Desmoid Tumors in Children

Abstract

Background/Objectives: Desmoid tumors (DT) are locally aggressive tumors with no known potential for metastasis. However, tumor invasion into vital structures can result in substantial morbidity and may be even fatal. DT are account for about 0.03 percent of all neoplasms and less than 3 percent of all soft tissue tumors. Clinical behavior and recommended treatments are often dictated by anatomic site. Design/Methods: Long-term results of treatment in Pediatric DT patients (pt) were evaluated in the Republic of Belarus from 1996 to 2013. A total of 40 children (boys-23, girls-17) with median age of 7,7 years (range 1-16) were treated and 39 of them underwent tumor excision and 1 -tumor biopsy. Tumor sites were: pelvic area- in 18 pt, lower extremity (ex)-8, upper ex-2, abdomen-5, head & neck-3 and others-4. Radiotherapy was given in 14 patients including 6, who received radiotherapy with local electromagnetic hyperthermia. Chemotherapy (usually vinblastin+metotrexate) was administrated in 10 cases, 6 of them additionally received tamoxifen. Tamoxifen without chemotherapy was given in 4 patients as well. Results: In 14 pts tumor relapsed, particularly in 5 pts two and more times during 12 months after wide excision. Conservative treatment curried out in 10 pts with non operable relapsed tumors. PR registered in 1 pt, SD- 8 and PD-1 with Gardner syndrome who died. 39 pts are alive. Ten years overall and relapse-free survival rate were 94 % and 49% respectively (follow up 74 months). Conclusion: Our results based on considerable number of patients suggest that radical surgical excision is the method of choice in the treatment of DT patients. Additional chemo, radio as well as hormone therapy could be used in locally advanced and recurrent forms of disease.