Desmoid tumors: clinical features and treatment options: a case report and a review of literature

Abstract

Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion and may even result in a fatal outcome when located around vital organs. Their clinical presentation, biological behavior and natural history can be quite varied and is incompletely understood at the present time. The optimal therapeutic approach depends on various factors, and a multidisciplinary approach is necessary to achieve local control with acceptable morbidity. Despite progress in the understanding of these tumors and the treatment options, local recurrence remains a major problem.

Value of imaging findings in predicting post‑operative recurrence of desmoid‑type fibromatosis

“…Women were more likely to develop the disease compared with men (20), and therefore, estrogen is an influencing factor. The most common sign is a painless, progressive mass, but symptoms/complaints including neurological disorders, joint stiffness or abdominal discomfort due to tumor growth may also occur (9). Desmoid-type fibromatosis is likely to recur after surgery.…”

“…Due to the variety of location and clinical symptoms of desmoid-type fibromatosis, it often leads to difficult diagnosis, and there are many diseases that need to be differentiated, such as inflammatory lesions, liposarcoma and fibrosarcoma (8). The most common complaints of patients are painless progressive growth of the mass, neurological dysfunction, joint stiffness and abdominal discomfort due to the tumor growth (9). The imaging findings of desmoid-type fibromatosis depend on the number of fibroblasts proliferating in the tumor, as well as the fiber composition, collagen content and tumor supply (10).…”

Can ovaries be preserved after an ovarian arteriovenous disconnection? One case report and a review of surgical treatment using Da Vinci robots for aggressive ovarian fibromatosis

“…The diagnosis can be confirmed by additional examinations, such as an MRI, which is the method of choice when fibromatosis and recurrence are suspected [10], as well as pathology and immunohistochemistry [11, 12]. Presently, surgery is the main and most effective method of treatment, with the standard surgical goal to completely excise the lesion with negative surgical margins [7, 8]. The postoperative recurrence rate is 13–68% and the recurrence generally occurs more than 2 years after the initial surgery [13].…”

“…Most of these incidences are sporadic cases, occurring mainly in women, and especially in patients aged 10–40 years [5, 6]. At present, surgery is the primary method of treatment with the standard surgical goal to completely resect the lesion of negative margins [7, 8]. Aggressive fibromatosis occurring in the posterior peritoneum can invade peripheral blood vessels and nerves.…”

Familial Adenomatous Polyposis and Desmoid Tumor Treated with Multivisceral Transplantation and Kidney Autotransplantation: Case Report and Literature Review

“…Surgical excision is the most common treatment for DT that are symptomatic or close to vital structures. However, this is complicated by the proximity of tumours to major vessels, multiple organ involvement, and a high rate of local recurrence [5][6][7]. Consequently, multivisceral transplantation (MVTx) may be considered a more effective curative approach, especially given the extensive bowel pathology, multiple prior resections, and liver steatosis (as a result of TPN) commonly seen in end-stage FAP patients [8].…”