๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
Desmoid fibromatosis
Desmoid fibromatosis
Clinical features Desmoid fibromatosis represents a group of deep-seated fibrous neoplasms which can present in a variety of clinical settings.1โ5 Although most cases are sporadic and solitary, some can be familial or associated with familial adenomatous polyposis (FAP, Gardner syndrome) and rare examples can be multicentric.6,7 A further subgroup presents in children.8 According to their anatomical distribution, desmoid tumors are classified into:
โข extra-abdominal (around 60% of cases),
โข abdominal (20โ25%),
โข intra-abdominal (15%). All of these anatomical subsets typically occur between the second and fourth decades of life with a predilection for females. Extra-abdominal fibromatoses are often sporadic and solitary.1,2 Most patients are young adults presenting with a slowly growing mass that may occasionally be painful. Tumors arise most often around the shoulder girdle or on the proximal lower limbs and rarely in the head and neck area, the latter group being commonest in children.8 Subcutaneous involvement is an occasional feature. A small proportion of tumors arise in association with a previous scar or post radiotherapy. Desmoid tumors arising in the anterior abdominal wall are particularly common in females, especially during or after pregnancy.3,5 Often, those arising after pregnancy appear at the site of the scar from a cesarean section. Many cases associated with FAP are intra-abdominal. Local recurrence is common even when excision is believed complete.9,10