|Year : 2015 | Volume
| Issue : 2 | Page : 326-328
Teratoid Wilms tumour with chemotherapy resistance
Renuka Gahine, Shruti Srivastava, Rabia Parveen Siddiqui, Vikas Bombeshwar
Department of Pathology, Pt JNM Medical College, Raipur, Chattisgarh, India
|Date of Web Publication||16-Dec-2015|
Department of Pathology, Pt JNM Medical College, Raipur, Chattisgarh
Source of Support: None, Conflict of Interest: None
We present a case of Teratoid Wilms tumour (a rare histologic variant) in a 4 year old male who presented with an abdominal lump. Wilms Tumour with paracaval lymphadenopathy and tumour thrombi in right renal vein and inferior vena cava was made radiologically. FNAC report was suggestive of Wilms tumour and patient was subjected to 6 cycles of chemotherapy with not much reduction in size. Post nephrectomy histological diagnosis of Teratoid Wilms tumour was established. Resistance to chemotherapy and radiotherapy is thought to be due to presence of well differentiated histologic appearance. Teratoid Wilms tumour is usually not an aggressive neoplasm and prognosis is comparatively neoplasm and prognosis is comparatively good if the tumour is excised completely thus surgery being the best treatment.
Keywords: Nephroblastoma, squamous differentiation, teratoid Wilms tumor
|How to cite this article:|
Gahine R, Srivastava S, Siddiqui RP, Bombeshwar V. Teratoid Wilms tumour with chemotherapy resistance. Arch Med Health Sci 2015;3:326-8
| Introduction|| |
Teratoid Wilms tumor is a rare histologic variant of nephroblastoma reported mainly in pediatric patients. To the best of our knowledge, less than 32 cases have been described in literature. ,, We are presenting another case with review of literature. Metastasis is rare in Wilms tumor. Our patient had nodular lesions in lung suggestive of metastasis at the time of presentation.
| Case Report|| |
A 4-year-old boy presented in the Department of Pediatrics with complaints of abdominal lump and intermittent fever since 2 months. There was no associated complaint of hematuria or dysuria; blood pressure was normal. Palpation revealed a well-defined, non-tender, hard abdominal lump extending from right hypochondrium to lumbar region. Hemoglobin was 9.0 g/dl. Urine examination showed microscopic hematuria and culture was sterile.
Contrast-enhanced computed tomography (CECT) abdomen showed a heterogeneously enhancing solid mass lesion measuring 102 × 95 × 101 mm replacing nearly the whole of the right kidney just sparing its upper pole. There was moderate hydronephrosis with poor contrast excretion. Left kidney was normal in size, shape with good excretory function. A diagnosis of right renal mass? Wilms tumor with paracaval lymphadenopathy and tumor thrombi in right renal vein and inferior vena cava (IVC) was made with incidental findings of nodular lesion in both lung bases most probably representing metastasis.
A diagnosis of Wilms tumor was made on ultrasonography (USG)-guided fine-needle aspiration (FNA) smears and patient was referred to oncology department to receive 6 cycles of chemotherapy. Post-chemotherapy CECT showed tumor measuring 9.2 × 7.8 × 7.3 cm with thrombus in the IVC and renal vein. There was a decrease in the size of lung nodularities but a small pleural-based nodular lesion was still there.
Laparatomy was done and right radical nephrectomy was performed with removal of paracaval lymph nodes. The contralateral kidney was found to be normal on examination. Post-operative period was uneventful.
Grossly, the tumor measured 13 × 9 × 5 cm, well-circumscribed. Cut surface was gray white with small cystic and yellowish areas [Figure 1]. Adrenal glands were not seperately identifiable. Ureter was not involved by tumor.
Microscopically, multiple sections examined from different areas showed classical triphasic pattern of Wilms tumor with blastemal element as tightly packed blue cells and stromal element as spindle cells. Heterologous elements were predominant and constituted about 70% area of the tumor. Mesenchymal differentiation was present in the form of skeletal muscle, adipose tissue, smooth muscle, and cartilage along with epithelial differentiation in the form of squamous epithelium and cysts lined by flattened epithelium [Figure 2] [Figure 3] [Figure 4]. Tumor was seen infiltrating the surrounding parenchyma, IVC, and adjacent lymph nodes.
|Figure 4: Figure showing large areas of cartilagenous, and spindle cell differentiation|
Click here to view
| Discussion|| |
Teratoid Wilms tumor, a rare histologic variant of Wilms tumor, is primarily a pediatric tumor with most of the patients reported between 2-4 years of age. Only one case has been reported in an adult.  Careful review of literature showed less than 35 cases reported in literature. The term teratoid Wilms tumors was first used by Variend et al.,  in 1984. Criteria of more than 50% heterologous component for the diagnosis of teratoid Wilms tumor was proposed by Fernandez et al., in 1988. 
The differential diagnosis of teratoid Wilms tumor is intrarenal teratoma, metastatic germ cell tumor and retroperitoneal infiltration by teratoma.  In our case normal renal parenchyma was present at one end, with classical blastemal pattern of Wilms tumor. Skin adnexal structure formation was not present. Patient had normal testis and radioimaging showed no infiltration of kidney by a retroperitoneal mass. Thus, all other differential diagnosis were ruled out.
Behavior of teratoid Wilms tumor is usually not aggressive and surgery has been recommended as the treatment of choice due to teratoid Wilms tumor being relatively resistant to chemotherapy. ,,,, However, a few authors recommend chemotherapy in these cases regardless of the tumor size, stage, age at diagnosis, and histologic appearance.  According to one of the reports in literature, out of 15 reported cases of teratoid Wilms tumor, chemotherapy was given in nine cases and only one case showed cytoreductive response.  Resistance to chemotherapy and radiotherapy is thought due to the presence of well-differentiated histologic appearance. Our patient also showed resistance to chemotherapy.
Teratoid Wilms tumor with a predominant squamous component and higher potential for metastasis are reported in literature.  In our patient, squamous component was present though not predominant but patient had nodular opacities in lung suggestive of metastasis.
To conclude Teratoid Wilms is a rare histological variant of Wilms tumor reported mainly in pediatric patients. Teratoid Wilms tumor is usually not an aggressive neoplasm and prognosis is comparatively good if the tumor is excised completly, for this reason surgery seems to be the best treatment. We suggest that presence of squamous component whether normal or dysplastic may be associated with metastasis and teratoid Wilms tumor with squamous differentiation should be considered at a higher stage.
Further research regarding treatment protocols and association of squamous component with higher stage of tumor is required.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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