This information is intended to provide a general overview of Desmoplastic Small Round Cell Tumours (DSRCT). It is not a substitute for professional medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider for any health concerns or before making any decisions related to your health.
Desmoplastic small round cell tumours (DSRCT) are a rare and aggressive type of soft-tissue sarcoma. This condition leads to the formation of multiple tumours, primarily located in the abdomen and pelvic area, though the exact site of origin is often unknown.
The first documented case of DSRCT was recorded in 1989, and approximately 200 cases have been diagnosed globally since then. The disease is most common in Caucasian boys. Although researchers suspect that a genetic problem may be the underlying cause of DSRCT, this link has not yet been definitively proven.
Because DSRCT is often not diagnosed until the cancer is at an advanced stage, the tumours typically grow large and spread (metastasise) through the lymph system or bloodstream to other parts of the body. Common areas for spread include the lymph nodes, lungs, bone, and liver. Other types of cancer, such as rhabdomyosarcoma, may also be found in the abdomen alongside DSRCT.
DSRCT is considered a dangerous form of cancer that frequently shows resistance to treatment and often recurs (comes back) after initial treatment.
In rare cases, DSRCT may be passed down genetically from one generation to the next. Genetic counselling may be appropriate for some families.
Desmoplastic small round cell tumours (DSRCT) typically do not present with any early symptoms. In the majority of cases, the first sign noticed is a hard, rounded mass in the abdomen, which can grow quite large before it is discovered. The child may also experience abdominal pain. Due to the rarity of DSRCT, it is frequently misdiagnosed by general practitioners and paediatricians.
It is important to remember that DSRCT is rare. If a child experiences any of these symptoms, they are most likely caused by a less serious condition. Nevertheless, it is advisable to consult a doctor, as these signs may indicate other health concerns.
As part of one of the nation's largest cancer centres, the SSCHRC Children's Cancer Hospital possesses more experience with desmoplastic small round cell tumours (DSRCT) than most other hospitals. SSCHRC utilises the latest, most accurate diagnostic technology, and its physicians are highly specialised.
Given that desmoplastic small round cell tumours are rare, few paediatricians or general practitioners are familiar with the disease. Even many oncologists (cancer doctors) have not treated a case of DSRCT.
An early and precise diagnosis significantly improves the chances for successful treatment. However, DSRCT is often misdiagnosed as other types of abdominal tumours until it has already spread to other areas of the body.
If your child presents with desmoplastic small round cell tumour symptoms, the doctor will conduct a physical examination and ask questions about your child's health and your family's medical history.
One or more of the following diagnostic tests may be used to determine if your child has DSRCT and if it has spread. These tests may also be used to monitor if treatment is working.
A biopsy is almost always necessary to confirm a DSRCT diagnosis. This procedure involves removing a small sample of cells from the tumour or fluid in the abdomen for examination under a microscope. One of these methods is typically used:
Desmoplastic small round cell tumours can be challenging to diagnose. The pathologists at SSCHRC Children's Cancer Hospital are highly specialised in diagnosing complex cancers, including DSRCT. They welcome the opportunity to provide second opinions for DSRCT.
SSCHRC's Children's Cancer Hospital is among the few cancer centres in the nation with extensive experience treating desmoplastic small round cell tumours, a rare and aggressive form of paediatric cancer.
Using the latest research and supported by modern technology and techniques, Children's Cancer Hospital physicians create a customised, comprehensive course of treatment to address the child's specific issues. The objective is to provide the best chance for effective treatment while minimising the impact on the child's body.
Surgery is usually the first line of treatment for DSRCT. As with all operations, DSRCT surgery is most successful when performed by a specialist with considerable experience in the specific procedure. SSCHRC Children's Cancer Hospital surgeons are highly skilled and renowned globally.
HIPEC, or hyperthermic peritoneal perfusion with chemotherapy, is an innovative surgical procedure pioneered at SSCHRC Children's Cancer Hospital and has proven to be safe and effective for many children with DSRCT. SSCHRC is one of the few hospitals in the world offering this therapy.
Adapted from a surgical procedure used for adult abdominal tumours, HIPEC involves debulking—surgically removing as much of the tumour(s) as possible. Following this, heated chemotherapy is circulated within the abdomen.
The specialists at SSCHRC Children's Cancer Hospital are actively researching new ways to treat DSRCT, including targeted therapies designed to help the body fight cancer at a cellular level. Clinical trials are available for novel agents to treat this rare disease, many of which are only offered at SSCHRC Children's Cancer Hospital.
After carefully evaluating your child's case, the expert team will discuss a recommended course of treatment for DSRCT. Since DSRCT is complex and rarely encountered, there is no single standard treatment protocol.
Surgery is almost always a component of DSRCT treatment. Procedures may include:
Some DSRCT patients respond to chemotherapy, but most will relapse. Long-term, low-dose chemotherapy may be beneficial for patients who are in remission or who have a tumour that cannot be surgically removed.
Radiation therapy (or radiotherapy) uses high-energy beams to destroy cancer cells. New radiation therapy techniques and the remarkable skill of SSCHRC Children's Cancer Hospital doctors allow them to target tumours more precisely, delivering the maximum amount of radiation with the least damage to healthy cells.
SSCHRC Children's Cancer Hospital is advancing the future of DSRCT treatment by developing innovative targeted therapies. These agents are specifically designed to treat each cancer's unique genetic/molecular profile to help the child's body fight the disease. The doctors who treat DSRCT at SSCHRC are dedicated researchers who have pioneered and actively lead national and international clinical trials involving novel targeted agents.