Desmoid tumors: need for an individualized approach

Abstract

Desmoid tumor, also known as aggressive fibromatosis or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. Although histologically benign, desmoids are often locally invasive and associated with a high local recurrence rate after resection. Since it is a heterogeneous disease, in particular regarding clinical presentation, anatomic location and biological behavior, treatment should be individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life. Many issues regarding optimal treatment of desmoids remain controversial. However, wide surgical excision remains the treatment of choice, except when surgery is mutilating and is associated with considerable function loss or major morbidity. Involvement of surgical margins is probably associated with an increased risk of local recurrence. Postoperative radiotherapy results in a significant reduction of the local recurrence rate, but only in the case of involved surgical margins. Radiotherapy for gross disease is considerably effective, but is associated with a relatively high rate of complications, which are usually mild or moderate and radiation dose dependent. Radiotherapy should only be applied where anatomic constraints preclude complete resection and radiotherapy is not too toxic. Risk factors for local tumor control failure include young age, large size, presentation as recurrent disease, limb/girdle or intra-abdominal location, involved surgical margins, omission of radiotherapy, radiation dose less than 50 Gy and insufficient radiation field size. Increased comprehension of the pathogenesis and biological behavior of desmoids resulted in the emerging applicability of systemic therapies and a wait-and-see policy. Systemic treatment may be indicated in patients that have anatomic barriers to effective surgery or radiotherapy. Considering the significant morbidity of surgery and/or radiotherapy for certain locations, especially mutilation and loss of function, and the tumor's natural history, which is often characterized by prolonged periods of stability or even regression, a period of watchful waiting may compose the most appropriate management in selected asymptomatic patients. Attempts to complete eradication of the disease may be worse than the disease itself.

Results of surgical treatment in patients with abdominal desmoid fibromas

“…К примеру, средние показатели КЖ жителей РФ для 8 шкал опросника SF-36 составляют от 54,1 до 79,6 балла [22,40].…”

Hepatocellular Cancer Arising From Ectopic Liver Tissue on Diaphragm in Association with Desmoid Mesenteric Tumor

“…Mesenteric desmoid tumors are often asymptomatic, but they can result in abdominal pain, palpable abdominal mass, fever, intestinal obstruction, perforation or ischemia [12]; although histologically benign, desmoid tumors are often locally invasive and associated with a high local recurrence rate after resection; many issues regarding optimal treatment of this disease remain controversial; surgical excision remains the treatment of choice; anyway, involvement of margins leads to an high risk of recurrence; for this reason some studies points out the need of considering other approaches, such as watchful waiting or surgery in association with other treatments (in particular radiotherapy) [13].…”

Aggressive Resection of Cervical Desmoid Tumor Invading the Paraspinal Muscles without Recurrence at Eight Year Followup: A Case Report and Review of the Literature

“…While DTs do not spread metastatically, they can invade local tissue and frequently recur after surgical resection [2][3][4]; for this reason they are often categorized with low-grade soft tissue sarcomas [5]. Approximately 2-4 cases of DTs per million people occur each year, and account for 0.03% of all neoplasms [6] and 3% of soft tissue tumors [7].…”

“…Development of DTs is thought to be associated with female sex hormones, as the tumors occur more commonly in females, their growth rate is directly related to endogenous estrogen levels in female patients, and estradiol receptors have been found to be present in the tumor cytosol [12]. Indeed, tumor regression has been reported with the use of the antiestrogen compound tamoxifen [5,13,14]. Interestingly, in a study reviewing the use of endocrine therapy for desmoid tumors, Wilcken and Tattersall After the occiput was identified, a periosteal was used to scrape the tumor from the bone.…”