Acinar cell carcinoma of the pancreas

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.

Acinar cell carcinoma of the pancreas
Other namesAcinar cell carcinoma
Micrograph of an acinar cell carcinoma of the pancreas. H&E stain.
SpecialtyOncology

Signs and 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.[2]

Pathology

ACC are associated with increased serum lipase and manifest in the classic presentation known as the Schmid triad (subcutaneous fat necrosis, polyarthritis, eosinophilia).[3]

ACC are typically large, up to 10 cm, and soft compared to pancreatic adenocarcinoma, lacking its dense stroma. They can arise in any part of the pancreas.[2]

Histomorphologically, the tumour resembles the cells of the pancreatic acini and, typically, have moderate granular cytoplasm that stain with both PAS and PASD.[4]

Diagnosis
Light microscopy of acinar cell carcinoma.
Relative incidences of various pancreatic neoplasms, with acinar cell carcinoma annotated near top right.[5]

Light microscopy of an acinar cell carcinoma biopsy typically shows granular appearance.[6] Immunohistochemistry is usually positive for trypsin, chymotrypsin and lipase.[6] On genetic testing, altered genes/proteins are typically found for p53, SMAD4, APC, ARID1A and GNAS.[6]

Treatment

ACC can be treated with a Whipple procedure or (depending on the location within the pancreas) with left partial resection of pancreas.

See also

References
  1. Tobias Jeffrey S., Hochhauser, Daniel, Cancer and its Management, p. 276, 2010 (6th edn), ISBN 1118713257, 9781118713259
  2. Von Hoff": Daniel D. Von Hoff, Douglas Brian Evans, Ralph H. Hruban, eds. Pancreatic Cancer, 2005, Jones & Bartlett Learning, ISBN 0763721786, 9780763721787
  3. 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.
  4. 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. S2CID 19317244.
  5. Wang Y, Miller FH, Chen ZE, Merrick L, Mortele KJ, Hoff FL; et al. (2011). "Diffusion-weighted MR imaging of solid and cystic lesions of the pancreas". Radiographics. 31 (3): E47-64. doi:10.1148/rg.313105174. PMID 21721197.{{cite journal}}: CS1 maint: multiple names: authors list (link)
    Diagram by Mikael Häggström, M.D.
  6. Pishvaian MJ, Brody JR (2017). "Therapeutic Implications of Molecular Subtyping for Pancreatic Cancer". Oncology (Williston Park). 31 (3): 159–66, 168. PMID 28299752.
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