Assessment of Physician’s Systemic Treatment Preferences for Patients with Advanced Desmoid-Type Fibromatosis: Experience-Based Medicine in the Absence of High-Level Evidence.

We assessed systemic treatment preferences for advanced DF among European experts, with the aim to define a control treatment for prospective randomized trials. Treament preferences for sporadic DF versus DF associated with Gardner’s syndrome were similar. Physicians use at least 5 different classes of drugs (27 agents). The most frequently used compounds were anti-estrogens and non-steroidial anti-inflammatory agents (NSAIDs), in combination or as single agents. The second and third more common systemic approach was chemotherapy based on methotrexate or an anthracycline. Trial activity was limited to 1 country/1 multicentric study.

Here, we examined the effects of sorafenib on patient-derived DT cell lines, with the aim of characterizing the efficacy and molecular mechanism of action. Taken together, our results suggest that sorafenib suppresses DT proliferation and invasion via inhibition of Ras/MEK/ERK and PI3K/Akt/mTOR signaling pathways with additional effects on translation. Sorafenib may be a promising therapeutic option in the treatment of desmoid tumors. Additional studies in DT patients are warranted to examine the efficacy of combination therapy using sorafenib.

Controlled ovarian hyperstimulation (COH) followed by oocyte retrieval is a leading option for fertility preservation before chemotherapy, yet this procedure causes excessive serum levels of estradiol (E2), which are often detrimental for cancer patients. Aromatase inhibitors are often used in breast cancer patients during COH to prevent elevated levels of E2. Of the five patients treated, two had an aggressive abdominal desmoid tumor, one had endometrial carcinoma, one had uterine sarcoma, and one patient had a brain oligodendroglioma. In all cases the treating oncologist suggested an association between estrogen and possible tumor progression. COH with aromatase inhibitors is apparently effective in non-breast cancer patients and spares exposure to high E2 levels.

A 32-year-old woman, who underwent cesarean section three years ago came to the hospital for finding a mass on abdominal wall for half a month. Underwent surgery. The lesion is aggressive fibromatosis of abdominal wall (ligament tumor of abdominal wall). We discussed the particularity of its clinical characteristics, treatment strategies and prognosis combined with literature review, and we think the surgeons need to pay high attention to this disease and make more patients get timely, correct and reasonable treatment, so as to improve the quality of life.

Intermediate tumors of the head and neck fall in the borderline category. The standard care is surgical excision with wide margins. However, surgeons are challenged with the anatomic complexity of the head and neck, increasing the susceptibly of satellite cells being left behind. Ki-67 and MMP-9 are proliferative index and extracellular matrix degradation biomarkers, respectively. They are directly correlated to malignant tumors, whereas less associated with the benign ones. Our main objective was to correlate between Ki-67 and MMP-9 expressions and the recurrence rates in these borderline tumors. We performed a retrospective immunohistochemical study comparing the immunoexpression of Ki-67 and MMP-9. Tumors of interest were aggressive fibromatosis (AF, n=70), epithelioid hemangioendothelioma (EHE, n=25), hemangiopericytoma (HP, n=25), benign fibrous histiocytoma (BFH, n=80) and juvenile ossifying fibroma (JOF, n= 40). Our results revealed that AF followed by HP showed significant high levels of MMP-9 expression, with an average positive area percentage of 40% and 37.4% respectively, compared with other tumors (P<0.05). Ki-67 immunoreaction was significantly the lowest in AF (2.3%, P<0.05) and the highest in JOF (24.7%). To conclude, MMP-9 can be used as a possible target in these tumors as an adjuvant therapy to minimize recurrence rates.

The current report presents a case of an omental fibromatosis discovered incidentally in a 46-year-old woman with no particular medical history and few symptoms. A surgical biopsy was performed initially, and microscopic examination revealed myofibroblastic proliferation. After additional immunohistochemical and molecular analyses, omental fibromatosis was diagnosed. Omental fibromatosis, also called intra-abdominal desmoid, is a rare and benign tumour but can be locally aggressive.