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05.08.2019 | Gastrointestinal Oncology

Surveillance of Low-Grade Appendiceal Mucinous Neoplasms With Peritoneal Metastases After Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy: Are 5 Years Enough? A Multisite Experience

Zeitschrift:
Annals of Surgical Oncology
Autoren:
MD Daniel Solomon, MD Eliahu Bekhor, MD Natasha Leigh, BA Yash M. Maniar, BS Linsay Totin, BS Margaret Hofstedt, PA Samantha N. Aycart, MD Jacquelyn Carr, MD Samuel Ballentine, MD Deepa R. Magge, MD Benjamin J. Golas, MD Reetesh K. Pai, MD, PhD Alexandros D. Polydorides, MD David L. Bartlett, MD Daniel M. Labow, MD Haroon A. Choudry, MD Umut Sarpel
Wichtige Hinweise
The original version of this article was revised: Margaret Hofstedt’s last name was misspelled.
A correction to this article is available online at https://​doi.​org/​10.​1245/​s10434-019-07739-4.

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Abstract

Background

Low-grade appendiceal mucinous neoplasms (LAMNs) are tumors that often present with widespread mucin in the peritoneal cavity (pseudomyxoma peritonei [PMP]). Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are effective treatment, but no published recommendations exist regarding surveillance.

Methods

Data from prospective databases of patients who underwent CRS-HIPEC from 2001 to 2017 at two high-volume institutions were retrospectively analyzed. Patients who underwent complete CRS-HIPEC for PMP secondary to LAMN were included in the analysis. Pathologic examination confirmed the diagnosis of LAMN. Cases of mucinous adenocarcinomas and neuroendocrine tumors (goblet cell carcinoids) were excluded.

Results

The study enrolled 156 patients. The median peritoneal cancer index (PCI) was 18 (interquartile range IQR1–3, 12–23), and 125 patients (80.1%) had a CC0 cytoreduction. According to American Joint Committee on Cancer (AJCC) grading, 152 patients (97.4%) presented with acellular mucin or G1 implants, 2 patients (1.3%) presented with G2 disease, and 2 patients (1.3%) presented with G3 disease. During the follow-up period (median, 45 months; IQR1–3 23–76 months), 23 patients (14.7%) experienced recurrence. All the recurrences were peritoneal and occurred within 5 years. The 1-, 3-, and 5-year disease-free survival (DFS) rates were respectively 95.5%, 83.4%, and 78.3%. Univariate Cox regression analysis showed that higher PCI scores (p < 0.001), a CC1 cytoreduction (p = 0.005), and higher preoperative levels of carcinoembryonic antigen (CEA) (p = 0.012) and CA-125 (p = 0.032) correlated with a shorter DFS. Only higher PCI scores independently predicted earlier recurrences (p < 0.001).

Conclusion

Most patients had recurrence within 3 years after CRS-HIPEC, and none after 5 years. High PCI was the only independently significant variable. The study findings support intensive surveillance (every 3–6 months) with tumor markers and imaging methods during the first 3 years, and annual surveillance thereafter, with follow-up assessment after 5 years yielding limited benefit.

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