Acinar Cell Carcinoma of the Pancreas

Klimstra, David S.; Heffess, Clara S.; Oertel, James E.; Rosaí, Juan

doi:10.1097/00000478-199209000-00001

Cited by 515 publications

(96 citation statements)

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“…Though acinar cells comprise most of the normal pancreas, acinar neoplasms are very rare, accounting for 1%-2% of pancreatic tumors in adults and 15% in children. Mean age in adults is 58 years, with a 3.6:1 male predominance [1,2] . Patients generally present with nonspecific abdominal symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…ACC accounts for less than 1% of all pancreatic neoplasms [1,2] . The tumor is typically seen in men over age 50 (mean age of 58 years) with no distinctive clinical presentation [3][4][5] .…”

Section: Introductionmentioning

confidence: 99%

“…The tumor is typically seen in men over age 50 (mean age of 58 years) with no distinctive clinical presentation [3][4][5] . Most patients may present with nonspecific symptoms such as skin rashes, weight loss, nausea, vomiting, jaundice, polyarthralgias, fevers, and fat necrosis [2] . Symptoms relating to the overproduction and release of lipase into the circulation are present in 10%-15% of patients [2,3] .…”

Section: Introductionmentioning

confidence: 99%

“…Most patients may present with nonspecific symptoms such as skin rashes, weight loss, nausea, vomiting, jaundice, polyarthralgias, fevers, and fat necrosis [2] . Symptoms relating to the overproduction and release of lipase into the circulation are present in 10%-15% of patients [2,3] . Approximately 50% of patients present with metastasis, typically to the liver and regional lymph nodes, when original diagnosis is made [2] .…”

Section: Introductionmentioning

confidence: 99%

“…Symptoms relating to the overproduction and release of lipase into the circulation are present in 10%-15% of patients [2,3] . Approximately 50% of patients present with metastasis, typically to the liver and regional lymph nodes, when original diagnosis is made [2] . The prognosis of ACC is intermediate between well-differentiated neuroendocrine tumors (PanNETs) and pancreatic ductal adenocarcinoma (PDA) with an average survival of 56.9 months in patients with localized, resectable disease and 19 months in patients with advanced disease [6] .…”

Section: Introductionmentioning

confidence: 99%

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SALL4 is expressed in pancreatic acinar cell carcinoma

Ding

Cho

Chan

et al. 2014

CRCP

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Pancreatic acinar cell carcinoma (ACC) is a rare, malignant epithelial neoplasm. SALL4 is a zinc-finger transcription factor and a member of the SALL gene family that has been previously expressed in numerous malignancies including breast, lung, colorectal, and hepatocellular carcinomas but not in pancreatic adenocarcinoma. The expression of SALL4 is related to early clinical stage in breast and lung carcinomas but with early metastasis and an unfavorable prognosis in liver and colorectal carcinomas. In this study, we report two ACC cases with SALL4 expression. Interestingly, one of these two cases also had elevated alpha-fetoprotein (AFP) while both cases showed neuroendocrine features. Clinically, these two patients responded very well to chemotherapy, and the second patient is alive without recurrence and metastasis for more than four years. To our knowledge, these are the first reported cases of SALL4 expression in pancreatic acinar cell carcinoma, indicating a role of SALL4 in this tumor.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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SALL4 is expressed in pancreatic acinar cell carcinoma

Ding

Cho

Chan

et al. 2014

CRCP

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A Man With Widespread Arthritis and Ill-Defined Cutaneous Lesions

2020

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A man in his 30s presented with a 3-month history of fourth finger right hand articular heat, pain, redness, and swelling. Similar symptoms subsequently spread to the left hallux and ankle, and he developed a fever. Despite many medical consultations and various attempts at treatment with nonsteroidal anti-inflammatories, the patient developed widespread arthritis within the following month, which was predominant in the knee, shoulder, and hip. In the month before presentation the articular symptoms worsened, the joints had become tender, and ill-defined skin nodules had appeared predominantly on the legs (Figure , A and B). An acute abdominal pain in the left hypochondrium developed, accompanied by nausea and vomiting, which led to a diagnosis of pancreatitis. The patient was admitted to the hospital and clinical measures relieved the pain. Laboratory test results revealed strikingly high levels of lipase and amylase, as well as normal levels of cancer antigen 19-9. Bone computed tomography (CT) of the right shoulder and knee demonstrated cortical bone destruction and intraosseous medullary necrosis (Figure , C and D). An abdominal CT was performed (Figure , E), and the patient underwent surgery. Diagnosis B. Cancer-associated PPP syndrome Discussion Clinical Review & Education

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