a 33 year old healthy male of afro - caribbean origin presented to the emergency department with acute generalised abdominal pain of three hours duration .
he had not noticed any recent change in bowel habit , bleeding per rectum or any systemic symptoms .
it was of note that there was no family history of inflammatory bowel disease or gastrointestinal malignancy .
he was a non - smoker and did not consume alcohol . on examination he had a heart rate of 110 beats per minute , temperature of 37.2c , stable blood pressure but he was cold and clammy .
bowel sounds were absent and rectal exam revealed an empty rectum and no palpable masses .
blood tests revealed a neutrophilia of 25,000 but urea and electrolytes were within normal range .
he was resuscitated with intravenous fluids and an emergency laparotomy was performed . during the laparotomy , a 0.5 cm perforation was noted in the ileum 25 cm proximal to the ileocaecal valve on the mesenteric border .
an inflammatory mass measuring 5  3 cm was noted directly adjacent to the perforation in the mesentry .
30 cm of small bowel was resected with a right hemicolectomy , hence removing the mass and the perforation .
no obvious cause of sepsis was identified and it was thought that he may have a subphrenic collection . therefore a computed tomography ( ct ) scan was arranged .
however , he had a severe reaction to the intravenous contrast medium which resulted in hypotension .
the patient underwent a second laparotomy to drain the subphrenic abscess and no masses were seen during this procedure .
histological examination of the resected specimen from the first laporotomy revealed mesenteric fibromatosis ( desmoid tumour ) incompletely excised .
the resected right hemi - colon did not show evidence of dysplasia , malignancy or polyposis .
the estrogen receptor status was not reported . due to continuing abdominal discomfort on the ward after the second laparotomy , an ultrasound scan was arranged that revealed the following : a rather ill - defined , fairly ovoid , inhomogeneously - hypoechoic mass lesion is identified in the lower abdomen ( infra- umbilical ) para - sagittal plane .
it measures about 5  3  4.5 cm in maximum dimensions surrounded by excess peritoneal fat and some bowel loops representing a residual of a previously resected abdominal desmoid .
the pain eventually resolved and after making a good recovery , he was commenced on oral tamoxifen , 20 mg once a day .
although estrogen receptor status was not reported , the patient was commenced on this therapy as it was assumed that most desmoid tumours have estrogen receptors .
he was subsequently referred to a specialist in a distant tertiary unit for further evaluation of the residual tumour and for the prospect of further elective surgery .
a 33 year old healthy male of afro - caribbean origin presented to the emergency department with acute generalised abdominal pain of three hours duration .
he had not noticed any recent change in bowel habit , bleeding per rectum or any systemic symptoms .
it was of note that there was no family history of inflammatory bowel disease or gastrointestinal malignancy .
he was a non - smoker and did not consume alcohol . on examination he had a heart rate of 110 beats per minute , temperature of 37.2c , stable blood pressure but he was cold and clammy .
bowel sounds were absent and rectal exam revealed an empty rectum and no palpable masses .
blood tests revealed a neutrophilia of 25,000 but urea and electrolytes were within normal range .
he was resuscitated with intravenous fluids and an emergency laparotomy was performed . during the laparotomy , a 0.5 cm perforation was noted in the ileum 25 cm proximal to the ileocaecal valve on the mesenteric border .
an inflammatory mass measuring 5  3 cm was noted directly adjacent to the perforation in the mesentry .
30 cm of small bowel was resected with a right hemicolectomy , hence removing the mass and the perforation .
no obvious cause of sepsis was identified and it was thought that he may have a subphrenic collection . therefore a computed tomography ( ct ) scan was arranged .
however , he had a severe reaction to the intravenous contrast medium which resulted in hypotension .
the patient underwent a second laparotomy to drain the subphrenic abscess and no masses were seen during this procedure .
histological examination of the resected specimen from the first laporotomy revealed mesenteric fibromatosis ( desmoid tumour ) incompletely excised .
the resected right hemi - colon did not show evidence of dysplasia , malignancy or polyposis .
due to continuing abdominal discomfort on the ward after the second laparotomy , an ultrasound scan was arranged that revealed the following : a rather ill - defined , fairly ovoid , inhomogeneously - hypoechoic mass lesion is identified in the lower abdomen ( infra- umbilical ) para - sagittal plane .
it measures about 5  3  4.5 cm in maximum dimensions surrounded by excess peritoneal fat and some bowel loops representing a residual of a previously resected abdominal desmoid .
the pain eventually resolved and after making a good recovery , he was commenced on oral tamoxifen , 20 mg once a day .
although estrogen receptor status was not reported , the patient was commenced on this therapy as it was assumed that most desmoid tumours have estrogen receptors .
he was subsequently referred to a specialist in a distant tertiary unit for further evaluation of the residual tumour and for the prospect of further elective surgery .
desmoid tumours ( fibromatosis ) are benign fibrous neoplasms originating from the musculo - aponeurotic structures throughout the body .
desmoid tumours can arise from any skeletal muscle but commonly affect the rectus abdominus in post - partum females and in old surgical scars of the abdomen .
peripheral tumours are smooth , firm and mobile . they are adherent to the surrounding structures
gardner s syndrome and familial adenomatous polyposis coli ( fap ) should be suspected in patients with such soft tissue growths ( 3 ) .
an intra - abdominal desmoid , also known as mesenteric fibromatosis , is the most common solid primary neoplasm of the mesentry .
approximately 80% of intra - abdominal desmoids involve small bowel mesentry , as was the case with our patient .
involvement of the transverse mesocolon , retroperitoneum , omentum and the ligamentum teres have also been reported ( 1 , 4 ) .
intra - abdominal desmoids are usually asymptomatic until their growth and infiltration causes compression of the viscera .
this can lead to intestinal obstruction , ischemic bowel secondary to vascular compression and hydronephrosis due to ureteric compression .
bowel perforation is extremely rare and my research has yielded one previous case report ( 1 ) .
other rare manifestations reported in the literature include deep vein thrombosis , pyrexia of unknown origin , gastrointestinal bleeding and intra - abdominal abscess formation ( 5 , 6 ) .
the subphrenic abscess that our patient developed is very likely to have been a complication of the initial laparotomy .
histologically , these tumours are composed of collagen that surrounds spindle cells which are poorly circumscribed ( figure 12 ) .
exact etiology is uncertain but hormonal factors are implicated as there are estrogen eceptors present in some desmoid tumours .
the adenomatous polyposis coli ( apc ) gene located on chromosome 5 is responsible for familial adenomatous polypsois coli ( fap ) .
biallelic mutations of the apc gene induces desmoid tumour formation , hence the association between these two disorders ( 8) . in our patient , there was no polyposis in the resected right hemi - colon .
trauma has also been suggested as another causative factor but there was no such history in our patient ( 1 ) .
both ct and magnetic resonance imaging ( mri ) are useful in diagnosing and monitoring recurrence .
they allow accurate staging and also enable one to delineate accurate anatomy prior to embarking upon major resection .
mri has the advantage of defining the extent of involvement and monitoring post operative recurrence , which can be as high as 70% ( 6 ) .
these patients should also undergo colonoscopy and examination of the eye to exclude gardner s syndrome .
colonoscopy may reveal multiple colonic polyps and fundoscopy may demonstrate multiple pigmented lesions affecting the fundus of the eye ( 2 , 3 ) . complete surgical excision may be the only effective way of providing a cure but complete excision is often impossible and therefore adjuvant treatments have been employed with various degrees of success ( 1 , 2 ) .
other treatment modalities include radiotherapy , if there is recurrence or as primary treatment to avoid radical surgery ( 9 ) .
anti - estrogens ( e.g. tamoxifen ) , prostaglandin inhibitors and non - steroidal anti - inflammatory drugs ( nsaids ) have also been used .
cytotoxic chemotherapy may be useful in those with disease recurrence or where surgery is contraindicated ( 10 ) .
desmoid tumours originate from the musculo - aponeurotic structures of the body and can be peripheral or intra - abdominal .
patients should be screened for gardner s syndrome and familial adenomatous polyposis coli since these can often present with dermatoid , epidermoid or other benign tumours .
emergency presentations are not common , but are usually secondary to mass effect on the viscera .
ct and mri are the imaging modalities of choice , particularly if surgery is contemplated and also in postoperative follow up to detect recurrence .
surgery is usually the mainstay of treatment but recurrence is common and adjuvant therapy is used .
some tumours possess estrogen receptors , hence they regress in response to anti - estrogens , such as tamoxifen .
 desmoid tumours are benign tumours originating from the musculoaponeurotic structures of the body . 
 they are mainly composed of collagen . 
 these tumours commonly occur in post - partum women in whom they originate from the rectus abominus and in old surgical incisions . here 
 we present a case report of a young gentleman who presented with an acute surgical abdomen and subsequently underwent a laparotomy and was found to have an inflammatory mass . 
 histological analysis of this mass revealed mesenteric fibromatosis ( desmoid tumour ) . 
 i wish to present this interesting case because the patient was neither female nor someone who had previously undergone any surgery . 
 it is rare for mesenteric fibromatosis to present with intestinal perforation and only one case been reported in the literature thus far ( 1 ) . an important learning point from this case 
 is that rare pathology can manifest itself with common signs and symptoms . 
CASE
History & examination
Investigations
Treatment
Post-operative course
DISCUSSION
CONCLUSION