Okada K, Hasegawa T, Tateishi U, Endo M, Itoi E
Department of Orthopedic Surgery, Akita University School of Medicine, Akita, Japan.
J Clin Pathol. 2006 Nov;59(11):1200-2. doi: 10.1136/jcp.2005.029629.
A 35-year-old Japanese man was admitted to the National Cancer Center, Tokyo, Japan, in December 2000, with a 2-month history of pain around the left thigh. Radiographs showed a poorly demarcated osteolytic lesion with focal mineralisation and endosteal scalloping in the left proximal femur. Biopsy showed a proliferation of highly anaplastic cells without any cartilaginous component. A wide excision of the left proximal femur with a replacement by endoprosthesis was carried out in February 2001 after treatment with methotrexate and 20 Gy radiation therapy. Pathological examination of the surgical specimen showed a focus of low-grade chondrosarcoma and the coexistence of telangiectatic osteosarcoma-like features. The patient was diagnosed with dedifferentiated chondrosarcoma with telangiectatic osteosarcoma-like features. Lung metastasis appeared in July 2001 despite an adjuvant chemotherapy including methotrexate, cis-platinum and doxorubicin. The latest follow-up study in June 2004 showed multiple lung metastases. Establishing a definitive diagnosis of dedifferentiated chondrosarcoma may be difficult with limited small biopsy specimens. Dedifferentiated chondrosarcoma should be included in the differential diagnosis of osteolytic tumours with focal calcification and endosteal scalloping even if an extraosseous tumour component is not identified.
一名35岁的日本男性于2000年12月被收治入日本东京国立癌症中心，其左大腿周围疼痛已有2个月。X线片显示左股骨近端有一个边界不清的溶骨性病变，伴有局灶性矿化和骨内膜扇贝样改变。活检显示高度间变细胞增殖，无任何软骨成分。2001年2月，在接受甲氨蝶呤和20 Gy放射治疗后，对左股骨近端进行了广泛切除并植入假体。手术标本的病理检查显示有一个低级别软骨肉瘤病灶，并伴有毛细血管扩张性骨肉瘤样特征。该患者被诊断为具有毛细血管扩张性骨肉瘤样特征的去分化软骨肉瘤。尽管进行了包括甲氨蝶呤、顺铂和阿霉素在内的辅助化疗，但2001年7月仍出现了肺转移。2004年6月的最新随访研究显示有多处肺转移。对于有限的小活检标本，可能难以明确诊断去分化软骨肉瘤。即使未发现骨外肿瘤成分，去分化软骨肉瘤也应列入伴有局灶性钙化和骨内膜扇贝样改变的溶骨性肿瘤的鉴别诊断中。
