Pediatric desmoid tumor: retrospective analysis of 63 cases.

Abstract

A surgical approach aiming at clear margins is presently the best treatment option. When this cannot be accomplished without severe disfigurement or function impairment, partial resection is an acceptable alternative, but one associated with a high risk of regrowth. Whether adjuvant strategies should be used in this situation remains to be addressed.

Pediatric aggressive fibromatosis

“…8,12 Including the current analysis, 85 patients underwent primary surgery and had information available regarding surgical margin status and disease recurrence (Table 4). 5,6,8,10,12 The recurrence rate among patients with SN margins was 16%, compared with 67% among patients with SP margins. In patients with SP margins, 74% of those who did not receive additional therapy developed recurrent disease, compared with 40% of patients who received adjuvant treatment.…”

“…None of the pediatric AF studies reported patients with familial history of AF or FAP, and only two patients with Gardner syndrome were reported. 6 In 3 studies of pediatric AF, 20% of patients (n ϭ 108 patients) had a history of local trauma before they developed AF. 5,6,8 Seven of 10 pediatric AF studies, including the current investigation, reported treatment of the primary tumor, and all patients generally underwent initial surgery (n ϭ 168 patients) ( Table 3).…”

“…5,6,8 Seven of 10 pediatric AF studies, including the current investigation, reported treatment of the primary tumor, and all patients generally underwent initial surgery (n ϭ 168 patients) ( Table 3). [5][6][7][8]10,12 The other 3 series reported treatment of recurrent tumors, including 2 series in which patients initially were ; n ϭ 15), 9,11 and a third study in which radiotherapy was administered (n ϭ 4 patients). 4 The disease recurrence rate in the reviewed children with primary AF was approximately 50% (Table 3).…”

“…The majority of disease recurrences (89%), including those occurring among the patients in the current study, were observed within 3 years, and nearly all recurrences (97%) were observed within 6 years, although recurrences after 10 years have been reported. 6,8,10,12 All reported disease recurrences were local or regional with a pattern consistent with infiltrative growth. Three deaths were reported that were caused by invasive tumor destruction of vital organs; all three patients had head and neck lesions.…”

Fibromatosis in a Young Bernese Mountain Dog: Clinical, Imaging, and Histopathological Findings

“…Motor weakness is possible if the tumor infiltrates or encases large nerves. Rare cases of multicentric lesions have been described 7,9,14 ; in most cases, the second growth develops proximal to the primary lesion. Microscopically, deep fibromatosis is poorly circumscribed and infiltrates the surrounding tissue, usually striated muscle.…”