4.5 Malignant Rhabdoid Tumor of the Kidney

149

kidney. It has been hypothesized that a germ-

•

To try to improve survival, a new regimen of

line INI mutation may predispose a child to

chemotherapy consisting of carboplatin-

these tumors.

etoposide alternating with cyclophosphamide

•

All

rhabdoid tumors share deletions in the

was used. This however did not improve

long arm of chromosome 22, mapped to the

outcomes.

INI1 gene, believed to be a tumor suppressor.

•

Recent reports have documented successful

• The following features help differentiate

outcomes in patients with metastatic malig-

Malignant rhabdoid tumor of the kidney from

nant rhabdoid tumor treated with ifosfamide-

Wilms tumor:

carboplatin-etoposide

(ICE)

or

– Subcapsular fluid collections: a rare find-

ifosfamide-etoposide (IE) alternating with

ing in other renal tumors.

vincristine-doxorubicin-cyclophosphamide

– Tumor lobules separated by hypoattenuat-

(VDC).

ing areas of necrosis or hemorrhage

•

On

the

basis

of

these

reports,

– Calcifications

cyclophosphamide-carboplatin-etoposide

• More common than in Wilms tumors

(CCE) alternating with VDC is the main treat-

•

Typically

linear distribution

outlining

ment now.

tumor lobules

•

Radiation therapy is a cornerstone of treat-

– Vascular and local invasion is more

ment for CNS malignant rhabdoid tumor of

common

the kidneys.

• High dose chemotherapy with stem cell rescue

are used to treat non-CNS malignant rhabdoid

4.5.6

Treatment and Outcome

tumor.

•

The prognosis for patients with a rhabdoid

•

The

treatment of children with malignant

tumor of the kidney is much worse than that of

rhabdoid tumor of the kidney is surgical resec-

patients with other malignant renal tumors.

tion followed by chemotherapy.

•

In contrast to a Wilms’ tumor, malignant rhab-

• The tumor should be removed en bloc to avoid

doid tumor of the kidney is characterized by:

tumoral spillage into the peritoneal cavity

– Early onset of local and distant

because this spillage increases the stage of the

metastases.

tumor. If the mass involves the upper pole of

– Resistance to chemotherapy.

the kidney, the adrenal gland should be

– Whereas the overall survival rate for

removed.

Wilms’ tumors exceeds 85 %, the survival

• Lymph nodes from the iliac, para-aortic, and

rate for rhabdoid tumor of the kidney is

celiac areas should be sampled, even if they do

only 20–25 %.

not appear abnormal.

– Gross hematuria is a presenting feature in

• If the tumor is bilateral or unresectable, biopsy

approximately 60 % of patients with malig-

should be performed and preoperative chemo-

nat rhabdoid tumor of the kidney. By

therapy is started to shrink the tumor and facil-

contrast, only 20 % of patients with Wilms’

itate subsequent resection.

tumor have gross hematuria.

• Chemotherapy for malignant rhabdoid tumor

– Fever is a presenting symptom in 50 % of

of the kidney was historically based on ther-

patients with a malignant rhabdoid tumor

apy for a Wilms’ tumor.

of the kidney, compared with 25 % of

•

This

included

vincristine,

actinomycin, and

patients with a Wilms’ tumor.

doxorubicin

with

or

without

– Hypertension is observed in up to 70 % of

cyclophosphamide.

patients with malignant rhabdoid tumor of

• With these agents, the estimated survival rate

the kidney.

for patients with malignant rhabdoid tumor of

•

Malignant rhabdoid tumor of the kidney is a

the kidney was only 23 %.

rapidly progressive tumor, with most deaths