Aggressive approach to acinar cell carcinoma of the pancreas: a single-institution experience and a literature review

Abstract

ACC represents a rare solid tumor of the pancreas. Prognosis is dismal, although, compared to the more common ductal adenocarcinoma, survival appears to be longer. Patients with metastatic disease might benefit from aggressive multimodality treatments.

Acinar Cell Carcinoma of the Pancreas: A Literature Review and Update

“…The long-term survival for patients with ACC is significantly better when compared to the long-term survival of patients with PA. Matos et al [23] 17 (2009) Acinar cell carcinoma of the pancreas is rare and appears to have a presentation and outcome distinct from the more common pancreatic DCA Lee et al [24] 29 (2010) In Korea, the clinical features of ACC include young age, large size, tail location and non-specific tumour markers. Surgery should be actively performed in the treatment of ACC regardless of size Hartwig et al [25] 17 (2011) ACC of the pancreas is a relatively rare tumour entity for which resection may result in long-term survival even in limited metastatic disease Butturini et al [26] 9 (2011) ACC represents a rare solid tumour of the pancreas. Prognosis is dismal, although, compared to the more common ductal adenocarcinoma, survival appears to be longer.…”

Multimodal approach and long-term survival in a patient with recurrent metastatic acinar cell carcinoma of the pancreas: A case report

“…It is important to note that the literature of acinar cell carcinoma is non-uniform, since ACC has been mixed to pancreatoblastoma and mixed acinar-endocrine tumor [11]. At presentation, approximately half of the patients have metastatic disease [5,15] with the liver as the most frequent site for distant spread [15].…”

“…The diagnosis of ACC is challenging due to various morphologic characteristics [7] and nonspecific clinical symptoms [5,6,8e11]. A distinctive but rare syndrome called Schmid's Triad is characterized by subcutaneous fat necrosis, eosinophilia and polyarthralgia, secondary to lipase hypersecretion [11]. Associated subcutaneous nodules can be misdiagnosed as erythema nodosum [10].…”

Pancreatic acinar cell carcinoma with bilateral ovarian metastases, panniculitis and polyarthritis treated with FOLFIRINOX chemotherapy regimen. A case report and review of the literature

“…Cases with resectable disease should be treated with surgery, which provides the highest survival rates, with a median overall survival achieving 123 months in some reports [5,6,8]. Optimal treatment should involve a multidisciplinary approach [5,6,8e10,30].…”