30-Years-Outcome Following Limb Salvage with Endoprosthetic Reconstruction in a Ewing Sarcoma of the Lower Limb Involving Multiple Revisions and Complications

Introduction: The primary malignant bone tumors of the lower extremities are rare tumor entities occurring mostly amongst adolescent and young adult age groups. In a metastasis free situation with adequate therapy, the overall survival rate can reach up to 90%. Since the end of the 20th century limb salvage has become the standard therapeutic procedure partially as a result of improved prosthetic reconstruction techniques.

Late consequences of limb salvage are seldom investigated; however, when assessing the quality of life, we may include the functionality of the extremity, the risk of complications and everyday suitability of the limb.

Case presentation: Here we present a 36-year-old male patient that was diagnosed with a primary Ewing sarcoma of the distal diametaphyseal region of the right femur at the age of nine. Subsequent to radical tumor resection an endoprosthetic reconstruction with distal femur replacement including knee joint was performed. After skeletal maturity a definitive prosthesis was implanted. The patient had several revisions due to mechanical failures of the prosthesis and implant associated infections in the following years with an overall hospital stay of 437 days with multiple changes of the prosthesis. Despite, the patient reports being satisfied with the outcome, enjoying excellent function and a high quality of life 30 years after the initial diagnosis. The patient confirmed at the last follow-up that he would never choose any other therapeutic option, only to keep his own leg.

Conclusion: This case report highlights that limb salvage, despite its potentially great financial costs, personal effort, and possible complications, can be an effective approach for achieving functionally beneficial outcome and patient satisfaction in lower extremity sarcoma. Nonetheless, in certain instances, multiple interventions spanning from minor adjustments to highly intricate revisions may be necessary to preserve the functionality of the limb.

The primary malignant bone tumors of the lower extremities are rare tumor entities. Beside osteosarcoma, Ewing sarcoma is the one of most prevalent type of bone sarcoma. The age range at which patients typically seek medical attention for this condition is primarily during adolescence and young adulthood [1,2]. Since the primary treatment approach is multidisciplinary and multimodal, patients with lower extremity sarcoma are typically treated at specialized sarcoma centers [3].

Limb salvage and amputation are the two main surgical approaches performing radical resection. Both methods involve a range of techniques aimed at retaining or restoring lower limb function [4,5]. Since the end of the 20th century, limb salvage has emerged as the standard therapeutic procedure due to advancements in diagnostics, therapy, and surgical techniques [2,5-7] Similar overall survival rates have been demonstrated between limb salvage and amputation in various studies [1,5,8]. According to the current standards, limb salvage is the preferred method whenever feasible [6,7,9,10].

Following appropriate treatment and successful recovery from the disease, a close to normal life expectancy is possible [9,11], which is characterized by the consequences of the primary treatment modality. Apart from oncological considerations, other factors such as the risk of further revisions or complications, functionality of the limb, psycho-socio-economic consequences, and the costs of treatment and aftercare need to be taken into account [11,12].

We present a male patient, who was 9-years-old when local swelling and tenderness on the tight were recognized. Based on imaging studies (Figure 1) and confirmed through a bone biopsy, Ewing sarcoma of the right femur in the distal diametaphyseal region was diagnosed (Table 1). After 4 months of neoadjuvant chemotherapy through a permanent venous port, radical resection of the tumor was performed with implantation of the first endoprosthesis (distal femur with knee joint). The pathological examination report showed a 10 cm, high-grade tumor mass without involvement of the lymph nodes and with tumor free resection margins. Metastasis or local recurrence was not detected in the follow-ups. After one tumor-free year, an expandable prosthesis was implanted. During the growth phase, the prostheses needed extending. The tibia component was exchanged as a consequence of aseptic loosening; in addition, the extensor apparatus needed refixation due to extensive bone loss with segmental defect of the proximal tibia. Up until the end of the patient’s growth phase, 24 operations-ranging from simpler to more complex-had been performed before the definitive endoprosthesis was implanted.

Figure 1: Anteroposterior plain x-ray image of the right distal femur and knee joint at the initial presentation in the 9-year-old child shows spiculated periosteal reactions and decent bone expansion with central cystic components. A bone biopsy confirmed the diagnosis of a Ewing sarcoma in the distal diametaphyseal region of the right femur.

The patient had an active lifestyle and had several occurrences in the following years. One of which was an implant associated infection with streptococci 9 years after primary surgery-probably due to a septic pneumonia acquired on a canal cruise which was treated with prosthesis retention. Almost two years later the prosthesis broke due to mechanical stress on a tractor tour. The second acute infection at age 24 was treated with implant retention. However, a later prolonged, burdensome chronic infection was treated with a two-stage prosthesis exchange. For definitive infection control, a prosthesis with silver coating was implanted at the age of 31. Finally, this procedure resulted in long-term quiescence.

However, at the age of 36, the patient presented unplanned for a consultation due to pain and dysfunction as he was unable to bear weight on the prothetised leg. The x-ray showed a prothesis breakage at the proximal modular part and an instability of the constrained knee prosthesis (Figure 2), which was treated with two consecutive operations. First, the broken component was removed and the remaining intact distal part of the modular endoprosthesis was temporary stabilized insitu. During surgery, a non-odorous fluid with a gray pseudocapsule was observed; although an infection was ruled out, a local metallosis was confirmed. During the second surgery, the meanwhile custom-made proximal part of the prosthesis was successfully implanted and the whole femoral part including the coupling mechanism of the knee joint exchanged (Figure 3). Surgeries were performed by the senior author (AMN). The postoperative course was incidence-free with rapid recovery of the function of the lower extremity. Eighteen months after surgery, during routine follow-up, the patient showed his meticulously collected self-written notes, documenting all his diagnoses, operations, names of attending surgeons, hospital stays, and prescribed antibiotics. In addition, he had satisfying functionality in activities of daily living (Figure 4), and see also the supplementary video), and proudly let us know that he will soon reach the 1000km distance-mark with his e-bike. We quote: “Despite the many operations I have had, I would repeat the procedure and take the same path again to keep my own leg”. 

Figure 2: At age of 36 the ap x-ray shows the breakage and dislocation of the proximal part of the megaprosthesis at the level of the junction between the well osseointegrated intramedullary shaft and the modular diaphyseal part (arrowhead). In addition, the knee joint replacement shows severe instability (arrow). X-ray ruler in centimeters.

Figure 3: 2.5 years after the last revision surgery the ap x-ray shows the proximal part of the right femur megaprosthesis with the previously implanted custom made intramedullary proximal part still in place (a, arrowhead), and a stable knee joint (b, arrow). X-ray ruler in centimeters.

Figure 4: Clinical image shown concurrently with figure 3 the patient uses his own leg with excellent function in activities of daily living. Note the typical skin discoloration of the right operated lower extremity, well known due to the use of a silver coated prosthesis.

The diagnosis of a bone sarcoma at a young age and its definitive treatment option has a significant long-term implication. In addition to purely oncological considerations, the risk of further revision or complications, the functionality of the extremity, the psycho-socio-economic consequences, and the cost of treatments and aftercare need to be considered [11,12]. Especially since after adequate treatment and a tumor free recovery from the disease a near normal life expectancy can be anticipated [6,7].

The objective comparison of all different therapeutic options is likely impossible due to the disease’s rarity and many variables. As a consequence, the scientific literature offers low level of evidence, making it challenging to decide on the proper surgical path [13-15].

The use of endoprosthesis to preserve the lower limb at a young age raises complex questions. Due to the enormous complexity, there has not been and probably will not be a study in the future that covers all treatment aspects and enables an evidence-based decision on optimal treatment [1,13-15]. A patient's psychological well-being may be enormous negatively affected by amputation, especially if they had hoped to preserve the limb. Regarding to overall quality of life, patient satisfaction or psycho-socio-economic outcome, there is no significant difference between limb salvage and amputation over the long-term. Although, limb salvage may offer functional advantages in daily life [1,11,12,14,16-18].

Further operations are anticipated as part of the treatment process [11,13]. These additional procedures could range from trivial adjustments to the soft tissue or prosthesis to more complex interventions. In case of major complications, such as infections, prosthesis breakage or loosening, or periprosthetic fractures, more substantial reconstructive interventions may be necessary. The proposed classification system of limb salvage failures by the International Society of Limb Salvage according to Henderson et al. encompasses a proper identification of the problem and aids in the comparison of outcomes in a scientific manner. In principle, it describes six basic types of failures, which are further categorized as follows: Type 1 soft-tissue failure (A: functional, B: coverage), Type 2 aseptic loosening (A: <2 years, B: >2 years after implantation), Type 3 structural failure (A: implant, B: bone), Type 4 infection (A: <2 years, B: >2 years after implantation), Type 5 tumor progression (A: soft-tissue, B: bone), and Type 6 pediatric failure (A: physeal arrest, B: joint dysplasia) [19].

In such complex cases, complications evoke strenuous challenges in preserving the previous functionality of the limb [20]. Current orthopedic practice, with close collaboration with other medical disciplines and industry, can effectively address these challenges in most cases. These treatment approaches are often associated with staged operations, resulting in longer hospital stays, a greater burden on both the patient and medical staff, and increased costs.

In the case of our patient, the complications occurred to an extreme extent. Although we are not able to present a summary of medical costs, the authors certainly assume that in the demonstrated case the cost of limb salvage significantly exceeded what an amputation and their follow-up would have cost, counter to the literature [21]. This was an enormous personal, medical, and financial investment on behalf of the patient, the medical team, and insurance company to avoid an ablative surgery. We have demonstrated this with a satisfied patient, who will fight for his own leg later on, and would probably be never opt for an amputation; even in the full knowledge that a seemingly stable condition of health may once again require revision in the future.

At sarcoma centers, professionals have the responsibility to guide patients in selecting the most appropriate therapeutic path. In the informed consent process, effective communication regarding the long-term risks, benefits, and drawbacks of various treatment options is crucial.

Limb salvage is an effective approach to achieving high functionality and patient satisfaction in malignant bone tumors of lower extremity. The measures often require high efforts from both patients and medical caregivers and are associated with high costs; however, the overall outcome can be favorable even in the case of multiple revisions and complications.

The authors thank Ms. Tamara Horn Lang PhD, and Mr. Finbar Dineen for the kind interim reviewing of the manuscript.

Conflicts of Interest

The authors declare no conflict of interest.

Funding

This research did not receive any specific grant.

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