Aggressive fibromatosis of the head and neck (desmoid tumours)

Abstract

Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.

Aggressive Fibromatosis of Head and Neck in a Child

“…They were first described by Stout and represent a distinct entity of intermediate lesions arising from the musculoaponeurotic system. They are characterized by high propensity to invade adjacent structures without distant metastasis [2].…”

Extraabdominal desmoid tumors

“…10,17 The rarity of these tumors is reflected in the case series available in the literature. Fasching 1,[20][21][22] Desmoid tumors were noted by Pack and Ehrlich 15 to account for 0.03% of all neoplasms. 6 They are generally thought to comprise fewer than 3% of all soft tissue neoplasms.…”

“…1), with scanty or absent mitotic activity or other atypical cytological features. 1,5,17 Differentiation of this tumor type from fibrosarcoma is essential, as desmoid tumors do not possess the tendency for metastasis. However, their local invasion along facial planes is ubiquitous and often involves nearby vascular and/or neural structures.…”

“…23 On computed tomography scans, the lesion appears hyperattenuated with significant enhancement after the application of contrast material. 1 Magnetic resonance imaging characteristics include a lesion that is hypointense to isointense relative to muscle on T1-weighted imaging, and with a signal intensity similar to fat on T2-weighted images 23 ( Fig. 2).…”

“…6,14 When used, the radiation dose fell within the range of 45 to 65 Gy. 1,5,6,17 Kiel and Suit 6 noted that complication rates after radiotherapy can be minimized if careful attention is given to a technique including secondary field shaping and reducing field techniques. Most authors would agree and recommend further treatment in the setting of known positive resection margins with infiltrative disease, as there is some evidence of decreased local recurrence.…”