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Keywords:

  • differential diagnosis;
  • immunohistochemistry;
  • myofibroma;
  • myofibromatosis

Oudijk L, den Bakker M A, Hop W C J, Cohen M, Charles A K, Alaggio R, Coffin C M & de Krijger R R

(2012) Histopathology 60, E1–E11

Solitary, multifocal and generalized myofibromas: clinicopathological and immunohistochemical features of 114 cases

Aims:  To report a large series of solitary and multiple myofibromas with systematic clinicopathological correlations.

Methods and results:  We report on 114 patients with myofibromas, 97 of which were solitary and 17 multifocal. The age at presentation ranged from newborn to 70 years. All multifocal myofibromas and 91% of solitary myofibromas occurred in children. The head and neck region was the most common site (n = 43), followed by the trunk (n = 24), lower limbs (n = 14), upper limbs (n = 11), and viscera (n = 4). Solitary and multifocal myofibromas stained positively for smooth muscle actin (SMA) in 95% and 92% of cases, muscle-specific actin (MSA) in 75% and 50% of cases, and desmin in 10% and 14% of cases, respectively. Regressive features were seen in 34 solitary myofibromas and in nine multifocal myofibromas. Most patients were treated with complete excision (n = 79) or partial excision (n = 12). There were no recurrences after treatment.

Conclusions:  Solitary and multiple myofibromas are benign tumours that predominantly occur in infancy and childhood. Myofibromas occur especially in the head and neck region, and are characterized by SMA and, to a lesser extent, MSA expression. The clinical course is self-limiting, and local excision appears to be sufficient.