Skip to main content

Advertisement

Log in

Discordant Diagnostic Terminology and Pathologic Grading of Primary Appendiceal Mucinous Neoplasms Reviewed at a High-Volume Center

  • Peritoneal Surface Malignancy
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Diagnostic terminology and grading of primary appendiceal mucinous neoplasms lacks uniformity. We sought to identify discordance in pathologic reporting by reviewing pathology slides for cases referred to our institution.

Methods

Using guidelines from Peritoneal Surface Oncology Group International (PSOGI) and American Joint Committee on Cancer 8th edition (AJCC8), we compared diagnostic terminology/grading of primary appendiceal mucinous neoplasms (n = 115) between pathology reports from referring institutions and review of slides by pathologists at our high-volume institution.

Results

There was discordance in pathologic terminology and grading of primary appendiceal mucinous neoplasms between referring institutions and our institution in 28% and 50% of patients, respectively. In particular, 24% of patients referred with mucinous adenocarcinoma (MACA) had LAMN on our review, and a higher grade MACA was found in 48% of patients referred with low-grade (G1) MACA and 16% of patients referred with high-grade (G2) MACA following our review. Discordance in tumor grade between primary and metastatic disease was seen in 19% of cases based on referred primary tumor grading compared with only 4% following our review. Systemic chemotherapy was unnecessarily administered to four cases of LAMN (6%) and inappropriately not administered to four cases of MACA (6%) before referral due to inaccurate diagnosis/grading by referring institutions.

Conclusions

We found significant discordance in diagnostic terminology/grading of primary appendiceal mucinous neoplasms following review of referred cases. Inaccurate pathologic assessment was associated with overtreatment or undertreatment with chemotherapy. These data highlight the need for pathologic review of such rare cases at high-volume centers.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7970 appendectomies. Dis Colon Rectum. 1998;41(1):75–80.

    Article  CAS  PubMed  Google Scholar 

  2. McCusker ME, Cote TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973–1998. Cancer. 2002;94(12):3307–12.

    Article  PubMed  Google Scholar 

  3. Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol. 2012;19(5):1379–85.

    Article  PubMed  Google Scholar 

  4. Amini A, Masoumi-Moghaddam S, Ehteda A, Morris DL. Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects. Orphanet J Rare Dis. 2014;9:71.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Choudry HA, O’Malley ME, Guo ZS, Zeh HJ, Bartlett DL. Mucin as a therapeutic target in pseudomyxoma peritonei. J Surg Oncol. 2012;106(7):911–7.

    Article  CAS  PubMed  Google Scholar 

  6. O’Connell JT, Tomlinson JS, Roberts AA, McGonigle KF, Barsky SH. Pseudomyxoma peritonei is a disease of MUC2-expressing goblet cells. Am J Pathol. 2002;161(2):551–64.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Sugarbaker PH. New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? Lancet Oncol. 2006;7(1):69–76.

    Article  PubMed  Google Scholar 

  8. Bradley RF, Stewart JHT, Russell GB, Levine EA, Geisinger KR. Pseudomyxoma peritonei of appendiceal origin: a clinicopathologic analysis of 101 patients uniformly treated at a single institution, with literature review. Am J Surg Pathol. 2006;30(5):551–9.

    Article  PubMed  Google Scholar 

  9. Carr NJ, Sobin LH. Epithelial noncarcinoid tumors and tumor-like lesions of the appendix. Cancer. 1995;76(11):2383–4.

    Article  CAS  PubMed  Google Scholar 

  10. Davison JM, Choudry HA, Pingpank JF, et al. Clinicopathologic and molecular analysis of disseminated appendiceal mucinous neoplasms: identification of factors predicting survival and proposed criteria for a three-tiered assessment of tumor grade. Mod Pathol. 2014;27(11):1521–39.

    Article  PubMed  Google Scholar 

  11. Misdraji J, Yantiss RK, Graeme-Cook FM, Balis UJ, Young RH. Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases. Am J Surg Pathol. 2003;27(8):1089–103.

    Article  PubMed  Google Scholar 

  12. Pai RK, Beck AH, Norton JA, Longacre TA. Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol. 2009;33(10):1425–39.

    Article  PubMed  Google Scholar 

  13. Pai RK, Longacre TA. Appendiceal mucinous tumors and pseudomyxoma peritonei: histologic features, diagnostic problems, and proposed classification. Adv Anat Pathol. 2005;12(6):291–311.

    Article  PubMed  Google Scholar 

  14. Reu S, Neumann J, Kirchner T. Mucinous neoplasms of the vermiform appendix, Pseudomyxoma peritonei, and the new WHO classification. Pathologe. 2012;33(1):24–30.

    Article  CAS  PubMed  Google Scholar 

  15. Ronnett BM, Yan H, Kurman RJ, Shmookler BM, Wu L, Sugarbaker PH. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer. 2001;92(1):85–91.

    Article  CAS  PubMed  Google Scholar 

  16. Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM. Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to “Pseudomyxoma peritonei.” Am J Surg Pathol. 1995;19(12):1390–1408.

    Article  CAS  PubMed  Google Scholar 

  17. Shetty S, Natarajan B, Thomas P, Govindarajan V, Sharma P, Loggie B. Proposed classification of pseudomyxoma peritonei: influence of signet ring cells on survival. Am Surg. 2013;79(11):1171–6.

    PubMed  Google Scholar 

  18. Carr NJ, Bibeau F, Bradley RF, et al. The histopathological classification, diagnosis and differential diagnosis of mucinous appendiceal neoplasms, appendiceal adenocarcinomas and pseudomyxoma peritonei. Histopathology. 2017;71(6):847–58.

    Article  PubMed  Google Scholar 

  19. Carr NJ, Cecil TD, Mohamed F, et al. A Consensus for Classification and Pathologic Reporting of Pseudomyxoma Peritonei and Associated Appendiceal Neoplasia: The Results of the Peritoneal Surface Oncology Group International (PSOGI) Modified Delphi Process. Am J Surg Pathol. 2016;40(1):14–26.

    Article  Google Scholar 

Download references

Acknowledgment

This work was partially funded by generous support from Valarie Koch and the New Era Cap Company. The project was supported by the National Institutes of Health through Grant Number UL1-TR-001857, using a Redcap maintained database.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Haroon A. Choudry MD.

Ethics declarations

Disclosures

None

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Choudry, H.A., Pai, R.K., Parimi, A. et al. Discordant Diagnostic Terminology and Pathologic Grading of Primary Appendiceal Mucinous Neoplasms Reviewed at a High-Volume Center. Ann Surg Oncol 26, 2607–2614 (2019). https://doi.org/10.1245/s10434-019-07447-z

Download citation

  • Received:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1245/s10434-019-07447-z

Navigation