Abdominal Fibromatosis in a Young Child: A Case Study and Review of the Literature

Abstract

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear β-catenin staining.

Lesiones ocupantes de espacio en pared abdominal (no herniaria). La visión del patólogo

“…Los tumores desmoides representan una proliferación fibroblástica y miofibroblástica agresiva con variable producción de colágeno que, frecuentemente, aparece en la pared abdominal anterior [17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] . Estas lesiones se caracterizan por tener un crecimiento infiltrativo, ausencia de evidente atipia nuclear y bajo índice mitótico.…”

“…Aunque la morfología permite distinguir generalmente la fibromatosis de otros procesos, en ocasiones -debido a escasez de tejido, artefacto etc.-es necesario acudir a la clínica y al estudio IHQ con ␤-catenina. La tinción nuclear positiva para ␤-catenina es un marcador que actualmente se considera como primordial en la distinción entre los tumores desmoides y otros tumores con morfología similar [17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] .…”

Uncommon abdominal wall mass in a young boy: Desmoid tumor

“…DT tends to occur in young women. Conversely, in children is an extremely rare condition 4 . The exact incidence of DT in children is unknown.…”

“…Aggressive fibromatosis is an often-used term to emphasize its frequently aggressive behavior. DT accounts for 0.03% of all neoplasms and 3% of soft tissue tumors, with a reported incidence of [2][3][4] They usually arise in the mesentery, omentum or retroperitoneum, while the abdominal wall is very rare 3 . DT tends to occur in young women.…”

“…Chemotherapy or radiotherapy has been suggested as adjuvant therapy in patients with residual tumors, as the potential for morbidity is high after the second operation 4 .…”