Urrea Yuly Ramirez, Epstein Jonathan I
The Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21231.
The Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD 21231; The Department of Urology, The Johns Hopkins Medical Institutions, Baltimore, MD 21231; The Department of Oncology, The Johns Hopkins Medical Institutions, Baltimore, MD 21231.
Hum Pathol. 2017 Sep;67:169-175. doi: 10.1016/j.humpath.2017.08.008. Epub 2017 Aug 19.
The association of sarcomatoid carcinoma (SC) with small cell carcinoma (SCC) has not been systematically studied. We identified 39 consult cases between 2001 and 2016 with available slides for review in 28 cases. There were 19 men and 9 women (mean age: 78 years [51-89]). In 26 (92.8%) cases, the sarcomatoid component had nonspecific malignant spindle cells, 4 (14%) chondrosarcoma, 2 (7%) myxoid sarcomatous, 1 (3.5%) osteosarcoma, and 1 (3.5%) rhabdomyosarcoma. The predominant component was SCC in 11 (39%) cases, urothelial carcinoma in 6 (21%), sarcomatoid in 3 (10%), and equal sarcomatoid and SCC in 8 (29%). There were 3 morphological groups: group 1 (18/28 [64%]) showed a gradual transition from SCC to other components; group 2 (5/28 [18%]) had an abrupt transition from SCC to other components; and in group 3 (5/28 [18%]), the SCC was separate from other components. In group 1, 12 (66%) cases of SCC showed a gradual transition to sarcomatoid areas; 3 (17%) to urothelial carcinoma; and 3 (17%) to multiple components including squamous cell carcinoma, urothelial carcinoma, and sarcomatoid. Mortality did not differ based on pathological groups. The 36-month actuarial risk of death was 64.3%. The multitude of different components in these tumors is further evidence of the remarkable ability of carcinoma of the bladder to show divergent differentiation with, in some cases, gradual transition between SCC and other elements including sarcomatoid. Greater recognition of this entity with chemotherapy targeted to the various histological elements may have important therapeutic implications.
肉瘤样癌（SC）与小细胞癌（SCC）的关联尚未得到系统研究。我们在2001年至2016年间确定了39例会诊病例，其中28例有可用切片以供复查。患者中有19名男性和9名女性（平均年龄：78岁[51 - 89岁]）。在26例（92.8%）病例中，肉瘤样成分具有非特异性恶性梭形细胞，4例（14%）为软骨肉瘤，2例（7%）为黏液样肉瘤，1例（3.5%）为骨肉瘤，1例（3.5%）为横纹肌肉瘤。主要成分在11例（39%）病例中为SCC，6例（21%）为尿路上皮癌，3例（10%）为肉瘤样，8例（29%）为肉瘤样与SCC相等。存在3种形态学分组：第1组（18/28 [64%]）显示从SCC到其他成分的逐渐过渡；第2组（5/28 [18%]）从SCC到其他成分有突然过渡；在第3组（5/28 [18%]）中，SCC与其他成分分离。在第1组中，12例（66%）SCC病例显示逐渐过渡到肉瘤样区域；3例（17%）过渡到尿路上皮癌；3例（17%）过渡到包括鳞状细胞癌、尿路上皮癌和肉瘤样在内的多种成分。死亡率在不同病理组之间无差异。36个月的精算死亡风险为64.3%。这些肿瘤中多种不同成分进一步证明了膀胱癌细胞具有显著的分化能力，在某些情况下，SCC与包括肉瘤样在内的其他成分之间存在逐渐过渡。对该实体有更深入的认识并针对各种组织学成分进行化疗可能具有重要的治疗意义。
