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Solid Pseudopapillary Tumors of the Pancreas. Clinical Features, Surgical Outcomes, and Long-Term Survival in 45 Consecutive Patients from a Single Center

  • Original Article
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Journal of Gastrointestinal Surgery

Abstract

Background

Solid pseudopapillary tumors of the pancreas (SPT) are rare neoplasms, and the natural history is poorly defined. The aim of this study was to define the natural history and compare patient and tumor factors between patients with malignant and non-malignant disease.

Methods

Data for all patients with SPT who underwent surgical exploration at MSKCC between 1987 and 2009 were collected and analyzed. Patient, tumor, treatment, and survival variables were examined. Malignant tumors were defined as any tumor that was locally unresectable, metastatic, or recurrent. Differences between groups were analyzed by Fisher’s exact, chi-squared, Wilcoxon, and log-rank tests.

Results

Forty-five patients had an SPT during the study period. Median age was 38 years (10–63) and 38 (84%) were women. At the time of diagnosis, 38 were symptomatic, with the most common symptom being abdominal pain (n = 35). The most frequent imaging characteristic was a solid and cystic tumor (n = 29), most commonly located in the tail of the pancreas (n = 23). Resection of the primary tumor (n = 41) (41/2,919 = 1.4% of all resections) included distal pancreatectomy in 26, pancreatoduodenectomy in 11, central pancreatectomy in two, and enucleation in two. Nine patients had malignant disease defined by a locally unresectable tumor in three, liver metastases in three, locally unresectable tumor and liver metastases in one, local recurrence and liver metastases in one, and local recurrence in another. Patients with malignant disease presented with larger tumors (7.8 vs. 4.2 cm) (p < 0.005). After median follow-up of 44 months, 34 patients were without evidence of disease, four patients were alive with disease, three patients died of disease, and four patients died of other causes.

Conclusions

These results demonstrate that SPT occurs in young women, and the majority of patients will experience long-term survival following resection. The only feature associated with malignant disease was tumor size at presentation. The majority of patients are alive at last follow-up, and a low percentage experienced disease recurrence or death from disease.

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References

  1. Papavramidis T., Papavramidis S. Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg 2005; 200: 965–972.

    Article  PubMed  Google Scholar 

  2. Adams A., Siegal G., Jhala N. Solid pseudopapillary tumor of the pancreas. A review of salient clinical and pathologic features. Adv Anat Pathol 2008; 15: 39–45.

    Article  PubMed  Google Scholar 

  3. Allen P, D'Angelica M, Gonen M, et al. A selective approach to the resection of cystic lesions of the pancreas: results from 539 consecutive patients. Ann Surg 2006; 244: 572–582.

    PubMed  Google Scholar 

  4. Lichtenstein L. Papillary cystadenocarcinoma of the pancreas. Am J Cancer 1934; 21: 542–553.

    Google Scholar 

  5. Reddy S, Wolfgang C. Solid pseudopapillary neoplasms of the pancreas. Adv Surg 2009; 43: 269–282.

    Article  PubMed  Google Scholar 

  6. Frantz V. Tumors of the pancreas. Atlas of tumor pathology. Washington, DC: US Armed Forces Institute of Pathology; 1959: 32–33

    Google Scholar 

  7. Chung Y, Kim M, Choi J, et al. Differentiation of benign and malignant solid pseudopapillary neoplasms of the pancreas. J Comput Assist Tomogr 2009; 33: 689–694.

    Article  PubMed  Google Scholar 

  8. Reddy S, Cameron J, Scudiere J, et al. Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series. J Am Coll Surg 2009; 208: 950–957.

    Article  PubMed  Google Scholar 

  9. Yang F, Jin C, Long J, et al. Solid pseudopapillary tumor of the pancreas: a case series of 26 consecutive patients. Am J Surg 2009; 198: 210–215.

    Article  PubMed  Google Scholar 

  10. Wang D, Wang Q, Chai W, Chen K, Deng X. Imaging features of solid pseudopapillary tumor of the pancreas on multi-detector row computed tomography. World J Gastroenterol 2009; 15: 829–835.

    Article  PubMed  Google Scholar 

  11. Machado M, Machado M, Bacchella T, Jukemura J, Almeida J, Cunha J. Solid pseudopapillary neoplasm of the pancreas: distinct patterns of onset, diagnosis, and prognosis for male versus female patients. Surgery 2008; 143: 29–34.

    Article  PubMed  Google Scholar 

  12. Sun C, Lee W, Choi J, Oh J, Choi S. Solid-pseudopapillary tumours of the pancreas: 14 years experience. ANZ J Surg 2005; 75: 684–689.

    Article  Google Scholar 

  13. Martin R, Klimstra D, Brennan MF, Conlon KC. Solid-pseudopapillary tumor of the pancreas: a surgical enigma?. Ann Surg Oncol 2002; 9: 35–40.

    Article  PubMed  Google Scholar 

  14. Tang L, Aydin H, Brennan M, Klimstra D. Clinically aggressive solid pseudopapillary tumors of the pancreas: a report of two cases with components of undifferentiated carcinoma and a comparative clinicopathologic analysis of 34 conventional cases. Am J Surg Pathol 2005; 29: 512–519.

    Article  PubMed  Google Scholar 

  15. Vin Y, Sima C, Getrajdman G, Brown K, Covey A, Brennan M, Allen P. Management and outcomes of postpancreatectomy fistula, leak, and abscess: results of 908 patients resected at a single institution between 2000 and 2005. J Am Coll Surg 2008; 207: 490–498.

    Article  PubMed  Google Scholar 

  16. Salvia R, Bassi C, Festa L, et al. Clinical and biological behavior of pancreatic solid pseudopapillary tumors: report on 31 consecutive patients. J Surg Oncol 2007; 95: 304–310.

    Article  PubMed  Google Scholar 

  17. Lahat G, Ben Haim M, Nachmany I, Sever R, Blachar A, Nakache R, Klausner J. Pancreatic incidentalomas: high rate of potentially malignant tumors. J Am Coll Surg 2009; 209: 313–319.

    Article  PubMed  Google Scholar 

  18. Goh B, Tan Y, Cheow P, Chung A, Chow P, Wong W, Ooi L. Solid pseudopapillary neoplasms of the pancreas: an updated experience. J Surg Oncol 2007; 95: 640-644.

    Article  PubMed  Google Scholar 

  19. Jani N, Dewitt J, Eloubeidi M, et al. Endoscopic ultrasound-guided fine needle aspiration for diagnosis of solid pseudopapillary tumors of the pancreas: a multicenter experience. Endoscopy 2008; 40: 200–203.

    Article  CAS  PubMed  Google Scholar 

  20. Klimstra D. Nonductal neoplasms of the pancreas. Mod Pathol 2007; 20: S94–112.

    Article  PubMed  Google Scholar 

  21. Comper F, Antonello D, Beghelli S, et al. Expression pattern of claudins 5 and 7 distinguishes solid-pseudopapillary from pancreatoblastoma, acinar cell and endocrine tumors of the pancreas. Am J Surg Pathol 2009; 33: 768–774.

    Article  PubMed  Google Scholar 

  22. Lee S, Jang J, Hwang D, Park K, Kim S. Clinical features and outcome of solid pseudopapillary neoplasm: differences between adults and children. Arch Surg 2008; 143: 1218–1221.

    Article  PubMed  Google Scholar 

  23. Choi S, Kim S, Oh J, Park J, Seo J, Lee S. Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases. J Pediatr Surg 2006; 41: 1992–1995.

    Article  PubMed  Google Scholar 

  24. Matos J, Grützmann R, Agaram N, Saeger H, Kumar H, Lillemoe K, Schmidt C. Solid pseudopapillary neoplasms of the pancreas: a multi-institutional study of 21 patients. J Surg Res 2009; 157: e137–e142.

    Article  PubMed  Google Scholar 

  25. Kang C, Kim K, Choi J, Kim H, Lee W, Kim B. Solid pseudopapillary tumor of the pancreas suggesting malignant potential. Pancreas 2006; 32: 276–280.

    Article  PubMed  Google Scholar 

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Correspondence to Peter J. Allen.

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Butte, J.M., Brennan, M.F., Gönen, M. et al. Solid Pseudopapillary Tumors of the Pancreas. Clinical Features, Surgical Outcomes, and Long-Term Survival in 45 Consecutive Patients from a Single Center. J Gastrointest Surg 15, 350–357 (2011). https://doi.org/10.1007/s11605-010-1337-1

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  • DOI: https://doi.org/10.1007/s11605-010-1337-1

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