The tumor was obtained, leaving unharmed the neural elements as well as the large vessels of this area. The excised tumor, measuring10 cm ?8 cm ?4.5 cm, had well defined smooth, round borders and was relatively firm (Figure 2). Histological examination of the mass revealed the presence of elongated spindle-shaped cells, surrounded by abundant collagen, features consistent with fibromatosis. Immunostaining was positive for desmin, vimentin, non-specfic enolase and b-catenin and negative for acinin and S-100. TheFigure 2. (A) Gross total resection of the tumor. (B) Macroscopic Staurosporine appearance of the excised mass.Page 2 of(page number not for citation purposes)Cases Journal 2009, 2:http://casesjournal.com/casesjournal/article/view/patient had an uneventful postoperative course and was discharged on the 4th postoperative day. During his followup MRI study, 6 months after his operation, no evidence of tumor recurrence were detected.DiscussionDesmoid tumors are rare fibroblastic tumors that are characterized by the presence of proliferating normalappearing fibroblastic cells in an abundant collagen stroma. There is a female predominance while the highest incidence arises among patients between the ages of 15 to 60 years old [5]. Desmoids arise sporadically, nevertheless in an incidence ranging from 3 to 35 these tumors can occur in patients with familial adenomatous polyposis [6,7]. Desmoid fibromatosis may occur at abdominal, intraabdominal and extraabdominal locations. Extraabdominal fibromatosis most frequently occurs in the limbs, followed by the head and neck region [8]. Although these tumors are benign their clinical behavior is unpredictable. Rock et al, in a series of 194 patients with extra-abdominal desmoid tumors, report a 68 recurrence rate PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/6833145 at an average of 1.4 years after the first treatment [9]. Such an aggressive behavior combined with the presence of many vital structures in a confined area in the head and neck, consists of a major therapeutic challenge. At present the management of desmoid tumors includes the use of surgery, radiation therapy (RT) as well as cytotoxic and noncytotoxic chemotherapy. Given the benign nature of these tumors, treatment strategies aim at achieving local control while preserving proper function and providing with an adequate cosmetic result [4]. When medically and technically feasible, complete resection of the tumor with negative microscopic margins is the firstline treatment [10]. Nevertheless, it must be noted that the importance of positive margin status to local recurrence rates, is still a matter of debate. In contrast to other published series [2,10], a study by Gronchi et al showed that presence of microscopic disease did not affect longterm disease-free survival in patients with primary presentation of extra-abdominal desmoid tumors [11]. Primary RT is an appropriate alternative for patients who are not good surgical candidates [12]. On the other hand the use of postoperative RT is unclear. After taking under consideration the controversy regarding the significance of positive resection margins and the potential for late radiation toxicity, particularly in younger patients, some researchers advocate that RT should not be pursued in patients with primary disease and either negative or positive surgical margins, but only in cases with gross residual disease [13,14]. In agreement with this approach our patient did not receive adjuvant RT. Systemic therapy includes a variety of noncytotoxic (ta.