Sporadic Retroperitoneal Aggressive Fibromatosis: Report of a Case

Abstract

We describe herein a case of sporadic retroperitoneal aggressive fibromatosis. A 54-year-old man with a palpable abdominal mass was referred to our hospital for investigation and treatment. Abdominal ultrasonography and computed tomography revealed a solid mass with relatively well-defined borders in the left abdominal retroperitoneum. At surgery, a large tumor (14 x 13 x 11 cm) was found, arising from the retroperitoneal space and involving the wall of jejunum. Complete removal was achieved. Histological examination of the resected specimen revealed the presence of changes consistent with aggressive fibromatosis. The patient was not scheduled for any further treatment. Two years after surgery, the patient is without any signs of recurrent disease. Although extremely rare, aggressive fibromatosis should be considered in the differential diagnosis of retroperitoneal masses. Complete surgical resection with negative pathological margins remains the first line management of these neoplasms. Careful follow-up is indicated because recurrence may occur every time after surgery.

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] .…”

Sporadic Case of Desmoid Tumor in Outcomes of Lombotomic Nephrectomy

“…Relativamente allo sviluppo dei tumori dermoidi sono stati identificati alcuni fattori di rischio, fra i quali la fibromatosi extraddominale, i fattori genetici, endocrini. può superare anche il 70% (6). Tuttavia anche se è stata descritta una parziale e spesso completa regressione dei tumori desmoidi dopo radioterapia adiuvante, diversi autori sostengono che questi tumori non sono responsivi alle radiazioni.…”

Practical Issues for Retroperitoneal Sarcoma

“…46 Retroperitoneal desmoid tumor has been described in 11 articles since 1991. [127][128][129][130][131][132][133][134][135][136][137] These tumors are characterized by proliferation of spindle (fibroblast) cells, with a moderate amount of collagen fibers, bland cellular appearance, scant mitosis, and lack of metastasis. 130, 135 Expression of beta catenin in tumor cells is helpful for confirming the diagnosis.…”