Review
Current diagnosis and management of unusual pancreatic tumors

https://doi.org/10.1016/j.amjsurg.2008.02.005Get rights and content

Abstract

Background

The finding of a solid or cystic mass in the pancreas is becoming more common secondary to the increasing use of cross-sectional imaging and the improved sensitivity of such studies for the detection of pancreatic abnormalities. Because of the aggressive natural history of pancreatic cancer, this has caused concern that all pancreatic abnormalities may be cancer as well as confusion over proper diagnostic and treatment algorithms. This review provides an overview of the natural history, diagnostic considerations, and treatment recommendations for the less common tumors of the pancreas which can be misinterpreted as pancreatic cancer including: solid pseudopapillary tumors (SPT), acinar cell carcinoma (ACC), lymphoplasmacytic sclerosing pancreatitis (LPSP), primary pancreatic lymphoma (PPL), and metastatic renal cell carcinoma to the pancreas.

Data sources

A Medline search was conducted to identify studies investigating the clinicopathologic features, molecular genetics, pathogenesis, diagnosis, and treatment of SPT, ACC, LPSP, PPL, and pancreatic metastases.

Conclusions

It is often possible to obtain an accurate pretreatment diagnosis for these unusual pancreatic tumors and to successfully differentiate them from the more common pancreatic malignancies.

Section snippets

Natural history

Initially reported by Franz in 1959,1 solid pseudopapillary tumor (SPT) has historically been described by multiple names, most of which have been descriptive of the cystic and papillary nature of these tumors.2, 3 In 1996, the World Health Organization classified these tumors as SPT and further defined malignant SPT as those tumors with angioinvasion, perineural invasion, or deep invasion of the surrounding pancreatic parenchyma.4 The number of patients diagnosed with SPTs of the pancreas has

Natural history

Although acinar cells make up the majority of the pancreas, acinar cell carcinoma (ACC) accounts for only 1% to 2% of all pancreatic tumors.24 ACC is characterized by the systemic release of pancreatic enzymes including trypsin, chymotrypsin, amylase, and lipase,25 although the serum levels are not always elevated. ACC is twice as common in men than women, and the disease usually occurs in the fifth to seventh decade of life, although there have been several case reports of ACC in children.25

Natural history

Autoimmunity as a possible pathological mechanism in pancreatitis was first described in 1961 by Sarles et al.38 Since that time, a distinct form of autoimmune pancreatitis has been described by a number of names, including lymphoplasmacytic sclerosing pancreatitis (LPSP), autoimmune pancreatitis, and sclerosing pancreatitis.39 This disease is characterized by diffuse inflammatory infiltrates involving both the pancreatic ducts and the pancreatic parenchyma; however, many patients present with

Natural history

Primary pancreatic lymphoma (PPL) is exceedingly rare, comprising less than 0.5% of pancreatic tumors.65 The majority of PPLs are diffuse large-cell lymphomas of intermediate to high grade. The disease occurs more often in men than women and usually presents in the 5th to 6th decade of life.66 PPL should be differentiated from secondary involvement of the pancreas by lymphoma, which may occur in up to one third of patients with non-Hodgkin's lymphoma.

Unfortunately, the clinical presentation

Natural history

Isolated metastatic cancer to the pancreas accounts for approximately 2% to 3% of all pancreatic neoplasms.69 The most common primary cancer to metastasize to the pancreas is renal cell carcinoma (RCC). RCC metastatic to the pancreas is unique in that there is generally a long disease-free interval (DFI) (often 10 years or more) from nephrectomy to the recognition of metastatic disease.73 Although very rare, solid tumor metastases to the pancreas carry a poor prognosis with the exception of

Conclusions

The difficulty in differentiating PDA from other unusual pancreatic tumors can create a challenging diagnostic dilemma in some patients. An accurate pretreatment diagnosis is crucial to the development of an optimal treatment strategy because this determines appropriate therapy and prognosis. This is best achieved by a multidisciplinary team of pancreatic tumor specialists who successfully integrate the information from all aspects of the diagnostic evaluation. Accurate analysis of

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