Acinar cell carcinoma of the pancreas

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Acinar cell carcinoma of the pancreas
Diagnosis in short

Acinar cell carcinoma. H&E stain.

LM cells reminiscent of pancreatic acinus cells (granular, basophilic cytoplasm - usu. abundant; round/oval nucleus with prominent nucleolus); variable architecture (nests, sheets, trabecular, glandular)
LM DDx pancreatic neuroendocrine tumour, invasive ductal carcinoma of the pancreas
Stains PAS +ve, PASD +ve
IHC trypsin +ve (key stain), lipase +ve, chromogranin +ve (scattered, focal)
Site pancreas

Associated Dx subcutaneous fat necrosis, polyarthritis
Symptoms +/-joint pain
Prevalence very rare
Blood work +/-eosinophilia
Radiology mass lesion - typically head of pancreas
Prognosis poor - median survival ~18 mo.
Clin. DDx other pancreatic tumours

Acinar cell carcinoma of the pancreas is a rare malignant epithelial tumour of the pancreas.

It is also known as pancreatic acinar cell carcinoma[1] and acinar cell carcinoma.

It should not to be confused with acinic cell carcinoma, a tumour of the salivary gland.

General

  • Rare.
  • Solid epithelial exocrine tumour.[2]
  • Poor prognosis; mean survival of 18 months in one series.[3]

Clinical:[3]

  • Increased serum lipase.
    • Associated with arthralgias (joint pain).
  • Classic presentation - Schmid triad:[4]
    1. Subcutaneous fat necrosis.
    2. Polyarthritis.
    3. Eosinophilia.

Gross

  • Usually head of pancreas.

Microscopic

Features:[3]

  • Cells reminiscent of pancreatic acinus cells:
    • Granular, basophilic cytoplasm - usu. abundant.
    • Round/oval nucleus.
      • Nucleolus prominent.
  • Architecture:
    • Nests, sheets, trabecular, glandular.

DDx:

Images

www:

Stains

Features:[3]

  • PAS +ve (granular).
  • PASD +ve.

IHC

  • Trypsin +ve -- key stain.
  • Lipase +ve.
  • Chromogranin +ve (scattered, focal).
  • CD56 -ve. (?)

See also

References

  1. Thomas, PC.; Nash, GF.; Aldridge, MC. (2003). "Pancreatic acinar cell carcinoma presenting as acute pancreatitis.". HPB (Oxford) 5 (2): 111-3. doi:10.1080/13651820310001153. PMID 18332967.
  2. URL: http://brighamrad.harvard.edu/Cases/bwh/hcache/380/full.html. Accessed on: 15 January 2012.
  3. 3.0 3.1 3.2 3.3 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. PMID 1384374.
  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. PMID 20168061.
  5. Klimstra, DS. (Feb 2007). "Nonductal neoplasms of the pancreas.". Mod Pathol 20 Suppl 1: S94-112. doi:10.1038/modpathol.3800686. PMID 17486055.