Case presentation: A 61-year-old woman diagnosed with long standing, therapy resistant CRPS-1 fourteen years prior was referred to our center for assessment on her wish to have her limb amputated, due to recent worsening of the symptoms. A soft tissue lesion was detected on the affected limb and she was sent back to her primary hospital for further study of this lesion and exclusion of an alternative diagnosis. The biopsies were inconclusive, and no underlying causes were determined for her symptoms in her primary hospital. Thus, back at our center, elective amputation of the affected limb was carried out. Results: Post-operative histology of the amputated limb revealed a myxofibrosarcoma, a rare malignant tumor. Corresponding dissemination study, follow-up and treatment was conducted. The patient died from metastatic complications 17 months post-amputation. Conclusion: This case exemplifies how various confusing factors can lead to misdiagnosis of a malignant tumor. Low incidence of sarcomas, a previous CRPS-1 diagnosis of 14 years and inconclusive biopsies conducted in a primary hospital led to this misdiagnosis. Primary amputation was appropriate due to a dysfunctional limb, and size and local invasion degree of the tumor. The most important take away lessons in this case report are the importance of never discarding a neoplasm in presence of a growing mass even in the case of other possible causes and the fact that it merits a thorough diagnostic procedure, as well as the recommendation to conduct diagnostic tests in reference hospitals specialized in the relevant techniques.
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