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Low-grade Appendiceal Mucinous Neoplasm of Uncertain Malignant Potential (LAMN-UMP): Prognostic Factors and Implications for Treatment and Follow-up

  • Gastrointestinal Oncology
  • Published:
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Abstract

Background

Low-grade appendiceal mucinous neoplasm of uncertain malignant potential are poorly understood lesions characterized by extraluminal mucin or fibrosis with neoplastic cells confined to the appendiceal lumen. The purpose of this study is to investigate the clinical and pathologic parameters of these lesions to optimize our understanding and management of these tumors.

Methods

Subjects with these tumors were identified from the appendiceal tumor databases at the University of Texas MD Anderson Cancer Center. Univariate and multivariate Cox proportional hazards regression analyses assessed relationships between clinicopathologic variables [including age, gender, margin status and serum levels of the tumor markers carcinoembryonic antigen (CEA), cancer antigen (CA)-125, and CA19-9] disease-free survival, postrecurrence survival and overall survival.

Results

Ninety-eight subjects with this disease were identified. Most patients did not experience disease recurrence after initial appendectomy. At last follow-up, 25 (26 %) had disease recurrence or died. Of the 20 patients who had disease recurrence, 5 (25 %) died, and 15 (75 %) were alive. Disease-free survival was significantly reduced with positive margin status (p = 0.02) and elevated serum levels of CEA (p < 0.001), CA19-9 (p = 0.01), or CA-125 (p = 0.002) at the time of appendectomy. The median postrecurrence survival time was 4.7 years and the 5-year postrecurrence survival rate was 41 % (standard error = 18 %).

Conclusions

Patients with Low-grade appendiceal mucinous neoplasm of uncertain malignant potential who have negative margins and normal tumor marker levels have a lower risk for recurrence. In these patients, expectant management is sufficient. Elevated tumor marker levels at the time of appendectomy marks an increased risk of recurrence or death and signals the need for closer monitoring or intervention.

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Disclosure

The authors declare no conflict of interest.

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Authors and Affiliations

  1. Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Keith Fournier MD, Safia Rafeeq MD, Paul Kanaby BA, Paul Mansfield MD & Richard Royal MD

  2. Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Melissa Taggart MD

  3. Department of Biostatistics, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Jing Ning PhD

  4. Department of Gastrointestinal Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Hsiang-Chun Chen PhD, Michael Overman MD, Kanwal Raghav MBBS & Cathy Eng MD

Authors
  1. Keith Fournier MD
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  2. Safia Rafeeq MD
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  3. Melissa Taggart MD
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  4. Paul Kanaby BA
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  5. Jing Ning PhD
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  6. Hsiang-Chun Chen PhD
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  7. Michael Overman MD
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  8. Kanwal Raghav MBBS
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  9. Cathy Eng MD
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  10. Paul Mansfield MD
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  11. Richard Royal MD
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Correspondence to Richard Royal MD.

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Fournier, K., Rafeeq, S., Taggart, M. et al. Low-grade Appendiceal Mucinous Neoplasm of Uncertain Malignant Potential (LAMN-UMP): Prognostic Factors and Implications for Treatment and Follow-up. Ann Surg Oncol 24, 187–193 (2017). https://doi.org/10.1245/s10434-016-5588-2

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  • DOI: https://doi.org/10.1245/s10434-016-5588-2

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