Our Experience with Desmoid Tumors

Abstract

Aim: Desmoid tumors,which originate from musculoaponeurotic tissue, progress locally and agresively, but they do not make distant metastasis. Its frequency rises up with polyposis coli syndrome. In this article, we aim to evaluate the results of the patients treated for desmoid tumors. Method: Fifteen patients, who were diagnosed with desmoid tumors between 2000-2013, were evaluated in terms of demographical and clinical data, surgical interventions, complication and long terms folllow-up. Results: : The avarage age of patients was 35 (20-51) and 9 (%60) of them were women. Thirteen patients underwent colorectal surgery because of polyposis syndrome [6 (%40) restorative proctocolectomy, 5 (%33) total colectomy and ileorectal anastomosis]. Two patients were inoperable at the time of diagnosis. The mean time of occuring desmoid tumor after the surgery for polyposis coli were 24,5 (4-48) months. The most common location of tumor was intra-abdomen with 11 cases. Eight (%54) patients were evaluated as inoperable, whereas 7 patient underwent surgical resection, but all of them had recurrence in their follow-up. Mortality rate was found 74% (n=11) and the survival was 79,7 months (5-312). One patient was performed multivisceral organ transplantation. Conclusion: After performing proflactic colectomy/proctocolectomy in early decades, desmoid tumor has been becoming the most common reason of the mortality in polyposis syndrome. At the time of diagnosis, most patients are inoperable because of the invasion to adjacent organs espacially small-bowel meso. Although multivisceral organ transplantation is one of the most complicated surgical procedure, it seems a convenient treatment option for desmoid tumor patients.

Keywords

• Desmoid tumors
• Polypozis Coly
• Multivisceral Organ Transplantation