Ordóñez N G, el-Naggar A K, Ro J Y, Silva E G, Mackay B
Department of Pathology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Hum Pathol. 1993 Aug;24(8):850-65. doi: 10.1016/0046-8177(93)90135-4.
The clinical, microscopic, and immunohistochemical features of 22 intra-abdominal desmoplastic small cell tumors are presented. Ten tumors also were studied by electron microscopy, and flow cytometry was performed in 11 cases. Nineteen patients were male and three were female; their ages ranged from 17 to 38 years (mean, 27 years). With the exception of one case, which arose in the scrotum, all the tumors originated within the abdomen or pelvis and multiple peritoneal nodules were typical. Five tumors also involved the retroperitoneum. The study demonstrates that the range of histology these tumors can display is broader than has been realized previously. The immunohistochemical and ultrastructural findings show evidence for epithelial, mesenchymal, and neuroendocrine phenotypes, raising the possibility that the tumors may be blastomas. Nine tumors were diploid, one tetraploid, and one aneuploid. Sixteen patients died of widespread metastases 8 to 50 months (mean, 25 months) from the time of diagnosis, and five were alive with known evidence of disease. One patient had no evidence of recurrence, but the follow-up of the case was only 10 months.
本文呈现了22例腹腔促结缔组织增生性小细胞瘤的临床、显微镜及免疫组化特征。10例肿瘤还进行了电镜研究，11例进行了流式细胞术检测。19例患者为男性，3例为女性；年龄范围为17至38岁（平均27岁）。除1例发生于阴囊外，所有肿瘤均起源于腹部或盆腔，典型表现为多个腹膜结节。5例肿瘤还累及腹膜后。该研究表明，这些肿瘤可呈现的组织学范围比之前认识到的更广泛。免疫组化和超微结构结果显示存在上皮、间充质和神经内分泌表型，提示肿瘤可能为母细胞瘤。9例肿瘤为二倍体，1例为四倍体，1例为非整倍体。16例患者在诊断后8至50个月（平均25个月）死于广泛转移，5例患者存活且有已知疾病证据。1例患者无复发证据，但该病例的随访仅10个月。
