Zhu 

et al. BMC Medical Imaging (2021) 21:181

https://doi.org/10.1186/s12880-021-00715-z

RESEARCH Open Access

Imaging features and differences

among the three primary malignant non‑Wilms
tumors in children
Yupeng Zhu1, Wangxing Fu1, Yangyue Huang2, Ning Sun2 and Yun Peng1* 

Abstract 
Background:  The pathology, treatment and prognosis of malignant non-Wilms tumors (NWTs) are different, so it
is necessary to differentiate these types of tumors. The purpose of this study was to review the clinical and imaging
features of malignant NWTs and features of tumor metastasis.
Methods:  We retrospectively analyzed the CT images of 65 pediatric patients with NWTs from March 2008 to
July 2020, mainly including clear cell sarcoma of the kidney (CCSK), malignant rhabdomyoma tumor of the kidney
(MRTK) and renal cell carcinoma (RCC). Available pretreatment contrast-enhanced abdominal CT examinations were
reviewed. The clinical features of the patients, imaging findings of the primary mass, and locoregional metastasis pat-
terns were evaluated in correlation with pathological and surgical findings.
Results:  The study included CCSK (22 cases), MRTK (27 cases) and RCC (16 cases). There were no significant differ-
ences observed among the sex ratios of CCSK, MRTK and RCC (all P > 0.05). Among the three tumors, the onset age of
MRTK patients was the smallest, while that of RCC patients was the largest (all P < 0.05). The tumor diameter of CCSK
was larger than that of MRTK and RCC (all P < 0.001). For hemorrhage and necrosis, the proportion of MRTK patients
was larger than that of the other two tumors (P = 0.017). For calcification in tumors, the proportion of calcification in
RCC was highest (P = 0.009). Only MRTK showed subcapsular fluid (P < 0.001). In the arterial phase, the proportion of
slight enhancement in RCC was lower than that in the other two tumors (P = 0.007), and the proportion of marked
enhancement was the highest (P = 0.002). In the venous phase, the proportion of slight enhancement in RCC was
lower than that in the other two tumors (P < 0.001). Only CCSK had bone metastasis. There was no liver and lung
metastasis in RCC.
Conclusions:  NWTs have their own imaging and clinical manifestations. CCSK can cause vertebral metastasis, MRTK
can cause subcapsular effusion, and RCC tumor density is usually high and calcification. These diagnostic points can
play a role in clinical diagnosis.
Keywords:  Children, Renal tumor, Clear cell sarcoma of kidney, Malignant rhabdomyoma tumor of kidney, Renal cell
carcinoma, Imaging

Background
Malignant non-Wilms tumors (NWTs) in children
include clear cell sarcoma of the kidney (CCSK), malig-
*Correspondence: ppengyun@yahoo.com
1
Department of Radiology, Beijing Children’s Hospital, Capital Medical
nant rhabdomyoma tumor of the kidney (MRTK), renal
University, National Center for Children’s Health, 56 Nanlishi Road, cell carcinoma (RCC) and other extremely rare malignant
Xicheng District, Beijing, China 100045 tumors [1–3]. Preoperative identification of the tumor
Full list of author information is available at the end of the article

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Zhu et al. BMC Medical Imaging (2021) 21:181 Page 2 of 8

type relies primarily on imaging or diagnostic needle performed first to determine the lesion site, and then as
biopsy. Diagnostic puncture biopsy is an invasive exami- enhanced scanning was performed. The contrast medium
nation with low repeatability and poor patient compli- was iohexol (300 MGI/ml) at a dose of 1.1–1.6 ml/kg. The
ance. In daily diagnosis and treatment of tumor cases, arterial phase was scanned 15–18  s after injection, and
imaging examination is an essential and noninvasive test the venous phase was performed at 45–55 s.
that has a very crucial role in identifying the heterogene- Two pediatric imaging diagnostic doctors assessed the
ity characteristics inside the tumor. With the continu- anatomical location, size, margin, homogeneity and con-
ous development of diagnostic imaging technology, the trast enhancement of the renal mass with respect to the
detection rate of renal tumors in children is high. How- normal renal parenchyma. CT images were reviewed and
ever, when the tumor volume is large, it is difficult to dis- diagnosed independently by two radiologists and reread
tinguish renal tumors from primary adrenal tumors (such for cases with discordant diagnostic results.
as neuroblastoma), but some studies have identified them
by radionuclide imaging and achieved certain results [4, Statistics
5]. At present, there are difficulties in the identification of Statistical analyses were performed using SPSS version
renal tumors. Although MR examination is safe and radi- 24.0 software (IBM Corp., Armonk, NY USA). Continu-
ation free, it has not been well popularized within clini- ous and quantitative data (such as age and tumor diam-
cal practice. CT remains the imaging modality of choice, eter) were analyzed by one-way ANOVA and P values of
and low-dose scanning methods do not increase radia- less than 0.05 indicated that the difference was statisti-
tion doses to children. Because the pathology, treatment cally significant. Categorical and qualitative data (such as
and prognosis of malignant NWTs are distinctly different gender, age group and image features) were analyzed by
[3, 6–10], it is necessary to conduct CT imaging exami- chi-square test of row × column data. When comparing
nations before surgery to determine differential diagno- the two groups of data, P values of less than 0.05 indi-
ses. The purpose of this study was to review the clinical cated that the difference was statistically significant. For
and imaging features of malignant NWTs and features of pairwise comparisons among the three groups of data, P
tumor metastasis. values of less than 0.017 indicate that the data difference
was statistically significant.
Methods
Patients Results
This study was a retrospective case–control investigation Our study cohort included 65 patients with malignant
approved by the Ethics Committee of the hospital, and NWTs. Twenty-two patients had CCSK, 27 patients had
the requirement for the consent of patients was waived. MRTK and 16 patients had RCC. In the current cohort,
The images of malignant NWTs confirmed through abdominal imaging was performed with enhanced CT
biopsy or surgical resection were searched from the pic- in all patients. All enhanced CT scans included three
ture archiving and communication system (PACS) of our phases, including the plain phase, arterial phase and
hospital from March 2008 to July 2020. Inclusion crite- venous phase. Each tumor was grouped according to age,
ria included: (1) Age younger than 18 years; (2) Primary named less than 1 year old, 1 to 4 years old, 5 to 9 years
unilateral renal tumor; (3) The pathological results were old and 10 to 14  years old. The demographic character-
renal tumor; (4) The phase of the enhanced CT scan was istics of the various tumors are shown in Table  1. The
intact; (5) Did not receive treatment before CT examina- imaging characteristics of the NWT patient cohort at
tion. The exclusion criteria were as follows: (1) pathologi- presentation are shown in Table 2.
cal results obtained by tumor puncture only and (2) cases
with failed CT image calling. Demographic characteristics
There were no significant differences observed among
Imaging examination the sex ratios of CCSK, MRTK and RCC (all P > 0.05)
All patients underwent enhanced CT scans. In CT exam- (Table  1), and the sex ratios (male vs. female) were
inations, GE Healthcare 16 slice spiral CT, GE Healthcare 1.75:1, 1.45:1 and 0.78:1, respectively. For the onset age
64 slice spiral CT and GE Discovery CT750 HD (General of tumors, there were significant differences observed
Electric Company, USA) were used. The scanning range among the groups (all P < 0.05) (Table  1). Among the
was from the top of the diaphragm to the pelvic entrance. three tumors, the onset age of MRTK patients was the
The thickness of the acquisition layer was 5  mm, the smallest, while that of RCC patients was the largest. For
interval between layers was 5  mm, and the reconstruc- the age group of onsets (Table  1), CCSK patients were
tion interval was 0.625  mm. The patients were placed distributed in all age groups. All MRTK patients were
in the supine position, a nonenhanced CT scan was distributed in the age group under 10  years old, while
 Zhu et al. BMC Medical Imaging (2021) 21:181 Page 3 of 8

Table 1  Demographic characteristics of the NWTs patient cohort at presentation

Parameter CCSK MRTK RCC​ P values P values P values P values
(n = 22) (n = 27) (n = 16) C vs. M C vs. R M vs. R

Sex (M/F) 14/8 (1.75:1) 16/11 (1.45:1) 7/9 (0.78:1) 0.450 0.754 0.223 0.324
Age range (years) 0.6–10 0.3–6.2 2.9–14.6 < 0.001 0.012 < 0.001 < 0.001
Median age (years) 2.55 1.2 11.35
Age group (years)
< 1 4.5% (1/22) 44.4% (12/27) 0 < 0.001 0.002 0.291 < 0.001
1–4 77.4% (17/22) 51.9% (14/27) 12.5% (2/16) < 0.001 0.066 < 0.001 0.010
5–9 13.6% (3/22) 3.7% (1/27) 25.0% (4/16) 0.118 0.460 0.375 0.107
10–14 4.5% (1/22) 0 62.5% (10/16) < 0.001 0.202 < 0.001 < 0.001
P values indicates whether the difference between the three groups of tumors is statistically significant. C, CCSK; M, MRTK; R, RCC​

Table 2  Imaging characteristics of the NWTs patient cohort at presentation

Parameter CCSK MRTK RCC​ P values P values P values P values
(n = 22) (n = 27) (n = 16) C vs. M C vs. R M vs. R

Diameter (cm) 9.8 (range: 4.4 to 18.1) 5.9 (range: 1.5 to 11.1) 5.3 (range: 2.2 to 12.5) < 0.001 < 0.001 < 0.001 0.462
Breaking through the renal fascia 5% (1/22) 26% (7/27) 19% (3/16) 0.136 0.044 0.158 0.590
Hemorrhage and necrosis 64% (14/22) 85% (23/27) 44% (7/16) 0.017 0.081 0.223 0.004
Calcification 14% (3/22) 7% (2/27) 44% (7/16) 0.009 0.809 0.037 0.005
Subcapsular fluid 0 33% (9/27) 0 < 0.001 < 0.001 / 0.002
Arterial phase
Slight enhancement 68% (15/22) 70% (19/27) 25% (4/16) 0.007 0.869 0.007 0.004
Moderate enhancement 14% (3/22) 15% (4/27) 25% (4/16) 0.607 1.000 0.375 0.671
Marked enhancement 5% (1/22) 4% (1/27) 38% (6/16) 0.002 1.000 0.008 0.004
Venous phase
Slight enhancement 77% (17/22) 56% (15/27) 6% (1/16) < 0.001 0.112 < 0.001 0.001
Moderate enhancement 14% (3/22) 30% (8/27) 50% (8/16) 0.052 0.182 0.014 0.182
Clear enhancement 14% (3/22) 4% (1/27) 25% (4/16) 0.118 0.460 0.375 0.107
Metastasis
Lymph node metastasis 9% (2/22) 19% (5/27) 19% (3/16) 0.603 0.348 0.388 0.985
Vein tumor thrombus 9% (2/22) 4% (1/27) 13% (2/16) 0.553 0.855 0.737 0.635
Bone (the vertebral body) metastasis 9% (2/22) 0 0 0.108 0.069 0.132 /
Liver and lung Metastases 5% (1/22) 22% (6/27) 0 0.022 0.079 0.291 0.013
P values indicates whether the difference between the three groups of tumors is statistically significant. C, CCSK; M, MRTK; R, RCC​

RCC patients were mainly distributed in the age groups P < 0.001), while the proportion of patients with CCSK
of 5–9 and 10–14. For the less than 1 age group, the pro- and MRTK was similar (P = 0.202).
portion of patients with MRTK was larger than that with
CCSK and RCC (P = 0.002 and P < 0.001, respectively), Imaging findings
while the proportion of patients with CCSK and RCC Primary mass
was similar (P = 0.291). For the 1–4 age group, the pro- The tumor diameter of the CCSK was larger than that of
portion of RCC patients was lower than that of the other MRTK and RCC (all P < 0.001), while the tumor diam-
two groups (P < 0.001 and P = 0.010, respectively), while eter of the MRTK and RCC was not significantly differ-
the proportion of patients with CCSK and MRTK was ent (P = 0.462) (Table  2). CCSK, MRTK and RCC could
similar (P = 0.066). For the 5–9 age group, there were no break through the renal fascia, but no significant differ-
significant differences observed among the groups (all ences were observed (P = 0.136). However, MRTK was
P > 0.05). For the 10–14 age group, the proportion of RCC more likely to break through the renal fascia than the
patients was larger than that of the other two groups (all other two tumors. For hemorrhage and necrosis, the
Zhu et al. BMC Medical Imaging (2021) 21:181 Page 4 of 8

proportion of MRTK patients was larger than that of

the other two tumors (P = 0.017) (Fig.  1a). The propor-
tion of hemorrhage and necrosis in RCC was lowest, so
the overall density of RCC was higher than that of CCSK
and MRTK (Fig. 2a). For calcification in tumors, the pro-
portion of calcification in RCC was highest (P = 0.009)
(Fig.  2b), while there was no significant difference in
the incidence of tumor calcification between CCSK and
MRTK (P = 0.809). Among the three tumors, only MRTK
tumors showed subcapsular fluid (Fig. 1b), accounting for
approximately 33% (P < 0.001).
The enhancement characteristics of CCSK, MRTK
and RCC tumors were different. In the arterial phase,
only 25% of RCCs showed slight enhancement, which
was lower than that of CCSK and MRTK (P = 0.007
and P = 0.004, respectively), while there was no signifi-
cant difference in the proportion of mild enhancement
between CCSK and MRTK (P = 0.869) (Fig. 3a). However,
the proportion of marked enhancement in RCC was the
highest among the three tumors, accounting for approxi-
mately 38% (P =  0.002; CCSK vs. MRTK, P = 0.008;
MRTK vs. RCC, P = 0.004). There were no significant
differences observed among the patients with moderate
enhancement (all P > 0.05). In the venous phase, the pro-
portion of slight enhancement in RCC was lowest, only
6% (P < 0.001), while there was no significant difference in
the proportion of slight enhancement between CCSK and
MRTK (P = 0.112). The proportion of moderate enhance-
ment in RCC was higher (50%; CCSK vs. RCC, P = 0.014).
There were no significant differences observed among the
patients with clear enhancement (all P > 0.05). Fig. 2  RCC in an 11-year-old boy. A The internal density of the left
renal tumor was uniform, and the tumor density did not decrease
Metastases based on imaging (white arrow). B Rim calcification (white arrow) was seen in the tumor
The metastasis characteristics of the three tumors were
also different. For lymph node metastasis and vein tumor
thrombus, there were no significant differences observed
(Fig.  3b). Both CCSK and MRTK had liver and lung
among the three types of tumors (all P > 0.05). Among
metastasis, but the proportion of MRTK was higher,
the three types of tumors, only CCSK had bone metas-
accounting for 22% (Fig. 1c and 1d).
tasis (9%), and the tumor metastasized to vertebral bone

Fig. 1  MRTK in a 7-month-old girl. A Large necrotic areas (black arrow) and hemorrhagic lesions (white arrow) were visible within the tumor, and
the tumor density was uneven. B The sagittal reconstructed image showed subcapsular effusion (white arrow) at the edge of the tumor. C, D Tumor
metastasis (white arrow) was found in the lung and liver
 Zhu et al. BMC Medical Imaging (2021) 21:181 Page 5 of 8

renal neoplasms and has a marked male predilection.

CCSK is commonly diagnosed in children 1 to 4 years of
age [2]. In our CCSK study, the ratio of males to females
was approximately 1.75:1, and the median age of onset
was 3.1  years (range of 0.6 to 10  years). The results of
our study were consistent with the literature. MRTK is
an aggressive embryonal tumor that arises from primi-
tive cells in the renal medulla and frequently involves
the hilum and collecting system. MRTK accounts for
less than 2% of childhood renal tumors [13]. MRTK was
originally described in the kidneys of young children by
Beckwith and Palmer in 1978 and is considered to be the
most aggressive malignant renal tumor in childhood [18].
The median age of patients with renal RT ranges from 11
to 18  months, with a mean age of 11 to 18  months [13,
19, 20]. MRTK affects boys slightly more commonly than
girls (1.5:1) [13, 20]. In our study, the ratio of males to
females was approximately 1.45:1, and the median age
of onset was approximately 1.4  years (range of 0.3 to
6.2 years). Therefore, if the child is younger (usually less
than 1  year old), MRTK should be considered first [1].
Although well recognized, RCC is an uncommon tumor,
as it is the second most common renal malignancy diag-
nosed among pediatric and adolescent patients, account-
ing for 2% to 6% of renal cancers [1, 7, 14]. The onset age
of RCC is usually older, generally more than 10 years old,
with an average age of 11–12  years [11, 14, 21]. In our
study, the median age of RCC onset was 10.1 years, rang-
ing from 2.9 to 14.6  years, but most patients were over
10  years old. The age of RCC onset is significantly dif-
ferent from that of CCSK and MRTK, which is also one
of the important differentiation points of the three renal
Fig. 3  CCSK in a 5-year-old boy. A Large tumor tissue was seen
in the left kidney area, and the density of the tumor was uneven.
tumor types.
Tumor tissue showed slight enhancement, less than the normal renal CCSK is usually highly malignant, so it is of great sig-
parenchyma (white arrow). B Sagittal bone window images showed nificance to identify it from NWTs [1, 12]. CCSK is
low-density lesions (white arrow) in lumbar vertebrae, suggesting usually unilateral, with necrosis and cystic lesions of dif-
tumor metastasis ferent sizes and numbers. Hemorrhage and necrosis are
frequent findings [22]. The blood supply of the tumor is
abundant. Enhanced scans can have mild to moderate
Discussion enhancement (> 20 Hu), and approximately 25% have
Renal tumors in children mainly originate in the poste- calcification [1, 23]. In our study, CCSK tumors did not
rior renal germ or posterior interrenal lobe, which are easily break through the renal fascia. More than half of
common abdominal tumors in children. Malignant renal the tumors showed evidence of hemorrhage and necrosis
tumors accounted for 5.2% of malignant tumors in chil- but rarely showed calcification. Most CCSK tumors had
dren [11]. NWTs account for approximately 10% of pedi- mild enhancement, which may be related to CCSK not
atric renal tumors. However, there are many kinds of easily invading blood vessels [13]. The results of our study
tumors, such as CCSK, MRTK, RCC and other extremely are similar to those in the literature. Although CCSK is
rare malignant tumors [1, 12–16]. a highly malignant tumor, only one CCSK tumor broke
CCSK is a rare malignant renal tumor that primar- through the renal fascia in our study, which may be one
ily occurs in children and was first described by Kidd of the differentiation points between CCSK and other
et  al. in 1970 [17]. CCSK has recently been regarded as tumors. At the same time, our study also found that the
a malignancy distinct from Wilms tumors (WTs) [12, tumor volume of CCSK is generally larger than that of
15]. CCSK represents approximately 4% of all childhood MRTK and RCC, which can distinguish CCSK.
Zhu et al. BMC Medical Imaging (2021) 21:181 Page 6 of 8

The CT findings of MRTK are relatively nonspecific, bone metastasis (the vertebral body). Bone metasta-
but some imaging features can also prompt the diagno- sis is a prominent feature of CCSK. If bone metastasis
sis. The MRTK tumors are relatively large and heteroge- occurs (especially vertebral metastasis), the first consid-
neous. Tumors in the central area often involve the renal eration should be CCSK rather than other renal tumors.
hilum. Most of these tumors are accompanied by hemor- Although CCSK tumors do not easily break through the
rhage, necrosis and subcapsular effusion [1, 13]. Subcap- renal fascia, they still metastasize locoregionally and dis-
sular effusion is a more specific imaging manifestation, tantly, which further indicates their invasiveness. Lung
which further confirms the characteristics of the MRTK metastasis is most common in MRTK, which is also dif-
tumor hemorrhage and necrosis [1, 13]. In our study, ferent from other renal tumors, including WTs [22, 30].
85% of the tumors had hemorrhage and necrosis, and In our study, approximately 22% of tumors developed
approximately 33% of the tumors had subcapsular effu- pulmonary metastases, indicating that MRTK tumors
sion. These two factors can be used as important factors mainly metastasize distantly. It is true that lung metas-
in the diagnosis of MRTK. An enhanced CT scan showed tases in very young patients could be an indication of
uneven enhancement of the tumor, and the enhancement MRTK, where a biopsy should be considered. In the lit-
degree was lower than that of muscle tissue [13]. Approx- erature, distant metastasis of RCC tumors is not char-
imately 85% of the tumors in our study showed mild to acteristic [31]. In our study, no distant organ metastasis
moderate enhancement. Approximately 10% to 20% of was found in RCC tumors, indicating that RCC tumors
affected children have primary brain tumors [13, 18, 24]. mainly metastasize locoregionally. Lymph node metas-
However, some studies have suggested that brain tumors tasis is not specific in the differential diagnosis of renal
are metastatic tumors rather than primary tumors [13, tumors.
25]. In our study, only 2 patients had tumor lesions in This study had several limitations. Most of the cases
the brain, so the diagnosis of brain tumors as metastatic in this study belonged to the previous cases, when MR
tumors remains to be studied. examination was not yet in clinical popularity, so most
In CT imaging, the density of RCC was higher than of the patients underwent CT examination. With the
that of the normal renal parenchyma, which was sepa- popularity of MR in recent years, MR has been gradually
rate from CCSK and MRTK. Calcification, hemorrhage applied in the clinic to evaluate renal tumors. However,
and necrosis can be found in most RCC tumors [1, 14, we believe that CT-related studies will be instructive for
26, 27]. In our study, approximately 44% of the tumors the future study of MR, and at the same time, CT find-
showed evidence of hemorrhage and necrosis, and ings will have important clinical implications for the eval-
approximately 44% of the tumors showed calcification. uation of tumor metastasis.
However, calcification was rare in CCSK and MRTK.
In contrast-enhanced CT imaging, approximately 63%
of the tumors showed moderate to clear enhancement, Conclusions
which is also different from the enhancement degree of CCSK, MRTK and RCC have their own imaging and
CCSK and MRTK. This finding is not consistent with the clinical manifestations. The volume of CCSK tumors is
known literature results [14], which may be related to the large, most of the enhanced scans show slight enhance-
sample size of patients because approximately 25% of the ment, and vertebral metastases can occur. The onset
tumors in our study showed slight enhancement. At pre- age of MRTK was younger, and hemorrhage and necro-
sent, some studies have used MRI images combined with sis were common in the tumor. Subcapsular effusion
radiomics to predict the Fuhrman classification of RCC was seen in most cases of MRTK. Most MRTK patients
in adult patients and have achieved good results [28]. showed slight to moderate enhancement in enhanced
This study suggests that we can use radiomics or deep scanning, and distant metastases, such as liver and lung
learning methods to predict the type of renal tumors in metastases, were common. The onset age of RCC is older.
the future and believe that it will achieve better results Hemorrhage and necrosis within the tumor are relatively
combined with image features. rare, and calcification is more common. Enhanced scans
CCSK, MRTK and RCC have different characteristics are mostly moderate and clear enhancement. The differ-
in tumor metastasis. CCSK is well known for its aggres- ent clinical and imaging features of CCSK, MRTK and
sive nature. Bone metastasis is a prominent feature of RCC can provide help for the diagnosis and treatment of
CCSK [16, 22, 29]. However, data from the American tumors.
National Wilms Tumor Study suggest that bone metasta-
ses are infrequent in CCSK, and indeed, bone metastases Abbreviations
accounted for only 6% of metastases among 351 patients WTs: Wilms tumors; NWTs: Non-Wilms tumors; CCSK: Clear cell sarcoma of the
kidney; MRTK: Malignant rhabdomyoma tumor of the kidney; RCC​: Renal cell
[6]. In our study, approximately 9% of tumors developed carcinoma; PACS: Picture archiving and communication system.
 Zhu et al. BMC Medical Imaging (2021) 21:181 Page 7 of 8

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1
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