Childhood sarcoma is a rare and aggressive form of cancer that is caused by the abnormal growth of tumors in soft tissues or bones. Sarcomas primarily affect children and adolescents, making early detection and treatment crucial for improved outcomes. Despite significant advances in pediatric oncology, sarcoma remains a complex disease, with multiple subtypes presenting unique diagnoses. The lack of distinctive symptoms in the early stages often leads to delayed diagnoses, further complicating treatment strategies.
Current research efforts focus on identifying genetic and molecular factors that contribute to the development of childhood sarcomas, aiming to develop targeted therapies for more effective treatment options.
Dr Akshay Tiwari, Director and Head – Musculoskeletal Oncology, Max Super Speciality Hospital, Saket explains early detection and support in children with sarcoma.
Sarcoma In Children
Dr Tiwari explains sarcoma is a cancer of connective tissue, that is, bones (bone sarcomas) or soft tissues like muscles, tendons, ligaments, nerves, vessels, etc ( soft tissue sarcomas). As far as children are concerned, the commonest sarcomas are osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma.
The upper and lower limbs are the most commonly affected organs, although they can affect any part of the body. That said, there are close to a hundred subtypes of bone and soft tissue sarcomas, he further explains.
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Symptoms Of Sarcoma
Dr Tiwari shares, while the majority of soft tissue sarcomas present with painless swelling or mass, sarcomas of bone usually present with swelling and/or pain in the involved bone, which can sometimes even fracture following trivial trauma. Particularly in playful children and adolescents indulging in sports, these symptoms are commonly mistaken for injuries, leading to delays in diagnosis and treatment.
Hence, it is said that any swelling larger than 5 cm in size or increase in size over time, or pain from an “injury” not improving with regular treatment in a child, should be investigated further after consultation with a specialist.
Sarcoma – Diagnosis And Treatment
Dr Tiwari explains, once clinically suspected, diagnosis of a sarcoma is confirmed with imaging (including X-ray, MRI, and sometimes CT scan) and biopsy. It is highly recommended that a biopsy of a suspected sarcoma be done at a centre where a specialised sarcoma team is available.
Once diagnosed, treatment will consist of a surgical “wide excision” in most cases, where the tumor is carefully removed along with a layer of surrounding normal tissue, followed by appropriate reconstruction.
This “limb salvage surgery” forms the mainstay of sarcoma surgery in today’s times, and is possible only if the patient reaches a sarcoma unit early enough. For children, particularly when they are still growing, this reconstruction becomes even more challenging because of small bones and asymmetrical growth of the limbs leading to discrepancies in limb length.
Patients may also be advised to undergo chemotherapy and/or radiation therapy, depending on the diagnosis. As with all rare diseases, planning, and execution of sarcoma management are best performed by specialised multidisciplinary sarcoma teams.
Sarcoma Awareness
Dr Tiwari shares, parents and caregivers need to know that children can have cancer in their bones, which can be effectively cured with limb-saving surgery and chemotherapy. Any swelling or lump that is more than 5 cm in size (more than the size of a lemon), increasing in size over time, and/or painful, should be considered a sarcoma and investigated accordingly.
Once suspected, patients affected by sarcoma should be referred to specialised sarcoma units. Healthcare providers, patient advocacy groups, NGOs, schools, and colleges should create an environment of awareness about this rare cancer.
