Clinical Characteristics and Outcomes From an Institutional Series of Acinar Cell Carcinoma of the Pancreas and Related Tumors

Abstract

The survival curves suggest a more aggressive cancer than pancreatic endocrine neoplasms but one that is less aggressive than ductal adenocarcinoma of the pancreas. Those patients who present with localized disease have a much better prognosis than those who present with metastases. There is a high recurrence rate after complete surgical resection, suggesting that micrometastases are present even in localized disease and that adjuvant therapies may be indicated. Chemotherapy and radiation afford disappointing results, however, and novel therapies are needed.

PAX8 expression reliably distinguishes pancreatic well-differentiated neuroendocrine tumors from ileal and pulmonary well-differentiated neuroendocrine tumors and pancreatic acinar cell carcinoma

“…The distinction between acinar cell carcinoma and pancreatic neuroendocrine tumors is important clinically as acinar cell carcinomas typically have a poor prognosis. 42 Histologically, acinar cell carcinomas and well-differentiated neuroendocrine tumors can be virtually indistinguishable, and the differentiation between these two entities often requires immunohistochemical analysis. Chymotrypsin and trypsin are relatively sensitive immunohistochemical markers of acinar differentiation, 43 but a significant subset of pancreatic neuroendocrine tumors can focally express these markers.…”

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

“…However, the knowledge of the efficiency of any neoadjuvant or adjuvant with radiation or chemotherapy for acinar cell carcinomas is limited as less data are available regarding the efficacy of systemic therapy in the management of these carcinomas, with the published experience to date limited to small case series or case reports. One of the largest single-institution study was published from the Memorial Sloan Kettering cancer centre in 2002 [5]; at that time, the reported experience with systemic chemotherapy for ACC was not promising and concluded that the high recurrence rate of acinar cell carcinoma indicated that micrometastasis are likely present at the time of surgery, making adjuvant therapy necessary. Acinar cell carcinomas are moderately chemoresponsive to agents that have activity in pancreatic adenocarcinoma and colorectal carcinoma.…”

“…Approximately 10-15 % of patients develop lipase hypersecretion syndrome, in which massive quantities of lipase are released by the neoplasm into the bloodstream with levels reaching over 10,000 U/dl, with clinical symptoms including multiple nodular foci of subcutaneous fat necrosis and polyarthralgia due to sclerotic lesions in cancellous bone because of fat necrosis in the bone [5,6]. Peripheral blood eosinophilia and parathyropathies also occur.…”

“…Common non-specific symptoms include weight loss (52 %), abdominal pain (32 %), nausea and vomiting (20 %), melena (12 %), and weakness, anorexia or diarrhoea (8 %) [5]. In contrast to ductal adenocarcinoma, acinar cell carcinoma rarely results in bile duct obstruction and jaundice, which occurs only in 12 % of patients [3].…”

“…Acinar cell carcinomas are highly aggressive neoplasms, with a median survival for patients with localised disease and metastatic disease of 38 and 14 months, respectively, and an overall 5-year survival rate of less than 10 %; but, this neoplasm is not quite as rapidly lethal as conventional ductal adenocarcinoma [3,5,11]. Approximately 50 % of patients have metastatic disease at the time of diagnosis, and more than half of the remaining patients develop metastatic disease following surgical resection of the primary tumour but it is not uncommon for patients with distant metastases to survive for 2-3 years.…”