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 Journal Article


Authors: Tang, L. H.; Aydin, H.; Brennan, M. F.; Klimstra, D. S.
Article Title: 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
Abstract: Solid pseudopapillary tumors (SPTs) are unusual neoplasms of the pancreas of uncertain histogenesis that occur mostly, but not exclusively, in young women. The pathologic features and immunophenotype of SPT are unique and well characterized. Despite its low malignant potential, proximately 15% of patients with SPT develop metastatic disease, mostly involving the liver or peritoneum. Even in the presence of disseminated disease, the clinical course is usually protracted, and the overall 5-year survival is reportedly 97%. We have encountered 2 cases of SPT possessing unusual pathologic features and exhibiting an aggressive clinical course. At the time of presentation, 1 patient had liver metastasis, and the other had a lymph node metastasis and developed liver metastases within 3 months. Both died of disease at 6 and 16 months, respectively, following the initial diagnosis. Review of other cases of SPT treated at Memorial Sloan-Kettering Cancer Center (New York, NY) revealed that 5 of 34 cases (15%) with conventional histologic features developed liver metastases. In contrast to the 2 cases reported here, all 5 patients survived for a mean of 106 months (39-193 months), and only 2 died of disease 5 and 10 years, respectively, following the initial resection. The pathologic features of the two rapidly fatal cases, which might have been indicative of their aggressive behavior, included a diffuse growth pattern, extensive tumor necrosis, significant nuclear atypia, an unusually high mitotic rate (35-70/50 high power fields), and in one a component of sarcomatoid carcinoma. However, regions displaying the typical histologic features of SPT were also evident. Abnormal β-catenin distribution and markedly increased M1B1 expression were detected by immunohistochemistry in both cases. The immunohistochemical staining patterns were otherwise similar to those of conventional SPTs. Although precise pathologic criteria suggesting a high risk for aggressive behavior are uncertain, recognition of some of the unusual pathologic features displayed in these 2 cases may be useful in the prediction of potentially more aggressive SPTs. The possibility that these tumors represent high-grade malignant transformation of a conventional low-grade SPT is proposed. Copyright © 2005 by Lippincott Williams & Wilkins.
Keywords: immunohistochemistry; adolescent; adult; cancer survival; clinical article; human tissue; school child; aged; middle aged; human cell; disease course; histopathology; review; solid tumor; liver neoplasms; comparative study; pancreatic neoplasms; lymph node metastasis; undifferentiated carcinoma; pancreas; ki 67 antigen; ki-67 antigen; carcinoma, papillary; tumor markers, biological; cancer mortality; liver metastasis; pancreas tumor; pancreatectomy; immunophenotyping; mitosis rate; sex difference; trans-activators; malignant transformation; fatal outcome; peritoneum metastasis; beta catenin; cytoskeletal proteins; histogenesis; fatality; aggression; tumor necrosis; pancreatic neoplasm; pancreas solid pseudopapillary tumor; solid and pseudopapillary tumor
Journal Title: American Journal of Surgical Pathology
Volume: 29
Issue: 4
ISSN: 0147-5185
Publisher: Lippincott Williams & Wilkins  
Date Published: 2005-04-01
Start Page: 512
End Page: 519
Language: English
DOI: 10.1097/01.pas.0000155159.28530.88
PUBMED: 15767807
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 102" - "Export Date: 24 October 2012" - "CODEN: AJSPD" - "Source: Scopus"
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MSK Authors
  1. Hakan Aydin
    2 Aydin
  2. Murray F Brennan
    750 Brennan
  3. David S Klimstra
    835 Klimstra
  4. Laura Hong Tang
    313 Tang