Pseudomyxoma Peritonei of Extra-Appendiceal Origin: A Comparative Study

Dario Baratti, Shigeki Kusamura, Massimo Milione, Filippo Pietrantonio, Marta Caporale, Marcello Guaglio, Marcello Deraco

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Abstract

Background: Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown. Methods: A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary. Results: In 19 patients (8.4 %), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 % confidence interval [CI] 53.9–80.1), and 10-year overall survival was 63.4 % (median 148.2 months; 95 % CI 131.2–165.2) for appendiceal PMP, and 62.0 % (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate (p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 % CI 0.78–3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival. Conclusions: Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.

Original languageEnglish
Pages (from-to)1-9
Number of pages9
JournalAnnals of Surgical Oncology
DOIs
Publication statusAccepted/In press - Jun 28 2016

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Pseudomyxoma Peritonei
Drug Therapy
Teratoma
Confidence Intervals
Appendiceal Neoplasms
Keratin-20
Keratin-7
Survival
Survival Analysis
Cervix Uteri
Ovary
Histology
Multivariate Analysis
Databases

ASJC Scopus subject areas

  • Surgery
  • Oncology

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Pseudomyxoma Peritonei of Extra-Appendiceal Origin : A Comparative Study. / Baratti, Dario; Kusamura, Shigeki; Milione, Massimo; Pietrantonio, Filippo; Caporale, Marta; Guaglio, Marcello; Deraco, Marcello.

In: Annals of Surgical Oncology, 28.06.2016, p. 1-9.

Research output: Contribution to journalArticle

Baratti, Dario ; Kusamura, Shigeki ; Milione, Massimo ; Pietrantonio, Filippo ; Caporale, Marta ; Guaglio, Marcello ; Deraco, Marcello. / Pseudomyxoma Peritonei of Extra-Appendiceal Origin : A Comparative Study. In: Annals of Surgical Oncology. 2016 ; pp. 1-9.
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abstract = "Background: Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown. Methods: A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary. Results: In 19 patients (8.4 {\%}), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 {\%} confidence interval [CI] 53.9–80.1), and 10-year overall survival was 63.4 {\%} (median 148.2 months; 95 {\%} CI 131.2–165.2) for appendiceal PMP, and 62.0 {\%} (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate (p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 {\%} CI 0.78–3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival. Conclusions: Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.",
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T2 - A Comparative Study

AU - Baratti, Dario

AU - Kusamura, Shigeki

AU - Milione, Massimo

AU - Pietrantonio, Filippo

AU - Caporale, Marta

AU - Guaglio, Marcello

AU - Deraco, Marcello

PY - 2016/6/28

Y1 - 2016/6/28

N2 - Background: Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown. Methods: A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary. Results: In 19 patients (8.4 %), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 % confidence interval [CI] 53.9–80.1), and 10-year overall survival was 63.4 % (median 148.2 months; 95 % CI 131.2–165.2) for appendiceal PMP, and 62.0 % (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate (p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 % CI 0.78–3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival. Conclusions: Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.

AB - Background: Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown. Methods: A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary. Results: In 19 patients (8.4 %), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 % confidence interval [CI] 53.9–80.1), and 10-year overall survival was 63.4 % (median 148.2 months; 95 % CI 131.2–165.2) for appendiceal PMP, and 62.0 % (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate (p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 % CI 0.78–3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival. Conclusions: Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.

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