Desmoid Fibromatosis of the Triceps and Sternocleidomastoid-A Report of Two Cases

Summary

Introduction: Desmoid fibromatosis (DF) is a rare, fibrous neoplasm arising from the musculoaponeurotic structures. It is also named aggressive fibromatosis owing to its rapid progression in spite of the nature. In this case report we want to highlight the unusual site of presentation, the age group, the surgical challenges, behavior of the tumor, and the various treatment methods. Owing to the rarity of diagnosis and unusual sites in our cases, they can be missed which eventually causes significant morbidity. Case report: We present two cases of gradually progressing soft tissue tumors posing significant deformity with functional restrictions, at unusual sites where diagnosis was delayed causing difficulty and loss of function. A 7-year-old female child presented with a history of swelling over the right arm and restriction of movements at the right elbow and shoulder joint approximately for the past 2 years or more. She had a previous history of incision and drainage at jucntion, and middle third arm around 3 years ago, following which a swelling and restriction of movements steadily increased. On presentation to us, higher Imaging revealed a large soft tissue mass involving the triceps, but not infiltrating the bone. Case 2, an 8-month-old child presented with a slow-growing tumor of the right neck causing a severe torticollis deformity. Parents were advised physiotherapy and also had a steroid injection intramuscularly elsewhere, which showed no improvement. On examination, a large, firm swelling along the entire length of the sternocleiodomastoid muscle was causing severe restriction of movements of the neck. An open biopsy done proved both to be a DF which can be locally aggressive if not treated in time. Surgical resection was done for functional improvement. This is the youngest age group reported ever in literature . Conclusion: A simple, safe, and clear surgical resection alone can bring about restoration of functional limitations and can have a disease-free survival in not very aggressive DF tumors provided close monitoring is guaranteed for check of recurrence.