22. TERATOID WILMS TUMOR: A CASE REPORT OF A POOR CHEMOTHERAPY-RESPONSIVE DISEASE WITH A PROMINENT FATTY COMPONENT

Tran Phan Ninh1, Nguyen Thanh Tam1, Tran Thi Thuy1
1 National Children’s Hospital

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Abstract

Teratoid Wilms tumor is a rare variant of Wilms tumor. This entity is characterized by atypical histopathological features, with the presence of various mature heterologous elements resembling those seen in teratomas, including adipose tissue, striated muscle, cartilage, glial tissue, intestinal epithelium, and respiratory epithelium. On imaging, the presence of fat within a renal mass in a pediatric patient may lead to diagnostic confusion with benign lesions such as angiomyolipoma or teratoma. We report a case of a large renal mass predominantly composed of adipose tissue, without calcification, and demonstrating poor response to chemotherapy. Initial core needle biopsy suggested a stromal-predominant Wilms tumor; however, the final diagnosis following complete surgical resection was confirmed as teratoid Wilms tumor.

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References

[1] Wei X, Li T, Wu X. Recurrent extrarenal teratoid Wilms tumor in sacrococcygeal region of children after initial surgery: A rare case. Urol Case Rep, 2025, 60: 102931. doi:10.1016/j.eucr.2025.102931.
[2] Williams M.A, Schropp K.P, Noe H.N. Fat Containing Renal Mass in Childhood: A Case Report of Teratoid Wilms Tumor. J Urol, 1994, 151 (6): 1662-1663. doi: 10.1016/S0022-5347(17)35339-9.
[3] Park C.M, Kim W.S, Cheon J.E et al. Teratoid Wilms tumor in childhood: CT and ultrasonographic appearances. Abdom Imaging, 2003, 28 (3): 0440-0443. doi: 10.1007/s00261-002-0044-0.
[4] Variend S, Spicer R.D, Mackinnon A.E. Teratoid Wilms’ tumor. Cancer, 1984, 53 (9): 1936-1942. doi: 10.1002/1097-0142(19840501)53:9<1936::aid-cncr2820530922>3.0.co;2-w.
[5] Fernandes E.T, Parham D.M, Ribeiro R.C, Douglass E.C, Kumar A.P.M, Wilimas J. Teratoid Wilms’ tumor: The St Jude experience. J Pediatr Surg, 1988, 23 (12): 1131-1134. doi: 10.1016/S0022-3468(88)80328-2.
[6] Sultan I, Ajlouni F, Al-Jumaily U et al. Distinct features of teratoid Wilms tumor. J Pediatr Surg, 2010, 45 (10): e13-19. doi: 10.1016/j.jpedsurg.2010.06.035.
[7] Gupta R, Sharma A, Arora R, Dinda A.K. Stroma-predominant Wilms tumor with teratoid features: report of a rare case and review of the literature. Pediatr Surg Int, 2009, 25 (3): 293-295. doi: 10.1007/s00383-009-2333-y.
[8] Wu W, Wu Y, Xu W, Liu J, Lv Z. Teratoid Wilms Tumor and Classical Wilms Tumor: A Retrospective 10-year Single-Center Study and Literature Review. Front Surg, 2021, 8: 781060. doi: 10.3389/fsurg.2021.781060.
[9] Tchaprassian Z, Mognato G, Paradias G, D’Amore E.S.G, Tregnaghi A, Cecchetto G. Renal angiomyolipoma in children: diagnostic difficulty in 3 patients. J Urol, 1998, 159 (5): 1654-1656. doi: 10.1097/00005392-199805000-00083.
[10] Batur A, Kerimoglu U. Fat containing bilateral synchronous Wilms’ tumor: Imaging findings. East J Med, Published online 2013.