Ayala A G, Ro J Y, Raymond A K, Jaffe N, Chawla S, Carrasco H, Link M, Jimenez J, Edeiken J, Wallace S
Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston 77030.
Cancer. 1989 Nov 15;64(10):2162-73. doi: 10.1002/1097-0142(19891115)64:10<2162::aid-cncr2820641031>3.0.co;2-p.
We report a study of 27 patients with small cell osteosarcoma (SCO), 17 from the M. D. Anderson Cancer Center (MDAH) and ten from the Pediatric Oncology Group (POG). There were 12 male patients and 15 female patients; 19 were white, five were black, and three were Hispanic. They ranged from 6 to 28 years of age with a median of 14 years. Histologically there were three patterns: Ewing's-like, lymphoma-like, and spindle cell. All cases showed osteoid formation and a few had chondroid areas. There was cytoplasmic glycogen in ten cases. Initial treatment for MDAH patients included intraarterial infusion of cisplatin in ten, amputation in four, partial mandibulectomies in two, and biopsy with local radiotherapy and systemic chemotherapy in one. All POG patients had resection or amputation followed by adjuvant chemotherapy. Twelve patients are alive, of whom nine have had significant follow-ups for 25 to 90 months. Fourteen patients are dead of lung, spine, and brain metastases from 1 to 23 months after initial diagnosis. One patient is alive with lung relapse at 4 months. In summary, SCO is a high-grade variant of osteosarcoma, with an incidence of up to 4% of all osteosarcomas, that affects patients of the same age group and has the same anatomic location as conventional osteosarcoma. Currently, SCO appears to have a prognosis that is the same as or slightly worse than that of conventional osteosarcoma. Furthermore, although intraarterial infusion is effective for the primary tumors in the bone, distant metastases are difficult to control.
我们报告了一项对27例小细胞骨肉瘤（SCO）患者的研究，其中17例来自MD安德森癌症中心（MDAH），10例来自儿童肿瘤学组（POG）。患者中有12名男性和15名女性；19人为白人，5人为黑人，3人为西班牙裔。年龄范围为6至28岁，中位数为14岁。组织学上有三种模式：尤文氏肉瘤样、淋巴瘤样和梭形细胞。所有病例均显示骨样形成，少数病例有软骨样区域。10例病例中有细胞质糖原。MDAH患者的初始治疗包括10例动脉内输注顺铂、4例截肢、2例部分下颌骨切除术以及1例活检加局部放疗和全身化疗。所有POG患者均接受了切除或截肢手术，随后进行辅助化疗。12例患者存活，其中9例进行了25至90个月的有效随访。14例患者在初次诊断后1至23个月死于肺、脊柱和脑转移。1例患者在4个月时出现肺部复发仍存活。总之，SCO是骨肉瘤的一种高级别变体，在所有骨肉瘤中发病率高达4%，影响相同年龄组的患者，且与传统骨肉瘤具有相同的解剖位置。目前，SCO的预后似乎与传统骨肉瘤相同或略差。此外，尽管动脉内输注对骨原发性肿瘤有效，但远处转移难以控制。
