Difference between revisions of "Acinar cell carcinoma of the pancreas"

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#redirect [[Pancreas#Acinar_cell_carcinoma_of_the_pancreas]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Acinar_cell_carcinoma_of_the_pancreas_-_very_high_mag.jpg
| Width      =
| Caption    = Acinar cell carcinoma. [[H&E stain]].
| Synonyms  =
| Micro      = cells reminiscent of pancreatic acinus cells (granular, basophilic cytoplasm - usu. abundant; round/oval nucleus with prominent nucleolus); variable architecture (nests, sheets, trabecular, glandular)
| Subtypes  =
| LMDDx      = [[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)
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[pancreas]]
| Assdx      = subcutaneous [[fat necrosis]], poly[[arthritis]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  = +/-joint pain
| Prevalence = very rare
| Bloodwork  = +/-eosinophilia
| Rads      = mass lesion - typically head of pancreas
| Endoscopy  =
| Prognosis  = poor - median survival ~18 mo.
| Other      =
| ClinDDx    = other pancreatic tumours
| Tx        =
}}
'''Acinar cell carcinoma of the pancreas''' is a rare malignant epithelial tumour of the [[pancreas]].
 
It is also known as '''pancreatic acinar cell carcinoma'''<ref name=pmid>{{Cite journal  | last1 = Thomas | first1 = PC. | last2 = Nash | first2 = GF. | last3 = Aldridge | first3 = MC. | title = Pancreatic acinar cell carcinoma presenting as acute pancreatitis. | journal = HPB (Oxford) | volume = 5 | issue = 2 | pages = 111-3 | month =  | year = 2003 | doi = 10.1080/13651820310001153 | PMID = 18332967 }}</ref> 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.<ref>URL: [http://brighamrad.harvard.edu/Cases/bwh/hcache/380/full.html http://brighamrad.harvard.edu/Cases/bwh/hcache/380/full.html]. Accessed on: 15 January 2012.</ref>
*Poor prognosis; mean survival of 18 months in one series.<ref name=pmid1384374/>
 
Clinical:<ref name=pmid1384374>{{Cite journal  | last1 = Klimstra | first1 = DS. | last2 = Heffess | first2 = CS. | last3 = Oertel | first3 = JE. | last4 = Rosai | first4 = J. | title = Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases. | journal = Am J Surg Pathol | volume = 16 | issue = 9 | pages = 815-37 | month = Sep | year = 1992 | doi =  | PMID = 1384374 }}</ref>
*Increased serum lipase.
**Associated with arthralgias (joint pain).
*Classic presentation - Schmid triad:<ref name=pmid20168061>{{Cite journal  | last1 = Jang | first1 = SH. | last2 = Choi | first2 = SY. | last3 = Min | first3 = JH. | last4 = Kim | first4 = TW. | last5 = Lee | first5 = JA. | last6 = Byun | first6 = SJ. | last7 = Lee | first7 = JW. | title = [A case of acinar cell carcinoma of pancreas, manifested by subcutaneous nodule as initial clinical symptom]. | journal = Korean J Gastroenterol | volume = 55 | issue = 2 | pages = 139-43 | month = Feb | year = 2010 | doi =  | PMID = 20168061 }}</ref>
*#Subcutaneous [[fat necrosis]].
*#Polyarthritis.
*#Eosinophilia.
 
==Gross==
*Usually head of pancreas.
 
==Microscopic==
Features:<ref name=pmid1384374/>
*Cells reminiscent of pancreatic acinus cells:
**Granular, basophilic cytoplasm - usu. abundant.
**Round/oval nucleus.
***Nucleolus prominent.
*Architecture:
**Nests, sheets, trabecular, glandular.
 
DDx:
*[[Pancreatic neuroendocrine tumour]].
*[[Invasive ductal carcinoma of the pancreas]].
 
===Images===
<gallery>
Image:Acinar_cell_carcinoma_of_the_pancreas_-_high_mag.jpg | Pancreatic acinar cell carcinoma - high mag. (WC/Nephron)
Image:Acinar_cell_carcinoma_of_the_pancreas_-_very_high_mag.jpg | Pancreatic acinar cell carcinoma - very high mag. (WC/Nephron)
</gallery>
www:
*[http://path.upmc.edu/cases/case377.html Acinar cell carcinoma - several images (upmc.edu)].
*[http://brighamrad.harvard.edu/Cases/bwh/hcache/380/full.html Acinar cell carcinoma - several images (harvard.edu)].
*[http://www.nature.com/modpathol/journal/v20/n1s/fig_tab/3800686f12.html#figure-title Acinar cell carcinoma - 1 (nature.com)].
*[http://www.nature.com/modpathol/journal/v20/n1s/fig_tab/3800686f13.html#figure-title Acinar cell carcinoma - 2 (nature.com)].<ref name=pmid17486055>{{Cite journal  | last1 = Klimstra | first1 = DS. | title = Nonductal neoplasms of the pancreas. | journal = Mod Pathol | volume = 20 Suppl 1 | issue =  | pages = S94-112 | month = Feb | year = 2007 | doi = 10.1038/modpathol.3800686 | PMID = 17486055 }}</ref>
==Stains==
Features:<ref name=pmid1384374/>
*PAS +ve (granular).
*PASD +ve.
 
==IHC==
*Trypsin +ve -- '''key stain'''.
*Lipase +ve.
*Chromogranin +ve (scattered, focal).
*CD56 -ve. (?)
 
==See also==
*[[Pancreas]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Pancreas]]

Latest revision as of 05:15, 21 October 2014

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

  • Pancreatic acinar cell carcinoma - high mag. (WC/Nephron)

  • Pancreatic acinar cell carcinoma - very high mag. (WC/Nephron)

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.
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