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Challenging Limb Salvage in a Crural Malignant Peripheral Nerve Sheath Tumor with Pre-existing Long-Stem Implants: A
Behic Celik1, Hasan Tufan Celik1, Omer Yonga1
1Department of Orthopedics and Traumatology, Yeditepe University Hospital, Istanbul, Turkey.
Introduction:
Malignant peripheral nerve sheath tumors (MPNST) are uncommon soft-tissue sarcomas that typically arise in proximal segments of the extremities or pelvis. Their management requires precise biopsy planning, careful surgical assessment, and coordinated multidisciplinary care. Tumors involving multiple longitudinal levels of the leg are rare and pose significant challenges for limb salvage.
Case Report:
We report the case of a 79-year-old male presenting with a rapidly progressive mass extending along the posterior cruris, in the setting of a prior hip hemiarthroplasty and a revision total knee arthroplasty with long femoral and tibial stems. Magnetic resonance imaging demonstrated two heterogeneous solid masses in the posterolateral proximal right cruris and biopsy revealed a high-grade MPNST (Fédération Nationale des Centres de Lutte Contre le Cancer grade 3). The tumor board concluded that limb-sparing resection was unsafe due to tumor extent, limited soft-tissue envelope, and anticipated difficulty achieving durable wound closure. The presence of existing long-stem implants further complicated determination of a safe osteotomy level. Above-knee amputation was therefore selected as the most reliable method for achieving oncologic control while minimizing post-operative complications.
Discussion/Results:
The amputation was performed with fluoroscopy-guided planning of the osteotomy level to avoid stem interference, followed by myoplasty and myodesis for stable stump reconstruction. Histopathology confirmed a high-grade MPNST with diffuse S100 and SOX10 positivity, extensive necrosis, high mitotic activity, and a Ki-67 index of 50%.
Conclusion:
This case illustrates the critical importance of methodical biopsy planning and multidisciplinary coordination in managing sarcomas of the lower extremity. The unusual longitudinal distribution of the tumor, combined with the constraints imposed by pre-existing long-stem implants, made limb salvage infeasible. Individualized surgical planning, incorporating both oncologic and reconstructive considerations, is essential when managing complex MPNSTs in reconstructively compromised limbs.
