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Outcomes of Surgical and Chemotherapeutic Treatments of Goblet Cell Carcinoid Tumors of the Appendix

  • Gastrointestinal Oncology
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Goblet cell carcinoids (GCCs) of the appendix are rare mucinous neoplasms, for which optimal therapy is poorly described. We examined prognostic clinical and treatment factors in a population-based cohort.

Methods

Patients diagnosed with GCC from 1984 to 2014 were identified from the British Columbia Cancer Agency and the Vancouver Lower Mainland Pathology Archive.

Results

Of 88 cases with confirmed appendiceal GCCs, clinical data were available in 86 cases (annual population incidence: 0.66/1,000,000). Median age was 54 years (range 25–91) and 42 patients (49%) were male. Metastasis at presentation was the strongest predictor of overall survival (OS), with median OS not reached for stage I–III patients, and measuring 16.2 months [95% confidence interval (CI) 9.1–29] for stage IV patients. In 67 stage I–III patients, 51 (76%) underwent completion hemicolectomy and 9 (17%) received adjuvant 5-fluorouracil-based chemotherapy. No appendicitis at initial presentation and Tang B histology were the only prognostic factors, with inferior 5-year recurrence-free survival (53 vs. 83% with appendicitis, p = 0.02; 45% Tang B vs. 89% Tang A, p < 0.01). Of 19 stage IV patients, 10 (62.5%) received 5-fluorouracil-based chemotherapy and 11 (61%) underwent multiorgan resection (MOR) ± hyperthermic intraperitoneal chemotherapy (HIPEC). Low mitotic rate and MOR ± HIPEC were associated with improved 2-year OS, but only MOR ± HIPEC remained significant on multivariate analysis (hazard ratio 5.4, 95% CI 1.4–20.9; p = 0.015).

Conclusions

In this population-based cohort, we demonstrate excellent survival outcomes in stage I–III appendiceal GCCs and clinical appendicitis. Hemicolectomy remains the standard treatment. In metastatic disease, outcomes remain poor, although MOR ± HIPEC may improve survival.

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Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Division of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada

    Erica S. Tsang MD & Hagen F. Kennecke MD, MHA, FRCPC

  2. Department of Medicine, University of British Columbia, Vancouver, BC, Canada

    Erica S. Tsang MD

  3. Puyallup General Surgery, Proliance Surgeons, Puyallup, WA, USA

    Yarrow J. McConnell MD, MSc, FRCSC

  4. Department of Surgery, University of British Columbia, Vancouver, BC, Canada

    Yarrow J. McConnell MD, MSc, FRCSC & Siham Zerhouni MD

  5. Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada

    David F. Schaeffer MD, FRCPC & Lawrence Lee MD

  6. Division of Anatomic Pathology, Vancouver General Hospital, Vancouver, BC, Canada

    David F. Schaeffer MD, FRCPC & Lawrence Lee MD

  7. Gastrointestinal Cancer Outcomes Unit, British Columbia Cancer Agency, Vancouver, BC, Canada

    Yaling Yin PhD & Caroline Speers BA, CHIM

  8. Provincial Pharmacy, British Columbia Cancer Agency, Vancouver, BC, Canada

    Kimberly Schaff RPhT

  9. Virginia Mason Cancer Institute, Virginia Mason Hospital and Seattle Medical Center, Seattle, WA, USA

    Hagen F. Kennecke MD, MHA, FRCPC

Authors
  1. Erica S. Tsang MD
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  2. Yarrow J. McConnell MD, MSc, FRCSC
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  3. David F. Schaeffer MD, FRCPC
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  4. Lawrence Lee MD
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  5. Yaling Yin PhD
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  6. Siham Zerhouni MD
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  7. Kimberly Schaff RPhT
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  8. Caroline Speers BA, CHIM
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  9. Hagen F. Kennecke MD, MHA, FRCPC
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Corresponding author

Correspondence to Hagen F. Kennecke MD, MHA, FRCPC.

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Disclosures

Erica S. Tsang, Yarrow J. McConnell, David F. Schaeffer, Lawrence Lee, Yaling Yin, Siham Zerhouni, Kimberly Schaff, Caroline Speers, and Hagen F. Kennecke declare that no conflicts of interest exist.

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Tsang, E.S., McConnell, Y.J., Schaeffer, D.F. et al. Outcomes of Surgical and Chemotherapeutic Treatments of Goblet Cell Carcinoid Tumors of the Appendix. Ann Surg Oncol 25, 2391–2399 (2018). https://doi.org/10.1245/s10434-018-6560-0

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  • DOI: https://doi.org/10.1245/s10434-018-6560-0

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