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Desmoplastic Small Round Cell Tumors: An Overview of Diagnosis and Treatment

  • sunshine4cancerkid
  • Aug 6
  • 29 min read
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Word Count: 8298


Calvin Le, Eva Saadi, Judy McClure-Anim, & Thalita Marpaung


Calvin Le | Writer/Researcher

College of Southern Nevada & Nevada State High School

Eva Saadi | Writer/Researcher

Hodan Nalayeh Secondary School

Judy McClure-Amin | Writer/Researcher

Spelman College

Thalita Marpaung | Writer/Researcher

Sampoerna Academy

Table Of Contents

Table Of Contents...................................................................................................................... 2

Abstract...........................................................................................................................................5

Introduction....................................................................................................................................6

Soft Tissue Sarcoma...................................................................................................................6

Desmoplastic Small Round Cell Tumor (DSRCT)....................................................................6

Discussion....................................................................................................................................... 8

Why Desmoplastic Small Round Cell Tumor?..........................................................................8

Methods...........................................................................................................................................9

How to Diagnose Patients............................................................................................................10

Signs and Symptoms of DSRCT..............................................................................................10

Medical Equipment Used For Diagnosing DSRCT................................................................. 10

Biopsy.......................................................................................................................................11

Molecular Testing ................................................................................................................... 11

Immunohistochemistry and Genetic Testing........................................................................... 12

Diagnostic Laparoscopy...........................................................................................................12

Medical Professions..................................................................................................................... 13

Neurosurgeons......................................................................................................................... 13

Neurologists and Neuro-oncologist......................................................................................... 13

Radiation and Medical Oncologists.........................................................................................13

Pathologist............................................................................................................................... 14

Treatments.................................................................................................................................... 15

Overview of Desmoplastic Small Round Cell Tumor............................................................. 15

Introduction to Desmoplastic Small Round Cell Tumor .................................................. 15

Importance of Understanding Treatment Options............................................................. 15

Early Detection........................................................................................................................ 15

The Importance of Early Detection....................................................................................15

Challenges in Early Detection........................................................................................... 15

Methods Used For Detection............................................................................................. 16

Imaging........................................................................................................................ 16

Biopsy.......................................................................................................................... 16

Genetic Testing............................................................................................................ 16

Surgery.....................................................................................................................................16

Radiation Therapy....................................................................................................................17

Whole-Abdominopelvic Radiotherapy (WAP-RT)............................................................18

Intensity-Modulated Radiotherapy (IMRT).......................................................................18

Palliative Radiotherapy......................................................................................................18

Chemotherapy..........................................................................................................................19

P6 Protocol...............................................................................................................................19

HER2 Antibody-Drug Conjugates...........................................................................................19

Immunotherapy........................................................................................................................19

CAR T-cell Therapy.................................................................................................................19

Targeted Drugs Delivery..........................................................................................................20

Complementary Therapies.......................................................................................................20

Statistics........................................................................................................................................ 21

Demographics of DSCRT........................................................................................................ 21

Rate of Diagnosis....................................................................................................................22

Diagnosis based on Age...........................................................................................................23

Diagnosis based on the influence of the EWS-WT1 fusion gene............................................23

Survival rate of Desmoplastic Small Round Cell Tumor........................................................ 25

Different Stages of DSRCT..................................................................................................... 26

Prognosis of DSRCT based on Age, Race, and Gender.......................................................... 26

Impacts..........................................................................................................................................27

Introduction..............................................................................................................................27

Physical and Functional Symptoms........................................................................................27

Tumor Burden and Abdominal Distress ........................................................................... 27

Weight Loss and Malnutrition........................................................................................... 27

Urinary and Reproductive Symptoms................................................................................27

Neurological and Cognitive Symptoms...................................................................................27

Neurological Symptoms.....................................................................................................27

Cognitive Impairments.......................................................................................................28

Psychological and Emotional Symptoms.................................................................................28

Psychological Distress....................................................................................................... 28

Support Systems................................................................................................................ 28

Social Impacts..........................................................................................................................28

Social Isolation...................................................................................................................28

Impact on Daily Activities.................................................................................................28

Management Strategies............................................................................................................28

Medical Intervations.......................................................................................................... 28

Supportive Therapies......................................................................................................... 29

Summary..................................................................................................................................29

Patient Impacts...................................................................................................................29

Importance of Integrated Care........................................................................................... 29

Conclusion.................................................................................................................................... 30

Summary of DSRCT................................................................................................................30

Early Detection, Treatment, and Prognosis............................................................................. 30

Awareness Effort .....................................................................................................................30

Bibliography................................................................................................................................. 31

Acknowledgements...................................................................................................................... 38


Abstract

In this comprehensive overview, we analyze “Desmoplastic Small Round Cell Tumors,”

also known as “DSRCT,” a rare malignancy commonly affecting children and young adults.

Covering topics like clinical outcomes, treatment approaches, medical diagnoses, and health

professionals relevant to the disease. We conducted a literature review using various online

databases such as PubMed and Google Scholar, as well as reputable sites such as the National

Institute of Health (NIH), Mayo Clinic, and SarcomaUK, to gather information on DSRCT.


Our analysis suggests that DSRCT mainly affects cautious young Caucasian males from

ages 10 to 30 and has a survival rate of 15% over a 5-year period. Our analysis identified

multiple required combination therapies, including surgical approaches, chemotherapy, and

radiation therapy, with optimal outcomes achieved through individualized multidisciplinary

treatment plans. With an incidence of 1 to 1 billion cases worldwide, it is by far the rarest cancer

affecting children and young adults, resulting in limited clinical knowledge and research. This

comprehensive analysis demonstrates the importance of early diagnosis and multidisciplinary

care among DSRCT patients.


Introduction

Soft Tissue Sarcoma

Soft tissue sarcoma is a rare type of malignant tumor that arises in the tissues that

connect, support, and surround other body structures. A malignant tumor is a collection of cancer

cells that can invade and destroy surrounding tissue. The tumor may also metastasize to other

areas of the body. It can develop at any age, but it’s most common in individuals older than 50

years old. It begins as the growth of cells in the body’s soft tissues, such as muscles, fat, blood

vessels, nerves, tendons, and joint linings. It can transpire anywhere in the body, mainly

happening in the arms, legs, and stomach, although more than 50% of them develop in one arm

or leg. Signs of soft tissue sarcoma include a lump or swelling in an area of soft tissue, but

sometimes, no signs or symptoms occur until the tumor is big enough to press on nearby nerves

or other parts of the body. However, soft tissue sarcoma can be diagnosed with a biopsy. One of

the strongest risk factors for soft tissue sarcoma is radiation therapy, particularly used to treat

cancers like Hodgkin lymphoma, non-Hodgkin lymphoma, or breast cancer. Soft tissue sarcoma

often develops in the area of the body that was treated with radiation. The average time between

coming into contact with radiation and a diagnosis of soft tissue sarcoma is 10 years. Less than

5% of sarcomas are caused by radiation therapy. The benefit of using radiation therapy to treat

cancer outweighs the increased risk of developing soft tissue sarcoma. (Canadian Cancer Society,

n.d.) More than 50 types of soft tissue sarcoma exist, some types are more likely to affect

children. Others affect mostly adults. These cancers can be hard to diagnose because they may be

mistaken for many other types of growths. Soft tissue sarcoma treatment usually involves

surgery. Other treatments might include radiation therapy and chemotherapy. Treatment depends

on the size, type and location of the cancer and how quickly it grows. (Mayo Clinic, n.d.) Cells

in soft tissues might alter and no longer develop or act normally. These alterations may result in

benign tumors such as lipomas (fat cells) and hemangiomas (blood vessels).


Desmoplastic Small Round Cell Tumor (DSRCT)

Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue sarcoma with

extremely aggressive behavior, typically developing in the abdomen and pelvic areas, forming

aggressive tumors in connective and soft tissues. DSRCT begins as the growth of cells then

further envelopes on the tissue lining of the abdomen and pelvis called peritoneum. The cancer

cells can then quickly spread to other parts of the body including the colon, bladder, and liver.

(Mayo Clinic, n.d.) DSRCT are extremely rare and most often occur in young white males

between the ages of 10 and 30, nevertheless, it can happen to anyone. However, some studies

suggest that only 200 cases of DSRCT have been reported since its first initial discovery in 1989.

(National Cancer Institution, n.d.) It was discovered by William L. Gerald and Juan Rosai,

described as a new clinicopathologic entity. The disease is currently managed using a

combination of chemotherapy, radiation, and rigorous surgical resection. Even with intensive

multimodal therapies such as chemotherapy, radiation, and surgery, DSRCT has a high

recurrence rate, with the majority of patients relapsing or dying within three years after

diagnosis. Due to the low survival rate, DSRCT highlights the urgent need for more effective and

creative therapy alternatives. Despite thorough treatment for newly diagnosed patients, the cure

rate remains poor. A distinguishing feature of DSRCT is the chromosomal translocation

t(11;22)(p13;q12), which causes the EWSR1 gene on chromosome 22 to exchange parts with the

WT1 gene on chromosome 11. This EWSR1-WT1 fusion gene produces a faulty gene that

promotes tumor growth, and its presence serves as a key diagnostic marker for DSRCT. There is

limited data on second and third line therapy for relapsed or progressing illness, and new

medicines have demonstrated only modest success. However, recent discoveries of molecular

changes in DSRCT suggest intriguing possibilities for future treatment trials. (Mello et al., 2021)

This research will examine Desmoplastic Small Round Cell Tumor (DSRCT) as a rare and

aggressive pediatric cancer, focusing on its molecular features, challenges in treatment, and the

effects it has on adolescents and young adults.


Discussion

Why Desmoplastic Small Round Cell Tumors?

  • A very rare and highly aggressive tumor, with an incidence of approximately 0.2-0.3

cases per million annually and fewer than 200-450 cases reported in the literature.

(Gonzalez et al., 2024). The survival and cure rate of this tumor is extremely low, in

which the five year survival rate is around 15-25% even with intensive multimodal

therapies. These poor outcomes highlight the urgent need for further research into

DSRCT. By deepening our understanding of its biology, we hope to contribute to the

development of more effective treatment strategies and improved survival rates.


  • The t(11;22)(p13;q12) chromosomal translocation, which produces the EWSR1-WT1

fusion gene, is a defining feature of DSRCT. (Ladanyi et al., n.d.) This unique genetic

signature is not only necessary for diagnosis, but it also paves the way for molecular

study and potential treatments. Understanding how the fusion corresponds with the

biology of the disease provides insights into how targeting a single, defining gene could

lead to novel therapeutics, which drew our interest due to its uniquely complex diagnostic

profile.


  • DSRCT is challenging to distinguish from other small round cell tumors because of its

co-expression of epithelial, mesenchymal, and neural markers, requiring molecular

testing for an accurate diagnosis. (Blejis et al., 2021) This diagnostic complexity piqued

our interest and drove us to learn how integrated molecular and histological methods

enable correct identification.


  • DSRCT research has historically been hindered by scarce tumor samples and cell models.

Recently, the development of new cell lines and patient-derived xenografts has enabled

better preclinical research, including the identification of molecular vulnerabilities like

NTRK3, EGFR, CDK4/6, and immunotherapy targets such as B7-H3. (Frontiers, n.d.)

This opens new possibilities for targeted and immune-based therapies.


Methods


A comprehensive literature review and search strategy were utilized, involving databases

like PubMed, Google Scholar, and the CSN Library. The review process and paper selection

were conducted as follows. The initial search included phrases like “Desmoplastic small round

cell tumor (DSRCT)” and “DSRCT causes.” Continued search strings included “DSRCT’s

linkage with the fusion gene” and “DSRCT prognosis.” Furthermore, information provided by

the Mayo Clinic, the National Cancer Institute, Johns Hopkins University, the Canadian Cancer

Society, and the National Library of Medicine was also utilized.


While having a range of 2020–2025 was ideal in gathering the most up-to-date sources

and literature, there was simply not enough data in that time frame, so the search range

expanded. The data that was used shows multiple pieces of information related to DSRCT’s

correlation with gender, age, and race when it comes to diagnosing the cancer, as well as

perceiving its prognosis. Data was extracted from multiple studies and sources with emphasis on

key search words related to “gender, age, and race,” which also expanded to include information

regarding the EWS-WT1 fusion gene, which is linked with the diagnosis rate of DSRCT. The

findings from the studies that were chosen were collected, analyzed, summarized, and presented

in a complete and comprehensive case study of DSRCT.


How to Diagnose Patients

Symptoms and Signs of DSRCT


DSRCT is a rare, fast-growing soft tissue sarcoma that commonly arises in the abdominal

and pelvic areas, spreading rapidly through connective tissues. DSRCT can be mistaken for other

soft tissue sarcomas such as Wilma's tumor and Ewing sarcoma due to overlapping symptoms

and similar tumor locations. Some symptoms include:


Abdominal Distension and Pain: Patients often experience bloating or swelling due to

tumor growth. Typically arises from peritoneal involvement, often found late when large masses

are present. (Jordan et al., 2017)


Palpable Mass: A growth/lump in the body that can be felt by touch. In this case, upon

touching a tumor, it can feel firm and hard in the abdomen during physical exams, often after

subtle growth. (Fagouri et al., 2018)


Weight Loss: Patients commonly experience significant, often rapid weight loss, tied to

the disease's burden and metabolic disruption. (Fagouri et al., 2018)


Constipation: Constipation is a common gastrointestinal indication that results from

intestinal compression or obstruction caused by tumour lumps.


Nausea and Vomiting: Reflect irritation or blockage in the bowel resulting from tumor

spread along the interstitial surfaces.


Ascites: The accumulation of fluid in the abdomen is frequently observed in advanced

stage presentations due to peritoneal involvement. (Fagouri et al., 2018)


Medical Equipment Used for Diagnosing DSRCT


One of the main and most important medical equipment used is imaging tests. These include:


Ultrasound: Ultrasounds are one of the first imaging tests patients will undergo,

especially when the patient is present with abdominal discomfort or swelling. In DSRCT,

ultrasound can reveal ascites (fluid buildup), cystic-solid masses, and tumors on the serosal

surface of the organs. It may also reveal internal features, such as necrosis or hemorrhage within

the tumour, which can help raise initial suspicion of a malignant process (National Cancer

Institute, n.d.).


Computed Tomography Scanner (CT): CT scans are commonly used to diagnose

abdominal and pelvic masses associated with DSRCT. They provide cross-sectional images that

reveal the size, quantity, and location of tumours. CT is also useful for assessing metastases and

organ involvement, such as the liver or lymph nodes. Intravenous contrast can improve picture

quality and help define tumours more clearly. (PubMed, n.d.)


Magnetic Resonance Imaging (MRI): In terms of soft tissue detail, MRI outperforms CT.

It is especially beneficial for determining the tumor's connection to the surrounding tissues and

organs. MRIs can better define tumour boundaries and detect abdominal distribution, making

them crucial for surgical planning. Contrast-enhanced MRIs also increase tissue distinction.


Positron Emission Tomography (PET): PET scans are used with CT scans (PET/CT) to

determine the metabolic activity of DSRCT tumours. Since these tumours are often aggressive

and hypermetabolic, PET scans aid in identifying both the main tumor and any secondary

tumours. (Clarke et al., n.d.) This is extremely useful for staging cancer and arranging treatment.


Biopsy

Tissue Biopsy: A tissue biopsy is the most definitive way to identify DSRCT. A sample

of tumour tissue is removed, either by needle biopsy or surgical excision, and inspected under a

microscope. This enables pathologists to evaluate the tumor's histological characteristics, such as

the distinctive nests of small, round, blue cells surrounded by desmoplastic stroma. (National

Cancer Institution, n.d).


Core Needle Biopsy: A larger needle is used to extract a core of tissue from the tumours.

This is more prevalent than fine needle aspiration for DSRCT because it allows for extensive

histological and molecular investigation.


Fine Needle Biopsy: A thin needle extracts cells from the tumour for cytological analysis.

While less invasive, this method may provide limited information since it gathers fewer cells and

lacks tissue structure, making it less definitive for the diagnosis of DSRCT.


Molecular Testing

Following tissue extraction, molecular testing is performed to discover genetic anomalies

unique to DSRCT. The most distinguishing trait is the EWSR1-WT1 gene fusion, which results

from a chromosomal translocation (t(11;22)(p13;q12). Detecting this fusion using methods such

as RT-PCR or FISH helps confirm the diagnosis and distinguish DSRCT from other small round

blue cell tumors. (PubMed, n.d.)


Immunohistochemistry and Genetic Testing

Immunohistochemistry (IHC) is a laboratory technique for detecting certain proteins in

tumour tissue using antibodies that bind to those proteins. This procedure aids in the

identification of the types of cells present as well as their features, which is critical in

distinguishing various tumours. In DSRCT, IHC labelling indicates the presence of numerous

important protein markers, including desmin (which has a unique dot-like pattern), cytokeratin,

WT1, vimentin, and epithelial membrane antigen. These markers reveal that DSRCT cells

exhibit characteristics of many tissue types, including epithelial, mesenchymal, and neural,

highlighting the tumor's complexity. Along with IHC, genetic testing is performed to detect the

EWSR1-WT1 gene fusion, a unique genetic defect that validates DSRCT diagnosis (National

Cancer Institution, n.d.).


Diagnostic Laparoscopy

A minimally invasive surgical procedure in which a camera is placed into the belly to

directly see tumours and perform a biopsy. This is occasionally employed when imaging is

unclear or to help stage disease. (PubMed, n.d.)


Medical Professions

Neurosurgeons

Though surgery is the main treatment for DSRCT, the inclusion of neurosurgeons is less

common unless the cancer spreads to the brain or spinal cord. If cancer has spread to the brain or

spinal cord, which is fortunately less likely to spread than to other areas like lymph nodes and the

liver, a neurosurgeon may be needed to operate for surgical removal or management of these

tumors.


  • Microsurgical resection is a technique that surgeons use to precisely eliminate spinal cord tumors while decreasing damage to surrounding tissues.

    • Stereotactic radiosurgery, the next method, can be used to treat spinal tumors, at times in combination with surgery, to reduce tumors and mitigate symptoms.


In some very extreme rare cases of DSRCT, there would be instances where the disease can form

as an intracranial tumor; in this case, a neurosurgeon would be very involved in the treatment.


Neurologists & Neuro-oncologists

Neurologists focus on the treatment of the nervous system, including the brain, spinal cord, and

nerves. Whereas a neuro-oncologist focuses on the cancer of the nervous system.

  • Neurologists become more involved in DSRCT cases when:

    • When the tumor begins to affect the whole nervous system

    • Spreading towards the brain, skull, or spinal cord

    • When the tumor starts to affect affect structures like the brachial plexus

  • Neuro-oncologists are involved when:

    • The tumor has spread to the central nervous system

    • There are neurological problems from chemotherapy or radiation

    • The tumor primarily affects neural structures


Radiation and Medical Oncologist

Medical oncologists are the head of the DSRCT management, as they oversee the systemic

chemotherapy that is the foundation of the treatment given to DSRCT patients.

  • Chemotherapy Management

    • Coordinating high-dose of chemotherapy protocols

    • Administering stem cell rescue procedures when indicated

    • Controlling the P6 regiment, needed for the DSRCT treatment


Radiation oncologists play a very major role in patient treatment against DSRCT, especially in

managing local disease control.

  • Managing radiation to identify recurrence trend of the disease

  • Using intensity modulated radiation therapy (IMRT) to decrease the toxicity compared to the conventional 2D radiation therapy

Both specialties work within an integrated treatment where a multidisciplinary strategy of

high-dose chemotherapy, extensive surgical resection, radiation, and stem cell rescue increases

the survival rates of some patients.


Pathologists

Examine tissues, cells, and biological fluids to learn more about disease causes and effects. In

relation to DSCRT, pathologists help distinguish DSRCT from other small cell tumors, which

can be a challenging task, so required expertise is a must.


Treatment

Overview of desmoplastic small round cell tumor


Introduction to Desmoplastic small round cell tumor


Desmoplastic small round cell tumor (DSRCT) is a tumor described to be a hostile, rare, and

deadly type of sarcoma. Treatment strategies were developed based on their characteristics of

chromosomal translocation and pathology. DSRCT can occur anywhere in the body, but it mostly

occurs in the abdomen whereas it is mostly associated with children and young adults. DSRCT

has been described to be a nest of tiny round cells separated by desmoplastic stroma. DSRCT

stains with desmin, cytokeratin, and S100 which includes mesenchymal, epithelial, and neural

markers. Researchers have identified translocation resulting in an active fusion protein involving

ewing sarcoma and wilms tumor. These translocations are identified through an essential open

biopsy in order to diagnose DSRCT. If EWS translocation is not identified, accurate diagnosis

would be difficult. DSRCT could be treated through combinations of several treatments.


Treatment options


Due to the rarity and aggressive nature of DSRCT, treatment is often complex and may

include additional therapies like Hyperthermic Intraperitoneal Chemotherapy (HIPEC) and

clinical trials. Desmoplastic small round cell tumor (DSRCT) treatment typically involves a

multimodal approach, combining surgery, chemotherapy, and radiation therapy.


Early Detection


Importance of Early Detection


Early detection of Desmoplastic Small Round Cell Tumor (DSRCT) is crucial due to its

aggressive nature and tendency to spread, but it's often challenging because early symptoms are

vague and non-specific. Symptoms such as abdominal pain, swelling, and weight loss may not

appear until the tumor has grown significantly.


Challenges in Early Detection


Early detection of Desmoplastic Small Round Cell Tumors (DSRCT) is challenging due to

the lack of early warning signs and the tendency for symptoms to be nonspecific, often leading to

misdiagnosis. The tumors often grow to a significant size within the abdominal cavity before

being detected, and can be mistaken for other conditions or benign masses. DSRCT can be easily

mistaken for more common malignancies like rhabdomyosarcoma, neuroblastoma, lymphoma, or

germ cell tumors, especially in young patients which can lead to inappropriate treatment,

delaying effective treatments.


The presence of widespread disease at the time of diagnosis further complicates treatment

and reduces the chances of successful outcomes.


Methods used for early detection

Early detection of Desmoplastic Small Round Cell Tumor (DSRCT) is challenging due to

its rarity and lack of specific symptoms. However, imaging techniques like CT scans, MRI, and

PET scans, along with biopsies, are crucial for diagnosis and assessing the extent of the disease.

Genetic tests, specifically those identifying the EWSR1-WT1 fusion gene, are also essential for

confirming the diagnosis.


Imaging

CT scans Useful for visualizing the tumor and its spread within the abdomen and other areas.

MRI provides detailed images of the tumor and surrounding tissues, particularly helpful for

assessing tumor extent and location. PET scans can help detect active cancer cells and identify

distant metastases.


Biopsy

A sample of the tumor is taken and examined under a microscope to confirm the presence of

DSRCT cells. It involves obtaining a tissue sample for microscopic examination, often through

fine needle aspiration, core biopsy, or surgical removal. This allows pathologists to identify the

specific genetic mutation (EWSR1-WT1 fusion) characteristic of DSRCT.


Genetic Testing

Identifying the EWSR1-WT1 fusion gene is a key diagnostic marker for DSRCT. This fusion

arises from a chromosomal translocation which brings together the EWSR1 gene on

chromosome 22 and the WT1 gene on chromosome 11. The resulting fusion protein is believed

to play a crucial role in the development and progression of DSRCT.


Surgery


Abdominal sarcomatosis is a common finding with tumor implants ranging from 1 mm to 40

cm or more. Typically, omental disease is found in most patients in addition to peritoneal

studding on the diaphragm, spleen, Morrison’s pouch, abdominal wall peritoneum, small bowel

mesentery, and almost certainly in the pelvis. Surgeons will remove the tumor and will aim to

take out an area of normal tissue. Complete surgical resection, including cytoreduction and

hyperthermic intraperitoneal chemotherapy (HIPEC), is standard therapy for appendiceal

carcinoma pseudomyxoma peritonei and other diseases. HIPEC surgery is performed shortly

after cytoreductive surgery, though the effectiveness of both surgeries are not yet confirmed due

to their complex nature. HIPEC surgery after cytoreduction is essential, as it kills remaining

micrometastases and tumor cells after the surgery. To administer HIPEC, drains are inserted in

the abdominal cavity, allowing high-dose chemotherapy.


Figure 1. These images depict a surgical procedure done to treat DSRCT

(“Presentation of a rare, highly aggressive peritoneal disease: desmoplastic small round cell tumor”)

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Radiation Therapy

For desmoplastic small round cell tumors that affect the abdomen, radiation might be an

option to kill cancer cells that remain after surgery. The advent of intensity-modulated radiation

therapy (IMRT) has resulted in a significant reduction in radiotherapy-related morbidity,

specifically gastrointestinal and hematologic toxicity. Radiotherapy uses high-energy radiation to

destroy cancer cells. Most relapses were intraperitoneal, suggesting that intraperitoneal therapy

in addition to conventional surgery, chemotherapy, and radiotherapy is necessary to improve

outcomes. Radiotherapy gives an easy way for tumor removal and kills excess cancer cells.

Radiotherapy assists in controlling signs and symptoms such as pain.


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Figure 2. This image shows how radiotherapy is used for DSRCT

(“Treating DSRCT”).


Whole-Abdominopelvic Radiotherapy (WAP-RT)

WAPRT involves delivering radiation to the entire abdominal and pelvic region. It's a

common approach for DSRCT, especially when tumors are localized to the abdomen or have

spread within the peritoneal cavity. It can also be used as a consolidated chemotherapy method

for small abdominal lesions. The average dose of patients receiving WART is 26.95gy. The main

adverse reactions involve gastrointestinal reactions such as nausea, diarrhea, and acute

hematological toxicity.


Intensity-Modulated Radiotherapy (IMRT)

IMRT therapy delivers radiation from multiple angles, allowing a more precise dose

distribution and minimizing side effects. It is most used with WAP-RT to prevent further damage

to healthy tissues. Two isocenters are placed in the upper and lower abdomen. This treatment

uses nine coplanar beams for the upper abdomen and seven coplanar beams for the lower

abdomen. The combined dose from both plans is then assessed to determine the radiation

delivered to the target area and nearby organs at risk.


Palliative Radiotherapy

Radiation therapy could be used in a palliative setting to help manage symptoms such as

pain, especially when the cancer has spread to other areas of the body.


Chemotherapy

Chemotherapy may be used before surgery to shrink the cancer or after surgery to kill

remaining cells. Chemotherapy uses powerful drugs to kill cancer cells. Total abdominal

radiation therapy, and high-dose chemotherapy followed by autologous stem cell rescue have all

been used in the treatment of DSRCT, with little improvement in survival. Control of DSRCT

with chemotherapy is most effective in children treated with Ewing’s type chemotherapy, which

has become the standard after efficacy of this regimen was demonstrated by Kushner et al.

Chemotherapy for DSRCT often involves a combination of drugs, with the P6 protocol

(cyclophosphamide, doxorubicin, vincristine, ifosfamide, and etoposide) being a common

regimen. Other chemotherapy options include vincristine, ifosfamide, doxorubicin, and etoposide

(VIDE), and vincristine, actinomycin-D, ifosfamide, and adriamycin (V AIA).


P6 Protocol

This regimen includes cyclophosphamide, doxorubicin, vincristine, etoposide, and

ifosfamide. Irinotecan, topotecan, carboplatin and cisplatin can be added in selected patients

depending on the extent of the disease. However due to the presence of stromal composition,

these tumors show little response to chemotherapy. Whole abdomen radiotherapy (WART)

emerges as a post-chemotherapy treatment option, despite potential complications.


HER2 Antibody-Drug Conjugates

HER2 was detectable in DSRCT samples from patients and PDXs, with higher sensitivity

RNA assays and improved IHC detectability. Treatment of /HER2-expressing DSRCT PDX, cell

line, and organoid models with T-DXd or trastuzumab emtansine resulted in tumor regression.

HER2 ADCs may represent options for managing aggressive sarcoma, possibly fulfilling an

urgent need for more effective clinical therapy.


Immunotherapy

Immunotherapy is an emerging treatment approach for desmoplastic small round cell tumors

(DSRCT) particularly in cases where surgery and chemotherapy have limited success. Various

immunotherapy strategies, including CAR T-cell therapy, checkpoint inhibitors, and bispecific

antibodies, are being explored to target and destroy cancer cells.


CAR T-cell Therapy

CAR T-cells are made to target specific antigens on cancer cells. Anti-B7H3 CAR-T cell

immunotherapy is being evaluated in a study for recurrent/refractory solid tumors, including

DSRCT. One major determinant of successful CAR-T therapy is the specificity of the mAb, and

as such the ScFv regions used to generate the CAR-T against the cell surface expression of the

target antigen.


Checkpoint Inhibitors

Drugs such as nivolumab and ipilimumab, which block immune checkpoints (like PD-1 and

CTLA-4), have shown potential in a subset of DSRCT cases, although PD-L1 expression in

DSRCT is generally limited. Pembrolizumab, an anti-PD-1 agent, is being evaluated in a phase 2

clinical trial for rare sarcomas, including DSRCT.


Targeted Drug Therapy

Targeted drug treatments attack specific chemicals present within cancer cells. By blocking

these chemicals, targeted drug treatments can cause cancer cells to die. Targeted therapy might

be recommended if the cancer comes back after treatment. It may also be offered if the cancer

has spread to other parts of the body. Targeted therapy can be used alone or combined with

chemotherapy.


Complementary Therapies

Complementary therapies for Desmoplastic Small Round Cell Tumors (DSRCT) focus on

managing symptoms and side effects of cancer and its treatment, often alongside standard

medical care. These therapies include mind-body approaches like relaxation techniques,

acupuncture, and mindfulness-based stress reduction, which may help reduce anxiety, stress, and

pain while also improving coping mechanisms and quality of life. Techniques such as deep

breathing and progressive muscle relaxation can help manage stress and anxiety associated with

cancer and its treatment.


Statistics

Demographics of DSCRT


Table 1. An overview of known demographic data and the proportion of different demographics

diagnosed with DSRCT.

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This data collected from the Surveillance, Epidemiology, and End Results (SEER)

program by the National Cancer Institute shows an overview of the percentage of different

demographics and the rate of which they are diagnosed with Desmoplastic Small Round Cell

Tumors (DSRCT). This data represents 192 diagnosed cases of DSRCT. Some of the most

common sites of the cancer are found in the regions of the pelvis and abdomen.


Rate of Diagnosis

The number of cases for DSRCT has grown over the last twenty years, with rates of

DSRCT diagnosis being between 0.22 and 0.05 cases per million people. DSRCT has only been

studied since the late 1980s, and it has been noted as an extremely rare condition, with only 1 in

1 billion people being affected with this condition. Notably, the condition affects distinct

demographics in very different ways, with white males in adolescence to early adulthood being

the most commonly diagnosed demographic.


Diagnosis Based on Age


Figure 3. This figure represents the range of ages that are commonly diagnosed with DSRCT.

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The data taken from Table 1. It was used to create this figure, which shows that the

majority of diagnosed cases of DSRCT occurred within the range of adolescence to early

adulthood. This age range accounts for over 50% of all diagnosed cases within this study.

Furthermore, it also portrays a downward trend in the diagnosis of cases of the 20-29 year age

range.


Diagnosis based on the influence of race and gender on patients linked with the EWS-WT1

fusion gene


THE EWS-WT1 fusion protein is commonly linked as a driver for DSRCT. The EWS

gene is found on chromosome 22, while the WT1 gene is found on chromosome 11. When

linked, the EWS-WT1is seen as being specific towards DSRCT. This protein fusion is a

transcription factor, a protein regulating gene expression, which helps in the development of

DSRCT.


Figure 4. This data represents 3 sets of data: age, gender, and race.

ree

Source: Lettieri, Christina K., et al. “Incidence and Outcomes of Desmoplastic Small Round

Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database.” Journal

of Cancer Epidemiology, vol. 2014, 2014, pp. 1–5, https://doi.org/10.1155/2014/680126.

Accessed 4 Dec. 2019.


The data from this figure was taken from Table1. which is the same data collected from

SEER by the National Cancer Institute. This data is meant to take into account incidents

(diagnosis of DSRCT) while considering DSRCT’s correlation to the EWS-WT1 fusion gene.

The age range still portrays a downward trend after 30 years of age. On the other hand, males

and whites are shown to be the gender and race that makes up the majority of incidents.


Figure 5. Depiction of the translocation of the EWS gene and the WT1 gene, specifically in the

gastrointestinal tract.

ree

Source: Gonzalez, Jeffrey, et al. “Desmoplastic Small Round Cell Tumors of the Gastrointestinal

Tract.” Cancers, vol. 16, no. 23, July 2024, p. 4101, pubmed.ncbi.nlm.nih.gov/39682287/,


This figure shows translocation of the EWS gene and the WT1 gene in the

gastrointestinal tract. WT1, referring to the Wilms tumor gene while EWS refers to the Ewing

sarcoma gene. The translocation shown in this figure, is where one part of a chromosome breaks

off and attaches to another chromosome. In this case, translocation of these two genes produce

the EWS-WT1 fusion gene.


Survival Rate of DSRCT


Patients with DSRCT undergo extensive treatment and therapy. The median survival rate

is expected to be anywhere from 17 to 25 months after diagnosis. The actual percentage of

5-year survival rates is actually close to 15%. Again, race and age are all factors that affect

survivability but taking consideration of the location of a tumor and one’s individual health is

also necessary when determining survivability of DSRCT. While gender certainly has an

influence on the rate of diagnosis for DSRCT, it does not have an impact on one’s survivability

rate after being diagnosed with the disease. As for the outcome of DSRCT treatment, surgery that

is combined with chemoradiotherapy leads to improved chances of survival as opposed to only

surgery and chemotherapy.


Different Stages of DSRCT


Researcher Hayes-Jordan and her colleagues at MD Anderson Cancer Center in Houston,

Texas proposed a staging system that is being used on a trial basis as a means of studying

DSRCT. It is important to note that this system is not fully validated yet as it is still closely being

studied.


Stage I DSRCT: The patient’s disease is still localized and it is only contained, usually in

the abdomen, in one or two areas.

Stage II DSRCT: The patients’ disease is no longer localized but it has spread to the

lining of the abdominal cavity.

Stage III DSRCT: This stage includes metastasis of the liver and the spread of the cancer

cells outside of the abdominal cavity.

Stage IV DSRCT: The disease has spread throughout the abdominal cavity, the liver, and

even the lymph nodes.


Prognosis of DSRCT based on Age, Race, and Gender


Prognosis is determined by factors that include the location of tumors, where they spread,

and how treatment of the disease. There is still not enough research on this rare disease to have

developed an exact prediction of survival. According to the Cleveland Clinic, 15% to 38% of

DSRCT patients are alive after five years of their initial diagnosis.


Impacts

Introduction

DSRCT is an aggressive and rare soft tissue sarcoma that predominantly affects young

males. It typically originates in the peritoneal cavity, leading to a variety of symptoms due to its

rapid growth and tendency to metastasize. Common symptoms include abdominal pain, swelling,

weight loss, and gastrointestinal disturbances such as constipation and diarrhea.


Physical and Functional Impacts


Tumour Burden and Abdominal Distress

As DSRCT frequently occurs in the abdomen and pelvis, it can cause severe symptoms

such as discomfort, distention, constipation, or urine problems due to mass impact. These

symptoms greatly reduce mobility and comfort, necessitating pain management and palliative

care. In some situations, big tumours or peritoneal dissemination cause ascites, intestinal

blockage, or organ dysfunction.


Weight Loss and Malnutrition

Tumor metabolism, combined with stomach involvement and treatment side effects,

frequently causes weight loss, anorexia, and nutritional deficits. These issues can cause treatment

to be delayed or less effective, worsening the patient's weariness and weakness.


Urinary and Reproductive Symptoms

The mass effect of pelvic or abdominal tumours might interfere with the bladder and

reproductive organs, causing urine difficulties, retention, or hematuria. Male patients'

reproductive structures may be impacted, potentially leading to infertility or sexual dysfunction,

which can have serious emotional and developmental consequences in teenage and young adult

populations.


Neurological and Cognitive Impacts


Neurological Symptoms

While DSRCT primarily affects the abdomen, its aggressive nature can cause metastases

in other organs, including the brain. If the tumour extends to the central nervous system, it can

cause neurological symptoms such headaches, seizures, and cognitive difficulties.


Cognitive Impairments

Patients with DSRCT may develop cognitive problems such as memory loss and trouble

concentrating, especially if the tumour affects the brain regions that control these abilities. These

limitations can have a major influence on everyday activities and overall quality of life.


Psychological and Emotional Impacts


Psychological Distress

The diagnosis of DSRCT can cause severe psychological discomfort, including anxiety

and sadness. The aggressive nature of the disease and the extensive treatment regimens add to

emotional stress.


Support Systems

Patients and their families can benefit from emotional support, coping methods, and a

feeling of community by participating in support groups and receiving counselling. Individuals

afflicted by DSRCT benefit from psychological treatment as part of their overall care strategy.


Social Impacts


Social Isolation

The physical and mental obstacles of DSRCT sometimes lead to social isolation. Patients

may withdraw from social activities owing to exhaustion, discomfort, or emotional suffering,

affecting their connections and support systems.


Impact on Daily Activities

Cognitive and physical disabilities caused by the disease might disrupt daily activities

such as work, education, and personal care. Rehabilitation treatments and occupational therapy

can help to maintain independence and improve quality of life.


Management Strategies


Medical Interventions

DSRCT is frequently treated with a combination of surgery, chemotherapy, and radiation

therapy. Because of the tumor's aggressive nature, prompt and extensive treatment is required.

However, the success of various treatments varies, and the prognosis is still dismal.


Supportive Therapies

Aside from medical treatments, supporting therapies such as pain management,

dietary support, and psychological counselling are critical components of patient care.

These therapies are intended to relieve symptoms, improve quality of life, and promote the

patient's general well-being.


Summary


Patient Impacts

DSRCT has a considerable impact on patients' physical health, emotional well-being, and

social functioning. The disease's aggressiveness and potential for extensive metastases contribute

to a difficult prognosis. Comprehensive treatment plans that cover physical, psychological, and

social issues are critical for treating the effects of DSRCT.


Importance of Integrated Care

An integrated care approach, involving a diverse team of healthcare providers, is critical

in meeting the unique needs of DSRCT patients. This strategy guarantees that all elements of the

patient's health are considered, leading to more effective care and a higher quality of life.


Conclusion

Summary of DSRCT

DSRCT is a type of soft tissue sarcoma characterized by its very aggressive behavior,

linked with the EWS-WT1 fusion gene. Diagnosed cases are predominantly associated with

white adolescent and young adult males. The location of its growth is primarily within the

abdominal and pelvic regions, resulting in tumors in connective and soft tissues. The cancer cells

can metastasize and spread to the colon, bladder, and liver, and even the lymph nodes. This

disease is still not widely understood, and more research is needed to better understand its full

nature.


Early Detection, Treatment, and Prognosis

The early detection of Desmoplastic Small Round Cell Tumor (DSRCT) is vital to

treating a patient and fully addressing their health needs. This disease is aggressive and has a

chance of metastasis. Diagnosis and detection do not come without challenges, as the symptoms

for this disease are not fully understood, coupled with the fact that this disease has not nearly

seen enough research being conducted. It is still relatively new and quite rare, so gathering

enough data to fully understand the disease is a challenge in itself. Known symptoms include

abdominal pain, swelling, and weight loss; however, they may not be apparent until the disease

has already grown to a larger stage. Furthermore, treatment options vary but include surgery,

chemotherapy, and different forms of radiation therapy. As for the survivability and outcome, the

most prominent factor of influence would be treatment. Despite this, survival rates still remain

low after five years of a patient’s diagnosis.


Awareness Efforts

One in a billion is one of many statistics used to describe the nature of Desmoplastic

Small Round Cell Tumor (DSRCT). It is without a doubt one of the rarest types of cancer, and

the higher rate of diagnosis among children and young adults makes it a disease that is worth

being studied. Its rarity and lack of notoriety make it all the more important to raise awareness

for it. Some ways for one to be proactive and advocate for this disease include educational

campaigns, community lessons, fundraising, and awareness outreach events. At most, the more

concern and notability this disease has, the more likely it will receive the necessary funding and

support for continued research. In a world of pediatric cancers, passionate individuals are the

pillar to creating movements, advocacy efforts, and ultimately change.


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