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Image of the MonthDiagnosis
Arch Surg. 2005;140:1224.
Answer: Solid Pseudopapillary Tumor of the Pancreas
Solid pseudopapillary tumor of the pancreas (SPT) is an extremely rare neoplasm of the pancreas, accounting for only 1% to 2% of pancreatic neoplasms. An SPT is a tumor of the primitive pancreatic epithelial cells, with predominance of exocrine features but having the capacity for dual (exocrine and endocrine) differentiation.1 Solid pseudopapillary tumor of the pancreas usually occurs in young girls, with more than 50% of patients younger than 19 years,2 and are more common among black and Asian populations.3 Many of these tumors are asymptomatic and present as mild abdominal pain and a lump.
The characteristic sonographic findings of SPT are a well-encapsulated, cystic, and solid mass. Sometimes the mass looks solid, or it may have internal septations or calcifications.4 Contrast-enhanced computed tomography will show a large, hypodense lesion with a few low-attenuation areas. Because of such findings, these lesions are often mistaken for pseudocysts.5
Treatment of SPT includes the Whipple procedure for masses found at the head of the pancreas and distal pancreatectomy for distal growth.3 Solid pseudopapillary tumors of the pancreas usually grow slowly; however, metastasis to the liver and peritoneum has been described.6-7 The most distinctive feature on histopathologic examination is pseudopapillae covered by several layers of epithelial cells. Nuclei are ovoid and folded, with indistinct nucleoli and few mitoses. Hyaline globules and a collection of foam cells may be present.8 Solid pseudopapillary tumors of the pancreas show strong cellular immunoreactivity for vimentin and focal, weak keratin reactivity. Neuron-specific enolase, 1-antitrypsin, and 1-antichymotrypsin stains are also positive.9
AUTHOR INFORMATION
Correspondence: Chirag S. Desai, MS, DNB, Department of Surgery, Seth G. S. Medical College and King Edward Memorial Hospital, Acharya Donde Marg, Parel, Mumbai 400 012, India (csdesai{at}gmail.com).
Accepted for Publication: April 27, 2005.
REFERENCES
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1. Pettinato G, Manivel JC, Ravetto C, et al. Papillary cystic tumor of the pancreas: a clinicopathologic study of 20 cases with cytologic, immunohistochemical, ultrastructural, and flow cytometric observations, and a review of literature. Am J Clin Pathol. 1992;98:478-488. [published correction appears in Am J Clin Pathol. 1993;99:764].
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2. Todani T, Shimada K, Watanabe Y, Toki A, Fujii T, Urushihara N. Frantzs tumor: a papillary and cystic tumor of the pancreas in girls. J Pediatr Surg. 1988;23:116-121.
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3. Jeng LB, Chen MF, Tang RP. Solid and papillary neoplasm of the pancreas: emphasis on surgical treatment. Arch Surg. 1993;128:433-436.
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4. Lee DH, Yi BH, Lim JW, Ko YT. Sonographic findings of solid and papillary epithelial neoplasm of the pancreas. J Ultrasound Med. 2001;20:1229-1232.
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5. Patel VG, Fortson JK, Weaver WL, Hammami A. Solid-pseudopapillary tumor of the pancreas masquerading as a pancreatic pseudocyst. Am Surg. 2002;68:631-632.
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6. Lee YR, Kim Y, Koh BH, et al. Solid and papillary epithelial neoplasm of the pancreas with peritoneal metastasis and its recurrence: a case report. Abdom Imaging. 2003;28:96-98.
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7. Horisawa M, Niinomi N, Sato T, et al. Frantzs tumor (solid and cystic tumor of the pancreas) with liver metastasis: successful treatment and long-term follow-up. J Pediatr Surg. 1995;30:724-726.
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8. Lieber MR, Lack EE, Roberts JR Jr, et al. Solid and papillary epithelial neoplasm of the pancreas: an ultrastructural and immunocytochemical study of six cases. Am J Surg Pathol. 1987;11:85-93.
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9. Bardales RH, Centeno B, Mallery JS, et al. Endoscopic ultrasound-guided fine-needle aspiration cytology diagnosis of solid-pseudopapillary tumor of the pancreas: a rare neoplasm of elusive origin but characteristic cytomorphologic features. Am J Clin Pathol. 2004;121:654-662.
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