ReviewCurrent diagnosis and management of unusual pancreatic tumors
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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Cited by (64)
Diagnostic Pathology: Cytopathology
2018, Diagnostic Pathology: CytopathologyObstructive jaundice as primary presentation of a stage IIE Non-Hodgkin lymphoma: A decision making process between advanced lymphoma and locally advanced/metastatic pancreatic adenocarcinoma
2018, International Journal of Surgery Case ReportsCitation Excerpt :Secondary pancreatic tumors are uncommon and account for 2–5% of pancreatic cancer. Tumors that are characterized most commonly with pancreatic involvement are lymphoma, renal cell, lung, breast and colorectal carcinoma followed by melanoma and leiomyosarcoma [4]. Secondary pancreatic lymphoma can be seen up to 30% of patients with advanced lymphoma and it is far more common than primary pancreatic lymphoma, which accounts of less than 0.5% of pancreatic malignancies, and other secondary pancreatic malignancies [5].
Multimodal approach and long-term survival in a patient with recurrent metastatic acinar cell carcinoma of the pancreas: A case report
2016, PancreatologyCitation Excerpt :Her performance status is now 0, and her previously mentioned elevated laboratory values are all within normal limits. Pancreatic acinar cell carcinoma is a rare and aggressive malignancy with a reported median overall survival of 19 months for all patients and 14 months for those presenting with metastatic disease [10,15]. 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].
Comparison of F-18-FDG PET/CT findings between pancreatic solid pseudopapillary tumor and pancreatic ductal adenocarcinoma
2014, European Journal of RadiologyPerioperative safety and prognosis following parenchyma-preserving surgery for solid pseudopapillary neoplasm of the pancreas
2023, World Journal of Surgical OncologyRare Solid Pancreatic Lesions on Cross-Sectional Imaging
2023, Diagnostics
Supported by the Lockton Fund for Pancreatic Cancer Research and the Various Donors Fund for Pancreatic Cancer Research at The University of Texas M. D. Anderson Cancer Center.