Rhabdoid tumor of kidney

Introduction:
Rhabdoid tumor is one of the most aggressive tumor in children. Initially it was considered as an aggressive form (Rhabdomyoblastic variant) of Wilms’ tumor as it arises in kidney but later it is separately classified. When the survival rate of Wilms’ tumor exceeds more than 85%, Rhabdoid tumor survival rate is only 20-25% inspite of all therapies. Apart from kidney, it also occurs in brain, liver, soft tissues, lung, skin, and heart. The median age of occurrence is 11 months & majority cases occur below 3 years of age. Here we will describe only Rhabdoid tumor of kidney.

Clinical Presentation:
1. Haematuria
2. Abdominal distension/ mass
3. Fever
4. Symptoms of brain involvement
Enlarged head
Vomitings
Convulsions

Signs:
1. A palpable mass in abdomen
2. High blood pressure
3. signs of raised intracranial pressure (if associated with brain involvement)

Diagnosis:
1. Complete blood count
2. Urine- routine & microscopy
3. Renal function tests
4. Ultrasound of abdomen
5. USG Doppler
6. CT/MRI abdomen
7. CT/MRI brain
8. CT chest
9. Bone scan
10. CT guided FNAC/ Biopsy of mass

Staging:
Stage I
Tumor is limited to the kidney and completely excised. The renal capsule is intact. The tumor is not ruptured or sampled for biopsy before it is removed. (Fine-needle aspiration is excluded from this restriction.) The vessels of the renal sinus are not involved. No evidence suggests tumor at or beyond the margins of resection.
Stage II
The tumor extended beyond the kidney, but it was completely excised. The tumor may regionally extend into the renal sinus or penetrate the renal capsule. Blood vessels outside the renal sinus may contain tumor, but the tumor must be removed en bloc with the tumor. No evidence of tumor at or beyond the margins of resection is present.
Stage III
Residual nonhematogenous tumor is confined to the abdomen. Any of the following may occur: (1) Tumor involves abdominal lymph nodes. (2) The tumor has penetrated the peritoneal surface. (3) Tumor implants are found on the peritoneal surface. (4) Gross or microscopic tumor remains after surgery. (5) The tumor is not completely resectable because of local infiltration of vital structures. (6) Tumoral spillage occurs before or during surgery. (7) Tumor biopsy was performed before resection.
Stage IV
Hematogenous metastases or lymph node metastases are present outside the abdominal and/or pelvic cavity.
Stage V
Tumors are bilateral.

Management:
1. Chemotherapy: Neoadjuvant chemotherapy (not standardized)
ICE (Ifosphamide + Carboplatin + Etoposide) alternating with VAC
(Vincristine + Adriamycin + Cyclophosphamide)
2. Surgery – Radical nephrectomy with retroperitoneal lymph node
Sampling.
3. Radiotherapy- Flank radiation & lung bath in cases of pulmonary
metastasis

Prognosis:
Despite of all the multimodality treatment, the prognosis remains poor.