Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients.

Abstract

BACKGROUND:

The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients.

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.

• 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)

Chemical References

• Antineoplastic Agents

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