Less common Pediatric Cancers in India: Symptoms and Advances in Treatment

Pediatric cancers are a significant health concern worldwide. While awareness of common childhood cancers such as leukemia, lymphoma, and brain tumors is growing, rare pediatric cancers remain a challenge due to limited awareness and delayed diagnosis. While the medical field continues to advance treatment options for these rare cancers, having comprehensive knowledge about them can help parents recognize early symptoms and seek timely intervention.

What are the common Pediatric Cancers?

The most common childhood cancer is Acute Leukemia which accounts for around 30% of all pediatric cancers. The next most common cancers in children are those of the brain and spinal cord. The third most common is Neuroblastoma which has a high mortality rate. Apart from these Nephroblastoma or Wilms tumor is the most common kidney tumor of childhood. 

The above cancers are widely reported and studied, and there have been continuous efforts to advance the treatments for these cancers in children. However, apart from the above children may also present with other cancers which are not as common, but equally debilitating.

Rare Pediatric Cancers

Rare cancers can affect any portion of the body, and at rare times cancers of adults may also present in children. The list of rare cancers in children is exhaustive, but some of these rare pediatric cancers that can present in children are mentioned below. 

Retinoblastoma 

Retinoblastoma is a rare type of eye cancer that primarily affects young children, typically under the age of five. It originates in the retina, the light-sensitive tissue at the back of the eye, which helps one to see. Early detection of this cancer is crucial for effective treatment and for preserving vision.

Symptoms

Below symptoms if noticed should alert the parents-  

• Leukocoria- A white glow in the eyes, which can be noticed even more in photographs- 
• Strabismus- Crossed eyes or misalignment of the eyes
• Poor vision or loss of vision in one or both eyes. Though younger children may not be able to report poor vision clearly, parents should look for other symptoms and notice subtle signs like inability to locate an object, to know if vision could be a problem in the child. 
• Eye redness and swelling- Though redness and swelling are associated with any infection, recurrent problems can indicate a more serious pathology. 
• Enlargement of the eye in advanced cases

Treatment Options and Advances

While conventional treatments like enucleation are required for this cancer if it is large, targeted treatments that are less debilitating are also an option in today’s time. 

Targeted therapy is an advancement that can be used only to target the cancerous cells specifically, and can help in preventing vision. Similarly, laser therapy and cryotherapy too can be applied to target and destroy smaller tumors.

Age-old treatments like chemotherapy and radiotherapy can be used with modern approaches for the best outcomes in children with this cancer. 

 Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is less common  and aggressive form of cancer that arises from skeletal muscle tissue. A soft tissue sarcoma in children  is most frequently found in the head, neck, bladder, and extremities.

Symptoms

Below symptoms if noticed should alert the parents- 

• A lump or swelling that may be painful. This should specifically alert when such a lump is seen without any history of injury and doesn’t decrease in size. 
• Bulging of the eye- This can be seen if the tumor is in the orbit.
• Difficulty urinating or blood in urine- This occurs if the tumor is located in the bladder.

• Nasal congestion or bleeding- In case of a tumor in the sinus.
• Persistent pain or tenderness in affected areas

Any other unusual symptoms that persist can indicate a more serious pathology and it’s advised to consult a healthcare provider for the same. 

Treatment Options and Advances

Apart from conventional treatment which includes surgical therapy, chemotherapy, and radiotherapy, newer advances have emerged too. With modern treatment options side effects have reduced and survival rates have increased. These advances include: 

• Precision Medicine: Every tumor may have a specific underlying cause. Precision medicine helps in the genetic profiling of tumors to tailor treatments based on individual mutations. 
• Proton Beam Therapy: A targeted radiation approach that minimizes damage to healthy tissues.
• Immunotherapy: This involves harnessing the body’s immune system to attack cancer cells. 
• CAR-T Cell Therapy: This is a new and promising experimental approach that modifies immune cells to specifically target rhabdomyosarcoma.
• New Chemotherapy Combinations: Research is also ongoing for novel drug combinations to improve effectiveness and reduce toxicity of the medications used for cancers. 

Hepatoblastoma

Hepatoblastoma is majorly a pediatric  malignant liver tumor that primarily affects children under the age of three. Early detection and treatment significantly improve outcomes for this condition. 

Symptoms

Below symptoms if noticed should alert the parents and caretakers of the child- 

• Abdominal swelling or a noticeable mass in the abdomen region
• Unexplained weight loss in the child
• Loss of appetite
• Nausea and vomiting
• Jaundice (yellowing of the skin and eyes)
• Fatigue and weakness

Treatment Options and Advances

Some conventional treatment options for hepatoblastoma include surgical resection of the tumor if it can be localised or operated on. Chemotherapy to shrink the tumor or to prevent recurrence, and liver transplant in case of a more widespread cancer where removal of the tumor is not possible. 

Apart from the above emerging treatments are aimed at specific molecular pathways involved in tumor growth and can help limit the tumor. Recent advancements have improved survival rates and treatment efficacy for hepatoblastoma and include-

• Genomic Studies: These help in identifying mutations to personalize therapy
• Minimally Invasive Surgery: Laparoscopic techniques for tumor removal can help in excising the tumor with minimal invasion and reduced recovery time
• New Targeted Drugs: In current days investigational therapies are aimed at specific cancer-driving pathways

Ewing Sarcoma

Ewing’s sarcoma is a rare type of bone or soft tissue cancer that primarily affects children and young adults. Most commonly it arises in the long bones of the legs, pelvis, or chest wall.

Symptoms

The below symptoms if noticed should alert the parents- 

• Persistent bone pain, especially at night
• Swelling or tenderness in the affected area
• Unexplained fever
• Fatigue and weakness
• Unintended weight loss
• Fracture of bone with minimal trauma

Treatment Options and Advances 

Below are some treatment options and advances that can be used to treat Ewings sarcoma.

Chemotherapy: To shrink the tumor before surgery or radiation

Surgical Resection: For removal of affected bone or soft tissue 

Radiation Therapy: This is often employed when surgical removal of the tumor is not possible

Targeted Therapy and Immunotherapy: Such a treatment can target specific cancer cells, and reduce the side effects drastically. These are emerging treatments that are still under development. 

Oral Cavity Cancers in Children

Oral cavity cancers in children are rare but can have serious implications if not diagnosed early. These cancers affect the lips, tongue, gums, inner cheeks, and the roof or floor of the mouth. Unlike in adults, where tobacco and alcohol use are major risk factors, childhood oral cancers are often associated with genetic mutations, viral infections, or congenital conditions.

Symptoms

Below symptoms if seen should alert the parents- 

• Persistent mouth ulcers that do not heal
• Swelling or lumps inside the mouth
• Difficulty in swallowing or speaking
• Bleeding from the mouth without injury
• Loose teeth without apparent cause
• White or red patches inside the mouth
• Pain or numbness in the mouth or jaw

Treatment of Oral Cancers

Apart from surgical treatment, chemotherapy and radiotherapy ,development of targeted therapies is underway and can greatly improve the treatment and prognosis of oral cancers. In the case of oral cancers, reconstructive surgery may also be required after tumor removal to restore oral function.  

Advances in Cancer Treatment 

With advancements in pediatric oncology, the survival rate for children with rare cancers has improved significantly. Below are some of the major treatment breakthroughs to understand the advancements as a whole.

• Precision Medicine and Targeted Therapy

At a genetic level, a specific mutation may at times be responsible for the development of cancers. This technology thus employs genetic profiling of tumors to help identify those specific mutations. Once identified targeted therapy for cancers can be used which greatly reduces side effects and debility of cancer treatment. 

Drugs such as Crizotinib for neuroblastoma and Sorafenib for hepatoblastoma are being used.

• Immunotherapy

The use of the body’s immune system to fight cancer has shown promising results.

Chimeric Antigen Receptor (CAR) T-cell Therapy is being studied for pediatric leukemia and neuroblastoma.

• Proton Beam Therapy

Conventional radiation therapy may lead to undue exposure of tissues surrounding cancers, thus causing many unwanted health issues. Proton beam is a highly targeted form of radiation therapy with fewer side effects. It can especially be beneficial for brain tumors and sarcomas.

• Minimally Invasive Surgery

Laparoscopic and robotic-assisted surgeries can help in tumor removal with minimal invasion. Such a surgery can improve outcomes with reduced recovery time.

Wilms’ tumor and hepatoblastoma surgeries have been enhanced through this approach.

• Bone Marrow Transplantation

Bone marrow transplantation can be used in aggressive cases of leukemia, neuroblastoma, and Ewing sarcoma. Stem cell transplantation has significantly improved survival rates for children with high-risk cancers, making it a crucial component of pediatric oncology treatment.

Rare pediatric cancers in India pose a significant health burden, but advancements in medical technology, precision medicine, and collaborative research are improving survival rates. Continued efforts in awareness, funding, and access to cutting-edge treatment will help provide a better future for children battling these rare diseases. Parents and caregivers should stay informed about the occurrence of these cancers and seek medical consultation at the earliest signs of concern. Pediatric haematologists and oncologists are experts in the diagnosis and treatment of all childhood cancers. Early detection ensures timely intervention and access to the best possible treatment, improving the child’s longevity and overall health.

References

https://www.cancer.gov/types/childhood-cancers/patient/rare-childhood-cancers-pdq#_90_toc

https://www.thelancet.com/journals/lansea/article/PIIS2772-3682(23)00028-8/fulltext

https://www.nhsinform.scot/illnesses-and-conditions/cancer/cancer-types-in-children/rare-tumours/