Английская Википедия:Acinar cell carcinoma of the pancreas

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Шаблон:Infobox medical condition (new) 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.

Signs and symptoms

Файл:Azinuszellkarzinom des Pankreasschwanz - CT axial 001.jpg
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 a release of lipase.[2]

Pathology

ACC is associated with increased serum lipase and manifests 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

Файл:Histopathology of acinar cell carcinoma of the pancreas.jpg
Light microscopy of acinar cell carcinoma.
Файл:Relative incidence of pancreatic neoplasms.png
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.Шаблон:Citation needed

See also

References

Шаблон:Reflist

  1. Tobias Jeffrey S., Hochhauser, Daniel, Cancer and its Management, p. 276, 2010 (6th edn), Шаблон:ISBN, 9781118713259
  2. 2,0 2,1 2,2 Von Hoff": Daniel D. Von Hoff, Douglas Brian Evans, Ralph H. Hruban, eds. Pancreatic Cancer, 2005, Jones & Bartlett Learning, Шаблон:ISBN, 9780763721787
  3. Шаблон:Cite journal
  4. Шаблон:Cite journal
  5. Шаблон:Cite journal
    Diagram by Mikael Häggström, M.D.
  6. 6,0 6,1 6,2 Шаблон:Cite journal