Recent Advances in Desmoid Tumor Therapy

Here, we review the most recent advances in desmoid tumor therapy, including low-dose chemotherapy and treatment with tyrosine kinase inhibitors. We also explore the recent improvements in our knowledge of the molecular biology of this disease, which are leading to clinical trials with targeted agents.

A 17-year-old adolescent boy was referred by an orthopaedic physician to physical therapy for chronic left shoulder pain and a clinical diagnosis of labral tear. The physician ordered current radiographs, which were noncontributory. Findings during physical therapy examination warranted referral back to the physician, with a request for magnetic resonance imaging. Magnetic resonance imaging revealed a soft tissue abnormality in the infraclavicular and subcoracoid spaces. The patient was referred to orthopaedic oncology, where biopsy confirmed a desmoid tumor.

We report here the case of a 27yrs old male with aggressive fibromatosis that recurred following a surgical excision, and resulted in hampering the quality of life. The disease was managed using cytotoxic chemotherapy with doxorubicin, resulting in significant clinical and radiological response. It was followed by maintenance regimen using a less aggressive approach with non-steroidal anti-inflammatory drugs such as celecoxib that resulted in further clinical response at 2 months follow up. Therefore, the therapy was further continued.

Mediastinal tumors may cause a wide range of symptoms; therefore, it should be considered in the differential diagnosis, after excluding common causes, when dealing with pulmonary and cardiac symptoms such as dyspnea or even mitral regurgitation. The most common site of the desmoid tumors is the abdomen, but in very rare cases, it can be located in the mediastinum.

A 52?year?old healthy patient presented with abdominal pain, nausea, and vomiting. Physical examination revealed abdominal distension and diffuse tenderness. An abdominal CT scan revealed a mesenteric mass measuring 9 ? 6 cm in the pelvis and small bowel mechanical obstruction. The mass was in contact with last ileal loop. A mini?laparotomy was performed with small segmental resection and manual end?to?end anastomosis. The patient was discharged on postoperative day 4, and at follow?up at 1 month, he had no complaints.

We present a case of a desmoid tumor of the splenic hilum laparoscopically resected in a 70-year-old male with a previous history of chromophobe renal cell carcinoma and ocular spindle melanoma.