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Pediatric myofibromatosis of the head and neck
J C Beck1, K O Devaney, R A Weatherly
1Department of Otolaryngology-Head and Neck Surgery, University of Michigan Health System, Ann Arbor, USA.
Insights
Pediatric myofibroma of the head and neck is a distinct fibrous proliferation. While many lesions resolve with conservative surgery, some recur, necessitating further treatment.
Area of Science:
- Pediatric Pathology
- Head and Neck Surgery
- Oncology
Background:
- Pediatric myofibroma is a rare fibrous tumor with a predilection for the head and neck.
- Distinguishing myofibroma from other fibrous proliferations, including aggressive fibromatoses, is crucial for appropriate management.
Purpose of the Study:
- To characterize the clinical and pathological features of pediatric myofibroma in the head and neck.
- To identify diagnostic and therapeutic challenges associated with this condition.
Main Methods:
- Retrospective review of pathology and clinical records of pediatric patients diagnosed with myofibroma.
- Comprehensive literature search of English-language publications on pediatric myofibroma.
Main Results:
- Of 13 pediatric patients, 9 achieved cure with conservative surgical excision.
- Recurrence was observed in 31% of cases, requiring multiple surgical interventions.
- Spontaneous regression occurred in one-third of patients; histological features did not consistently predict clinical behavior, with some cases initially misdiagnosed as malignancy.
Conclusions:
- Pediatric myofibromatosis of the head and neck is a unique entity that must be differentiated from adult-type fibromatoses.
- Solitary lesions typically respond well to conservative surgery, but aggressive or recurrent tumors may require extensive management.
- Conservative management may be suitable for lesions without functional compromise, growth anomalies, or rapid progression, given the potential for spontaneous regression.
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) diagnosed 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 procedures. One third showed spontaneous regression clinically or by histological examination. The clinical course did not parallel the histological appearance, as high cellularity and mitotic figures were commonplace among the specimens. A misdiagnosis of malignancy was not unusual in this series, as 3 patients 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 particular predilection for the head and neck region. These lesions should be clearly distinguished from conventional adult-type fibromatoses (desmoid tumors), which are more aggressive. Most patients have solitary lesions that respond well to conservative surgical excision, whereas a few of these lesions behave more aggressively, requiring several surgical procedures for the management of recurrent or persistent tumor. Many of these lesions show spontaneous regression, suggesting that lesions not affecting vital functions, resulting in growth anomalies, or demonstrating rapid aggressive growth may be managed conservatively.
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