Aggressive fibromatosis of the head and neck (desmoid tumours)

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...  

Fibroid is a benign lump of growth, occurring within or outside the uterus. It is a rarity for such growths to occur intra-abdominally. Such cases are of aggressive fibromatosis which are marked by the presence of desmoid tumours. It is most commonly seen in patients with a history of familial adenomatous polyposis (FAP) or past surgical procedures. As FAP has a wide fibroepithelial growth spectrum, the symptomatology and therefore the prognosis varies. Unpredictable clinical behaviour, varied location and non-specific presentation are the factors accounting to diagnostic difficulty. Therefore, misdiagnosing the disease is not uncommon. Here we report a case ofa26-year-old pregnant female presenting with lower abdominal pain and mass. Preoperative diagnosis was of uterine fibroids as per radiological evidence, but on exploration it turned out to be a large right colonic mass. The patient underwent right hemicolectomy. Histopathological reports confirmed morphological and immuno-histochemical features indicating fibromatosis. This fibromatous mass though intra-abdominal was not growing from the mesentery/mesocolon or the pelvis. It appeared to grow from the wall of the colon. This is a confirmatory presentation of true colonic wall fibromatosis which is deep and isolated form of intra-abdominal desmoid tumours. As this lesion was isolated in nature, the treatment of choice was surgical resection. It is essential to have a multi-disciplinary team approach in the management of such a patient. This improves the treatment and the prognostic outcomes. Keywords: Fibroid, Colon, Fibromatosis, Hemicolectomy, Desmoid Tumour


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