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Pediatr Blood Cancer 2011;57:423– 428

Rhabdoid Tumors of the Liver: Rare, Aggressive, and PoorlyResponsive to Standard Cytotoxic Chemotherapy

Angela D. Trobaugh-Lotrario, MD,1* Milton J. Finegold, MD,2 and James H. Feusner, MD3

INTRODUCTION

Rhabdoid tumors in children are rare, aggressive tumors that

have been reported most commonly in the central nervous system

and kidneys, but can also occur in other organs. In general, the

diagnosis of rhabdoid tumor appears to confer a poor prognosis, but

little has been reported regarding those tumors which are primary

in the liver. Therefore, we undertook an investigation of all the

published reports of malignant rhabdoid tumor of the liver in

children.

PROCEDURE

Themedical literaturewas searched using PubMedfrom 1970 to2010 for reports of pediatric patients with rhabdoid tumors of the

liver. The search terms used included: children, pediatric, rhabdoid,

malignant rhabdoid tumor, liver, and hepatic. We abstracted

information only from articles written in English. In addition, we

performed searches of the bibliographies of identified articles. We

accepted as cases all those in which the authors stated the histology

of the tumor was rhabdoid. One of the authors (MF) was able to

review the histologic and/orultrastructural features illustrated for 28

of the tumors to confirm them as rhabdoid. Rhabdoid tumors are

cytologically discohesive with round cells with large, irregularly

shaped, and eccentrically placed nuclei in abundant eosinophilic

cytoplasm containing bundles of intermediate filaments that are

both cytokeratin- and vimentin-positive. Nuclei are often clear withprominent nucleoli and sharply defined nuclear membranes.

RESULTS

Ourliterature review identified34 cases of rhabdoid tumor of the

liver, abstracted from 25 separate publications. Demographics are

shown in Table I [1–25]. Patients ranged in age from birth to

15 years (median 8 months). There were 20 males and 14 females.

Two patients (patients 5 and 8) were born prematurely at 33 weeks

gestation. Twenty-one patients presented with metastatic disease

at diagnosis. Sites of metastases were lung (16), pleural effusions

(2), lymph node (3—mesenteric in patient # 14, porta hepatis in

patient # 29, hilar in patient # 31), and one report each of bone

marrow, hepatic artery, CNS, inferior vena cava, right atrium,

omentum, and skin. Five patients had multiple metastatic sites.

No cases warranted exclusion due to descriptions in the text

and/or figure presentation of the case appearing very atypical for

rhabdoid. Ten of the tumors had immunohistochemical evaluation

of INI (BAF-47), and all were typical of rhabdoid tumors in having

no nuclear expression.

The most commonly reported presenting symptoms in the

29 patients with data were fever in 13 patients, anorexia/vomiting in

5, and lethargy/malaise in 4. The most frequently reported signs

were a mass (14), abdominal distention (7) or hepatomegaly (4).

Of particular note is that of 29 patients with symptoms reported,

22 presented with systemic signs and/or symptoms. Spontaneoustumor rupture occurred in five patients.

Common laboratory abnormalities at the time of diagnosis in the

24 cases with reported lab data included anemia (12), thrombocy-

tosis (6), increased liver enzyme levels (7), and elevation of lactate

dehydrogenase (11). Two patients were reported to have significant

hypercalcemia, and one patient who presented with chronic diarrhea

had an elevated vasointestinal peptide level. Various treatment

regimens were reported and included the agents actinomycin,

carboplatin, cisplatin, cyclophosphamide, doxorubicin, epirubicin,

etoposide, 5-fluorouracil, ifosfamide, melphalan, methotrexate,

and vincristine. The most commonly used were doxorubicin

(16 patients), vincristine (14), etoposide (14), cisplatin (12),

carboplatin (11), and ifosfamide (10). Of the 31 cases with therapy

details given, only 3 patients were not treated, and none of these

3 survived. Two patients received radiation therapy but did

Background. Rhabdoid tumors of the liver are rare tumors thataredifficult to cure. We compiledall thecases previously reported inthe literature to review clinical data, treatments, and outcomes.Procedure. Patients were identified by literature review usingPubMed. Results. Thirty-four patients were identified. The medianage at presentation was 8 months. All patients with reported AFPresults exhibited normal or minimally increased serum AFP levels.All 10 tumors with reported INI1 immunohistochemistry results werereported as negative. Twenty-one patients presented with metastaticdisease at diagnosis. Thirty patients died of disease or treatmentcomplications. Most deaths occurred within 12 months afterdiagnosis. Five patients survived at the time of the reports with onepatient alive with disease. One patient relapsed and subsequently

died after the report was published. Of the four patients alive withoutdisease, all were treated with chemotherapy, and at least three hadsurgery or transplantation. Two patients received radiation therapybut did not survive. Conclusions. Rhabdoid tumors of the liver areaggressive, rare tumors of the infant liver that are often associatedwith metastases at the time of diagnosis. Mortality is high and oftenoccurs soon after diagnosis. Treatment with aggressive chemo-therapy in combination (especially an alkylating agents doxorubicin)with complete resection may lead to improved outcomes. Therapytargeted to the INI1 mutation of these tumors is currently beinginvestigated and may offer greater hope of cure. Pediatr BloodCancer 2011;56:423–428. 2010 Wiley-Liss, Inc.

Key words: liver; pediatric; rhabdoid tumor

2010 Wiley-Liss, Inc.DOI 10.1002/pbc.22857Published online 6 November 2010 in Wiley Online Library(wileyonlinelibrary.com).

——————1

Sacred Heart Children’s Hospital, Spokane, Washington; 2

Texas

Children’s Hospital, Baylor College of Medicine, Houston, Texas;3Children’s Hospital & Research Center Oakland, Oakland, California

Conflict of interest: Nothing to report.

*Correspondence to: Angela D. Trobaugh-Lotrario, Department of

Pediatric Hematology/Oncology, Sacred Heart Children’s Hospital,

101 West 8th Avenue, Spokane, WA 99204.

E-mail: [email protected]

Received 20 August 2010; Accepted 8 September 2010



Pediatr Blood Cancer DOI 10.1002/pbc

T A B L E

I . P a t i e n t C h a r a c t e r i s t i c s

P t

A g e a t

d i a g n o s i s

S e x

M e t a s t a

t i c

d i s e a s

e

A F P

( n g / m l )

I N I 1

s t a t u s ( n u c l e a r

s t a i n )

T r e a t m e n t

R e s e c t i o n

R e s p o n s e

O u t c o m e

R e f s .

S u r v i v o r s

1

6 m o n t h s

F

N o

N R

N e g a t i v e

C h e m o

t h e r a p y

N R

N R

A l i v e a t 1 7 m o n t h s

[ 1 ] ; p e r s o n a l

c o m m . w i t h

G a r c e s - I n i g o

a n d M c H u g h

2

1 2 m o n t h s

F

I V C , R A

,

l u n g

N R

N e g a t i v e ;

h o m o z y g o u s

d e l e t i o n

V A d r i a

C

5 c y c l e s ,

i f o s /

e t o p o s i d e

7 m o n t h s

P a r t i a l r e s e c t i o n

o f R A c o m p o n e n t

C o m p l e t e r e s p o n s e

N E D a t 2 6 m o n t h s

f r o m d i a g n o s i s

[ 2 ] ; p e r s o n a l

c o m m . w i t h

C h o w

3

1 3 m o n t h s

F

L N ( p o r t a

h e p a t i s )

N l

N R

I f o s f a m

i d e , V C R , a n d

a c t i n

o m y c i n

6 c o u r s e s

G r o s s t o t a l r e s e c t i o n ,

b u t w i t h r u p t u r e

f o u n d a t t i m e

o f s u r g e r y

C o m p l e t e r e s p o n s e

N E D a t 6 y e a r s

p o s t - r e s e c t i o n

[ 3 , 4 ]

4

3 6 m o n t h s

M

N o

N l

N e g a t i v e

I C E a n

d V A d r i a C

T r a n s p l a n t a t i o n

P a r t i a l

r e s p o n s e

t o

c h e m o t h e r a p y

N E D 3 y e a r s

p o s t - t r a n s p l a n t

[ 5 ] ; p e r s o n a l

c o m m . w i t h J a s t y

F a t a l i t i e s

5

0 m o n t h s

M

S k i n

N l

N R

N o n e

N o n e

N R

D i e d o f h e m o r r h a g e

a t 4 d a y s

p o s t - d i a g n o s i s

[ 6 ]

6

3 m o n t h s

F

P l e u r a l

e f f u s i o n s

N R

N R

C h e m o

t h e r a p y

R e s e c t i o n

P a r t i a l r e s p o n s e , t h e n

p r o g r e s s i v e d i s e a s e

D O D 8 m o n t h s


[ 7 ]

7

3 m o n t h s

M

B o n e

m a r r o w

N l

N R

C a r b o p

l a t i n , V C R ,

a n d e p i r u b i c i n

R e s e c t i o n

p o s t - r u p t u r e

P r o g r e s s i v e d i s e a s e

D O D a t 4 m o n t h s


[ 8 ]

8

3 m o n t h s

M

L u n g

1 3 , 3 8 1

N e g a t i v e ,

n o n c o d i n g

s e q u e n c e

m u t a t i o n

C a r b o p

l a t i n , c i s p l a t i n ,

a n d d o x o r u b i c i n

A t t e m p t e d r e s e c t i o n ,

b u t p e r i t o n e a l

i m p l a n t s

I n i t i a l r e s p o n s e

i n m e t a s t a s e s

( c i s / d o x o )



[ 9 ]

9

3 m o n t h s

M

L u n g

1 4

N R

C 5 V D

B i o p s y o n l y


D i e d 2 m o n t h s


[ 1 0 ]

1 0

3 m o n t h s

M

N o

N R

N e g a t i v e

N R

N R

N R

D i e d a t 1 m o n t h

[ 1 ]

1 1

3 m o n t h s

M

N o

N l

N R

C h e m o

t h e r a p y

B i o p s y o n l y ;

s p o n t a n e o u s

r u p t u r e

N R

D i e d 5 d a y s f r o m

d i a g n o s i s

[ 4 ]

1 2

4 m o n t h s

M

L u n g

N R

N R

C 5 V D

1 c o u r s e

B i o p s y o n l y

N o r e s p o n s e

D O D a t 1 m o n t h

[ 1 1 ]

1 3

5 m o n t h s

F

N o

N l

N R

C i s p l a t i n a n d V C R

C o m p l e t e r e s e c t i o n

R e l a p s e i n l i v e r



[ 1 2 ]

1 4

6 m o n t h s

F

N o

N R

N R

C i s p l a t i n , e t o p o s i d e ,

a n d d o x o r u b i c i n

G r o s s t o t a l r e s e c t i o n

w i t h p o s i t i v e

m a r g i n s


D O D 7 w e e k s


[ 1 3 ]

1 5

6 m o n t h s

F

L u n g

N R

N R

N o n e

U n r e s e c t a b l e


D O D 0 . 4 m o n t h s


[ 1 4 ]

1 6

7 m o n t h s

M

N o

N R

N R

I C E

3 c o u r s e s ,

l o w -

d o s e

m e t h

o t r e x a t e

C o m p l e t e r e s e c t i o n

w i t h m i c r o s c o p i c

r e s i d u a l

N o r e s p o n s e


[ 1 1 ]

424 Trobaugh-Lotrario et al.




1 7

7 m o n t h s

M

L u n g

N R

N e g a t i v e

N R

N R

N R

D i e d a t 4 m o n t h s

[ 1 ]

1 8

8 m o n t h s

M

L u n g

N l

N R

I f o s f a m i d e , V C R ,

a n d

a c t i n o m y c i n

R e s e c t i o n o f

l i v e r m a s s

I n i t i a l r e s p o n s e ,

t h e n r e c u r r e n t

d i s e a s e

D O D a t > 1 5 m o n t h s

p o s t - s u r g e r y

[ 1 5 ]

1 9

8 m o n t h s

M

N o

2 3

N e g a t i v e

I C E

1 0 c y c l e s

R i g h t l o b e c t o m y

R e c u r r e n t d i s e a s e

2 m o n t h s o f f

t h e r a p y

D O D 6 w e e k s a f t e r

r e c u r r e n c e

[ 1 6 ]

2 0

8 m o n t h s

M

L u n g

N l

N R

N o n e



D O D ( t i m e n o t

r e p o r t e d )

[ 4 ]

2 1

9 m o n t h s

F

P l e u r a l

e f f u s i o n

N l

N R

C i s p l a

t i n , e t o p o s i d e ,

a n d

d o x o r u b i c i n

B i o p s y a n d

p a r a c e n t e s i s

N R

D i e d 1 4 d a y s


[ 1 0 ]

2 2

1 0 m o n t h s

F

L u n g

N R

N R

C i s p l a

t i n , e t o p o s i d e ,

a n d


B i o p s y o n l y

N o r e s p o n s e

D O D a t 2 w e e k s

[ 1 1 ]

2 3

1 0 m o n t h s

M

N o

2 8 . 7

N e g a t i v e

V A d r i a C a n d I C E

N o n e


D O D 2 m o n t h s

p o s t - t r e a t m e n t

[ 1 6 ]

2 4

1 1 m o n t h s

M

L u n g

N l

N R

C h e m o t h e r a p y

B i o p s y o n l y

N o r e s p o n s e

D O D a t 8 w e e k s


[ 4 ]

2 5

1 2 m o n t h s

F

L u n g

1 , 2 0 8

N R

C i s p l a

t i n , C T X ,

a n d


H e p a t i c a r t e r y

e m b o l i z a t i o n

T u m o r r u p t u r e

D i e d o f h e m o r r h a g e

w i t h r u p t u r e

3 w e e k s


[ 1 7 ]

2 6

1 2 m o n t h s

M

L u n g s ,

o m e n

t u m

N R

N R

A c t i n o

m y c i n

N R

N R

D i e d a t 1 w e e k

[ 1 8 ]

2 7

1 6 m o n t h s

F

H i l a r l y m p h

n o d e s

‘ ‘ R a i s e d ’ ’

N R

C a r b o p l a t i n a n d




D i e d a t 2 m o n t h s

o f u p p e r G I

h e m o r r h a g e

[ 1 9 ]

2 8

1 7 m o n t h s

M

C N S , l u

n g

N R

N R

I C E

R e s e c t i o n o f l i v e r

m a s s a n d

d e b u l k i n g o f

C N S m a s s


( C N S )

D O D 1 1 m o n t h s a f t e

r

d i a g n o s i s

[ 2 0 ]

2 9

1 7 m o n t h s

F

N o

N l

N R

C h e m o t h e r a p y a n d

r a d i a t i o n

N R


D O D 6 w e e k s


[ 2 1 ]

3 0

1 8 m o n t h s

M

L u n g

N l

N e g a t i v e

V A d r i a C

5 c y c l e s ,

C T X

/ c a r b o p l a t i n /

e t o p

o s i d e

5 c y c l e s

B i o p s y o n l y



f r o m d i a g n o s i s

[ 2 ] ; p e r s o n a l

c o m m . w i t h

C h o w

3 1

2 1 m o n t h s

M

L u n g , L

N

2 0

N R

C 5 V , I C E , V A d r i a C ,

h i g h

- d o s e

c h e m o t h e r a p y

( e t o p o s i d e /

c a r b

o p l a t i n / C T X ;

m e l / C T X ) w i t h

t w o

a u t o l o g o u s

t r a n s p l a n t s


I n i t i a l r e s p o n s e ,

t h e n p r o g r e s s i v e

d i s e a s e , t h e n

r e s p o n s e t o I C E ,

t h e n p r o g r e s s i v e

d i s e a s e

p o s t - t r a n s p l a n t

D O D 9 m o n t h s


[ 2 2 ]

3 2

6 0 m o n t h s

F

H e p a t i c

a r t e r y

,

a l o n g

p o r t a

h e p a t i c

N R

N R

C 5 V D





[ 2 3 ]

Rhabdoid Tumors of the Liver 425



not survive. One patient underwent liver transplantation after

chemotherapy and survived.

Only 4 of these 34 patients (patients # 1, 2, 3, and 4) are alive

without evidence of disease at the time of the reports or on follow-

up. Deaths occurred early (range 4 days to 44 months from

diagnosis;median 2 months), with all buttwo deaths occurringprior

to 12 months from diagnosis. Nineteen patients clearly died of their liver tumor. Eleven others died presumably still with disease

(range 4 days to 4 months; median 22 days) after diagnosis.

Of those patients with reported details regarding the death, three

died as a result of hemorrhage, one of neurologic deterioration, and

one of congestive heart failure.

The four patients alive without evidence of disease were older at

diagnosis (ages 6 months, 12 months, 13 months, and 3 years) as

compared with the patients who did not survive (range birth to

15 years; median 8 months) (Table I). Patients 1 and 4 had localized

disease, patient 3 had regional lymph node metastasis only (porta

hepatis), and patient 2 presented with multiple metastases (lungs,

inferior vena cava, andright atrium). Patient 2 hada partial resection

of the right atrial mass after chemotherapy that included vincristine,

doxorubicin, cyclophosphamide, etoposide, and ifosfamide. One of

the survivors (patient # 4) underwent liver transplantation after

ifosfamide, carboplatin, etoposide, vincristine, doxorubicin, and

cyclophosphamide. One patient (patient # 3) survived in spite of

tumor rupture at the time of surgery.

CONCLUSIONS

Rhabdoid tumors of the liver are rare, aggressive tumors that are

difficult to treat. This review constitutes the largest collection of

such cases yet reported. The INI1 status was reported in only

10 cases, and we did not attempt a central pathology review, so it

could well bethatsomeof theothercasesin thisreport are not infact

true rhabdoid tumors, especially the three with elevation of AFP atdiagnosis. In spite of this, this compilation of cases represents the

total body of published information available on this subject and we

feel is of use in trying to improve our understanding of this rare but

highly lethal tumor.

Presenting symptoms and laboratory values can be helpful in

establishing the diagnosis of a patient presenting with a liver mass.

The differential diagnosis of a malignant mass in the liver in a

pediatric patient includes hepatoblastoma (HB), hepatocellular

carcinoma(HCC), rhabdoid tumor of the liver, cholangiocarcinoma,

and various sarcomas (angiosarcoma, undifferentiated or embry-

onal sarcoma, rhabdomyosarcoma).

Most patients with rhabdoid tumors of the liver present in

infancy with hepatomegaly, abdominal distention, or an abdominal

mass and have systemic symptoms, including fever, lethargy/

malaise, and anorexia/vomiting. Of potential significance, 17%

(5/29 with data) of these patients presented with spontaneous

rupture of their tumor, which is definitely more frequent than has

been reported for HB or HCC in this age group.

The major tumor to differentiate from these rhabdoid tumors in

this age group (infants and toddlers) is HB. Children with HB share

some of the clinical features described above for rhabdoid tumor

of the liver (male predominance, thrombocytosis, anemia, and only

moderate derangement of overall liver function). However, they

aredistinctin being somewhatolderat diagnosis (16monthsmedian

vs. 8 months) [26,27], less frequently present with systemic signs

or symptoms, a lesser incidence of spontaneous tumor rupture, and


3 3

8 4 m o n t h s

F

L u n g

N l

N R

C a r b o p

l a t i n , V C R ,

a n d e p i r u b i c i n

B i o p s y o n l y

N

R

D i e d

o f

n e u r o l o g i c

d e t e r i o r a t i o n

2 2 d a y s a f t e r

d i a g n o s i s

[ 2 4 ]

3 4

1 8 0 m

o n t h s

M

N o

N R

N e g a t i v e

I f o s f a m

i d e , V C R ,

a c t i n

o m y c i n

6

c y c l e

s ; d o x o r u b i c i n ,

c i s p l a t i n ; r a d i a t i o n ;

a n d e t o p o s i d e

L e f t h e p a t e c t o m y ;

s p o n t a n e o u s

r u p t u r e

R

e c u r r e n t d i s e a s e

D i e d a t 4 4 m o n t h s

o f c o n g e s t i v e h e a r t

f a i l u r e a f t e r

3 r d r e c u r r e n c e

[ 2 5 ]

P t , p a t i e n t ; A F P , a l p h a - f e t o p r o t e i n ; F , f e m a l e ; N l , n o r m a l ; N R , n o t r e p o r t e d ; C o m m . , c o

m m u n i c a t i o n ; I V C , i n f e r i o r v e n a c a v a ; R A , r

i g h t a t r i u m ; V A d r i a C , V i n c r i s t i n e / a d r i a m y c i n / c y c l o p h o s p h a m i d e ;

I f o s , i f o s f a m i d e ; N E D , n o e v i d e n c e o f d i s e

a s e ; L N , l y m p h n o d e ; V C R , v i n c r i s t i n e ; M , m

a l e ; I C E , i f o s f a m i d e / c a r b o p l a t i n / e t o p o s i d e ; D O D , d i e d o f d i s e a s e ; C 5 V D , c i s p l a t i n / 5 - fl u

o r o u r a c i l / v i n c r i s t i n e /

d o x o r u b i c i n ; C T X , c y c l o p h o s p h a m i d e ; G

I , g a s t r o i n t e s t i n a l ; C N S , c e n t r a l n e r v o u s s y s t e m ; C 5 V , c i s p l a t i n / 5 - fl u o r o u r a c i l / v i n c r i s t i n

e ; M e l , m e l p h a l a n .

T A B L E

I .

( C o n t i n u e d )

P t

A g e a t

d i a g n o s i s

S e x

M e t a s t a t

i c

d i s e a s e

A F P

( n g / m l )

I N I 1

s t a t u s ( n u c l e a r

s t a i n )

T

r e a t m e n t

R e s e c t i o n

R e s p o n s e

O u t c o m e

R e f s .




especially in having a significantly elevated AFP at diagnosis over

90% of the time.

For younger patients with liver tumors but without an elevated

AFP at diagnosis, detailed cytogenetic, immunohistochemical and/

or molecular analysis INI1 (BAF-47) should be conducted. A recent

report by Trobaugh-Lotrario et al. found that six of six patients with

HB of small cell undifferentiated histology who were tested for theINI1 mutation were negative [28]. Those patients were similar to the

patients presented in this review with low AFP and younger age at

diagnosis (median 8 months compared with 8 months for this

review).In the Trobaugh-Lotrario report, one patient withsmall cell

undifferentiated HB was found to have a deletion in the 22q12

region, the location of the SMARCB1 /INI1 gene by whole genome

comparative genomic hybridization of the INI1 gene locus [28].

Therefore, some of the small cell undifferentiated HBs should be

classified as rhabdoid tumors.

At least 15–30% of rhabdoid tumors reported in the literature

have been associated with germline abnormalities involving

chromosome 22q11.2 and specifically inactivating mutations in

the SMARCB1 gene [29]. SMARCB1 appears to act as a tumor

suppressor gene, with inactivation of both copies of the gene

resulting in tumor predisposition. Patients with germline mutations

of the SMARCB1 gene tend to develop these rhabdoid tumors

(especially brain, kidney, and soft tissues) within their first year of

life, and may have multiple primary tumors with a very poor

prognosis [30– 33].

Outcomes for patients with rhabdoid tumors of the liver are very

poor. In our series, the survivors tended to be older and were more

likely to be female (Table I). Of 4 survivors, 2 presented with

localized disease, whereas only 9 of 30 patients who did not survive

presented with localized disease. Of 11 patients presenting with

localized disease, 2 survived compared to only 2 of the 23 patients

presenting with nonlocalized disease.

Forthe patients who survived, chemotherapy generally includedvincristine, doxorubicin, cyclophosphamide, and ifosfamide, which

is consistent with typical treatment for non-CNS rhabdoid tumors

and has been used successfully in metastatic rhabdoid tumors [34–

36]. Recent reports have studied new potential therapeutic targets in

vitro via targeting tumor suppressor genes associated with

SMARCB1 via angiogenesis inhibition [37] and restoration of

SMARCB1 via histone deacetylase inhibitors [38]. The prospect of

targeted intervention is especially encouraging because of the

potentially harmful long-term consequences of current chemo-

therapy in very young infants.

In conclusion, rhabdoid tumors of the liver are rare and very

difficult to cure. Presenting features that might be distinctive are

systemic signs or symptoms such as fever, a very elevated LDH, a

normal or near normal AFP level at diagnosis, and perhaps the

occurrence of spontaneous tumor rupture. Given the few patients

cured with rhabdoid tumor of the liver, conclusions regarding

treatment are difficult. It is likely, however, that treatment utilizing

aggressive chemotherapy (such as vincristine, doxorubicin, cyclo-

phosphamide, ifosfamide, and etoposide) in combination with

aggressive surgery appears to be necessary for cure. Radiation has

been used in treatment for very few of these patients. Given the low

number of patients, strong conclusions cannot be drawn. However,

based on its lack of clear benefit in patients with these tumors at

other sites, it is unlikely to be of particular benefit for children with

rhabdoid tumors in the liver, with the possible exception of cases

with microscopic residual disease involving small amounts of the

liver. Given the toxicity to the liver,its use in rhabdoid tumors of the

liver is likely to be limited to rare cases with small lesions that are

unresectable due to proximity to blood vessels.

Children that present with this tumor should be enrolled on

biology and therapy treatment trials, so to best advance our

understanding of the pathogenesis of this tumor.

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