LETTER TO EDITOR
Year : 1999 | Volume
: 9 | Issue : 1 | Page : 35--37
Malignant rhabdoid tumor of kidney
KN Ramachandran, K Sasidharan, P Kusumakumary
Department of Imageology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India
K N Ramachandran
Department of Imageology, Regional Cancer Centre, Thiruvananthapuram, Kerala
|How to cite this article:|
Ramachandran K N, Sasidharan K, Kusumakumary P. Malignant rhabdoid tumor of kidney.Indian J Radiol Imaging 1999;9:35-37
|How to cite this URL:|
Ramachandran K N, Sasidharan K, Kusumakumary P. Malignant rhabdoid tumor of kidney. Indian J Radiol Imaging [serial online] 1999 [cited 2019 Sep 15 ];9:35-37
Available from: http://www.ijri.org/text.asp?1999/9/1/35/28374
Rhabdoid tumour of the kidney (RTK) is a highly malignant neoplasm of very young children. It is prone to widespread metastases.The outcome is poor even with modern forms of aggressive chemotherapy. There are no specific radiologic features to suggest a preoperative diagnosis. We report a case of malignant rhabdoid Tumor of the kidney in an infant, with its imaging features.
A three months old baby was referred to us for abdominal imaging. She had recurrent fever and hematuria of two months duration. She was born after a full-term normal delivery. There was no history of maternal illness during pregnancy. There was no consanguinity. General examination was unremarkable. Abdominal examination revealed a mass in the right lumbar region suggestive of a renal mass.
The hemogram, blood biochemistry and chest radiograph were within normal limits. Ultrasound revealed a 9.0cm x 5.0cm irregular hypoechoic mass almost replacing the right kidney [Figure 1]. There was no calcification in the mass. Intravenous urography showed a dilated right collecting system displaced upwards suggesting a lower pole renal mass [Figure 2]. CT of the abdomen with intravenous contrast revealed an 8.0cm x 6.0cm, hypodense mass in the lower pole of the right kidney with solid and cystic areas and irregular enhancement in the solid areas. The rim of renal tissue noted in the upper pole posteriorly showed uniform contrast enhancement. There was dilatation of the calyces with superior displacement and delay in excretion [Figure 3],[Figure 4] There was no free fluid or enlarged lymph nodes in the abdomen. The left kidney and other viscera were normal. CT of the chest ruled out the possibility of metastases.
At laparotomy there was a large tumor involving the lower pole of the right kidney, infiltrating the mesentery of the ascending colon. The inferior vena cava was encased by the tumor. A nephro-ureterectomy was done, but residual disease was left behind. Histopathological examination revealed typical features of a malignant rhabdoid tumor of the kidney.
RTK is an uncommon renal tumor in children. It is one of the most lethal neoplasms of early life . There are no known radiological features to distinguish it from other renal tumors . The tumor was first identified in 1978, by the pathologists of the National Wilm's Tumor Study . The term 'rhabdomyosarcoid' used in the initial paper was shortened to 'rhabdoid' in the subsequent paper. The classical rhabdoid morphology is characterized by solid proliferation of monotonous tumor cells with vesicular nuclei and prominent nucleoli, abundant cytoplasm and intra-cytoplasmic inclusions . Cells with rhabdoid features have been noted in other tumors, too . On gross pathology, RTKs presented as a bulky mass largely replacing the kidney. There is an association of this tumor with brain tumors of embryonal origin.
The imaging features of RTKs have been reported in small series' only . On US, RTK presents as a mass of mixed or increased echogenicity, although areas of decreased echogenicity may be seen. Calcification may present. Subcapsular fluid collection has been described as a sonographic sign . Our patient presented with mixed echotexture and showed no calcification or subcapsular fluid. The CT features are of a mixed density mass with low attenuation areas involving most of the tumor . A lobulated contour and foci of calcifications have been described. Our case demonstrated a well-defined mass with solid and cystic areas. The solid areas showed marked enhancement after contrast administration. No calcifications were noted. The plain radiographs and intravenous urography were not contributory. With the imaging features it was not possible to diagnose RTK in this child.
The child was put on a chemotherapy regime with vincristine, doxorubicin, dactinomycin etc. The disease progressed rapidly and the baby died two months after surgery.
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