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
Literatur
-
1
Averbach A M, Sugarbaker P H.
Recurrent intraabdominal cancer causing intestinal obstruction: Washington Hospital Center experience with 42 patients managed by surgery and intraperitoneal chemotherapy.
Cancer Treat Res.
1996;
81
133-147
-
2
Chuaqui R F, Zhuang Z, Emmert-Buck M R, Bryant B R.
Genetic analysis of synchronous mucinous tumors of the ovary and appendix.
Hum Pathol.
1996;
27 (2)
165-171
-
3
Cuatrecasa M, Matias-Guiu X, Prat J.
Synchronous mucinous tumors of the appendix and the ovary associated with pseudomyxoma peritonei. A clinicopathologic study of six cases with comparative analysis of c-Ki-ras mutations.
Am J Surg Pathol.
1996;
20 (6)
739-746
-
4
Gough D B, Donohue J H, Schutt A J, Gionchoroff N.
Pseudomyxoma peritonei. Long-term patient survival with an aggressive regional approach.
Ann Surg.
1994;
219 (2)
112-119
-
5
Hinson F L, Ambrose N S.
Pseudomyxoma peritonei.
Br J Surg.
1998;
85 (10)
1332-1339
-
6
Jahne J, Lang H, Meyer H J, Pichlmayr R.
Possibilities and limits of surgical therapy of pseudomyxoma peritonei.
Langenbecks Arch Chir.
1993;
378 (5)
292-296
-
7
Jahne J, Piso P, Schmoll E.
Intraoperative intraperitoneal chemotherapy-considerations and aspects of safe intra- and postoperative treatment with cytostatic drugs.
Langenbecks Arch Chir.
1997;
382 (1)
8-14
-
8
Lang H, Jahne F, Flemming P, Meyer H J, Pichlmayr R.
Pseudomyxoma peritonei of appendiceal origin - a report of seven cases and a review of published reports.
Eur J Surg.
1995;
161 (5)
355-560
-
9
Meden H, Conrad A, Brinck U, Rath W, Kuhn W.
Pathologic appendix findings in gynecologic operations.
Zentralbl Gynäkol.
1994;
116 (8)
456-460
-
10
Peek D F, Beets G L.
Pseudomyxoma peritonei in the pleural cavity: report of a case.
Dis Colon Rectum.
1999;
42 (1)
113-115
-
11
Prayson R A, Hartz W R, Petras R E.
Pseudomyxoma peritonei - a clinicopathologic study of 19 cases with emphasis on site of origin and nature of assotiated ovarian tumors.
Am J Surg Pathol.
1994;
18 (6)
591-603
-
12
Seidman J D, Elsayed A M, Sobin L H, Tavassoli F A.
Association of mucinous tumors of the ovary and appendix. A clinicopathologic study of 25 cases.
Am J Surg Pathol.
1993;
17 (1)
22-34
-
13
Sugarbaker P H, Zhu B W, Sese G B, Shmookler B.
Peritoneal carcinomatosis from appendical cancer: results in 69 patients treated by cytoreductive surgery and intraperitoneal chemotherapy.
Dis Colon Rectum.
1993;
36 (4)
323-329
-
14
Sugarbaker P H.
Pseudomyxoma peritonei.
Cancer Treat Res.
1996;
81
105-119
-
15
Sugarbaker P H, Fernandez-Trigo V M, Shamsa F.
Clinical determinants of treatment failure in patients with pseudomyxoma peritonei.
Cancer Treat Res.
1996;
8
121-132
-
16
Sullivan M H, Sugarbaker P H.
Treatment of pseudomyxoma peritonei in a geriatric patient population.
J Surg Oncol.
1995;
58 (2)
121-124
-
17
Teixeira M R, Qvist H, Giercksky K E, Bohler P J.
Cytogenetic analysis of several pseudomyxoma peritonei lesions originating from a mucinous cystadenoma of the appendix.
Cancer Genet Cytogenet.
1997;
93 (2)
157-159
-
18
Wertheim T, Fleischhacker D, McLachlin C M, Rice L W, Berkowitz R S.
Pseudomyxoma peritonei: a review of 23 cases.
Obstet Gynecol.
1994;
84 (1)
17-21
Dr. med. D. Dorn
Ludwigstraße 27
15345 Eggersdorf
