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Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei)

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Abstract

The epithelial appendiceal neoplasms are uncommon and are usually detected as an unexpected surgical finding. The general surgeon should be aware of the diversity of its clinical manifestations and biological behaviors along with the significance of the surgical treatment on the progression of the illness and the prognosis of the patients. The operative findings and, especially, tumor histology, determine the type of surgery. Intestinal histologic subtype behaves and should be treated similarly to the right colon neoplasms; while mucinous tumors, often discordant between histology and its aggressiveness, can be treated with a simple appendectomy or require complex oncological surgeries. Mucinous tumors are often associated with the presence of mucin or tumor implants in the abdominal cavity, being the clinical syndrome known as pseudomyxoma peritonei (PMP). PMP tends to present an indolent but deadly evolution and requires a multimodal approach as a single treatment with curative potential: complete cytoreductive surgery plus hyperthermic Intra-peritoneal chemotherapy (CCRS + HIPEC) now considered the standard of care in this pathology. The general surgeon should be aware of the governing principles of the treatment of appendiceal neoplasms with or without peritoneal dissemination, know the therapeutic frontiers in every situation (avoiding unnecessary or counterproductive surgeries) and sending early these patients to specialised centres in the radical management of malignant diseases of the peritoneum in the conditions and with the necessary information to facilitate a possible radical treatment.

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Acknowledgments

The authors are grateful to the members of Grupo Español de Tratamiento de los Tumores Digestivos (TTD) and Grupo Español de Cirugía Oncológica Peritoneal (GECOP) for their review and valuable contributions.

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Authors and Affiliations

  1. Consorci Sanitari Integral (H. Sant Joan Despí. Moisès Broggi), Universidad de Barcelona, Barcelona, Spain

    P. Barrios, F. Losa & I. Ramos

  2. Anderson Cancer Center, Madrid, Spain

    S. Gonzalez-Moreno & A. Rojo

  3. Hospital San José, Vitoria, Spain

    A. Gómez-Portilla

  4. Hospital Quirón-Salud Torrevieja, Alicante, Spain

    P. Bretcha-Boix

  5. Hospital de Torrecardenas, Almeria, Spain

    J. Torres-Melero

  6. Institut Català d’Oncologia (ICO-L’Hospitalet), Barcelona, Spain

    R. Salazar

  7. Hospital Regional Universitario, Málaga, Spain

    M. Benavides

  8. Hospital General de Alicante, Alicante, Spain

    T. Massuti

  9. Reina Sofía Hospital, University of Córdoba, Maimonides Institute of Biomedical Research (IMIBIC), Córdoba, Spain

    E. Aranda

  10. Spanish Cancer Network (RTICC), Instituto de Salud Carlos III, Córdoba, Spain

    E. Aranda

  11. Universidad de Barcelona, Barcelona, Spain

    P. Barrios

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  1. P. Barrios
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Barrios, P., Losa, F., Gonzalez-Moreno, S. et al. Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei). Clin Transl Oncol 18, 437–448 (2016). https://doi.org/10.1007/s12094-015-1413-9

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