| Literature DB >> 22791954 |
Francesca De Felice1, Daniela Musio, Rossella Caiazzo, Bartolomeo Dipalma, Lavinia Grapulin, Camilla Proietti Semproni, Vincenzo Tombolini.
Abstract
A desmoid tumor, also known as aggressive fibromatosis, is a rare benign neoplasm that arises from fascial or musculoaponeurotic tissues. It can occur in any anatomical location, most commonly the abdominal wall, shoulder girdle and retroperitoneum. The typical clinical presentation is a painless mass with a slow and progressive invasion of contiguous structures. It is associated with a high local recurrence rate after resection. Many issues regarding the optimal treatment of desmoid tumors remain controversial. Aggressive surgical resection with a wide margin (2-3 cm) remains the gold standard treatment with regard to preserving quality of life. Radiotherapy alone has been shown to be effective for the control of unresectable or recurrent lesions. Desmoid tumors tend to be locally infiltrative, therefore, the fields must be generous to prevent marginal recurrence. The radiation dose appropriate for treating desmoid tumors remains controversial. We present a 25-year-old Caucasian man with local recurrence of a desmoid tumor after repeated surgical resection, treated with radiotherapy. The patient achieved complete tumor regression at 4 mo after radiotherapy, and he is clinically free of disease at 12 mo after the end of treatment, with an acceptable quality of life. The patient developed short bowel syndrome as a complication of second surgical resection. Consequently, radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.Entities:
Keywords: Aggressive fibromatosis; Desmoid tumor; Fatty liver
Mesh:
Year: 2012 PMID: 22791954 PMCID: PMC3386332 DOI: 10.3748/wjg.v18.i24.3173
Source DB: PubMed Journal: World J Gastroenterol ISSN: 1007-9327 Impact factor: 5.742