992). "Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.". Am J Surg Pathol 16 (9): 815–37. doi:10.1097/00000478-199209000-00001. PMID 1384374.

External links

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