Clear Cell Sarcoma of the Kidney (CCSK); A Review of theDiagnostics, Treatments, and Physical and Emotional Impacts

Saawariya Shahriar, Sai Hinge, Polly Geddes

Sunshine 4 Cancer Kids Research Internship

August 30, 2025

Clear Cell Sarcoma of the Kidney (CCSK); A Review of the Diagnostics, Treatments, and Phys.

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Abstract

Clear Cell Carcinoma of the Kidney (CCSK) is a rare, malignant renal cancer, composing

approximately 5% of pediatric renal cancer cases, affecting patients in a 2:1 ratio of male to

female. It has significant impacts on the physical and emotional health of patients who navigate

struggles of social isolation, frustration, and fear. Explaining its original name as

“Bone-Metastasizing Renal Tumor of Childhood” (Lal & Singhai, 2011), CCSK has a high

tendency to metastasize, with the common regions of metastasis being the liver, bone, regional

lymph nodes, lung and brain. CCSK assumes many morphologies, including those similar to

Wilms Tumor, which comprises approximately 85% of pediatric renal cancers, and

Blastemal-Type Nephroblastoma,and other renal neoplasms that make identification and

diagnosis difficult. Diagnostic criteria and protocols have been developed by the International

Society of Pediatric Oncology - Renal Tumor Study Group (SIOP - RTSG) who produced the

UMBRELLA SIOP-RTSG 2016 Protocol (Brok et al., 2022). Although there is a lack of

universal genetic or molecular markers of CCSK, multiple mutations and irregularities within the

cell signalling pathways have been identified. Internal tandem duplications (ITD) in the BCOR

gene have been found in approximately 70% of CCSK patients, and fusion of the YWHAE gene

with either the NUTM2B or NUTM2E gene due to a translocation of 10:17 and q22:p13 has

been identified in approximately to 12% of CCSK patients. Both genetic markers are exclusive

to CCSK (Aldera & Pillay, 2020). For treatment, SIOP suggests preoperative chemotherapy,

using a combination of vincristine, actinomycin, and/or doxorubicin depending on the stage and

area of the neoplasm (local or metastasized) with surgery following afterwards. In contrast, the

North American Wilms Tumor Study Group (NWTS) recommends surgery as the first treatment

option if safe to carry out.

Discussion

● The unknown pathology and development of CCSK is interesting. Some cancers have

hereditary and genetic links, however, CSSK has not been associated with inheritance,

and instead research proposes the role of epigenetic divergence in the onset of CCSK.

● Misdiagnosis of CCSK for other pediatric renal tumors is common. Exploring the

characteristics of CCSK will be essential for the development of new diagnostic tests,

procedures and markers, in order to make more accurate diagnoses.

● CCSK is an aggressive neoplasm that has a propensity to metastasize, with a common

site being the brain. There are multiple treatment regimens, developed by the Society of

Pediatric Oncology including a combination chemotherapy drugs. Assessing the efficacy

survival, relapse outcomes and toxicity of treatment for patients using these regimens

ensures that patients are receiving effective treatment with a consideration for the risks of

these treatments as well.

● The social and emotional impacts of CCSK are significant and damaging to patient

mental health and quality of life. As well as examining the biological impacts of CCSK,

understanding the difficulties patients face when experiencing CCSK and treatment,

including fear of survival outcomes, social isolation and depression is important. This

will allow CCSK patients, including young children who are still developing socially and

emotionally, to receive better emotional support.

Introduction

Clear Cell Sarcoma of the Kidney (CCSK) is a rare and a very aggressive pediatric

kidney cancer, most commonly diagnosed in children under five, it affects boys more frequently

than girls. CCSK is known for its high strength to invade, especially the liver, bone, lungs, and

brain, which makes the treatment and prognosis highly complicated. Despite its rarity, CCSK has

a significant impact on both the physical and emotional health of child patients and families,

unfortunately often leading to social isolation, and extreme fear and anxiety due to the

uncertainty of the danger of the disease. Initially named "Bone-Metastasizing Renal Tumor of

Childhood," CCSK shares many traits with other renal tumors like Wilms tumor, making

diagnosis difficult. While there may be no universal genetic markers for CCSK, research has

uncovered specific mutations, including internal duplications in the BCOR gene and

translocations involving the YWHAE gene, that are unique to this condition (Aldera & Pillay,

2020). Treatment typically involves chemotherapy followed by surgery, but, like many other

cancers, varies based on the tumor’s stage and its potential to reach distant sites; however, while

medical advancements have helped improve survival rates, the emotional toll on patients is often

a factor that is overlooked. The fear of the cancer relapsing and the side effects of treatment can

lead to depression, anxiety, and reduced social interaction. This essay will explore the genetic,

diagnostic, and therapeutic challenges of CCSK while also addressing the emotional struggles

that children suffering from Clear Cell Sarcoma of the Kidney face. By discussing these aspects,

our goal is to highlight the need for integrated care that balances both the physical and

psychological needs of children with CCSK.

Genetic and Molecular Markers

There is a pressing need for research into markers of CCSK. As of now, imaging methods

including MRIs, X-Rays and CT scans, and blood and urine laboratory tests are employed to

identify and diagnose CCSK in patients. There is a scarcity of universal genetic and molecular

markers unique to CCSK, and as a result, in the 2001 SIOP clinical trials of pediatric

nephroblastoma, 27% of CCSK patients were originally misdiagnosed with other renal cancers

(Gooskens et al., 2018). There has, however, been multiple genetic mutations identified in

studies of CSSK. Somatic internal tandem mutations have been found on exon 16 of the BCOR

gene on chromosome X in around 70% of CCSK patients. One study by Uneno-Yokohata et al.,

identified 20/20 CCSK tumors with BROC internal tandem mutations. To conclude tandem

mutations of the BCOR gene are unique to CCSK, the study analyzed 193 non-CCSK pediatric

renal tumors, including rhabdoid tumor of the kidney and congenital mesoblastic nephroma

which both have similar morphologies to CCSK, and found that 0/193 tumors contained any

tandem mutations found in CCSK tumors (Ueno -Yokohata et al., 2015). The BCOR protein has

been found to form complexes with multiple polycomb proteins, which regulate gene expression

by altering the structure of chromatin, including PCGF1 and RYBP. BCOR is further involved in

the non-canonical PRCI complex which epigenetically alters the gene expression of genes that play a role in embryonic development and the functioning of mesenchymal stem cells. This

suggests that the BROC mutation which alters the non-canconical PRC1 complex, may be

associated with pathological gene expression.

Another genetic divergence identified in 12% of CCSK tumors in a study by O’Meara et

al. is a chromosomal translocation (10;17) (q22;p13) in which the YWHAE gene fuses with

either NUTM2B or NUTM2E of the NUTM2 gene family (O'Meara et al., 2011).

Cutcliffe et al. identified commonalities among a 14 CCSK tumors when compared to 15

Wilms tumors and 3 healthy fetal kidneys, including a diversity of greater than 30 neural

markers, (genes involved in neural activity, differentiation, expression and development) that

were significantly upregulated by CCSK cells compared to the Wilms tumors and the healthy

fetal kidneys. The proto-oncogene EGFR was found to be significantly upregulated by CCSK

tumors compared to the Wilms tumor subset which produced mainly negative results for the

immunohistochemistry using EGFR antibodies (Cutcliffe et al., 2005). The study also found that

the akt1 protein, active in the Akt pathway that inhibits apoptosis and necrosis and promotes cell

division, was upregulated in 3/6 CCSK tumors analyzed in western blot analysis, although the

other tumors potentially contained phosphorylated akt1 protein. Although neural markers and the

upregulation of the AKT1 signalling pathway are not exclusive to CCSK neoplasms, they offer

insight into the pathways and markers which can be further researched in order to develop

effective tests and therapies for CCSK diagnosis and treatment.

Diagnosis

Signs and Symptoms

Clear cell sarcoma of the Kidney (CCSK) is a form of cancer that initiates metastasis

throughout the body. Many symptoms may not be identifiable immediately, as tumors take

extensive time to grow. It maintains several symptoms that are associated with common

illnesses, such as the flu, so it is crucial to distinguish them from the development of CCSK.

A primary sign of CCSK is hematuria, which induces blood in the urine and develops

through a kidney infection. There are three main types of hematuria, including gross hematuria

(visible to the naked eye), microscopic hematuria (invisible to the naked eye), and dipstick

hematuria (change of color on a dipstick through oxidation) (Cleveland Clinic). Many patients

experience abdominal mass and constant flank pain, which are exacerbated with a growing tumor

in the kidney(s). Furthermore, the development of anemia (low blood cell count) is expected with

the condition, which results in extensive fatigue and reduced energy. CCSK weakens the immune

system and enables susceptibility to infection, thus initiating a fever in the body (99 to 99.5°F).

Cancerous cells within the kidney deprive the body of necessary nutrition, which can induce

weight loss. Moreover, CCSK triggers high calcium levels, leading to a number of significant

symptoms in the patient, such as weakness, thirst, constipation, and cognitive impairment (Patel

et al., 2024).

Medical Equipment and Tests

To identify kidney abnormalities and provide diagnosis for CCSK, a medical professional

is expected to conduct a number of urine tests, such as urinalysis and urine cytology. In the

urinalysis, the red/white blood cells, chemicals, and proteins are analyzed. This may identify

microscopic hematuria and regular functioning of the vital organs. The urine cytology examines

for potentially cancerous cells in the urine, which is conducted subsequent to the recognition of a

tumor in the renal pelvis.

Following the diagnosis of cancer, blood tests are held to identify if metastasis has

progressed and the body is prepared for surgery. A known symptom of CCSK is anemia, which

displays a low blood count. However, tests have the rare possibility of showing polycythemia

(high blood count). In addition to blood examinations, blood chemistry tests are conducted due to

the rise of particular chemicals in kidney patients, which are used to identify calcium, liver

enzymes, and lactate dehydrogenase (LDH) (American Cancer Society).

Magnetic Resonance Imaging (MRI) sequences are utilized to determine the nature of the

tumor and various characteristics that may lead to the development of CCSK. T1-Weighted

MRIs analyze matter within the tumor, which display clear contrast within the uneven

distribution of signal intensities or hypotensities. T2-Weighted MRIs exhibit potential cysts and

distinguish the hyperintensity of CCSK tumors. In addition, Diffusion-weighted MRIs locate

stromal tumor types, metastasis, cell density, and enhancement patterns in the kidney (Van Der

Beek et.al, 2023). Dynamic contrast-enhanced (DCE) MRIs use contrast agents to analyze tissue

perfusion and blood flow through the tumor. (Gordon et.al, 2014).

Computer tomography (CT) scans display cross-sectional imaging of the body and

potential metastasis. The abdomen is analyzed to determine the size, shape, and location of the

tumor. CT angiography provides clear focus into the kidney’s surrounding blood vessels and

abnormalities. Moreover, CT-guided needle biopsies gather a sample of the abdominal mass to

recognize cancerous matter (American Cancer Society).

Medical Professions

To treat and diagnose clear cell sarcoma of the kidney, a team of specialists is required to

provide extensive examinations of the body. Each professional analyzes a particular region to

detect and prevent abnormalities.

Nephrologists are one of the primary doctors involved in the diagnosis of CCSK. They

maintain expertise in kidney diseases and analyze the organ’s functions to identify potential

damage. To complete this, they may gather data from laboratory tests or conduct examinations

with medical equipment (Kidney Cancer Association Team). Salary is dependent upon the

location of the doctor’s practice. However, the average practitioner makes $267,826 per year

(ZipRecruiter). To become a nephrologist, it is critical to acquire an undergraduate degree (four

years of pre-medical school), a MD degree (four years of medical school), internal medicine

residency (3 years of specialization), and fellowship (2-3 years of nephrology) (Torres, 2024).

This is applicable to the following medical professionals, as every medical specialization is

required to take the same route. Overall, it is an extensive process and requires immense

dedication to accomplish expertise in the field.

Urologists are essential for treating urinary tract issues, which include the kidneys,

ureters, bladder, and urethra. In the specialty, Urologic oncologists are responsible for diagnosing

and providing treatment for urinary tract cancers. They may insert a needle into the tumor to

obtain a miniscule sample (Kidney Cancer Association Team). Urologists typically make an

average of $368,164 per year and Urologic Oncologists receive $368,086 per year

(ZipRecruiter).

Radiologists receive kidney imaging tests and spend extensive time for analysis of

abnormalities. Diagnostic radiologists inspect the results of imaging tests to determine necessary

options for further treatment. Whereas, interventional radiologists utilize imaging tests for

procedures, such as biopsy or ablation therapies (Kidney Cancer Association Team). Diagnostic

radiologists are known to receive an average of $267,432 annually and interventional

radiologists make $347,480 annually.

All such medical professionals are crucial for effective treatment and regimen for patients

with CCSK.

Treatments

Clear Cell Sarcoma of the Kidney (CCSK) is a really rare and aggressive form of kidney cancer

that has a higher effect on children. Because it spreads quickly to extensive organs, such as the

liver, lungs, bones, and brains, it has a requirement of urgent and effective treatment. Doctors use

a combination of chemotherapy, surgery, and sometimes radiation therapy to fight the disease.

Acting fast and using the right treatment approach is important for improving survival rates and

reducing serious complications in child patients.

Chemotherapy

Chemotherapy is one of the foundation treatments for Clear Cell Sarcoma of the Kidney

(CCSK). It is often used before surgery to help shrink the tumor, which makes it easier and safer

to remove. If the cancer has already extended greatly, chemotherapy can provide aid to slow it

down and keep it under control. The most common drugs used are vinscristine, actinomycin B,

and doxorubicin. While these medicines can be very effective, they also come with side effects

like nausea, hair loss, low blood cell counts, and nerve problems. That is why doctors continue to

examine the patients during treatment to help manage these side effects (CCS, 2024).

Surgery

Surgery is vital for removing localized CCSK tumors. In cases where the cancer is specifically in

the kidney, surgery can be performed after chemotherapy has reduced tumor size. For highly

invasive cases, surgery targets secondary tumors in areas like the lungs or bones. The goal is to

remove as much of the tumor as possible while also ensuring preservation of kidney function.

However, if metastasis is widespread, surgery may not even be an option, and chemotherapy and

radiation may be better to do.

Radiation Therapy

Radiation therapy is mostly used for more serious cases of CCSK, most importantly when the

cancer has spread to important areas like the brains, lungs, or bones. It helps shrink tumors that

cannot be removed with surgery or lower the chance of the cancer coming back in high-risk areas

of the body. Even though radiation can be helpful, it also comes with long-term risks for

children, including learning problems, delayed development, and a bigger chance of getting other

types of cancer later on in life (NRU, 2018).

Supportive Treatments

Children with Clear Cell Sarcoma in the Kidney also need supportive care to help balance the

side effects of both the cancer and its treatments. Pain management is important to reduce

discomfort caused by the tumor or therapy. Nutrition support helps kids stay strong and keep

their immune system working, especially since chemotherapy can lead to things such as nausea

and a loss of appetite. Mental and emotional support, such as counseling or therapy, is also

major. Going through cancer treatments is overwhelming, and many children experience anxiety,

depression, and feel isolated from others as a cause of their sickness.

Ongoing research into genetic markers and targeted therapies offers new hope for

improving the treatment of Clear Cell Sarcoma of the Kidney (CCSK) in children. Mutations in

the BCOR and YWHAE genes, a factor identified as unique to CCSK, could lead to more

personalized treatment approaches (Aldera & Pillay, 2020). Additionally, immunotherapy is

being introduced as an effective alternative to traditional chemotherapy, harnessing the body's

immune system to target cancer cells more impactfully and with fewer side effects. While current

treatments, including chemotherapy, surgery, and sometimes radiation therapy, have led to

increased survival rates, side effects remain a major conflict. The potential of genetic research

and targeted therapies to provide more customized treatments is an exciting direction for the

future. However, along with these advances, it’s crucial to provide extensive physical and

emotional support to ensure the overall well-being of children undergoing treatment for CCSK.

Statistics

Prevalence and Epidemiology

Clear Cell Sarcoma of the Kidney makes up 3-5% of pediatric renal cancers, with a mean

age of diagnosis at 36 months old (Argani et al., 2000). It is the second most common pediatric

renal tumor after Wilms tumor, and affects males and females in a 2:1 ratio respectively.

Prognosis

CCSK prognosis was originally poor, due to a high likelihood of metastasis and relapse,

and because of CCSKs aggressive behavior, however, starting from the development of the

NWTS treatment protocols in 1974 and SIOP protocols, which have been evolving since the

1980s, the overall patient survivorship and relapse-free patient outcomes have increased

(Gooskens et al., 2018). Between 1971 and 1979 in SIOP trials SIOP1, SIOP2 and SIOP5, the

overall survival of 43% and the 5-year event free survival of 30%. The increasing rigor of

chemotherapies is a notable adjustment made to the treatment regimens for CCSK. In the third

NWTS, more rigorous chemotherapy regimens, specifically including doxorubicin, increased 6

year patient relapse-free outcome from 25% to 64%. In the fifth NWTS treatment including

chemotherapy of cyclophosphamide, etoposide, vincristine, and doxorubicin, and by

radiotherapy produced an overall patient survival of 90% and a five year relapse free outcome

from 79% of patients (Zhang et al., 2024).

Metastasis and Relapse

Approximately 40% of metastasis cases occur in the brain, although the most common

location was previously bone. A review of the NWTS 1-4 further found that there was a 29%

metastasis rate A collaborative study from SIOP and the Associazione Italiana Ematologia

Oncologia Pediatrica (AIEOP) approximates that 16% of CCSK patients experience a relapse

(Zhang et al., 2022).

Physical Impacts

Individuals with CCSK maintain a great variety of symptoms, including those associated

with common illnesses, such as fever, constipation, and vomiting. In the abdominal region of the

body, a large mass, swelling, and pain is to be expected (Colorado Medicine). Through the

development of the tumor, children begin having concerns with blood in the urine, lower back

pain, weight loss, fatigue, and hypertension (Markman et.al ). Although some of these symptoms

may not be visible to the naked eye, it is significant to identify such changes in the body and

report them to a medical professional immediately.

Exposure to particular forms of treatment can increase the likelihood of acquiring certain

conditions, such as cancer or chronic kidney disease. Consistent chemotherapy and radiation

therapy may lead to a deterioration of other health aspects (Aldrink). Chemotherapy has a myriad

of side-effects as a result of its intensity, such as difficulty to process information, nerve damage

that induces numbness, low blood count-anemia, and bowel issues. The body may also have a

higher risk of infections and infertility. Many such issues can outlast the span of chemotherapy

and develop a second cancer (Jones). Thus, it is crucial to maintain a balance of treatment and

frequent regulation of their potential consequences.

CCSK is widely known for its development of metastasis (spread of cancer from the

original site) in the body. Despite chemotherapy and surgical options, the condition still

maintains the ability to metastasise to the bone. Within the bone regions, the skull and skeleton

are at highest risk (Alavi). In addition to regional metastasis, local areas of tissue and lymph

nodes can be impacted as well.

The neurological implications of CCSK are expanded through the process of brain

metastasis, which develops tumors. 10-13% of patients experience the conditions of this

establishment due to the vascularity of CCSK (ABTA Team). Based on the size and location of

the metastasis, a range of symptoms occur in the patient. This includes headaches, frequent

nausea, seizures, and/or cognitive difficulty. It is critical to identify the source of CCSK and

initiate methods to prevent the spread to vital organs.

Emotional Impacts

The majority of children with CCSK have profound mental health issues due to the

multitude of changes in their daily lives and inadequate strategies for stress management. Many

patients experience depression and anxiety, which can vastly reduce the possibility of an

effective treatment. Faulty expectations of the “cure” and a lack of knowledge tend to develop

such anxiety (Psychosocial effects of kidney cancer). Moreover, patients maintain uncertainty

over the removal of the tumor and a potential for recurrence (Goldberg).

Emotional concerns often lead to a substandard quality of life and reduced social

interactions. This is particularly evident when the child endures fatigue and a lack of connection

with others in their environment (Harding). While facing the demands and side-effects of cancer

treatment, it is common for children to isolate themselves.

Moreover, many treatments, including surgeries and chemotherapy may alter certain

aspects of the patient’s body. This can lead to low self-esteem and a struggle with accepting the

new changes. It also tends to reduce the patient’s likelihood of resorting to public spaces

(National Cancer Institute). Therefore, there is great significance in attending therapy sessions

and finding solace in prioritizing mental health.

Conclusion

Clear Cell Sarcoma of the Kidney (CCSK) is a rare and aggressive pediatric cancer that

significantly impacts both the physical and emotional well being of affected children. Despite

advancements in methods to help diagnose over time, such as detection techniques and genetic

testing, the disease continues to challenge to identify, due to the ways it is similar to other renal

tumors and its power to metastasize rapidly. Current treatment options, including chemotherapy,

surgery, and radiation therapy, have improved survival rates, but the emotional toll on patients

remains a critical concern as it is often overlooked. Children undergoing treatment for CCSK

often face fear, anxiety, and isolation, which can intervene with their overall well-being and

recovery.

The ongoing studies and research of genetic markers and targeted therapies offer hope for future

advancements in treatment, offering the potential for more personalized approaches with fewer

dangerous side effects. However, as we move toward more effective strategies, it is equally

important to provide emotional support to ensure that children not only survive but are also in the

joyful state every child deserves to be in, during and after their battle with CCSk. Extensive care

that addresses both the biological and psychological factors of the disease will be important for

improving the quality of life for these patients. Continued research and an inclusive approach to

care are major in the fight against CCSK, offering hope for better outcomes of the health of

young patients and a brighter future for affected children and their families.

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