Treatment and follow-up strategies in desmoid tumours: a practice guideline.

We set out to determine the optimal treatment options-surgery, radiation therapy (rt), systemic therapy, or any combinations thereof-for patients with desmoid tumours once the decision to undergo active treatment has been made (that is, monitoring and observation have been determined to be inadequate).provide clinical-expert consensus opinions on follow-up strategies in patients with desmoid tumours after primary interventional management. Surgery with or without rt can be a reasonable treatment option for patients with desmoid tumours whose surgical morbidity is deemed to be low.The decision about whether rt should be offered in conjunction with surgery should be made by clinicians and patients after weighing the potential benefit of improved local control against the potential harms and toxicity associated with rt. Depending on individual patient preferences, systemic therapy alone or rt alone might also be reasonable treatment options, regardless of whether the desmoid tumours are deemed to be resectable.

Patients referred to the soft tissue tumour group at Helsinki University Central Hospital (HUCH) for a desmoid tumour (primary or recurred) during 1987-2007 and receiving surgical treatment with or without adjuvant treatment were included in this retrospective review. All locations and also patients with a FAP-associated tumour were included. Extra-abdominal location showed lower local control despite the fact that 27% of patients also received radiation therapy. In this review, the occurrence (14%) of concurrent neoplasms was higher than expected with unusual tumour types noted including two GISTs. In those patients in whom surgical treatment is chosen, adjuvant radiation therapy should also be considered in order to decrease morbidity from aggressive surgery aiming at R0 resection. Further studies are suggested to illuminate the biological association between the desmoid tumour and other neoplasms.

The case of a 56-year-old male with a desmoid tumor 1 year after surgical removal of a gastrointestinal stromal tumor near the ligament of Treitz is described. Although he received adjuvant therapy of imatinib mesylate for 1 year after the surgery, a small nodule was found 1 year and 3 months after the surgery. The nodule was considered to be a recurrent tumor of gastrointestinal stromal tumor, and a wedge resection of the duodenum including the tumor was done. Histopathological examination by immunohistochemical staining revealed that it was a desmoid tumor. It would be useful to be aware of the possibility of desmoid tumor after removal of gastrointestinal stromal tumor. To accumulate further information on these rare diseases, a central database that includes rare diseases will be necessary.

The objective of this study was to examine the clincopathologic characteristics and outcomes of familial adenomatous polyposis (FAP) patients with and without desmoid tumors (DTs), including the risk factors for progression of FAP-related Dts. In univariate analysis, DT diagnosis before or simultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant risk factor for tumor progression at final follow-up.

The phase II “Desminib trial” included 40 patients with AF to evaluate the toxicity and efficacy of imatinib resulting in a 65% tumor control rate at 1 year. We investigated a potential predictive value of KIT exon 10 M541L variant (KITL541) on this prospective series.

The patient was a seven-year old boy with a large mandibular mass growing over a period of six months. His CT-scan showed a large mass, 13 cm in diameter in the cheek area extending to the neck and trachea. Biopsy was compatible with desmoid fibromatosis. Despite rarity desmoid tumors should be kept in mind of an otorhinolaryngologist as a differential diagnosis in children with head and neck mass.