Background: Low-grade myofibroblastic sarcoma (LGMS) is a rare subtype of soft tissue sarcoma of intermediate grade often representing fibromatosis-like features with a rare metastasing behavior. This type of tumor has a predilection for the head and neck region, but also occurs in the extremities. Confirming the diagnosis is difficult and treatment strategies have to be chosen individually.Methods: The objective of this study was to conduct a systematic review for LGMS of the extremities. The electronic databases PubMed and the Cochrane Library were searched for eligible studies. 141 abstracts were screened on PubMed, while 10 studies were identified as eligible. Cases were summarized in terms of clinical aspects, therapeutic regimen with the primary endpoint of follow-up controls regarding local recurrence or distant metastasis. In addition, we present the rare case and surgical management of a 28-year-old male patient with residual LGMS of the thumb after initial incomplete resection. Results: 33 cases of LGMS in the extremities were identified on PubMed. Cochrane library didn’t show any results. All of them were surgically resected. Only two cases of LGMS in the hand were described in literature so far. Treatment options varied from local excision to wide resection without exact definition of the safety distance. 26 cases provided follow-up information with local recurrence in 6 cases (23 %), while 4 cases (15 %) showed distant metastasis. Conclusions: Wide resection should be the surgical aim to avoid local recurrence and distant metastases. While the excision of tumors of the thumb and hand often require closer resection margins, due to the close proximity of anatomical structures, tumor-free margins are elementary even if tissue transfer from a donor site is needed for reconstruction.
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