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


CONCLUSIONS
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.
The Netherlands Foundation for the Detection of Hereditary Tumours, Rijnsburgerweg 10, Poortgebouw Zuid, 2333 AA Leiden, The Netherlands. m.nieuwenhuis@stoet.nl
M H
MH
Nieuwenhuis
E M
EM
Mathus-Vliegen
C G
CG
Baeten
F M
FM
Nagengast
J
J
van der Bijl
A D
AD
van Dalsen
J H
JH
Kleibeuker
E
E
Dekker
A M
AM
Langers
J
J
Vecht
F T
FT
Peters
R
R
van Dam
W G
WG
van Gemert
W N
WN
Stuifbergen
W R
WR
Schouten
H
H
Gelderblom
H F A
HF
Vasen
D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000628:therapy*; D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003082:Colectomy; D003131:Combined Modality Therapy; D005260:Female; D018221:Fibromatosis, Abdominal / Q000150:complications / Q000628:therapy*; D018222:Desmoid Tumors / Q000150:complications / Q000628:therapy*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009426:Netherlands; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
D000970:Antineoplastic Agents