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复发性滑膜软骨瘤病的恶性转化:一例报告及文献复习

Malignant Transformation of Recurrent Synovial Chondromatosis: A Case Report and Review.

作者信息

Urwin John W, Cooper Kumarasen, Sebro Ronnie

机构信息

Medicine, Beth Israel Deaconess Medical Center / Harvard Medical School, Boston, USA.

Pathology, Hospital of the University of Pennsylvania, Philadelphia, USA.

出版信息

Cureus. 2019 Oct 4;11(10):e5839. doi: 10.7759/cureus.5839.

Abstract

Chondrosarcoma is the second most common primary malignant bone tumor. While the majority arrive de novo, a minority arise from malignant transformation of benign neoplasms, such as osteochondromas. Rarely, chondrosarcomas have been found to originate from other preexisting lesions, such as synovial chondromatosis. A 44-year-old male with a history of a spinal osteochondroma presented with one year of left hip pain and decreased range of motion. On examination, he had a palpable, irregular fullness in the left groin that was minimally tender to palpation. Radiographs and CT of the hip showed extensive soft tissue calcifications and erosion of the femoral neck. The lesion was debulked surgically and histologically diagnosed as synovial osteochondromatosis with no evidence of atypia or cellularity. One year later, his residual disease progressed and resulted in increasingly limited range of motion. He underwent left total hip arthroplasty with simultaneous debulking and the lesion was once again diagnosed as synovial osteochondromatosis. Two months postoperatively, the patient developed a new focus of calcification around the hip joint that was thought to be recurrent disease. Six months later, due to worsening symptoms, he underwent a repeat CT scan. This scan demonstrated extensive intra-articular disease extending into the iliopsoas bursa and around total hip arthroplasty, as well as a new soft tissue nodule with foci of calcification in the left gluteus maximus. The new lesion was debulked surgically and diagnosed as a grade 1 chondrosarcoma. Chondrosarcoma arising from synovial chondromatosis is a rare presentation of the second most common primary malignant bone tumor. It typically presents as an indolent, slowly growing painful mass of large joints in middle aged men. Conventional radiography shows punctate opacities, while MRI and CT reveal diffuse soft tissue calcification and cortical erosion. Low-grade chondrosarcomas are treated with intralesional curettage and adjuvant therapy, while higher grade chondrosarcomas are treated with wide, en bloc excision. Malignant transformation should be considered in any patient presenting with worsening symptoms and a history of a benign bony lesion.

摘要

软骨肉瘤是第二常见的原发性恶性骨肿瘤。虽然大多数是新发的,但少数起源于良性肿瘤的恶性转化,如骨软骨瘤。很少有软骨肉瘤被发现起源于其他先前存在的病变,如滑膜软骨瘤病。一名44岁有脊柱骨软骨瘤病史的男性,出现左髋部疼痛1年,活动范围减小。检查时,他左腹股沟可触及不规则的饱满感,触诊时仅有轻微压痛。髋部的X线片和CT显示广泛的软组织钙化和股骨颈侵蚀。病变经手术切除,组织学诊断为滑膜软骨瘤病,无异型性或细胞增多的证据。1年后,他的残留疾病进展,导致活动范围越来越受限。他接受了左全髋关节置换术,同时进行了肿瘤切除,病变再次诊断为滑膜软骨瘤病。术后2个月,患者髋关节周围出现新的钙化灶,被认为是复发性疾病。6个月后,由于症状恶化,他接受了重复CT扫描。该扫描显示广泛的关节内疾病延伸至髂腰肌滑囊和全髋关节置换周围,以及左臀大肌内一个有钙化灶的新软组织结节。新病变经手术切除,诊断为1级软骨肉瘤。由滑膜软骨瘤病引起的软骨肉瘤是第二常见的原发性恶性骨肿瘤的罕见表现。它通常表现为中年男性大关节处缓慢生长的无痛性肿块。传统X线片显示点状opacity,而MRI和CT显示弥漫性软组织钙化和皮质侵蚀。低级别软骨肉瘤采用病灶内刮除术和辅助治疗,而高级别软骨肉瘤采用广泛整块切除术。对于任何出现症状恶化且有良性骨病变病史的患者,都应考虑恶性转化。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d93/6827705/ff8649798ef2/cureus-0011-00000005839-i01.jpg

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