Solid pseudo-papillary tumors of the pancreas: current update
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Abdominal Imaging
Abdom Imaging (2013) DOI: 10.1007/s00261-013-0015-7
Solid pseudo-papillary tumors of the pancreas: current update Dhakshina Moorthy Ganeshan,1 Erik Paulson,1 Eric P. Tamm,1 Melissa Wainwright Taggart,2 Aparna Balachandran,1 Priya Bhosale1 1
Department of Diagnostic Imaging, Body Imaging section, Unit 1473, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030-4009, USA 2 Department of Pathology Administration, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030-4009, USA
Abstract Solid pseudo-papillary tumors are rare pancreatic tumors, which occur in females and are typically indolent neoplasms. However, atypical, aggressive variants can occur with locally advanced disease or metastases. They have characteristic imaging features, which vary according to size. This article provides a current update on the molecular biology, histopathology, clinico-radiological features, and management of these tumors. Key words: Solid pseudo-papillary tumors—CT— MR—Pancreas
Solid pseudo-papillary tumor (SPT) is a rare pancreatic tumor, accounting for only 1%–2% of pancreatic exocrine neoplasms [1–4]. Initially called Frantz tumor after its discoverer, it has since been known by many names including Hamoudi tumor, papillary epithelial neoplasm of the pancreas, solid and papillary tumor, and papillary cystic tumor. It is currently known as SPT by the World Health Organization [5]. SPT is unique among other pancreatic tumors in that it occurs predominantly in women (85%–90%) [6, 7]. Although it has been reported in a wide age group ranging from 8 to 74 years, it typically occurs in young females in their 2nd to 3rd decade [3, 8] (Fig. 1). However, SPT has been reported in men, and compared to females, usually occurs in an older age group and tends to be more aggressive [9]. It is an indolent tumor with low grade malignant potential and is associated with
Correspondence to: Dhakshina Moorthy Ganeshan; email: dganeshan@ mdanderson.org
excellent prognosis following surgery. However, aggressive, atypical SPTs have been reported, which may be associated with metastases and local recurrence. The aim of this article is to review the pathogenesis, molecular biology, histology, imaging features, and management of SPT. We also illustrate the differences between small (£3 cm) and large SPT with radiologic– pathologic correlation.
Pathogenesis The pathogenesis of SPT is still unclear but they are thought to arise from pluripotential embryonic stem cells [10–13]. Its preponderance in young woman raises the possibility of a hormonal influence in its development [14–17]. Yet despite extensive investigations, only progesterone receptors have been consistently demonstrated in these tumors, and only a few reports suggest the presence of beta forms of estrogen receptors [3, 15, 18– 20]. Alternatively, its female proclivity has been speculated to be a result of SPTs possibly originating from genital ridges in close proximity to pancreatic anlage dur
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