Abstract | BACKGROUND: METHODS: Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids. RESULTS: Median follow-up was 8 years. For intra-abdominal desmoids (n=62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P=0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti- estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy. CONCLUSION: For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin.
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Authors | M H Nieuwenhuis, E M Mathus-Vliegen, C G Baeten, F M Nagengast, J van der Bijl, A D van Dalsen, J H Kleibeuker, E Dekker, A M Langers, J Vecht, F T Peters, R van Dam, W G van Gemert, W N Stuifbergen, W R Schouten, H Gelderblom, H F A Vasen |
Journal | British journal of cancer
(Br J Cancer)
Vol. 104
Issue 1
Pg. 37-42
(Jan 04 2011)
ISSN: 1532-1827 [Electronic] England |
PMID | 21063417
(Publication Type: Evaluation Study, Journal Article)
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Chemical References |
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Topics |
- Adenomatous Polyposis Coli
(complications, therapy)
- Adolescent
- Adult
- Antineoplastic Agents
(therapeutic use)
- Colectomy
- Combined Modality Therapy
- Female
- Fibromatosis, Abdominal
(complications, therapy)
- Fibromatosis, Aggressive
(complications, therapy)
- Humans
- Incidence
- Male
- Middle Aged
- Netherlands
- Radiotherapy Dosage
- Retrospective Studies
- Survival Rate
- Treatment Outcome
- Young Adult
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