Acinar cell carcinoma of the pancreas: New genetic and treatment insights into a rare malignancy Journal Article
| Authors: | Lowery, M. A.; Klimstra, D. S.; Shia, J.; Yu, K. H.; Allen, P. J.; Brennan, M. F.; O'Reilly, E. M. |
| Article Title: | Acinar cell carcinoma of the pancreas: New genetic and treatment insights into a rare malignancy |
| Abstract: | Background. Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm, accounting for 1% of all pancreatic neoplasms. There remains a lack of data regarding the use of systemic therapy in this disease. We present a series of 40 consecutive cases ofACCof the pancreas treated at Memorial Sloan-Kettering Cancer Center, with an emphasis on evaluation of activity of new therapeutic agents. Methods. Patients reviewed at our institution from January 2000 through January 2011 were identified from an institutional database with prior institutional review board approval. Pathology was confirmed in all cases as ACC or a closely related entity. Results. Forty patients were identified; 29 were male (73%). The median age at diagnosis was 65 years (range, 16 = 87 years). The median overall survival (OS) time for patients with localized, resectable disease was 56.9 months and the OS time for patients with metastatic ACC (n = 18) was 19.6 months. Six patients with metastatic or recurrent ACC had a partial response to chemotherapy and five patients had stable disease for ≥6 months on systemic chemotherapy. Clinical observation was made of a patient with ACC and hereditary nonpolyposis colorectal cancer and a patient with ACC and a BRCA1 germline mutation. Conclusions. ACC is moderately chemoresponsive to agents that have activity in pancreatic adenocarcinoma and colorectal carcinoma. A potential association between germline mutations in DNA mismatch repair genes and ACC warrants further evaluation. ©AlphaMed Press. |
| Keywords: | immunohistochemistry; adolescent; adult; clinical article; human tissue; treatment outcome; treatment response; aged; aged, 80 and over; middle aged; survival analysis; unclassified drug; gene mutation; overall survival; genetics; dna-binding proteins; histone deacetylase inhibitor; clinical feature; cisplatin; erlotinib; fluorouracil; antineoplastic agents; capecitabine; gemcitabine; paclitaxel; pancreatic neoplasms; recurrent cancer; pancreas; disease association; metastasis; brca1 protein; medical record review; cetuximab; histology; docetaxel; irinotecan; pancreas carcinoma; oncogene; genes, brca1; folinic acid; neoplasm metastasis; oxaliplatin; radiofrequency ablation; floxuridine; acinar cell carcinoma; carcinoma, acinar cell; brca; protein msh6; hereditary nonpolyposis colorectal cancer; drug intermittent therapy; acinar; pyroxamide |
| Journal Title: | The Oncologist |
| Volume: | 16 |
| Issue: | 12 |
| ISSN: | 1083-7159 |
| Publisher: | Oxford University Press |
| Date Published: | 2011-01-01 |
| Start Page: | 1714 |
| End Page: | 1720 |
| Language: | English |
| DOI: | 10.1634/theoncologist.2011-0231 |
| PROVIDER: | scopus |
| PUBMED: | 22042785 |
| PMCID: | PMC3248770 |
| DOI/URL: | |
| Notes: | --- - "Export Date: 1 February 2012" - "CODEN: OCOLF" - "Source: Scopus" |
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