Desmoid tumor of the mesentery in a patient after restorative proctocolectomy as a result of familial adenomatous polyposis – case reports

The aim of the paper is to present a case of a 33-year-old woman with diagnosed familial polyposis in the colon, who has been identified with mesenteric desmoid.

Papillary thyroid carcinoma with nodular fasciitis-like stroma (PTC-NFS) is a rare lesion with dual pathological features, that behaves more aggressively if positive for beta-catenin gene (CTNNB1) mutations. A 48-year-old female presented with a recurrent enlarging tumor with a diagnosis of desmoid-type fibromatosis 6 months after her initial operation for PTC-NFS. Over a 2-year observation period, she underwent remedial surgery to resect the recurrent tumor. Local invasion, beta-catenin aberrant staining, and CTNNB1 mutations were detected both in the primary tumor stroma and the recurrent tumor. This case indicates that CTNNB1 mutation screening and beta-catenin staining should be performed in PTC-NFS cases as a predictor for recurrence. If either of these are positive, a wider surgical resection should be adopted to achieve negative margins. Otherwise, radiotherapy should be considered. Furthermore, the lesion might be better known as papillary thyroid carcinoma with desmoid-type fibromatosis in such situations to reflect its local aggressive nature.

Aggressive fibromatosis comprises connective tissue tumours that represent 0.03% of all bodily neoplasms, occurring more often in the abdominal wall, mesentery, and extremities; its location in the breast constitutes a very infrequent type of lesion. Its pathogenesis is diverse and its relationship with augmentation mastoplasty is still unclear. Four cases of aggressive breast fibromatosis following augmentation mastoplasty are reported in this article.

A retrospective review was performed for 135 patients who underwent oncological chest wall resection during 1997-2015. Indications for resection were: advanced breast cancer n=44, soft tissue sarcoma n=38, bone or chondrosarcoma n=28, desmoid tumour n=11, metastasis from other cancers n=7, and other primary tumours n=7. There were 72 full-thickness and 63 partial-thickness resections (34 soft tissue resections only and 29 skeletal bone resections only). Resection margins were wide n=29, marginal n=82 and intralesional n=24. Reconstruction was warranted in 118 cases: chest wall stabilization and flap coverage n=57, chest wall stabilization only n=36 and soft tissue flap coverage only n=25. In total, 82 flaps were performed (17 free flaps and 65 pedicled/local flaps). There were no perioperative mortalities nor flap losses. One-year, 2-year and 5-year survival rates were 84%, 82% and 70%, respectively. With careful patient selection, appropriate peri- and postoperative care and accurate surgical technique, even extensive chest wall resections and reconstructions are safe.

Here, we report a rare case of a large aggressive fibromatosis(AF) in the axilla. Interestingly, 18 F-fluorodeoxyglucose-positron emission tomography/computed tomography showed significant increase in standard uptake value. Surgical resection yielded a spindle cell tumor likely of fibromatosis origin which was positive for beta-catenin expression.

We retrospectively reviewed the outcomes of patients who had been treated with meloxicam for the extra-abdominal desmoid tumors and evaluated the correlation between clinical outcome and clinic pathological variables. Twenty patients treated with meloxicam were followed up every 3 to 6 months. Meloxicam administration was planned at 15 mg/day orally for 6 months. Of the 20 patients evaluated, according to Response Evaluation Criteria in Solid Tumors criteria, there were five patients with partial response (25.0%), eight with stable disease (40.0%), and seven with tumor progression (35.0%). The present study suggests that conservative treatment would be a primary treatment option for this perplexing disease even though we were not able to determine that the use of a cyclooxygenase-2 inhibitor would have an additional influence on the natural course of a desmoid tumor.