Aggressive fibromatosis in the neck. A case treated effectively by testolactone with a long follow-up study

Extra-abdominal fibromatosis in the neck may occasionally cause serious sequelae when aggressive invasion of the vital structures occurs. However, the principle of wide excision is difficult to apply in some patients because of the important organs surrounding the tumor. We report herein, a woman followed up for 22 years, in whom testolactone showed a definite effect during the course of observation. She first presented, at the age of 52, with a painful tumor in the neck, located behind the right thyroid lobe and fixed to the adjacent structures. Incomplete excision of the tumor was followed by a local recurrence accompanied with recurrent laryngeal nerve palsy and Horner's syndrome on the right side. Prednisolone was administered for 2 1/2 months and external irradiation of 8,000 RAD was given. Enlargement of the recurrent tumor stopped but 2 months later an emergency tracheostomy was required for an airway obstruction. Over the next 3 years, the local findings remained unchanged, but then a painful tumor growth reappeared cephalad which gradually attained a size of 5 cm in diameter, and was fixed to the base. Testolactone 150 to 200 mg was given to the patient daily for 2 months, resulting in marked regression of the tumor. During the last 13 years the patient has been well with the tumor still decreasing in size.