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Image of the Month—Diagnosis FREE

Arch Surg. 2005;140(12):1224. doi:10.1001/archsurg.140.12.1224.
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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, α1-antitrypsin, and α1-antichymotrypsin stains are also positive.9

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@gmail.com).

Accepted for Publication: April 27, 2005.

Pettinato  GManivel  JCRavetto  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;98478- 488[published correction appears in Am J Clin Pathol. 1993;99:764]
PubMed
Todani  TShimada  KWatanabe  YToki  AFujii  TUrushihara  N Frantz’s tumor: a papillary and cystic tumor of the pancreas in girls. J Pediatr Surg 1988;23116- 121
PubMed Link to Article
Jeng  LBChen  MFTang  RP Solid and papillary neoplasm of the pancreas: emphasis on surgical treatment. Arch Surg 1993;128433- 436
PubMed Link to Article
Lee  DHYi  BHLim  JWKo  YT Sonographic findings of solid and papillary epithelial neoplasm of the pancreas. J Ultrasound Med 2001;201229- 1232
PubMed
Patel  VGFortson  JKWeaver  WLHammami  A Solid-pseudopapillary tumor of the pancreas masquerading as a pancreatic pseudocyst. Am Surg 2002;68631- 632
PubMed
Lee  YRKim  YKoh  BH  et al.  Solid and papillary epithelial neoplasm of the pancreas with peritoneal metastasis and its recurrence: a case report. Abdom Imaging 2003;2896- 98
PubMed Link to Article
Horisawa  MNiinomi  NSato  T  et al.  Frantz’s tumor (solid and cystic tumor of the pancreas) with liver metastasis: successful treatment and long-term follow-up. J Pediatr Surg 1995;30724- 726
PubMed Link to Article
Lieber  MRLack  EERoberts  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;1185- 93
PubMed Link to Article
Bardales  RHCenteno  BMallery  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;121654- 662
PubMed Link to Article

Figures

Tables

References

Pettinato  GManivel  JCRavetto  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;98478- 488[published correction appears in Am J Clin Pathol. 1993;99:764]
PubMed
Todani  TShimada  KWatanabe  YToki  AFujii  TUrushihara  N Frantz’s tumor: a papillary and cystic tumor of the pancreas in girls. J Pediatr Surg 1988;23116- 121
PubMed Link to Article
Jeng  LBChen  MFTang  RP Solid and papillary neoplasm of the pancreas: emphasis on surgical treatment. Arch Surg 1993;128433- 436
PubMed Link to Article
Lee  DHYi  BHLim  JWKo  YT Sonographic findings of solid and papillary epithelial neoplasm of the pancreas. J Ultrasound Med 2001;201229- 1232
PubMed
Patel  VGFortson  JKWeaver  WLHammami  A Solid-pseudopapillary tumor of the pancreas masquerading as a pancreatic pseudocyst. Am Surg 2002;68631- 632
PubMed
Lee  YRKim  YKoh  BH  et al.  Solid and papillary epithelial neoplasm of the pancreas with peritoneal metastasis and its recurrence: a case report. Abdom Imaging 2003;2896- 98
PubMed Link to Article
Horisawa  MNiinomi  NSato  T  et al.  Frantz’s tumor (solid and cystic tumor of the pancreas) with liver metastasis: successful treatment and long-term follow-up. J Pediatr Surg 1995;30724- 726
PubMed Link to Article
Lieber  MRLack  EERoberts  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;1185- 93
PubMed Link to Article
Bardales  RHCenteno  BMallery  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;121654- 662
PubMed Link to Article

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