When a lump isn't a lump: Understanding pediatric soft tissue tumors

Unlike those found in adults, childhood cancers are particular in many respects, they are rare with cumulative incidence up to 15 years varying only slightly from one country to another country (roughly 1-2.5 per 1000). Furthermore, the histology of these tumors differs considerably, and carcinomas are infrequent among the histological types. Many childhood cancers have histological features that resemble fetal tissue of various stages of development and are therefore designated as embryonal.

The short latent period and innocuous growth with varied presentation makes forth the importance of abnormal lump in kids to be seen by clinician with dedicated expertise. Most of the treatment is tailored as per the diagnosis and because of the genetic predisposition, they are classified on the basis of histology rather than the primary site of the tumor.

Soft tissue tumors can develop in many areas of the body like arms, legs, chest or abdomen. Paediatric soft tissue musculoskeletal masses encompass a wide array of pathology that ranges from benign fatty masses and self-involuting vascular tumors, all the way to aggressive malignant lesions that require a multidisciplinary approach and staging before intervention is performed. They can arise from:

• Fat tissue
• Bone and cartilage
• Connective tissue
• Muscle
• Nerve sheath
• Blood vessels

Signs and Symptoms

Symptoms of a pediatric soft tissue tumor vary depending on the type of tumor.

Common signs include a

• Lump or mass at any site which is progressive and persistent.
• Vascular boggy lesions over head, neck, face, tongue, trunk, limbs suggestive of Vascular tumors
• Compressible and pulsatile swelling suggestive of vascular malformations
• Compressible lesions over neck, axilla, chest walls suggesting lymphatic malformations
• Pain, swelling, numbness or tingling around the lesion suggestive of involvement of nerve sheaths (Ganglions, Ganglioneuromas)
• Infected soft tissue swelling around the nasal bridge, eyebrows, midline neck areas suggestive of Dermoid tumor

Diagnosis

Diagnosing a pediatric soft tissue tumors typically involves an expertise of dedicated clinician who tailors the supportive tests to support the most likely diagnosis which fits the scenario rather than rampant series of tests:

• Physical examination – Your child's doctor will examine your child for signs of tumors and related issues
• Imaging tests – Possible tests to run include ultrasound, CT scan, or MRI to get a closer look at tissue and surrounding organs
• Blood and urine tests aids with some special tumor markers to aid in diagnosis
• FNAC/Biopsy – A small sample of tissue from the tumor may be needed to confirm the diagnosis

Common treatment options are:

• Pharmacotherapy: By using certain drups which helps in involution of lesion , Vascular tumors like hemangiomas can be treated with pharmacotherapy unlike surgery previously
• Intralesional therapy: Injection of some agents which help in regressing the lesions are also the foremost approach foe lymphatic malformations and vascular malformations
• Surgery – This is commonly performed for pediatric soft tissue tumors. The goal is to remove the entire tumor, preserving as much healthy tissue as possibleand reconstructing the cosmesis
• Chemotherapy – This treatment uses powerful medications to destroy cancer cells
• Radiation therapy – Radiation is when high-energy X-rays target and kill cancer cells
• Targeted therapy – Newer drugs are used with chemotherapy to treat tumors

The smallest of the action is always better than the noblest of intentions. Everything starts with a powerful why and that’s how the way forward begins. Through the series of practices performed with consistency and repetition of desired result help us, choosing the right way. That’s how the clinical approach is formulated which adds value to the correct diagnosis.

The good news is that pediatric soft tissue tumors are highly treatable. The specific treatment plan will depend on the presentation, type, histology and stage of the tumor, as well as your child's age and overall health.

(Dr. Shweta Kumari, Consultant - Paediatric Surgery, Sarvodaya Hospital, Sector-8, Faridabad)