Desmoid tumors of the head and neck: A therapeutic challenge

Abstract

Desmoid tumor, or aggressive fibromatosis, is a rare, histologically benign, fibroblastic lesion that infrequently presents in the head and neck. Desmoid tumors often grow locally, invasively, and may, in rare instances, be fatal secondary to invasion into critical structures, such as airway or major vessels. The most common treatment is surgery, but desmoid tumors are characteristically associated with a high local recurrence rate after resection. Although the margin status seems to be of importance, operations that avoid function loss and esthetic disfigurement should be the primary goal. The efficacy of postoperative radiotherapy is controversial. Its potential benefit should be carefully balanced against possible radiation-induced adverse effects. Alternative treatment modalities, such as primary radiotherapy and medical treatment or a wait-and-see policy, may be preferable to mutilating surgery. Considering all the aforementioned, it seems obvious that desmoid tumors of the head and neck present a therapeutic challenge and require an individualized approach.

Aggressive Desmoid Fibromatosis of the Neck after Total Thyroidectomy

“…However, these tumors are considered a neoplastic rather than an inflammatory reactive process. 4 This is the third case of a desmoid tumor after thyroidectomy, 5,6 the second after total thyroidectomy, 5 and, to the best of our knowledge, the first with such a large tumor to be completely cured by surgery.…”

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

“…However, pharmacologic treatment of head, neck, and spine DTs has only been used infrequently, so robust data regarding its efficacy is lacking [7,34,35]. Although NSAID therapy has relatively low morbidity, there is currently no prospective randomize data to demonstrate that NSAIDs alone are efficacious for the treatment of DTs [36].…”

“…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]. DTs have been reported to occur in nearly every bodily location, with an estimated 7% to 15% of DTs occurring in the head and neck [8,9].…”

Periosteal desmoid tumour: a rare finding in the oral cavity

“…However, systemic treatment for head-and-neck desmoid tumours has been used infrequently. In few unresectable cases where local treatment leads to high morbidity, a protocol of watchful waiting until significant symptoms develop may be followed as these lesions tend to have periods of stability or may even regress 9. Since the lesion was relatively small in size, a wide surgical excision was planned for the current case.…”