Clinical case reveals ALK expression in desmoid fibromatosis, suggesting Crizotinib as effective treatment.
Objectives Desmoid fibromatosis (DF) is a rare myofibroblastic neoplasm with invasive growth and a tendency to local recurrence. A number of patients have been found to suffer from relapse after surgical treatment. Moreover, there are several instances where the DF treated with radiotherapy led to radio-induced sarcoma. Here in our practice, we have revealed that DF can express ALK and adjuvant therapy with Crizotinib is possible after the surgical treatment. Case presentation A young patient was initially diagnosed with DF in the projection of the right shoulder. Initially, the tumour mass was left under strict active surveillance. When the tumour grew aggressively after two years, the patient underwent an interscapulothoracic amputation of the right upper limb followed by targeted adjuvant therapy with Crizotinib. The patient is relapse free for five years. Conclusion DF has been found to have a tendency to local recurrence. Furthermore, radiation therapy has a risk of inadvertently transforming the tumour into sarcoma. Here, we have demonstrated that such patients can be checked for ALK mutation, and an adjuvant therapy with Crizotinib can be recommended.
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Kopylova et al. (2026) studied this question.
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