Abstract
Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) are an effective therapeutic modality for treating selected patients with peritoneal metastases (PM). There are uncommon situations in which primary peritoneal tumors which are rare give rise to PM or when PM are secondary to rare tumors or when isolated PM arise from common primary intra-abdominal and extra-abdominal tumors. CRS and HIPEC has the potential to provide therapeutic benefit to these patients that are conventionally treated with systemic chemotherapy, essentially a palliative approach. The natural history of many of these diseases is not known, and the evidence favoring the use of such procedures is limited. The collaborative efforts of surgeons treating peritoneal surface malignancies in pooling data and analyzing outcomes have generated some evidence to guide treatment decisions. In some situations like patients with mucinous tumors, intra-abdominal primary sites with limited peritoneal cancer spread, in patients who are in good general health, have tumors responsive to systemic therapy and the risk of morbidity is low, CRS and HIPEC can be used in the absence of level I/II evidence based on retrospective data and the recommendation of experts in the field. Such treatment should be offered in specialized centers and decisions taken in multidisciplinary meetings. It is ideal to enroll such patients in clinical trial or follow an institutional protocol or refer such patients to a specialized center. The respective roles of CRS and HIPEC should be considered separately for each tumor site and histology; where the benefit of intraperitoneal chemotherapy is less clear, the morbidity versus benefit should be considered. There is rapid progress in systemic therapies as well, with new and more effective therapies available, which should be used where indicated. New therapeutic modalities like pressurized intraperitoneal chemotherapy (PIPAC) can be used as a palliative option in combination with systemic chemotherapy where the therapeutic benefit of CRS and HIPEC is uncertain and the risk of morbidity is high.
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Bhatt, A., Seshadri, R.A. (2018). Rare Indications for Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy. In: Bhatt, A. (eds) Management of Peritoneal Metastases- Cytoreductive Surgery, HIPEC and Beyond. Springer, Singapore. https://doi.org/10.1007/978-981-10-7053-2_16
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DOI: https://doi.org/10.1007/978-981-10-7053-2_16
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Publisher Name: Springer, Singapore
Print ISBN: 978-981-10-7052-5
Online ISBN: 978-981-10-7053-2
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