Boushabi A, Benali H Ait, Shimi M
Department of Orthopedics and Trauma-surgery, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Tangier, Morocco.
Department of Orthopedics and Trauma-surgery, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Tangier, Morocco.
Int J Surg Case Rep. 2024 Mar;116:109421. doi: 10.1016/j.ijscr.2024.109421. Epub 2024 Feb 21.
The iliac origin Ewing's sarcoma is a highly malignant primitive bone tumor. Its occurrence in adults is very rare. The prognosis for this tumor in adult patients is unfavorable and has a high rate of local recurrence. The main treatment goals include preventing local recurrences and distant metastases. A secondary objective is to maintain the quality of life by avoiding major amputative surgery. The primary aim of this report is to underscore the rarity and significance of the combination of surgery with neoadjuvant chemotherapy for better outcomes.
We present a rare case of Ewing's sarcoma of the right ilium without metastasis in a 50-year-old woman, treated with initial chemotherapy followed by extensive local marginal resection of the pelvic lesion, complemented by perimeter radiotherapy, and concluded with additional chemotherapy. The patient's progress has been satisfactory, with no recurrence observed during a 6-month follow-up period.
The Ewing's sarcoma of the pelvis, being more aggressive than in other locations, presents an unfavorable prognosis, especially in cases of delayed diagnosis associated with large tumors and micrometastases. Therapeutic advancements, such as neoadjuvant chemotherapy, precise radiotherapy, and sophisticated preoperative planning, contribute to improving survival rates. Early diagnosis of Ewing's sarcoma of the ilium remains a challenge due to subtle changes difficult to detect on radiographs and nonspecific clinical symptoms. Ewing's sarcoma of the pelvis has an unfavorable prognosis due to the lack of a major anatomical barrier to tumor spread in this region. The treatment of this disease requires a multidisciplinary approach involving oncologists, radiation oncologists, surgeons, and radiologists. The effectiveness of surgery depends on the possibility of complete resection without excessive morbidity. The clarity of surgical margins influences the prognosis, although the presence of distant metastases remains the worst prognostic factor, with a limited long-term survival of 20 % despite aggressive treatment. Histological grades do not determine the prognosis, and long-term survival is generally reported between 60 % and 70 %, emphasizing the crucial importance of early detection and appropriate therapeutic intervention.
In summary, the case of Ewing's sarcoma in the right ilium highlights the rarity and challenges associated with this highly malignant bone tumor. Despite the unfavorable prognosis often linked to delayed diagnosis in the pelvic region, a multidisciplinary approach, including surgical resection along with neoadjuvant chemotherapy and precise radiotherapy, shows promise in improving outcomes. The satisfactory progress of the patient over 6 months underscores the potential effectiveness of this treatment plan.
髂骨原发尤因肉瘤是一种高度恶性的原发性骨肿瘤。其在成人中极为罕见。成年患者中这种肿瘤的预后不佳,局部复发率很高。主要治疗目标包括预防局部复发和远处转移。次要目标是通过避免大型截肢手术来维持生活质量。本报告的主要目的是强调手术联合新辅助化疗以获得更好疗效的罕见性及重要性。
我们报告一例罕见的50岁女性右髂骨尤因肉瘤,无转移,初始接受化疗,随后对盆腔病变进行广泛局部边缘切除,并辅以周边放疗,最后追加化疗。患者病情进展令人满意,在6个月的随访期内未观察到复发。
盆腔尤因肉瘤比其他部位的更具侵袭性,预后不佳,尤其是在与大肿瘤和微转移相关的诊断延迟的情况下。新辅助化疗、精确放疗和精细的术前规划等治疗进展有助于提高生存率。由于X线片上难以检测到细微变化且临床症状不具特异性,髂骨尤因肉瘤的早期诊断仍然是一项挑战。盆腔尤因肉瘤预后不佳,因为该区域肿瘤扩散缺乏主要的解剖屏障。这种疾病的治疗需要肿瘤学家、放射肿瘤学家、外科医生和放射科医生的多学科方法。手术的有效性取决于在不过度增加发病率的情况下完全切除的可能性。手术切缘的清晰度影响预后,尽管存在远处转移仍然是最糟糕的预后因素,尽管积极治疗,长期生存率也仅为20%。组织学分级不能决定预后,一般报道长期生存率在60%至70%之间,强调了早期检测和适当治疗干预的至关重要性。
总之,右髂骨尤因肉瘤病例凸显了这种高度恶性骨肿瘤的罕见性及挑战。尽管盆腔区域的诊断延迟通常与不良预后相关,但包括手术切除联合新辅助化疗和精确放疗在内的多学科方法在改善疗效方面显示出前景。患者6个月来的良好进展强调了该治疗方案的潜在有效性。