|Year : 2013 | Volume
| Issue : 1 | Page : 72-74
Teratoid Wilms' tumor in a child: A report of a rare case
Anuradha Sinha1, Jyoti Prakash Phukan1, Gautam Bandyopadhyay2, Sumana Mukherjee3
1 Department of Pathology, Bankura Sammilani Medical College, Bankura, India
2 Department of Pathology, RG Kar Medical College, Kolkata, India
3 Department of Pathology, NRS Medical College, Kolkata, West Bengal, India
|Date of Submission||23-Apr-2012|
|Date of Acceptance||19-Dec-2012|
|Date of Web Publication||18-May-2013|
Department of Pathology, Bankura Sammilani Medical College, P. O. Kenduadihi, Bankura - 722 102, West Bengal
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Teratoid Wilms' tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. We report a case of teratoid Wilms' tumor in a 2-year-old male. Right sided abdominal mass was the presenting complaint. Ultrasonography of the abdomen showed a mass in the right kidney. Histopathological examination revealed blastemal, epithelial, and mesenchymal components along with areas presenting heterologous elements. More than 75% predominance of squamous differentiation with the keratin pearl formation was observed. The patient underwent nephrectomy and was followed post-operatively for 1 year and was normal.
Keywords: Nephroblastoma, squamous differentiation, teratoid Wilms′ tumor
|How to cite this article:|
Sinha A, Phukan JP, Bandyopadhyay G, Mukherjee S. Teratoid Wilms' tumor in a child: A report of a rare case. Int J App Basic Med Res 2013;3:72-4
|How to cite this URL:|
Sinha A, Phukan JP, Bandyopadhyay G, Mukherjee S. Teratoid Wilms' tumor in a child: A report of a rare case. Int J App Basic Med Res [serial online] 2013 [cited 2020 Jan 29];3:72-4. Available from: http://www.ijabmr.org/text.asp?2013/3/1/72/112248
| Introduction|| |
Wilms' tumor is the most common primary renal tumor of childhood and characterized by recognizable attempts to recapitulate different stages of nephrogensis.  The term teratoid Wilms' tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates.  The classical triphasic combination of blastemal, stromal and epithelial cell types is observed in the vast majority of lesions, although, the percentage of each component is variable. Rarely, other heterologous elements are identified, including squamous or mucinous epithelium, smooth muscle, adipose tissue, cartilage, and osteoid and neurogenic tissue. Fewer than 30 cases of teratoid Wilms' tumor have been reported until date. 
| Case Report|| |
A 2-year-old male with a complaint of right sided abdominal mass of 1 month duration revealed a palpable right abdominal mass on the physical examination. Ultrasonographic examination of abdomen presented a large, sharply demarcated predominantly hyperechoic mass of 7 cm × 5.5 cm × 4 cm in right para vertebral region in the location of normal kidney with areas of hypoechogenicity. No normal kidney tissue was identified on the right side. No calcification is noted. No intravascular echogenic focus was identified on color Doppler sonography. Left kidney was normal in position and echotexture. However, computed tomography scan was not performed as the patient was very poor to afford the test. Pre-operative routine investigations; serum urea, creatinine, and the blood urea nitrogen were normal. Serum alpha feto-protein levels were within normal limits. With a provisional diagnosis of Wilms' tumor, right sided nephrectomy was carried out and the specimen was sent for the histopathological examination.
On gross examination, the tumor measured 6.5 cm × 5 cm × 4 cm and was weighing 250 g. It was well-circumscribed and encapsulated. Cut sections presented homogenous gray-white tumor tissue, soft, and granular in the texture. Ureter and renal veins were not involved by the tumor.
Microscopically multiple sections examined from different areas showed classic triphasic combination of blastemal, stromal, and epithelial cell types [Figure 1]. The tightly packed blue cells are the blastemal elements, immature tubule in the picture is the epithelial element and spindle cells are stromal elements. The heterologous elements are predominant and composed of squamous epithelium with abundant keratin pearl formation [Figure 2] and [Figure 3] and constitute about 75% of the area from where the sections were examined.
|Figure 1: Photomicrograph showing classic triphasic combination of blastemal, stromal and epithelial cell types (H and E, ×100)|
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|Figure 2: Photomicrograph showing heterologous squamous epithelium surrounded by blastemal component (H and E, ×100)|
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|Figure 3: Photomicrograph showing heterologous squamous pearl formation (H and E, ×400)|
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Considering the above histopathological features, a diagnosis of teratoid Wilms' tumor was arrived. The patient was referred to the medical oncology department. No post-operative chemotherapy was advised to consider the diagnosis of teratoid Wilms' tumor. Patient was doing well 1 year post-surgery. After that, the patient was not in contact for further follow-up.
| Discussion|| |
Wilms' tumor constitutes the prototypical example of a neoplastic process that faithfully recapitulates embryogenesis at the morphologic and molecular level. It is seen primarily in infants with 50% of the cases occurring before the age of 3 years and 90% before the age of 6 years. The classic location for Wilms' tumor is the kidney. Right and left kidneys are equally affected.
The term "teratoid Wilms' tumor" was first used by Variend et al. in 1984.  In 1988, Fernandes et al. proposed the criteria of more than 50% heterologous component for the diagnosis of teratoid Wilms' tumor.  Until date, fewer than 30 cases of teratoid Wilms' tumor have been reported.  About 38% of cases are bilateral.  In the present case, the tumor was unilateral and the contralateral kidney was normal. Raised alpha feto-protein levels in occasional cases were reported. ,, Alpha feto-protein levels were normal in the present case. Teratoid Wilms' tumor is predominately a tumor of childhood and only one case occurring in an adult has been reported until now. 
Behavior of teratoid Wilms' tumor is usually not aggressive with a favorable outcome. , Teratiod Wilms' tumor is relatively resistant to chemo and radiotherapy and surgery is the treatment of choice. ,,,, However, a few author recommend chemotherapy in these cases regardless of the tumor size, stage, age at diagnosis, and the histological appearance.  According to one of the reports in literature out of 15 reported cases of teratoid Wilms' tumor, chemotherapy was given in 9 cases and only 1 case showed cytoreductive response.  Resistance to chemotherapy and radiotherapy is thought to be due to the presence of well-differentiated heterologous components. ,, In the present case, as the tumor was limited to kidney and has been completely excised (Stage I), no post-operative chemotherapy was given after reviewing the literature. As there is still controversy regarding treatment, further, research should be carried out before concluding a tight treatment protocol in these cases.
| References|| |
|1.||Maitra A. Diseases of infancy and childhood. In: Kumar V, Abbas AK, Fausto N, editors. Robbins and Cotran Pathologic Basis of Disease. 7 th ed. New Delhi: Elsevier; 2004. p. 504. |
|2.||Fernandes ET, Parham DM, Ribeiro RC, Douglass EC, Kumar AP, Wilimas J. Teratoid Wilms' tumor: The St Jude experience. J Pediatr Surg 1988;23:1131-4. |
|3.||Treetipsatit J, Raveesunthornkiet M, Ruangtrakool R, Sanpaki K, Thorner PS. Teratoid Wilms› tumor: Case report of a rare variant that can mimic aggressive biology during chemotherapy. J Pediatr Surg 2011;46:e1-6. |
|4.||Variend S, Spicer RD, Mackinnon AE. Teratoid Wilms' tumor. Cancer 1984;53:1936-42. |
|5.||Myers JB, Dall'Era J, Odom LF, McGavran L, Lovell MA, Furness P 3 rd . Teratoid Wilms' tumor, an important variant of nephroblastoma. J Pediatr Urol 2007;3:282-6. |
|6.||Song JS, Kim IK, Kim YM, Khang SK, Kim KR, Lee Y. Extrarenal teratoid Wilms' tumor: Two cases in unusual locations, one associated with elevated serum AFP. Pathol Int 2010;60:35-41. |
|7.||Parikh B, Trivedi P, Shukla K. A unilateral teratoid Wilms› tumor with raised serum alpha-fetoprotein level. Indian J Pathol Microbiol 2007;50:317-9. |
|8.||Ashworth MT, Pizer BL, Oakhill A, Spicer RD, Berry PJ. A teratoid Wilms› tumor with raised serum alpha-fetoprotein level. Pediatr Pathol Lab Med 1996;16:853-9. |
|9.||Seo J, Suh YL, Choi HY. Adult teratoid Wilms' tumor with prominent neuroepithelial differentiation. Pathol Int 2009;59:44-8. |
|10.||Cecchetto G, Alaggio R, Scarzello G, Dall'Igna P, Martino A, Bisogno G, et al. Teratoid Wilms' tumor: Report of a unilateral case. J Pediatr Surg 2003;38:259-61. |
|11.||Inoue M, Uchida K, Kohei O, Nashida Y, Deguchi T, Komada Y, et al. Teratoid Wilms' tumor: A case report with literature review. J Pediatr Surg 2006;41:1759-63. |
|12.||Karaca I, Sencan A, Ortaç R, Bostanci-Sencan A, Mir E. Teratoid Wilms' tumor: A case report. Turk J Pediatr 2000;42:242-5. |
|13.||Mukhopadhyay B, Shukla RM, Mukhopadhyay M, Mandi S, Roy D, Bhattacharya MK. Teratoid Wilms› tumor-A rare renal tumor. Urol Ann 2011;3:155-7. |
|14.||Gupta R, Sharma A, Arora R, Dinda AK. Stroma-predominant Wilms tumor with teratoid features: Report of a rare case and review of the literature. Pediatr Surg Int 2009;25:293-5. |
[Figure 1], [Figure 2], [Figure 3]