Zytoreduktive Chirurgie und HIPEC bei peritoneal-metastasierten Tumoren im Kindesalter

 

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Zusammenfassung

Die Peritonealkarzinose ist ein weit fortgeschrittenes Tumorstadium mit reduzierter Überlebensrate. Durch die Einführung der zytoreduktiven Chirurgie (CRS) und der intraperitonealen hyperthermen Chemotherapie (HIPEC) konnten verbesserte Behandlungsergebnisse bei Erwachsenen im Vergleich zur alleinigen Chemotherapie erreicht werden. Die Hauptindikationen für die CRS und HIPEC bei Erwachsenen stellen primäre peritoneale Tumoren wie die Mesotheliome und Pseudomyxoma peritonei dar. Hinzu kommen die peritoneale Aussaat von gastrointestinalen Karzinomen und Ovarialkarzinomen. Bei Kindern ist die Inzidenz der Peritonealkarzinose vermutlich niedriger als bei Erwachsenen. Allerdings liegen hierzu keine verlässlichen Daten vor. Dennoch kann die Peritonealkarzinose/peritoneale Sarkomatose bei Kindern mit desmoplastischen klein- und rundzelligen Tumoren (DSRCT), Weichteilsarkomen (Rhabdomyosarkome, Leiomyosarkome, GIST oder Liposarkomen) und gastrointestinalen Karzinomen auftreten. Die CRS und HIPEC ist in jüngster Vergangenheit als neue Behandlungsalternative für Kinder mit Peritonealkarzinose/peritonealer Sarkomatose in einzelnen Zentren weltweit etabliert worden. In dieser Publikation werden die Indikationsstellung, Behandlungsregime und Komplikationen bei Kindern beschrieben.

Abstract

Peritoneal tumour dissemination represents an advanced tumour stage and survival rates are usually low. In the past, cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) has been established in adults leading to increased survival rates in comparison to chemotherapy alone. CRS and HIPEC are indicated in primary peritoneal tumours such as mesothelioma and pseudomyxoma peritonei, and in peritoneal metastases from gastrointestinal and ovarian cancers in adults. The incidence of peritoneal surface malignancies in children seems to be lower than in adults, but the incidence is unknown. Nevertheless, peritoneal carcinomatosis/sarcomatosis may occur in patients suffering from desmoplastic small round cell tumour (DSRCT), soft tissue sarcoma (rhabdomyosarcoma, leiomyosarcoma, GIST or liposarcoma), as well as in patients with gastrointestinal cancers. CRS and HIPEC have been established as a novel treatment option in children suffering from peritoneal carcinomatosis/sarcomatosis in very few centres worldwide. This paper reviews the indications, treatment regimens, and pitfalls of this approach in children.

^* Geteilte Erstautorenschaft.

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