Acinar cell carcinoma with PRKAR1A and PTEN alterations and paraneoplastic panniculitis

Zhizhou Yang, Jorge G. Zarate Rodriguez, Haley Beck, Kathleen Byrnes, Nikolaos A. Trikalinos, Chet W. Hammill

Research output: Contribution to journalArticlepeer-review


Pancreatic acinar cell carcinoma is a rare type of pancreatic malignancy, which can be confused with pancreatic neuroendocrine neoplasm. Here, we describe a woman in her 80s who presented with abdominal pain and bilateral lower extremity panniculitis. She underwent surgery for a presumed diagnosis of neuroendocrine tumour with PTEN and PRKAR1A alterations; 19 months, later, a recurrence of her pancreatic malignancy was discovered. The patient underwent repeat resection and this time immunohistochemical staining confirmed the diagnosis of acinar cell carcinoma. Staining for acinar cell carcinoma should be prompted based on clinical suspicion in context of PTEN or PRKAR1A mutation when appropriate.

Original languageEnglish
Article numbere251400
JournalBMJ Case Reports
Issue number12
StatePublished - Dec 22 2022


  • pancreatic cancer
  • surgical oncology


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