Myofibrosarcoma of the Head and Neck in Children

Abstract

We have identified a distinctive malignant soft tissue neoplasm that occurred in the head and neck region of six children. Histologically, these neoplasms presented an array of features ranging from low-grade spindle cell to high-grade fibrohistiocytic histologies and often had myoid characteristics. Ultrastructural and immunohistochemical studies indicated that they contained neoplastic myofibroblasts that were variably positive for vimentin (4 positive/4 tested), alpha-smooth muscle actin (4/5), muscle-specific actin (5/5), desmin (2/5), and v-src protein substrate p80/85 (4/5). Three patients died of rapidly progressive unresectable local disease, one died of metastatic and local disease, and two are alive 13 months and 8 years after wide resection. We conclude that these neoplasms form a distinctive subset of pediatric soft tissue sarcomas that display an aggressive clinical behavior, typically with local recurrence, and exhibit features of myofibroblastic differentiation.

Low-grade Sarcomas with CD34-Positive Fibroblasts and Low-Grade Myofibroblastic Sarcomas

“…Myofibroblasts were observed in well-differentiated fibrosarcoma in 1975 [109], and the first ''sarcoma of myofibroblasts'' was reported 3 years later [110]. About 50 cases have since appeared in the literature [104,[111][112][113][114][115][116][117][118][119][120][121][122].…”

Low Grade Myofibroblastic Sarcoma Of The Breast: Case Report And Review Of The Literature

“…(sMiTh Table 2. DM et al, 1995, eyden BP et al, 1991. All but 2 cases, developed in the head and neck, in soft tissue (post-auricular region, face, tonsils, scalp, neck and thyroid) and in bone tissue (maxilla and mandible).…”

Mandibular myofibrosarcoma of childhood: surgical resection & reconstruction with fibula flap

“…Myofibrosarcomas respond poorly to chemotherapy and radiation therapy 3 . In the pediatric population, reconstruction following surgical resection is challenging, both in terms of donor site morbidity and rebuilding the growing facial skeleton.…”