The generic term fibromatosis was proposed by Stout and Raffale for a group of related conditions having the following features in common: (1) proliferation of well differentiated fibroblasts, (2) infilterative pattern of growth, (') presence of variable (but usually abundant) amount of collagen between the proliferating cells, (4) lack of cytologic features of malignancy, scanty or absent mitotic activity and (5) aggressive clinical behaviour characterised by repeated local recurrences but without capacity to distant metastasis.
The tumour is ether a large firm mass with ill-defined borders or a diffuse plaque. The cut surface is irregularly whorled. Essentially a benign lesion, it may behave in a malignant fashion locally.
Fibromatosis can affect various parts of the body such as the anterior abdominal wall (desmoid tumour), the face, the neck (fibromatosis coli), the palms (Dupytren's contracture), the retroperitoneal tissues (retroperitoneal fibrosis), the gluteal region, the mediastinum and the abdominal cavity. When it affects children, it is called `Juvenile fibromatosis'.
A 52 year old man presented with attacks of epigastric discomfort, feeling of fullness, colicky pains and vomiting aggravated by overeating for 5 months. He was constipated and had lost weight but did not have fever, haematemesis, melena or backache.
On examination, he was well built and nourished; there was no pallor, icterus, lymphadenopathy, or any skin lesion. There was an intermittently felt, vague, nontender, mobile, intra-abdominal lump in epigastric and umbilical region. There was no ascites, The per rectal examination was normal. The other systems were normal.
Haemogram, urine and stool were normal. Blood biochemistry was normal. Plain X-ray abdomen and chest were normal. Barium meal studies showed a questionable widening of the 'C' loop of the duodenum and an upward displacement and partial volvulus of the stomach, attributable to distended colon [Fig. 1]. They also showed some dilated loops of jejunum. Hypotonic duodenography using 20 mg of intravenous hyoscine-N-butyl bromide, showed an extrinsic pressure defect on the inferior aspect of distal duodenum and the duodenojejunal flexure [Fig. 2].
While in the ward, the patient developed in testinal obstruction and an emergency laparotomy was performed.
At exploration, an obstruction to the transverse colon was detected caused by a firm irregular mobile mass, 10 x 8 x 8 cms, with well defined borders situated in the transverse mesocolon. The mass had engulfed the transverse colon towards the left side. The distal part of the duodenum was partly compressed. The vessels in the mesocolon and the superior mesenteric vessels were completely engulfed by the tumour. The mesentery of the small bowel was also adherent to the mass. The proximal transverse colon, the ascending colon and the entire small bowel were markedly distended. The stomach was pushed up and was placed transversely in the horizontal plane due to the distended colon. Diver and spleen were normal.
In view of the extensive involvement of important vessels, no attempt at excision was made. Anterior antecolic gastrojejunostomy was done to bypass the duodenal obstruction and ileotransverse colostomy was done well beyond. the colonic obstruction. Biopsy of the mass was taken which showed fibromatosis [Fig. 3]. The patient had a smooth post-operative course and is doing well at the time of publication of this report.
Fibromatosis of mesentery is uncommon, and has been only occasionally reported.
Yannapoulas and Stout reported 12 cases of mesenteric fibromatosis and in addition analysed 35 cases reported in the literature earlier. The chief complaints were abdominal mass, symptoms due to compression of organs, pain, discomfort, constipation and vomiting. Male/female ratio was 5: 7 in their series though the same ratio was not seen in other series. Age ranged from 1 year to 65 years. Of their 12 cases, one had the lesion situated in the gastrosplenic ligament. The tumour was always well circumscribed between the leaves of the mesentery. All were treated with excision, with good results. Das Gupta et al presented an analysis of extra-abdominal wall desmoids, a clinico-pathological study of 72 cases wherein they discussed all the desmoids other than those affecting anterior abdominal wall. Of 72 cases only six were involving the mesentery. Their observations were similar to those of Stout and Raffale but in addition, they noted poor vascular supply though some cases had a capillary network accompanying the proliferating fibrous tissue. At the advancing periphery some lymphocytic infiltration was noted. A few cases showed myxomatous transformation. Metaplastic bone formation was uncommon. In a case reported by Tahiliani et al there was calcification. Das Gupta et al, however, emphasised that the lesion was distinctly different from even a well differentiated fibrosarcoma.
Simon et al found 7 cases of mesenteric fibromatosis in their study of 280 patients of polyposis (with Gardner's syndrome-diffuse colonic polyposis, osteoma, sebaceous or epidermoid cysts and subcutaneous tumours). These patients were prone for intraperitoneal adhesions, particularly after an exploration; hence these workers do not advocate excision of the mass until it threatens life. They record two patients in whom a second laparotomy two years after the initial one for biopsy showed the mass to be unchanged in size.
Zayed and Dihmis reported three cases of familial fibromatosis suggesting strong hereditary predisposition.
Ackerman had a case following trauma.
Sacks et al presented two cases of isolated mesenteric fibromatosis; their second case resembled our patient. They emphasise upon certain radiological features. Plain X-ray may show soft tissue mass. Barium meal may show compression and angulation of distal duodenum. These masses also give separation, angulation, compression of the small bowel loops. Teethering of mucosa may be demonstrated rarely. Severe asymmetric involvement of the bowel will show formation of pseudo-diverticula. Barium enema may be normal or may show compression or polyposis.
Mesenteric angiography reveals displacement, separation and angulation of major and minor branches of superior mesenteric artery.
Radiologically, metastatic carcinoma, abdominal lymphoma, intestinal carcinoids, peritoneal neoplasms, post-operative adhesions, and retractile mesenteritis form the other differential diagnoses.
The ideal treatment is complete excision. When the lesion involves vital structures and resections become difficult bypass procedures have to be undertaken. Radiotherapy, chemotherapy, hormonal therapy have all proved ineffective.
Prognosis is good after complete excision. Local recurrences are known which are again to be treated by excision. When the tumour is left behind with a bypass procedure, it takes several years before it requires a second exploration. Subsequent surgery is made difficult by extensive intraperitoneal adhesions created by previous surgery. Morbidity and mortality increase with each subsequent operation. Extensive involvement. of abdominal viscera is the ultimate cause of death after a number of years.