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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Cellular Classification

Table 1. Indications for Germline Genetic Analysis (Screening) of Children and Adolescents with Renal Cell Carcinoma (RCC)a continued...

Desmoplastic Small Round Cell Tumor of the Kidney

Desmoplastic small round cell tumor of the kidney is a rare, small, round blue tumor of the kidney. It is diagnosed by its characteristic EWS-WT1 translocation.[70] (Refer to the PDQ summary on Childhood Soft Tissue Sarcoma Treatment for more information about desmoplastic small round cell tumor of the kidney.)

Cystic Partially Differentiated Nephroblastoma

Cystic partially differentiated nephroblastoma is a rare cystic variant of Wilms tumor (1%) with unique pathologic and clinical characteristics. It is composed entirely of cysts and their thin septa are the only solid portion of the tumor. The septa contain blastemal cells in any amount with or without embryonal stromal or epithelial cell type. Several pathologic features distinguish this neoplasm from standard Wilms tumor. Patients with stage I disease have a 100% survival rate with surgery alone. Patients with stage II disease have an excellent outcome with tumor resection followed by postoperative vincristine and dactinomycin.[10]

Multilocular Cystic Nephroma

Multilocular cystic nephromas are benign lesions consisting of cysts lined by renal epithelium. These lesions can occur bilaterally and a familial pattern has been reported. Multilocular cystic nephroma has been associated with pleuropulmonary blastomas, so radiographic imaging studies of the chest should be followed in patients with multilocular cystic nephroma.[71] Recurrence has been reported following tumor spillage at surgery.[72][Level of evidence: 3iiiA]

Primary Renal Synovial Sarcoma

Primary renal synovial sarcoma is a subset of embryonal sarcoma of the kidney and is characterized by the t(x;18)(p11;q11) SYT-SSX translocation. It is similar in histology to the monophasic spindle cell synovial sarcoma. Primary renal synovial sarcoma contains cystic structures derived from dilated, trapped renal tubules. Primary renal synovial sarcoma occurs more often in young adults and this type of renal tumor should be treated with different chemotherapy regimens than traditional Wilms tumor.[73]

Anaplastic Sarcoma of the Kidney

Anaplastic sarcoma of the kidney is a rare renal tumor that has been identified mainly in patients younger than 15 years. Patients present with a renal mass with the most common sites of metastases being the lung, liver, and bones. These tumors show pathologic features similar to pleuropulmonary blastoma of childhood (see the PDQ summary on Unusual Cancers of Childhood for more information) and undifferentiated embryonal sarcoma of the liver (see the PDQ summary on Childhood Liver Cancer for more information). Optimal therapy for this diagnosis is not clear. In the past, these tumors have been identified as anaplastic Wilms tumor and treated accordingly.[74]

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