Zusammenfassung
Das Pseudomyxoma peritonei ist eine seltene Komplikation nach Ruptur einer Mukozele
der Appendix oder muzinöser Zystadenome der Ovarien bzw. nach Ruptur muzinöser Zystadenokarzinome
niedriger Malignität im Bereich des Gastrointestinaltraktes oder der Ovarien. Trotz
aggressiver Behandlung der Erkrankung durch rezidivierende, ausgedehnte Operationen
und die Anwendung von Zytostatika haben die Patientinnen häufig Rezidive und eine
schlechte Prognose.
Fallvorstellung
Wir berichten über eine Patientin, der im Alter von 57 Jahren eine primär perforierte
Mukozele der Appendix entfernt wurde. 8 Monate später erkrankte sie an einem Pseudomyxoma
peritonei. Nach mehreren Rezidivoperationen und einer intraperitonealen Chemotherapie
mit Novantron verstarb die Patientin nach sechsjähriger Krankheitsdauer an Kachexie.
Schlussfolgerungen
Zur Vermeidung eines Pseudomyxoma peritonei müssen muzinöse Tumoren in toto ohne Rupturierung
der Kapsel entfernt werden. Bei muzinösen Tumoren der Appendix sollte immer das innere
Genitale begutachtet, bei einem primären Befall der Ovarien die Appendektomie durchgeführt
werden. Hat bereits eine Ausbreitung der Schleimmassen stattgefunden, ist die operative
Druckentlastung des Abdomens und die Reduktion der schleimbildenden Herde, gefolgt
von einer intraperitonealen Chemotherapie zu empfehlen. Studien fehlen aufgrund der
Seltenheit des Krankheitsbildes.
Summary
Objective
Pseudomyxoma peritonei is a rare complication after rupture of mucinous tumors of
the appendix, ovaries or colon. The condition tends to recur despite aggressive surgical
debulking and intraperitoneal chemotherapy and the prognosis is poor.
Case Report
A 57-year-old woman presented with pseudomyxoma peritonei 8 months after removal of
a mucocele of the appendix. After multiple operations and intraperitoneal chemotherapy
with mitoxantrone the patient died of cachexia 6 years after the initial surgery.
Conclusion
Mucinous tumors should be removed completely and intact to avoid pseudomyxoma peritonei.
The ovaries should be inspected in women with mucinous tumors of the appendix. Appendectomy
appears indicated in patients with mucinous tumors of the ovary. Tumor reductive surgery
and possibly intraperitoneal chemotherapy are the treatment of choice for patients
with mucinous tumors and deposits in the abdomen and pelvis.
Schlüsselwörter
Pseudomyxoma peritonei - Chemotherapie
Key words
Pseudomyxoma peritonei - Chemotherapy
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