Intra-abdominal Desmoid Tumor after Resection for Gastrointestinal Stromal Tumor of the Small Intestine: Case Report.

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 (F.A.P.) 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.

Patient example: 57 year old female with a newly diagnosed desmoid which extended from C3-T2. Current standard of care would be surgical resection followed by radiation therapy. Given the size of the lesion it was felt she would need cervical stabilization and possibly a latissimus flap. The patient was referred to our service to discuss less invasive options. Myelographic dye was placed for visualization, the spinal cord was hydrodissected away from the posterior elements using and epidural catheter, and neural monitoring was used and demonstrated no changes through the case. A total of 10 probes (Galil) were used and the lesion was cryoablated for 7 min, passively thawed for 10 min and reablated for 15 min. There was no post procedure weakness, and she was discharged postprocedure day 1, back to work on day 5.

Recurrence after surgical excision of a desmoid tumor in the upper extremity is common, especially in younger patients. Adjuvant radiation therapy tended to increase time to recurrence but not rate of recurrence.