Zusammenfassung
Hintergrund: Aufgrund des Fehlens fester anatomischer Kompartimente werden retroperitoneale Sarkome erst bei Vorliegen sehr großer Tumoren und Infiltration von Nachbarorganen diagnostiziert. Trotz Fortschritte der diagnostischen Möglichkeiten und chirurgischer Techniken kommt es auch nach kompletter Entfernung sehr häufig zu Lokalrezidiven mit ungünstiger Prognose. Ziel dieser Arbeit war die Analyse von Diagnostik, Therapie und Langzeitverlauf der retroperitonealen Sarkome im eigenen Krankengut über einen Zeitraum von 10 Jahren. Patienten und Methodik: Zwischen Januar 1995 und Januar 2005 wurde in unserer Klinik bei 379 Patienten ein operativer Eingriff wegen eines primären retroperitonealen Tumors durchgeführt. Unter den 67 (17,7 %) malignen Läsionen fand sich bei 35 Patienten ein Sarkom. Die vorliegende Untersuchung berücksichtigt den Langzeitverlauf der 21 Patienten mit primärem reseziertem retroperitonealem Sarkom, Rezidiveingriffe und Probelaparotomien ausgeschlossen. Ergebnisse: Das mediane Alter zum Zeitpunkt der Operation war 61 (25-86) Jahre, 57,1 % aller Patienten waren männlich. Die Dauer der Symptome betrug 3 (1-36) Monate. Eine pR0-Resektion konnte bei 20/21 Patienten erreicht werden. Unter den histopathologischen Tumortypen fanden sich am häufigsten Liposarkome (n = 10) und Leiomyosarkome (n = 6). Bei 12 von 21 Patienten kam es im Median 13,5 (5-42) Monate nach dem Ersteingriff zu einem Lokalrezidiv, bei 4 Patienten zu Fernmetastasen. Das Langzeitüberleben aller 21 Patienten mit primärem reseziertem retroperitonealem Sarkom betrug 24 (1-101) Monate. Dabei fanden sich günstigere Verläufe für Patienten mit Leiomyosarkom verglichen mit den Liposarkomen. Schlussfolgerung: Retroperitoneale Sarkome sind eine heterogene Entität und fanden sich im eigenen Krankengut bei 6,1 % aller primärer, operativ behandelter retroperitonealer Tumoren. Die Behandlungsstrategie ist komplex und abhängig von Stadium, Lokalisation und histologischen Charakteristika. Das Langzeitüberleben ist limitiert durch die hohe Rate an Lokalrezidiven.
Abstract
Background: Due to the fact that there are no distinct anatomical compartments, retroperitoneal sarcomas are moreover diagnosed with evidence of large tumors and infiltration of adjacent organs. In spite of improvement of the diagnostic facilities and surgical techniques, quite frequently local recurrences with unfavourable prognosis turn up even after complete removal. It was the aim of this study to analyze diagnosis, therapy and long-term prognosis in patients with retroperitoneal sarcomas over a period of 10 years. Patients and Methods: Between January 1995 and January 2005, 379 patients underwent surgery for a primary retroperitoneal tumor at our clinic. Among the 67 (17.1 %) malignant lesions, a sarcoma was found in 35 patients. The present study is focused on the long-term prognosis of those 21 patients with a primary resected retroperitoneal sarcoma, recurrent sarcomas and exploratory laparotomies excluded. Results: The median patient age at the time of surgery was 61 (25-86) years, 57.1 % were males. The duration of symptoms was 3 (1-36) months. A pR0-resection was achieved in 20/21 patients. Among the histopathological tumor types, liposarcomas (n = 10) and leiomyosarcomas (n = 6) were found most frequently. Local recurrence developed in 12 out of 21 patients at 13.5 (5-42) months after the first operation, and in 4 patients distant metastases were observed. The long-term survival of all 21 patients with primary resected retroperitoneal sarcoma was 24 (1-101) months. A more favourable prognosis was seen in patients with leiomyosarcoma as compared with liposarcoma. Conclusion: Retroperitoneal sarcomas are a heterogeneous entity, and they were found among our own patients in 6.1 % of all primary operatively treated retroperitoneal tumors. The strategy of treatment is complex and dependent on the stage, localization and histopathological characteristics. Long-term survival is limited as a result of the high rate of local recurrence.
Schlüsselwörter
retroperitoneale Sarkome - Differenzialdiagnostik - operatives Vorgehen - Langzeitverlauf
Key words
retroperitoneal sarcoma - differential diagnostic - operative procedure - long-term course
Literatur
-
1
Alvarenga J C, Ball A B, Fisher C, Fryatt I, Jones L, Thomas J M.
Limitations of surgery in the treatment of retroperitoneal sarcoma.
Br J Surg.
1991;
78
912-916
-
2
Berquist T H, Ehman R L, King B F, Hodgman C H, Ilstrup D M.
Value of MR imaging in differentiating benign from malignant soft-tissue masses: study of 95 lesions.
Am J Roentgenol.
1990;
155
1251-1255
-
3
Braasch J W, Mon A B.
Primary retroperitoneal tumors.
Surg Clin North Am.
1967;
47
663-678
-
4
Catton C N, O'Sullivan B, Kotwall C, Cummings B, Hao Y, Fornasier V.
Outcome and prognosis in retroperitoneal soft tissue sarcoma.
Int J Radiat Oncol Biol Phys.
1994;
29
1005-1010
-
5
Cody H S, Turnbull A D, Fortner J G, Hajdu S I.
The continuing challenge of retroperitoneal sarcomas.
Cancer.
1981;
47
2147-2152
-
6
Cormier J N, Pollock R E.
Soft tissue sarcomas.
CA Cancer J Clin.
2004;
54
94-109
-
7
Dalton R R, Donohue J H, Mucha Jr P, van Heerden J A, Reiman H M, Chen S P.
Management of retroperitoneal sarcomas.
Surgery.
1989;
106
725-733
-
8
Demas B E, Heelan R T, Lane J, Marcove R, Hajdu S, Brennan M F.
Soft-tissue sarcomas of the extremities: comparison of MR and CT in determining the extent of disease.
AJR Am J Roentgenol.
1988;
150
615-620
-
9
De Saint Aubain Somerhausen N, Fletcher C D.
Soft-tissue sarcomas: an update.
Eur J Surg Oncol.
1999;
25
215-220
-
10 DeVita Jr V T, Hellman S, Rosenberg S A. Cancer: Principles and Practice of Oncology. 6th ed. Lippincott Williams & Wilkins, Philadelphia, PA 2001; 1841-1891
-
11
Dupuy D E, Rosenberg A E, Punyaratabandhu T, Tan M H, Mankin H J.
Accuracy of CT-guided needle biopsy of muscoloskeletal neoplasms.
AJR Am J Roentgenol.
1998;
171
759-762
-
12
Fein D A, Corn B W, Lanciano R M, Herbert S H, Hoffmann J P, Coia L R.
Management of retroperitoneal sarcomas: does dose escalation impact on locoregional control?.
Int J Radiat Oncol Biol Phys.
1995;
31
129-134
-
13
Ferrario T, Karakousis C P.
Retroperitoneal sarcomas. Grade and survival.
Arch Surg.
2003;
138
248-251
-
14
Gielen J LMA, De Schepper A M, Vanhoenacker F, Parizel P M, Wang X L, Sciot R, Weyler J.
Accuracy of MRI in characterization of soft tissue tumors and tumor-like lesions. A prospective study in 548 patients.
Eur Radiol.
2004;
14
2320-2330
-
15
Gieschen J H, Spiro I J, Suit H D, Ott M J, Rattner D W, Ancukiewicz M, Willett C G.
Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma.
Int J Radiat Oncol Biol Phys.
2001;
50
127-131
-
16
Glenn J, Sindelar W F, Kinsella T, Glatstein E, Tepper J, Costa J, Baker A, Sugarbaker P, Brennan M F, Seipp C.
Results of multimodality therapy of resectable soft-tissue sarcomas of the retroperitoneum.
Surgery.
1985;
97
316-325
-
17
Goffman T, Tochner Z, Glatstein E.
Primary treatment of large and massive adult sarcomas with iododeoxyuridine and aggressive hyperfractionated irridation.
Cancer.
1991;
67
572-576
-
18
Gronchi A, Casali P G, Fiore M, Mariani L, Lo Vullo S, Bertulli R, Colecchia M, Lozza L, Olmi P, Santinami M, Rosai J.
Retroperitoneal soft tissue sarcomas. Patterns of recurrence in 167 patients treated at a single institution.
Cancer.
2004;
100
2448-2455
-
19
Gunderson L L, Nagorney D M, McIlrath D C, Fieck J M, Wieand H S, Martinez A, Pritchard D J, Sim F, Martenson J A, Edmonson J H, Donohue J H.
External beam and intraoperative electron irradiation for locally advanced soft tissue sarcomas.
Int J Radiat Oncol Biol Phys.
1993;
25
647-656
-
20
Hanna S L, Fletcher B D.
MR imaging of malignant soft-tissue tumors.
Magn Reson Imaging Clin N Am.
1995;
3
629-650
-
21
Hassan I, Park S Z, Donohue J H, Nagorney D M, Kay P A, Nasciemento A G, Schleck C D, Ilstrup D M.
Operative management of primary retroperitoneal sarcomas. A reapprisal of an institutional experience.
Ann Surg.
2004;
239
244-250
-
22
Herbert S H, Corn B W, Solin L J, Lanciano R M, Schultz D J, McKenna W G, Coia L R.
Limb-preserving treatment for soft-tissue sarcomas of the extremities. The significance of surgical margins.
Cancer.
1995;
72
1230-1238
-
23
Heslin M J, Smith J K.
Imaging of soft tissue sarcomas.
Surg Oncol Clin N Am.
1999;
8
91-107
-
24 Jaques D P, Coit D G, Brennan M F. Soft-tissue sarcoma of the retroperitoneum. In: Shiu MH, Brennan MF (eds). Surgical management of soft-tissue sarcoma. Lea & Febiger, Philadelphia, Pa 1989; 157-169
-
25
Jaques D P, Coit D G, Hajdu S I, Brennan M F.
Management of primary and recurrent soft-tissue sarcoma of the retroperitoneum.
Ann Surg.
1990;
212
51-59
-
26
Kaplan E L, Meier P.
Nonparametric estimation from incomplete observations.
J Am Stat Assoc.
1987;
34
187-202
-
27
Karakousis C P, Kontzoglou K, Driscoll D L.
Resectability of retroperitoneal sarcomas: a matter of surgical technique?.
Eur J Surg Oncol.
1995;
21
617-622
-
28
Karakousis C P, Velez A F, Emrich L J.
Management of retroperitoneal sarcomas and patient survival.
Am J Surg.
1985;
150
376-380
-
29
Karakousis C P, Velez A F, Gerstenbluth R, Driscoll D L.
Resectability and survival in retroperitoneal sarcomas.
Ann Surg Oncol.
1996;
3
150-158
-
30 Kiel K D, Won M H, Witt T R, Noyes D, Sause W T, Lanciano R M, Hanks G E. Preliminary results of protocol RTOG 85-07: phase II study of intraoperative radiation for retroperitoneal sarcomas. In: Abe M, Takahashi M (eds). Intraoperative radiation therapy. Proceedings of the third international symposium on intraoperative radiation therapy. Pergamon Press, New York 1991; 371-372
-
31
Kilkenny J W, Bland K I, Copeland E M.
Retroperitoneal sarcoma: the University of Florida experience.
J Am Coll Surg.
1996;
182
329-339
-
32
Kinsella T J, Sindelar W F, Lack E, Glatstein E, Rosenberg S A.
Preliminary results of a randomized study of adjuvant radiation therapy in resectable adult retroperitoneal soft tissue sarcomas.
J Clin Oncol.
1988;
6
18-25
-
33
Lewis J J, Leung D, Woodruff J M, Brennan M F.
Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution.
Ann Surg.
1998;
228
355-365
-
34
López-Ríos F, Pérez-Barrios A, Alberti N, Vargas J, Lozano F, de Augustín P.
Fine-needle aspiration biopsy of the retroperitoneum: a series of 111 cases not including specific organs.
Diagn Cytopathol.
2002;
27
85-89
-
35
Ma L D, Frassica F J, Scott Jr W W, Fishman E K, Zerbouni E A.
Differentiation of benign and malignant muscolosceletal tumors: potential pitfalls with MR imaging.
Radiographics.
1995;
15
349-366
-
36
McGrath P C, Neifeld J P, Lawrence Jr W, DeMay R M, Kay S, Horsley 3rd J S, Parker G A.
Improved survival following complete excision of retroperitoneal sarcomas.
Ann Surg.
1984;
200
200-204
-
37
Moulton J S, Blebea J S, Dunco D M, Braley S E, Bisset III G S, Emery K H.
MR imaging of soft-tissue masses: diagnostic efficacy and value of distinguishing between benign and malignant lesions.
Am J Roentgenol.
1995;
164
1191-1199
-
38
Pisters P W, Leung D H, Woodruff J, Shi W, Brennan M F.
Analysis of prognostic factors in 1 041 patients with localized soft tissue sarcomas of the extremities.
J Clin Oncol.
1996;
14
1679-1689
-
39
Sindelar W F, Kinsella T J, Chen P W, DeLaney T F, Tepper J E, Rosenberg S A, Glatstein E.
Intraoperative radiotherapy in retroperitoneal sarcomas. Final results of a prospective, randomized, clinical trial.
Arch Surg.
1993;
128
402-410
-
40
Singer S, Corson J M, Demetri G D, Healey E A, Marcus K, Eberlein T J.
Prognostic factors predictive of survival for truncal and retroperitoneal soft-tissue sarcoma.
Ann Surg.
1995;
221
185-195
-
41
Storm F K, Mahvi D M.
Diagnosis and management of retroperitoneal soft tissue sarcomas.
Ann Surg.
1991;
214
2-10
-
42
Tepper J E, Suit H D, Wood W C, Proppe K H, Harmon D, McNulty P.
Radiation therapy of retroperitoneal soft tissue sarcomas.
Int J Radiat Oncol Biol Phys.
1884;
10
825-830
-
43
Willett C G, Suit H D, Tepper J E, Mankin H J, Convery K, Rosenberg A L, Wood W C.
Intraoperative electron beam radiation therapy for retroperitoneal soft tissue sarcoma.
Cancer.
1991;
68
278-283
Dr. med. Ines Gockel
Klinik für Allgemein- und Abdominalchirurgie · Johannes-Gutenberg-Universität
Langenbeckstr. 1
55101 Mainz
Phone: +49/(0)61 31/17 72 91
Fax: +49/(0)61 31/17 66 30
Email: gockel@ach.klinik.uni-mainz.de
