Aggressive Fibromatosis

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The age of the patient, the soft tissue mass, the fast growth, and the destructive nature of the lesion are all in support of a condition that is aggressive in behavior. However, it is difficult to tell if the lesion is an aggressive soft-tissue neoplasm that is invading the bone or if this is an aggressive bone neoplasm perforating it and forming a soft tissue mass. The age and gender of this patient, the location, the soft tissue mass and the bony destruction are all supportive of aggressive fibromatosis but the histology is not.

Aggressive fibromatosis represents a group of fibrous neoplasms with various clinical behaviors and histologic presentations. Aggressive fibromatosis is known by many names, including juvenile aggressive fibromatosis, infantile desmoid fibromatosis, and nonmetastasizing fibrosarcoma. The last nomenclature best describes the behavior of this lesion; it is a benign but locally infiltrative and aggressive neoplasm with a potential for multiple local recurrences, but no potential for metastasis. Aggressive fibromatosis can be sporadic or familial, such as those associated with Gardner's syndrome. The latter tends to occur more in the abdominal area, and rarely in the head and neck area. Some investigators consider aggressive fibromatosis in children to be a separate entity than that which occurs in adults.

Head-and-neck aggressive fibromatosis usually occurs in children under 20 years of age. Among adults, it is more prevalent in females by a ratio of 2:1; in children, it occurs more often in males by a ratio of 2:1. It typically presents as a firm, painless, rapidly growing asymptomatic mass. Occasionally, it is slow-growing, but it is destructive in either case. It can destroy bone, infiltrate adjacent structures, displace teeth, and induce periosteal reaction, rendering it clinically and radiographically difficult to differentiate from a sarcoma. It presents in multiple locations within the oral cavity, including the gingiva, lips and other oral soft tissue areas. Several theories have been proposed regarding the etiology of aggressive fibromatosis, which is currently unknown. Hormonal, viral, chromosomal, and, occasionally, trauma-induced etiologies have all been described. Trauma-induced etiology is often described in the area of caesarian-section scars. There is a legitimate body of evidence to suggest that estrogen promotes the growth of fibromatosis, particularly in pregnant females. Association with the FAP (Gardner's Syndrome) gene is also well documented. The clinical behavior of aggressive fibromatosis is unpredictable and ranges from frequently recurring to spontaneously regressing; for that reason, a variety of treatment modalities have been employed. Surgery, which is the treatment of choice, ranges from complete removal with clean margins to debulking of the lesion to avoid loss of vital structures. The latter method is often necessary for oral cavity lesions. In the present case, care was taken during surgery to avoid sacrificing the lingual nerve; therefore, the surgeons opted for the more conservative approach of debulking of the primary lesion and very close watch of the surgical area. Several non-surgical alternative treatments have also emerged, such as radiotherapy, chemotherapy, hormonal and biological manipulation, and non-steroidal anti-inflammatory agents. Surgery combined with radiotherapy or chemotherapy has been used in recurring and persistent lesions. Radiotherapy is a secondary treatment method to surgery, and is sometimes used alone. However, concern over its possible adverse long-term effects, especially in children, makes it a less desirable mode of therapy. Chemotherapy, with single or multiple agents, and neoadjuvant or maintenance therapy have also been used. In children, chemotherapy is associated with long-term adverse effects and occasional serious complications and even death. Some cases have been successfully treated with alternative treatments such as estrogen antagonists and non-steroidal anti-inflammatory drugs. Prognosis and recurrences are difficult to predict. The recurrence rate ranges from 20% to 70% for aggressive fibromatosis in children, and most recurrences occur within the first five years. Some studies suggest that positive margins may be the only predictor for recurrence, if any. Other adverse prognostic factors may include an age of less than 18 years, recurrent disease, and surgical treatment alone.