Pediatric myofibromatosis of the head and neck

Objectives: To examine the clinical and pathological features of pediatric myofibroma of the head and neck and to discuss the challenges in diagnosis and treatment. Design: A retrospective search of pathology department and clinical records to identify patients with myofibroma and a retrospective review of English -language medical publications. Setting: Academic medical center. Patients: Thirteen pediatric patients (aged from birth to 8 years old) diag nosed as having myofibroma of the head and neck. Results: Nine of 13 patients were cured with conservative surgical excision . Four patients (31%) had recurrence, requiring multiple surgical procedure s. One third showed spontaneous regression clinically or by histological ex amination. The clinical course did not parallel the histological appearance , as high cellularity and mitotic figures were commonplace among the specim ens. A misdiagnosis of malignancy was not unusual in this series, as 3 pati ents had an initial diagnosis of fibrosarcoma, which on review was revised to myofibroma. Conclusions: Myofibromatosis is a distinct disorder among the great number of fibrous proliferations occurring in infants and children, with a particu lar predilection for the head and neck region. These lesions should be clea rly distinguished from conventional adult-type fibromatoses (desmoid tumors ), which are more aggressive. Most patients have solitary lesions that resp ond well to conservative surgical excision, whereas a few of these lesions behave more aggressively, requiring several surgical procedures for the man agement of recurrent or persistent tumor. Many of these lesions show sponta neous regression, suggesting that lesions not affecting vital functions, re sulting in growth anomalies, or demonstrating rapid aggressive growth may b e managed conservatively.