Patient-centered limb preservation in giant recurrent myxofibrosarcoma: a case-based review of goal-concordant care

Abstract

Myxofibrosarcoma is an infiltrative soft-tissue sarcoma of the elderly with a high propensity for local recurrence; giant, recurrent, infected tumors with sciatic nerve encasement may prompt consideration of amputation when adequate resection is expected to leave a non-functional limb. This report describes a 64-year-old man with a 32-cm recurrent high-grade myxofibrosarcoma (T4N0M0, Stage IIIB) involving the anteromedial and posterior compartments of the left thigh and encasing the sciatic nerve, complicated by Proteus mirabilis bacteremia, who declined hip disarticulation and chemotherapy in a documented living will grounded in functional concerns and personal values. After multidisciplinary counseling, sciatic nerve-sparing limb-salvage surgery was performed with epineural dissection and vascularized muscle coverage; pathology confirmed R1 margins, and intensity-modulated radiotherapy (66 Gy in 33 fractions) was delivered. The patient declined adjuvant chemotherapy. He progressed from a bedridden state to independent ambulation, and at 24 months showed no local recurrence on serial magnetic resonance imaging, with functional and quality-of-life measures documented as a single pre-progression snapshot. At 27 months, massive pulmonary metastasis developed; palliative doxorubicin and ifosfamide were initiated, and the patient died approximately 72 h later after developing suspected ifosfamide-related encephalopathy and respiratory syncytial virus pneumonitis in the setting of advanced metastatic disease. In this patient, durable local and functional control coexisted with fatal systemic progression, underscoring that local control is not cure. The case illustrates, for one individual whose priorities differed from the treatment initially recommended, both the value and the limits of aligning treatment with patient-defined goals.