Four different treatment strategies in aggressive fibromatosis: A systematic review

The treatment approach for aggressive fibromatosis is changing. We compared the local control rate for surgery, surgery with radiotherapy, radiotherapy alone and a wait and see policy in a systematic review. A comprehensive search of the databases PubMed/Medline, Embase and Cochrane, of the medical literature published in 1999 till March 2017 was performed by two reviewers, including articles about extra abdominal aggressive fibromatosis without the genetical variants. A total of 671 studies were assessed for eligibility, and 37 studies were included for analysis, representing 2780 patients. The local control rates for surgery alone, surgery and radiotherapy, radiotherapy alone and observation were 75%, 78%, 85% and 78%, respectively. For patients with recurrent disease observation had a better local control rate than surgery alone (p=0.001). In the observation group, stabilization of the tumor was seen in median 14 (range 12-35) months. The time to local recurrence in the treatment group was median 17 (range, 11-52) months. A watchful conservative first line approach with just observation and closely monitoring, by means of physical examination and MRI, appears to be justified in a subgroup of patients without clinical symptoms and no possible health hazards if the tumor would progress.

In this article we focus on approved treatments and newer therapeutic approaches for management of soft tissue sarcomas.

The case of a 46-year old female patient, who presented a desmoid tumor in the area of a previously excised accessory breast, is reported. A review of the international literature revealed no other similar case. The differential diagnosis and current opinion on treatment of these tumors is discussed.

In this article, a case of a patient in the pediatric age affected by desmoid fibromatosis localized in the orbit is presented. The management of desmoid fibromatosis is based on the function preservation and the maintenance of a good quality of life, but in case of symptomatic patients or aggressive course of the disease or risk of functional damages, the surgical approach may be considered. Therapeutic alternatives to surgical resection are radiotherapy and systemic therapy.

We encountered a case of retroperitoneal desmoid-type fibromatosis in a 45-year-old woman who presented with chief complaints of stomach ache and vomiting. She underwent total abdominal hysterectomy and left salpingo-oophorectomy due to uterine myoma and a paroophoritic cyst at 42 years of age. Abdominal computed tomography showed a 5 cm left retroperitoneal tumour and severe hydronephrosis of the left kidney. Therefore, we performed tumour resection, right salpingo-oophorectomy, ureterectomy, and ureterocystostomy. The tumour surrounded the left ureter and adhered to the left internal/external iliac artery, rectum, bladder, and the edge of the vagina. Histopathologic examination yielded a diagnosis of retroperitoneal desmoid-type fibromatosis. One month after the operation, transvaginal sonography showed a 2-cm mass in the pelvis. We suspected tumour recurrence and commenced pharmacotherapy with tranilast (300 mg/day, three times per day). Four months after the operation, the mass disappeared.

Desmoid-type fibromatosis (DF), also known as aggressive fibromatosis, is a locally aggressive benign fibroblastic neoplasm that can infiltrate or recur but cannot metastasize. Due to the high rate of recurrence, imaging plays an important role not only in diagnosis, but also in the management of DF. Although there are a number of studies describing CT and MRI findings of DF, there is no description of contrast-enhanced ultrasound findings.