Wu Haixiao, Ghanem Ahmed K, Abbas Kirellos Said, Abdalshafy Hassan, Abdelazeem Basel, Wang Hangchi, Zhang Jin, Zhang Chao
Department of Bone and Soft Tissue Tumors, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin's Clinical Research Center for Cancer, Tianjin, China.
The Sino-Russian Joint Research Center for Bone Metastasis in Malignant Tumor, Tianjin, China.
Heliyon. 2024 Aug 29;10(17):e36953. doi: 10.1016/j.heliyon.2024.e36953. eCollection 2024 Sep 15.
Liposarcoma of the bone is an extremely rare and aggressive primary bone tumor. We aimed to review all liposarcoma cases in the literature and present our new young female patient with liposarcoma.
of literature review and case presentation: Electronic databases (PubMed, Scopus, Web of Science (WOS), and Google Scholar) were searched to retrieve the related cases on liposarcoma. Extraction for important clinical data was done independently by two authors to present age, gender, site, histological type, the treatment used, and clinical outcomes of survival or recurrence. In our presented case, we followed the CARE checklist.
A total of 33 patients were included in the literature search: 19 (57.6 %) male and 14 (42.4 %) female, with a mean age of 42.85 ± 18.83. Seven patients (21.2 %) were less than the age of 20 years old. The most frequent subtype was primary pleomorphic liposarcoma. Most treatment options were resection, excision, and amputation with or without chemotherapy. Recurrence was reported in four cases. However, most patients died due to late diagnosis with distant organ metastasis.
we report a case of primary pleomorphic liposarcoma of the left distal femur of a twelve-year-old female. The patient presented with a painful lower thigh swelling, not responding to analgesics. Imaging studies were done and showed a lesion extended across the distal physeal plate laterally and centrally with scattered lesions medially. Histologically, the tumor showed a uniformly pleomorphic liposarcomatous pattern with extensive necrosis. Genetic analysis showed frequent mutations in LATS2, CREBBP, and SMAD2 genes in addition to deletions and amplifications in different genetic pathways. Two cycles of MAP chemotherapy were completed before tumor excision and total left knee replacement, followed by two other MAP cycles postoperatively. Multiple lung metastases were detected on chest CT 10 months postoperatively. The patient died 13 months postoperatively.
Primary pleomorphic liposarcoma can present as sclerotic and aggressive malignant bone tumors at an early age. Excision, MAP, and MAPI chemotherapy are not enough to treat this tumor.
骨脂肪肉瘤是一种极其罕见且侵袭性强的原发性骨肿瘤。我们旨在回顾文献中所有骨脂肪肉瘤病例，并介绍我们新收治的一位年轻女性骨脂肪肉瘤患者。
文献回顾与病例报告：检索电子数据库（PubMed、Scopus、科学网（WOS）和谷歌学术）以获取骨脂肪肉瘤相关病例。由两位作者独立提取重要临床数据，以呈现年龄、性别、部位、组织学类型、所用治疗方法以及生存或复发的临床结果。在我们呈现的病例中，遵循了CARE检查表。
文献检索共纳入33例患者：男性19例（57.6%），女性14例（42.4%），平均年龄42.85±18.83岁。7例患者（21.2%）年龄小于20岁。最常见的亚型是原发性多形性脂肪肉瘤。大多数治疗选择是切除、根治性切除和截肢，伴或不伴化疗。有4例报告复发。然而，大多数患者因诊断延迟伴有远处器官转移而死亡。
我们报告一例12岁女性左股骨远端原发性多形性脂肪肉瘤。患者表现为下大腿疼痛性肿胀，对镇痛药无反应。进行了影像学检查，显示病变横向和中央延伸穿过远端骨骺板，内侧有散在病变。组织学上，肿瘤呈现一致的多形性脂肪肉瘤模式，伴有广泛坏死。基因分析显示，除了不同基因途径中的缺失和扩增外，LATS2、CREBBP和SMAD2基因频繁突变。在肿瘤切除和全左膝关节置换术前完成了两个周期的MAP化疗，术后又进行了另外两个周期的MAP化疗。术后10个月胸部CT检测到多处肺转移。患者术后13个月死亡。
原发性多形性脂肪肉瘤在早期可表现为硬化性和侵袭性恶性骨肿瘤。切除、MAP和MAPI化疗不足以治疗该肿瘤。
