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Paper text:
desmoid tumours comprise 0.03% of all neoplasms , less than 3% of all soft tissue tumours ( 1 ) , with an incidence of 2 - 4 cases / 1000000 per year ( 2 ) .
they are usually detected relatively early on in life ( 10 - 40 years of age ) , with a slight female predominance . while normally occurring sporadically , they are more common in patients with familial adenomatous polyposis ( fap ) , where the incidence is 850 times higher than in the general population ( 3 ) .
most desmoid tumours typically tend to grow slowly and do not metastasise , although their growth pattern remains quite aggressive , with organ infiltration , local tissue destruction and organ compression / displacement .
the tumours often grow without causing any clinical symptoms and are therefore usually only detected relatively late or during a check - up for another ailment , when they may have already reached substantial sizes of 15 cm in diameter or more .
during a routine check - up for hypertension , the 43-year old female patient s general practitioner noted a painless mass in the abdomen .
the patient reported increased fatigue and a reduced overall performance for the previous two years . further questioning revealed occasional dyspepsia and an increased urge to urinate , although micturition was otherwise without any associated discomfort .
an abdominal computed tomography revealed a very large , well - defined intra - abdominal mass , resulting in the displacement of most intra - abdominal organs to the right of the patient s abdominal cavity ( fig .
1 ) . the abdominal ct scan reveals a large tumour , resulting in the displacement of the intra - abdominal organs to the right of the abdominal cavity .
radiological evaluation did not make it possible to tell from which organ the tumour originated .
intra - operative findings revealed a tumour spanning from the symphysis to the xyphoid ( fig .
2 ) and showed multiple adherences between the omentum and the caudal pole of the spleen . in order to achieve an r0-resection , a partial splenectomy and partial removal of the omentum was necessary .
diagnostic imaging includes abdominal sonography , computed tomography or magnetic resonance imaging ( mri ) .
mri usually is the examination of choice as it provides the surgeon with a better evaluation of exact tumour size , location and infiltration in to the surrounding tissues with tumour recurrences being more easily detected . upon diagnosis of a desmoid tumour
histologically , desmoid tumours are benign and characterised by a monoclonal fibroblastic proliferation typically arising from muscular or aponeurotic structures .
although upon initial inspection an intact capsule seems to surround the periphery , the tumour characteristically extends beyond this pseudo - capsule .
tumours are classified as intra- or extra - abdominal , with extra - abdominal tumours most often located around the neck and shoulders , the pelvis , or within the thoracic wall .
sporadic desmoid tumours are mainly located extra - abdominal and are relatively easy to resect .
high recurrence rates and difficult locations make treatment of the intra - abdominal sub - group particularly difficult .
treatment of desmoid tumours is challenging and the optimal therapeutic strategy remains a matter of debate . while spontaneous tumour regression has been observed , usually in menopausal women or post- oophorectomy , suggesting a certain estrogen - dependency ( 4,5 ) , rapid growing tumours require an aggressive therapy if cure is to be sought . their high recurrence rate and
their ability and tendency to infiltrate vital organs and structures can result in difficult and highly complex operations .
treatment for small , non - infiltrating tumours can be delayed in completely asymptomatic patients and / or in patients unfit for surgery , as the tumours can remain stable over years without any significant morbidity
. however , these patients require routine radiological imaging to exclude rapid growth or new organ infiltration .
surgery remains the main therapeutic option , with complete tumour resection providing the only potentially curative treatment .
although , as with classical cancer surgery , the aim is to achieve an r0 resection margin , surgery should nevertheless be kept to the required minimum , avoiding any mutilating intervention .
if resected early on , the removal of abdominal - wall desmoid tumours is associated with a low morbidity and mortality .
overall recurrence rates lie by 20% to 75% ( 6,7 ) and are more frequent for the rarer extra - abdominal tumours .
surgical interventions for intra - abdominal tumours are often associated with a high mortality and morbidity .
many are found at the root of the small bowel mesenterium and up to 65% of the tumours are unresectable upon intraoperative inspection ( 8) . there is little evidence for the benefit of pharmacological therapies , which include the use of non - steroidal anti - inflammatory drugs , such as sulindac or anti - estrogens such as tamoxifen ( 9 ) . the aim is tumour regression / growth stop and it can be used as a first - line treatment for multiple , intra - abdominal tumours .
the high recurrence rate can be reduced by adjuvant and / or neoadjuvant radiotherapy ( 10 ) both for abdominal wall and extra - abdominal tumours .
the usefulness of alternative therapies , such as radio frequency ablation is currently under scrutiny .
other options include placement of a percutaneous catheter for use in chemical ablation using acids .
desmoid tumours are rare and are either found sporadically or more commonly associated with fap . a classification into abdominal wall tumours as well as extra- and intra - abdominal tumours is important with regard to further treatment and outcome .
however , symptomatic tumours , especially if located intra - abdominal , should be treated aggressively .
surgery still remains the treatment of choice , although recurrence rates are high , even after r0 resection .
desmoid tumours are a rare entity and are usually detected with a certain delay , as they initially often grow without causing any specific clinical symptoms . 
 although these tumours grow slowly and do not metastasise , their growth pattern remains aggressive and local tissue displacement or even destruction is common . 
 surgery remains the only potentially curative treatment option . 
 here we report on the findings of a 43-year old female patient with a large intra - abdominal desmoid tumour , resulting in the displacement of most intra - abdominal organs to the right of the patient s abdominal cavity . 
 she successfully underwent surgical removal of the mass and recovered without complications .