[Intra-abdominal desmoid tumors: rare but important disease]

Desmoid tumors are rare benign neoplasms with high tendency to local recurrence, and they can be divided into extra- and intra-abdominal types (mesenteric fibromatosis). Eight cases have been treated in our Department from 1997 to 2006. Six patients (3 men and 3 women) affected by extra-abdominal desmoid tumors have been treated with radical excision. In two patients desmoid was intra-abdominal: 1) a 55 years old man admitted for acute abdomen and submitted, in emergency, to a laparotomy with excision of a mesenteric abscess including a jejunal loop at about one meter from Treitz; 2) 52 years old man, submitted to an elective excision of a capsulated neoplasm of the little omentum, which had caused an oppressive abdominal pain. In both cases the hystological diagnosis has been desmoid tumor. Surgical treatment of desmoid tumors must aim at radical excision to avoid frequent recurrences (25-65%); these have stimulated the research of other kinds of treatments, since a new surgical operation itself can lead to a further recurrence. Radiotherapy has been investigated with results in 79-96% of cases, antiestrogenic therapy has been used with success in 51% of patients, and high dose tamoxifen seemed to obtain a stable disease in non operable cases. Non steroidal anti-inflammatory drugs have been experimented in association with tamoxifen and chemotherapy. Conclusive results on the efficacy of these treatments have not been obtained yet, because of the rarity of the desmoid tumors even in greater Centres.