Ovarian Sarcoma with Pathologic Features of Clear Cell Sarcoma of the Kidney

We report a case of an unusual sarcoma arising in the ovary of an infant girl. Histologically, the tumor was composed of clear, undifferentiated cells set in an arborizing vascular stroma. Immunohistochemical staining was positive only for vimentin. Ultrastructural evaluation demonstrated undifferentiated cells with interdigitating broad cell processes that encompassed irregular electron lucent spaces that contained flocculent extracellular material. Light and electron microscopic features of the tumor resembled a clear cell sarcoma of the kidney. Although the cell of origin is unproven, both tumors may arise from primitive mesenchymal cells that may not be restricted to the kidney. Received April 29, 1999; accepted July 22, 1999.     

Expression of WT-1, Bcl-2, and CD34 by Primary Renal Spindle Cell Tumors in Children

The confident diagnosis of renal spindle cell tumors in children is often difficult. An immunohistochemical study of WT-1, Bcl-2, and CD34 was performed to determine their expression profiles and to assess the potential utility of these immunohistochemical markers in the differential diagnosis of 36 cases of renal spindle cell tumors of childhood. The cases included 11 stromal predominant Wilms tumors, 12 cellular mesoblastic nephromas, 9 clear cell sarcomas of the kidney (CCSK), and 4 monophasic synovial sarcomas. WT-1 was uniformly positive in primitive undifferentiated stromal Wilms tumors (6 of 6) and negative in the differentiating and differentiated stromal elements of Wilms tumors (0 of 5). WT-1 was also negative in cellular mesoblastic nephromas (0 of 12), CCSKs (0 of 12), and synovial sarcomas (0 of 4). Bcl-2 was expressed in all stromal Wilms tumors (11 of 11), all synovial sarcomas (4 of 4), some CCSKs (4 of 9), and none of the cellular mesoblastic nephromas (0 of 12). Although CD34 was absent in the tumor cells of all the tumors studies (0 of 36), CD34 immunohistochemistry nicely demonstrated the evenly distributed septal capillaries characteristic of CCSK in all 9 cases of this tumor. We conclude that a combination of WT-1 and Bcl-2 immunohistochemistry may aid in the distinction of stromal Wilms tumor, monophasic synovial sarcoma, cellular mesoblastic nephroma, and CCSK.     

Familial Adenomatous Polyposis Coli and Clear Cell Sarcoma of the Kidney

Familial adenomatous polyposis coli is an inherited multiple neoplasia syndrome that is associated with an increased risk for development of another primary tumor. We report a case of a 14-year-old boy who had a proctocolectomy for familial adenomatous polyposis coli. He had survived radical nephrectomy, chemotherapy, and radiotherapy for a congenital clear cell sarcoma of the right kidney. Perhaps the presence of the familial adenomatous polyposis gene induces chromosomal instability in affected persons.     

Carcinoma of the Tongue in a Long-Term Survivor of Clear Cell Sarcoma of the Kidney

Squamous cell carcinoma (SCC) of the tongue in persons younger than 30 years of age is an extremely rare neoplasm. We report an unusual case of SCC of the tongue as a second malignancy in a young adult with a history of a stage III clear cell sarcoma of the kidney (CCSK) treated 19 years earlier. Review of the literature reveals a spectrum of cytogenetic abnormalities in SCC; however, aberrations involving the long arm of chromosome 10 have been reported in both SCC and CCSK. Long-term follow-up, with particular attention to the head and neck, may be warranted in children with CCSK.     

Congenital Sacrococcygeal Teratomas: Effect of Gestational Age on Size, Morphologic Pattern, Ploidy, p53, and ret Expression

Prognosis of infants born with sacrococcygeal teratomas (SCTs) correlates with gestational age (GA). The survival rate after 30 weeks of gestation is 75%, compared to 7% before 30 weeks of gestation. Studies correlating GA with size, morphologic composition of teratomas, ploidy or expression of cell cycle control proteins such as p53, and ret [a tyrosine kinase receptor of the GDNF (glial cell line–derived neurotrophic factors)] receptor family may provide information explaining differences in survival. Seven SCTs (GA 21 to 41 weeks), ranging in size from 5 to 15 cm, were evaluated for morphologic composition. DNA ploidy was assessed in mature and immature neural elements. Immunohistochemical reactivity with monoclonal antibodies recognizing p53, and ret was quantitated and correlated with morphological pattern and GA. Relative size of teratomas to infants' weight and content of immature neural tissues correlated inversely with advancement of GA. Yolk sac tumor (YST) and immature tissues showed aneuploid cell populations. Nuclear p53 reactivity was apparent in the teratoma with YST in the microcystic patterns, the neuroectodermal rosettes, and the glandular patterns. Ret reactivity was seen in osteoclasts adjacent to bone formation surrounding developing teeth in an immature teratoma, and in rare mature neural cells of one SCT of 35 weeks GA. The rapid growth of SCT (GA <30 weeks) correlates with increase in immature neural tissues. Our study confirms aneuploidy in YST and suggests aneuploid populations within immature tissues. p53 accumulates in a variety of patterns of YST and may be seen in immature components of SCTs. To understand the possible role of ret, further studies comparing ret expression in immature human tissues are needed. Received February 9, 1999; accepted August 17, 1999.