Sporadic Desmoid-Type Fibromatosis Occurring at Laparoscopic Trocar Site: An Unusual Entity

腹腔镜套管穿刺部位散发性韧带样纤维瘤病:一种罕见疾病

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Abstract

Desmoid-type fibromatosis (DF) or desmoid tumours are rare benign soft tissue tumours of musculo-aponeurotic origin, which have no malignant potential but are locally aggressive and can result in significant morbidity. They may be hereditary (in association with FAP, i.e. familial adenomatous polyposis syndrome resulting from APC gene mutation) or sporadic, with hereditary cases having a much higher risk of developing DF compared to sporadic ones. Desmoids are known to occur in previous surgical scars. However, previous laparoscopic port site/trocar site is an extremely uncommon site for sporadic desmoids, with only two cases of sporadic laparoscopic trocar site desmoids (occurring in absence of FAP) reported in world literature. We thus describe a case of sporadic DF, occurring at the site of umbilical port 8 months following laparoscopic cholecystectomy for cholelithiasis, treated successfully by margin-negative resection. To the best of our knowledge, this appears to be the first such case in Indian literature and the first being reported after laparoscopic cholecystectomy. In the era of laparoscopic surgery, one must be aware of the existence of such an entity after laparoscopy as it may be confused with conditions such as metastatic deposit, port site tumour recurrence, port site hernia, scar site endometriosis, soft tissue sarcoma etc. and may result in diagnostic dilemma.

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