Large desmoid-type fibromatosis of the shoulder girdle: operative approach selection and clinic outcome.

Surgical resection of large desmoid-type fibromatosis (DTF) of the shoulder girdle is challenging. This article discusses the operative approach and evaluates the clinic outcome.

Desmoid tumors (DTs) are rare, mesenchymal tumors that exhibit features of an abundant wound healing process. Previously, we showed that mesenchymal stem cells (MSCs) are constituents of DTs and may contribute to desmoid tumorigenesis via activities associated with wound healing. Hyaluronan (HA) is a long-charged chain of repeating glucuronate and N-acetylglucosamine disaccharides that is synthesized by HA synthases (HAS) and degraded by hyaluronidases (HYAL). HA is secreted into the extracellular matrix by injured stroma and is important for normal tissue repair and neoplastic progression. Here, we investigated the presence of HA in DTs and the antitumor effects of the HA inhibitor, 4-methylumbelliferone (4-MU), on DT-derived mesenchymal cells.

Loco-regional treatments of breast DTs resulted in undesired disfigurement. Front-line observation yielded encouraging results and could enable the identification of patients who require loco-regional treatment. This strategy needs further evaluation.

The canonical Wnt signaling pathway is involved in a variety of biological processes like cell proliferation, cell polarity, and cell fate determination. This pathway has been extensively investigated as its deregulation is linked to different diseases, including various types of cancer, skeletal defects, birth defect disorders (including neural tube defects), metabolic diseases, neurodegenerative disorders and several fibrotic diseases like desmoid tumors.

The prognostic value of surgical resection margins in predictingrecurrence of desmoid-type fibromatosis remains inconclusive, and appropriate resection margins are not defined. We analyzed whether specific factors related to margin status, including the adequacy of pathologic assessment, affect the prognostic impact of surgical resection margins in a retrospective cohort study. Surgical resection margin status, as currently evaluated and reported, fails to predict recurrence-free survival for desmoid tumors. Instead, positive or close (<1 mm) resection margins are predictive of disease recurrence. In addition, multiple targeted sections are necessary for accurate assessment of surgical resection margins of desmoid-type fibromatosis.

A non-surgical approach to extra-abdominal wall DF allowed surgery to be avoided in the majority of patients. This approach can be safely proposed and surgery offered as an option in selected cases.