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familial fibrocystic pancreatic atrophy

Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma

Synopsis

- diabetes mellitus
- pancreatic exocrine insufficiency
- fibrocystic, lobulocentric pancreatic atrophy
- patchy to diffuse in distribution
- endocrine cell hyperplasia

- glandular epithelial dysplasia

  • low-grade dysplasia (pancreatic intraductal neoplasia grade II of III or PanIN II)
  • high-grade dysplasia (pancreatic intraductal neoplasia grade III of III or PanIN III)
  • multifocal dysplasia in small-to medium-sized duct-like structures within areas of acinar atrophy, microcystic change, and mucinous hyperplasia

- familial pancreatic carcinoma

  • small cell undifferentiated carcinoma
  • giant cell anaplastic carcinoma

See also

- pancreatic tumorigenesis

References

- Meckler KA, Brentnall TA, Haggitt RC, Crispin D, Byrd DR, Kimmey MB, Bronner MP. Familial fibrocystic pancreatic atrophy with endocrine cell hyperplasia and pancreatic carcinoma. Am J Surg Pathol. 2001 Aug;25(8):1047-53. PMID: 11474289