Acinar Cell Carcinoma of the Pancreas

Abstract

Confirming the diagnosis of ACC preoperatively is difficult, but this diagnosis should be kept in mind while planning surgery for ordinary pancreatic cancer. Once the diagnosis has been confirmed, a possibility of surgical resection should be pursued to achieve better prognosis. If ACC is unresectable or recurrent, chemotherapy is likely to prove useful. Multidisciplinary therapy centering on the role of surgery will need to be established.

Acinar cell carcinomas of the pancreas: a molecular analysis in a series of 57 cases

“…PACs have frequently metastasized at the time of diagnosis or develop metastases during follow-up [1][2][3][4]6]. When compared with PDACs, higher resectability rates can be achieved for PACs [1][2][3].…”

“…Radiotherapy and chemotherapy are only effective in a limited number of patients [6]. Five-year survival rates range between 25 and 50 %, depending on stage [7], and survival times between 3 and 123 months have been reported [1][2][3].…”

“…Pancreatic acinar cell carcinomas (PACs) are rare pancreatic exocrine tumors which most commonly occur in adults in the sixth and seventh decades of life but show a wide age range and may affect children as well [1][2][3][4][5]. At the time of diagnosis, approximately 50 % of PACs have metastasized, most frequently involving regional lymph nodes and/ or the liver [4,6].…”

“…The prognosis of these neoplasms, even if they are resectable, is therefore poor. Local, and especially metastatic, relapse occurs in up to 42 % of patients [1][2][3][4]6]. Radiotherapy and chemotherapy are only effective in a limited number of patients [6].…”

Oral Capecitabine in Gemcitabine-Pretreated Patients with Advanced Pancreatic Cancer

“…These patients do not only have different histological features compared with patients with (ductal) adenocarcinoma, they also seem to have a different prognosis [28, 29]. Both patients with metastatic acinar cell carcinoma in this study experienced prolonged disease control during treatment with Cap (TTP of 9.5 and >45.1 months, respectively), and they showed a favorable survival prognosis with an OS (from initial diagnosis) of 60.4 and >48.1 months, respectively.…”