The Wnt/β-catenin pathway in human fibrotic-like diseases and its eligibility as a therapeutic target.

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.

Neurosurgical cases of DT have been very scarce. We review the literature concerning neurosurgical DTs and describe the first case of a cicatricial DT after the resection of vestibular schwannoma, presenting as a painful swelling in the retrosigmoid scar. Contrary to other localizations in the body, standard-of-care wide margin resection cannot be performed in intracranial and spinal DTs. Therefore, maximally safe resection followed by radiotherapy when tumor margins are not free can be proposed as a treatment strategy in neurosurgical Dts

Imatinib, a tyrosine kinase inhibitor, is a major therapeutic option for the management of unresectable aggressive fibromatosis. Unfortunately, for most patients of low or very low average income countries, surgery often is the first treatment option. This is related to unavailability of chemotherapy or targeted therapy, and to a lack of financial resources or surgeons’ lack of knowledge of other therapeutic options.

A giant desmoid tumor arising from the left abdominal wall of a young female patient with rapid growth during pregnancy is described. Preoperative evaluation included ultrasonography and magnetic resonance imaging. Decision made by a multidisciplinary team was not to intervene before birth, and abdominal delivery at term was accomplished. Desmoid tumors should be part of the differential diagnosis in an abdominal wall tumor of rapid growth during pregnancy. Future studies are needed for better understanding of the pathogenesis, diagnosis, and treatment of desmoid tumors in pregnant women.