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Strahlentherapie und Onkologie

, Volume 193, Issue 4, pp 269–275 | Cite as

Radiotherapy in desmoid tumors

Treatment response, local control, and analysis of local failures
  • Kirsi SanttiEmail author
  • Annette Beule
  • Laura Tuomikoski
  • Mikko Rönty
  • Anna-Stina Jääskeläinen
  • Kauko Saarilahti
  • Hanna Ihalainen
  • Maija Tarkkanen
  • Carl Blomqvist
Original Article

Abstract

Background

Desmoid tumors (aggressive fibromatosis) are rare soft tissue tumors which frequently recur after surgery. Desmoid tumors arise from musculoaponeurotic tissue in the extremities, head and neck, abdominal wall, or intra-abdominally. Our aim was to examine the outcome of radiotherapy of desmoid tumors in a single institution series.

Patients and methods

We evaluated 41 patients with desmoid tumors treated with 49 radiotherapies between 1987 and 2012. Radiologic images for response evaluation were reassessed and responses to treatment registered according to RECIST criteria 1.1. For patients with local failures radiation dose distribution was determined in each local failure volume using image co-registration. Recurrences were classified as in-target, marginal, or out-of-target. Prognostic factors for radiotherapy treatment failure were evaluated.

Results

Radiotherapy doses varied from 20–63 Gy (median 50 Gy) with a median fraction size of 2 Gy. The objective response rate to definitive radiotherapy was 55% (12/22 patients). Median time to response was 14 months. A statistically significant dose-response relation for definitive and postoperative radiotherapy was observed both in univariate (p-value 0.002) and in multivariate analysis (p-value 0.02) adjusted for potential confounding factors. Surgery before radiotherapy or surgical margin had no significant effect on time to progression. Nine of 11 (82%) local failures were classified as marginal and two of 11 (18%) in-target. None of the recurrences occurred totally out-of-target.

Conclusions

Radiotherapy is a valuable option for treating desmoid tumors. Radiotherapy dose appears to be significantly associated to local control.

Keywords

Aggressive fibromatosis Radiotherapy Local recurrence Dose-response relationship, radiation 

Radiotherapie bei Desmoid-Tumoren

Therapieansprechen, lokale Kontrolle und Lokalrezidivauswertung

Zusammenfassung

Hintergrund

Desmoide (aggressive Fibromatosen) sind seltene Weichteiltumore der muskulären Membranen von Kopf, Hals, Extremitäten und Bauchwand. Ziel war es, die Wirksamkeit der Strahlentherapie bei aggressiver Fibromatose an einer einzelnen Klinik zu untersuchen.

Patienten und Methode

Ausgewertet wurden 41 Patienten mit aggressiver Fibromatose, die zwischen 1987 und 2012 mit 49 Strahlentherapien behandelt wurden. Das Ansprechen wurde anhand der radiologischen Aufnahmen neu beurteilt und der Behandlungserfolg gemäß RECIST-1.1-Kriterien registriert. Für Patienten mit Lokalrezidiv wurde die Dosisverteilung der Radiotherapie durch Fusion von diagnostischen CT- und MRT-Bildern mit den Planungs-CT-Aufnahmen für jedes Lokalrezidiv bestimmt. Die Rezidive wurden je nach Lage eingeteilt in: innerhalb, am Rande und außerhalb des ehemaligen Bestrahlungsvolumens gelegen. Prognostische Faktoren für ein Therapieversagen wurden ausgewertet.

Ergebnisse

Die Gesamtdosen der Strahlentherapie lagen zwischen 20 und 63 Gy (Median 50 Gy) mit einer medianen Fraktionierung von 2 Gy. Die objektive Ansprechrate war 55 % (12/22 Patienten). Die mediane Zeit bis zum Therapieerfolg betrug 14 Monate. Eine statistisch signifikante Dosis-Wirkungs-Beziehung für die definitive und die postoperative Strahlentherapie wurde sowohl in der univariaten (p-Wert 0,002) als auch in der für potentielle Störfaktoren korrigierenden multivariaten Analyse (p-Wert 0,02) beobachtet. Neun von 11 (82 %) Lokalrezidiven wurden als Feldrandrezidive und zwei von 11 (18 %) als innerhalb des Bestrahlungsfelds liegende Rezidive bewertet. Kein Lokalrezidiv trat komplett außerhalb des Bestrahlungsfelds auf.

Schlussfolgerung

Die Strahlentherapie ist eine wertvolle Therapiewahl zur Behandlung aggressiver Fibromatosen. Die Strahlentherapiedosis hat einen signifikanten Einfluss auf die lokale Kontrolle.

Schlüsselwörter

Aggressive Fibromatose Radiotherapie Lokalrezidiv Dosis-Wirkungs-Beziehung, Bestrahlung 

Notes

Acknowledgements

Finska Läkaresällskapet supported the corresponding author and the study was supported from the EVO funds. We thank Annette Beule, MD PhD and Claudia Lundgren, MD for writing and editing the German abstract.

Compliance with ethical guidelines

Conflict of interest

K. Santti, A. Beule, L. Tuomikoski, M. Rönty, A.-S. Jääskeläinen, K. Saarilahti, H. Ihalainen, M. Tarkkanen, and C. Blomqvist declare that they have no competing interests.

Ethical standards

This article does not contain any studies with human participants or animals performed by any of the authors.

Supplementary material

66_2016_1091_MOESM1_ESM.docx (36 kb)
Supplementary data

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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Kirsi Santti
    • 1
    Email author
  • Annette Beule
    • 1
  • Laura Tuomikoski
    • 1
  • Mikko Rönty
    • 2
  • Anna-Stina Jääskeläinen
    • 1
  • Kauko Saarilahti
    • 1
  • Hanna Ihalainen
    • 3
  • Maija Tarkkanen
    • 1
  • Carl Blomqvist
    • 1
  1. 1.Comprehensive Cancer CenterHelsinki University Hospital and University of HelsinkiHelsinkiFinland
  2. 2.Department of PathologyHUSLAB and University of HelsinkiHelsinkiFinland
  3. 3.Department of Plastic SurgeryHelsinki University Hospital and University of HelsinkiHelsinkiFinland

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