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Rare Childhood Cancers

Published on Jul 31, 2021 and last reviewed on Apr 23, 2022   -  5 min read


Cancer is considered to be a tragic health issue and is even more disastrous when it occurs in children. Even though there is constant advancement in the medical field, it still remains the second leading cause of death in children. Please read the article to know more.

Rare Childhood Cancers


Usually, rare diseases affect a small percentage of the population in a geographical location. All childhood cancers are considered rare, with a prevalence rate of 1,69,000 children under 15 years of age in the United States. These rare childhood cancers are measured for chronic conditions based on their prevalence rate.

Childhood cancers are subacute illnesses and are defined using age-specific incidence rates. Despite its rarity, cancer is considered the most common cause of disease-related death in children.

What Are Rare Childhood Cancers?

Rare childhood cancers are not seen in adults and are diagnosed before 20 years of age. Rare cancers are not that rare.

Amongst the rare childhood cancers, the following are more commonly seen:

1) Adrenocortical Carcinoma:

It is a rare tumor that accounts for 0.5 to 0.2% of all malignancies.


The total incidence rate in the US is 0.72 per million. The incidence rate in children is 0.2 per million, with 25 cases per year and 0.2% of pediatric cancers.


It occurs in children over 4 to 5 years of age with a large-sized tumor and is associated with Cushing’s syndrome, hypertensive crisis, and abdominal pain. The prognosis is 54 to 74% in children, with a five-year survival rate.


The treatment strategy includes surgery (stage 1), extended surgery (stage 2), and surgery with chemotherapy (stage 3 and 4).

Anatomy of the Adrenal Gland:

The adrenal cortex produces cortisol or aldosterone and testosterone or estrogen. The adrenal medulla produces adrenaline or noradrenaline, and it gives rise to medullary tumors such as,

2) Neuroblastoma:

Neuroblastoma is a cancer arising from the abnormal growth of immature nerve cells, disrupting the normal function of affected body parts. It spreads to skin, bones, bone marrow, liver, and lymph nodes. It also develops in the nerve tissue along the spine, neck, and abdomen.

3) Colon Cancer:

Adults - It is the third most common malignant tumor, with 90% of cases present > 50 years of age and preceded by adenomatous polyps. It slowly becomes malignant, and risk factors are obesity, alcohol, tobacco, and prior radiation.

Children - The annual incidence is 1 per million in children below the age of 20 in the US with 1% of pediatric malignancies. Children experience more predisposing syndromes than adults.

4) Nasopharyngeal Carcinoma:

It is an extremely rare cancer and is seen in children below 10 years of age. It is two to three times more common in males than females.


The annual incidence of nasopharyngeal carcinoma is 0.5-2 cases per 100,000 in the US, and there are 0.8 cases per million between 10 to 14 years of age and 1.3 cases per million between 15 to 19 years of age.


There are three types of nasopharyngeal carcinoma based on WHO (world health organization) classification. They are keratinizing, non-keratinizing, and undifferentiated. The risk factors associated are EBV (Epstein-Barr virus) infection, tobacco, alcohol, and high salted fish. In addition, it can lead to nausea, vomiting, hearing loss, xerostomia, and mucositis. The prognosis is 83% in children and 62% in adults, with a five to ten-year survival rate.


5) Pleuropulmonary Blastoma (PPB):

It is a rare, aggressive malignancy of pleuropulmonary mesenchyme. It occurs between the peak age of 1 to 4 years and is most commonly a primary malignancy of lungs in children. The classification of pleuropulmonary blastoma reflects a spectrum of malignant evolution over time:


It presents with multiple lesions in 50% of cases, bilateral lesions in 30% of cases, chest pain, pneumothorax, and incidental pulmonary cysts. It is said that germline mutations in DICER1 present with 66% of pleuropulmonary blastoma cases, and not all families with DICER1 mutations develop PPB. It metastasizes to the bone, liver, brain (11% on type 2 and 54% on type 3) and also has a vascular invasion.

Incidence and Prognosis:

It is an extremely rare cancer with approximately 15 cases per year in the United States.

The prognosis depends on the tumor type, metastases, and complete surgical resection. The five-year survival rates depend on the classification as 89% for type 1, 100% for type1r, 71% for type 2, and 52% for type 3.


6) Retinoblastoma:

It is a rare form of cancer that is exclusively found in young children and develops due to the immature cells of the retina. It is the most common primary malignant intraocular cancer in children.


The incidence of retinoblastoma is 3.7 cases per million children under 15 years of age. There are 300 new cases per year in the US, with 3% of all pediatric cancers and 11% of cancers <1 year of age. Mostly, 63% of cases affected are less than 2 years of age, and 95% of cases are less than 5 years of age.


Leukocoria and strabismus in infants are danger signals of retinoblastoma, which is a malignant retinal tumor. Symptoms are not common but include white color in the center of the eye (when light is shone or flash-photograph), poor vision, eye redness, and eye swelling. It is highly curable with a 97% survival rate. However, in case of the high incidence of late effects, it can lead to vision loss, hearing loss and can lead to death.


It needs a multidisciplinary approach with treatment options such as,

  1. Enucleation.

  2. Systemic chemotherapy.

  3. Local chemotherapies-

  1. Other local therapies-

  1. Radiation.


A prompt approach and thorough evaluation are essential to identify the type and extent of cancer. This is because each type of cancer needs a different treatment. Therefore, in order to determine the type of cancer, better testing is needed which leads to better treatment. Nowadays, childhood and adolescent cancers are more curable than past, and the overall survival rate of most cancers are now approaching 90%. So, consult a doctor at the early stage when you diagnose any unusual symptoms because the early diagnosis reduces the complications.

Last reviewed at:
23 Apr 2022  -  5 min read




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