The surgical management of metastatic pancreatic acinar cell carcinoma and associated pancreatic panniculitis—A case report and literature review

摘要

Introduction Pancreatic panniculitis is a rare manifestation of benign and malignant pancreatic disease. The presentation of pancreatic panniculitis is non-specific and thus diagnosis is often delayed. When associated with malignancy, pancreatic panniculitis confers a poor prognosis. This case demonstrates the successful surgical management of this paraneoplastic phenomenon following resection of the underlying pancreatic acinar cell carcinoma and associated liver metastasis. Presentation of case A 71-year-old female with debilitating subcutaneous lower limb lesions had a delayed diagnosis of pancreatic panniculitis. A formal diagnosis of pancreatic acinar cell carcinoma with liver metastasis was established and the disease was determined to be resectable. Pre-operatively, serum lipase measured 10,825 U/L. The patient proceeded to an open left hemihepatectomy and radical distal pancreatectomy with complete resection of malignant disease. Six days post-operatively the serum lipase levels normalised, and the panniculitis began to settle. The patient proceeded to adjuvant FOLFORINOX chemotherapy. Twenty months post-surgery, the patient remains disease-free and without any evidence of panniculitis. Discussion Due to the rarity of pancreatic acinar cell carcinoma, guidelines based on prospective data do not exist. Most management is based on retrospective analyses. A survival benefit may be achieved with more aggressive surgical management compared to other pancreatic cancer types. Pancreatic acinar cell carcinoma may show a slower rate of disease progression, an increased likelihood of resectability of disease at presentation and is more likely to undergo potentially curative resection. Conclusion Aggressive surgical management of resectable metastatic pancreatic acinar cell carcinoma can treat pancreatic panniculitis and provide sustained disease-free survival from pancreatic cancer.