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Adult Wilms’ Tumor

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Rare Genitourinary Tumors

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Abstract

Wilms’ tumor or nephroblastoma is an embryonal cancer of the kidney that occurs primarily in children younger than 5 years. It is an extremely rare tumor among adults, with an incidence of less than 0.2 per million per year. Histologically, adult WT is similar to the childhood counterpart. Presence of anaplasia is a known adverse prognostic factor. It is associated with somatic mutations in tumor suppressor genes such as WT1, WTX, CTNNB-1, and TP53. Wilms’ tumor usually presents with abdominal or flank pain and is clinically indistinguishable from the more common adult malignant renal neoplasms such as renal cell carcinoma. Due to rarity of this tumor in adults, there are no firmly established treatment regimens. Over the years, the use of pediatric treatment protocols, which incorporate multidisciplinary care utilizing surgery, chemotherapy, and radiation, has made a significant impact in improving survival and prognosis in adults. Both neoadjuvant and adjuvant chemotherapies are considered reasonable for localized high-risk disease. Radiation is used to palliate symptoms from metastatic disease and occasionally to debulk localized tumors preoperatively. Chemotherapeutic agents used in combination are vincristine and actinomycin-D with the addition of doxorubicin for high-risk localized disease and for advanced stages as first-line therapy. Salvage chemotherapy consists of ifosfamide, carboplatin, and etoposide as single agents or combination therapy.

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Acknowledgment

We thank Jennifer Beth Gordetsky, MD, and Mark Lockhart, MD, for providing histology and radiology images, respectively. We also acknowledge cancer.sanger.ac.uk and Forbes et al. (2014) COSMIC: exploring the world’s knowledge of somatic mutations in human cancer for providing mutation information.

Potential Conflicts

Ankit Madan: None

Guru Sonpavde: Research support from Onyx, Bayer; Consultant/advisory board of Sanofi, Merck, Genentech, Bayer

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  1. Department of Medicine, Section of Hematology-Oncology, University of Alabama at Birmingham (UAB), Birmingham, AL, USA

    Ankit Madan MD & Guru Sonpavde MD

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  1. Ankit Madan MD
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Correspondence to Guru Sonpavde MD .

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  1. Department of Oncology, Mayo Clinic, Division of Medical Oncology, Rochester, Minnesota, USA

    Lance Pagliaro

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Madan, A., Sonpavde, G. (2016). Adult Wilms’ Tumor. In: Pagliaro, L. (eds) Rare Genitourinary Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-30046-7_5

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