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多发性骨髓瘤相关性髂腰肌肌淀粉样变性瘤首发表现为双侧股神经嵌压。

Multiple myeloma-associated iliopsoas muscular amyloidoma first presenting with bilateral femoral nerve entrapment.

机构信息

Beijing Chao-yang Hospital, Capital Medical University, Beijing, China.

出版信息

Int J Hematol. 2012 Jun;95(6):716-20. doi: 10.1007/s12185-012-1063-4. Epub 2012 Apr 7.

DOI:10.1007/s12185-012-1063-4
PMID:22484545
Abstract

We report a 38-year-old man of multiple myeloma with bilateral femoral nerve entrapment caused by bilateral huge protruding masses in the inguinal areas. The masses were identified as iliopsoas muscular amyloidoma after the operation. He was diagnosed with multiple myeloma 1 year ago before he was admitted to our hospital. He complained of muscle weakness in the bilateral thigh and protruding lumps in the bilateral inguinal areas with tenderness for 6 month. The pelvic MRI revealed round masses in the iliopsoas muscles of bilateral inguinal areas. To implement the nerve decompression, the resection of the bilateral masses was done. The pathological result showed Congo red-positive substance with green birefringence to polarized light in a dense fibrous background. Before the operation, six cycles of chemotherapy with VAD (vincristine, adriamycin, dexamethasone) and two cycles of chemotherapy with PAD (bortezomib, adriamycin, dexamethasone) regimen were performed. One month after the operations, one cycle of chemotherapy with PADT (bortezomib, adriamycin, dexamethasone, thalidomide) regimen was used and the patient reached complete remission. The function of the bilateral femoral nerves restored to normal 7 months after the operation with a Karnofsky score of 100. Twenty-two months follow-up showed that there was no evidence of the recurrence of the iliopsoas muscular amyloidoma and no progression of multiple myeloma.

摘要

我们报告了一例 38 岁男性多发性骨髓瘤患者,双侧股神经受压,原因是腹股沟区有双侧巨大突出肿块。手术后,这些肿块被确定为髂腰肌淀粉样瘤。他在 1 年前被诊断为多发性骨髓瘤,然后住进了我们医院。他主诉双侧大腿肌无力,双侧腹股沟区有突出肿块,并伴有压痛,已经持续了 6 个月。骨盆 MRI 显示双侧腹股沟区髂腰肌有圆形肿块。为了实施神经减压,我们对双侧肿块进行了切除。病理结果显示刚果红阳性物质在致密纤维背景下呈绿色双折射偏光。在手术前,患者接受了 6 个周期的 VAD(长春新碱、阿霉素、地塞米松)化疗和 2 个周期的 PAD(硼替佐米、阿霉素、地塞米松)化疗方案。手术后 1 个月,患者接受了 1 个周期的 PADT(硼替佐米、阿霉素、地塞米松、沙利度胺)化疗方案,患者达到完全缓解。术后 7 个月,双侧股神经功能恢复正常,卡氏评分 100 分。22 个月随访显示,髂腰肌淀粉样瘤无复发,多发性骨髓瘤无进展。

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Int J Hematol. 2012 Jun;95(6):716-20. doi: 10.1007/s12185-012-1063-4. Epub 2012 Apr 7.
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A Case of Abdominal Aortic Retroperitoneal and Mesenteric Amyloid Light Chain Amyloidoma.一例腹主动脉、腹膜后及肠系膜轻链型淀粉样瘤病例。
Case Rep Rheumatol. 2016;2016:4146030. doi: 10.1155/2016/4146030. Epub 2016 Sep 26.

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Imaging of focal amyloid depositions in the head, neck, and spine: amyloidoma.头部、颈部和脊柱局灶性淀粉样沉积的影像学表现:淀粉样瘤。
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Treatment of light chain (AL) amyloidosis with the combination of bortezomib and dexamethasone.硼替佐米与地塞米松联合治疗轻链(AL)淀粉样变性。
Haematologica. 2007 Oct;92(10):1351-8. doi: 10.3324/haematol.11325.
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Muscular amyloidoma presenting as inguinal masses in multiple myeloma.肌肉淀粉样瘤表现为多发性骨髓瘤患者的腹股沟肿块。
Scand J Rheumatol. 2005 Mar-Apr;34(2):152-4. doi: 10.1080/03009740510026355.
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