Primary bone carcinosarcoma: Chondrosarcoma and squamous cell carcinoma with keratin pearl formation
Primary bone carcinosarcoma: Chondrosarcoma and squamous cell carcinoma with keratin pearl formation
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DOI：
10.1111/j.1440-1827.2005.01860.x
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发表时间：
2005-08-01
影响因子：
2.2
通讯作者：
Sakamoto, M
中科院分区：
文献类型：
--
作者：
Shiraishi, J;Mukai, M;Sakamoto, M
恶性骨肿瘤伴上皮分化是非常罕见的。原发性恶性骨肿瘤合并鳞状细胞癌及软骨肉瘤仅一例报告。本文报告一例原发性恶性骨肿瘤伴鳞状细胞癌和软骨肉瘤，即所谓的骨癌肉瘤，发生于一位53岁男性的股骨。肿瘤位于股骨内，经刮除诊断为分化良好的软骨肉瘤。在任何其他器官中均未检测到原发性肿瘤。在几个月内，肿瘤迅速向软组织生长，并进行了半骨盆切除术。手术标本的检查显示，肿瘤主要由未分化的梭形肉瘤细胞与散在的软骨肉瘤和鳞状细胞癌与角蛋白珠形成的病灶组成。患者于术后约6个月死亡。尸检时，在心脏、双肺、肌肉和淋巴结中检测到多个转移灶。有趣的是，在几个转移灶中观察到软骨肉瘤和鳞状细胞癌成分。先前报告的病例和本病例的肿瘤均含有软骨肉瘤和鳞状细胞癌的成分，并伴有角蛋白珠形成，这种组织学特征的组合可能是骨癌肉瘤的独特特征。
Malignant bone tumors with epithelial differentiation are extremely rare. Only one case of primary malignant bone tumor with distinct squamous cell carcinoma and chondrosarcoma has ever been reported. Reported herein is a case of primary malignant bone tumor with distinct squamous cell carcinoma and chondrosarcoma, so-called carcinosarcoma of bone, arising in the femur of a 53-year-old man. The tumor was located within the femur and was diagnosed by curettage as a well-differentiated chondrosarcoma. No primary tumor was detected in any other organ. Within a few months the tumor had rapidly grown toward the soft tissue, and hemipelvectomy was performed. Examination of the surgical specimen revealed that the tumor was mainly composed of undifferentiated spindle sarcoma cells with scattered foci of chondrosarcoma and of squamous cell carcinoma with keratin pearl formation. The patient died approximately 6 months postoperatively. At autopsy multiple metastases were detected in the heart, both lungs, muscles, and lymph nodes. Interestingly, the chondrosarcoma and squamous cell carcinoma components were observed in several metastatic foci. The tumors in both the previously reported case and the present case contained components of chondrosarcoma and squamous cell carcinoma with keratin pearl formation, and this combination of histological features may be a unique characteristic of carcinosarcoma of bone.