Desmoid fibromatosis of the chest wall

We report a case of desmoid fibromatosis of the chest wall. A 70-year-old woman was referred to our hospital with right shoulder blade pain and paresthesia over the right upper breast. Chest X-ray and computed tomography demonstrated a 5 cm right apical mass in the chest. Biopsy of the mass demonstrated features of desmoid fibromatosis. The patient subsequently underwent surgical resection of the mass and received adjuvant radiation therapy for microscopic positive margins. In conclusion, although desmoid tumour of the chest is rare, it is worth considering in the differential diagnoses of chest wall tumours.

A 65-year-old Chinese man presented with a 2-day history of left-sided abdominal pain with fever and watery diarrhea in February 2016. An 11 cm x 13 cm tumour was found at the mesenteric side of the proximal jejunum which had ruptured with abscess formation. Gross examination showed a multinodular tumour with an area of purulent material on the surface at the serosa, measuring up to 11 cm in diameter. On light microscopy, a circumscribed and non-encapsulated spindle cell neoplasm was seen centred at the subserosa and muscularis propria. On immunohistochemical staining, tumour cells exhibited a beta-catenin nuclear translocation pattern and were weakly positive for c-Kit. The features were compatible with a diagnosis of mesenteric fibromatosis (MF).

Percutaneous ablation of benign musculoskeletal tumors in children has become an area of significant clinical growth over the last decade. With growing research interest, the technologies available and techniques used are becoming increasingly safe and effective. We review some of the more common ablation technologies and their utility in several common benign bone and soft tissue tumors that occur in children and young adults.

Here we report a very rare entity of an infratemporal region aggressive fibromatosis in a 23-year-old Tharu man who had presented with the symptoms of painless but progressive trismus (lockjaw). Radiological imaging as well as an immunohistochemistry panel from a biopsy indicated a diagnosis of an aggressive fibromatosis. Since there was no aggravation in his trismus following surgery and because of his poor socioeconomic status, he was advised to attend regular follow-up visits without any adjuvant therapy. This case report adds to the notion of keeping the differential diagnosis of an aggressive fibromatosis in all patients presenting with progressive but painless trismus. The characteristic imaging findings as well an immunohistochemistry panel will help us clinch the correct diagnosis.

A 35-year-old Asian woman presented at our hospital with a chief complaint of abdominal pain. She had abdominal desmoid tumors that required complete resection of her rectus abdominis muscle. Due to necrosis in her own reconstructed tissue, we failed to cover her anterior abdominal wall; thus, we used an abdominal binder as a substitute material to avoid exacerbating the incisional hernia and help her generate intra-abdominal pressure. This case report may be informative and helpful for the treatment of patients with desmoid tumors, as managing desmoid-type fibromatosis is difficult.

Neovascularisation is a critical step in the progression of malignant tumors. Circulating endothelial progenitor cells (cEPC) have been proposed as surrogate markers of vasculogenesis in malignancies. In this project, we studied the impact of tumor-specific therapy on cEPC and associated angiogenic factors in patients with soft tissue tumors. Fifty-three patients with soft tissue tumors (25 soft tissue sarcomas, 19 GIST, 9 desmoids) and 15 healthy controls were included. The pre-treatment values of the angiogenic markers did not correlate with the clinical course of the disease. However, cEPCs levels were significantly higher in sarcoma patients with progressive disease compared to those with stable disease and should be further evaluated as early markers of disease progression in sarcoma patients. VEGF-A and angiopoetin-2 clearly play a role as mediators of the vasculogenesis contributing to tumor progression.