Rare Childhood Cancers

Creator: Dr. Anuthanyaa. R
Published: 2021-07-31T16:48:13

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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.

Written by

Dr. Anuthanyaa. R

Medically reviewed by

Dr. Naresh Chelakalapelli

July 31, 2021

April 4, 2023

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Contents

What Are Rare Childhood Cancers?
Adrenocortical Carcinoma
Neuroblastoma
Colon Cancer
Nasopharyngeal Carcinoma
Pleuropulmonary Blastoma (PPB)
Retinoblastoma

Introduction:

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.

11% of childhood cancers are less than 20 years of age.
75% occur in patients aged 15-19 years.

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

Adrenocortical Carcinoma.
Neuroblastoma.
Colon Cancer.
Melanoma.
Desmoid Tumors.
Gastrointestinal Stromal Tumors (GIST).
Germ Cell Tumors.
Liver Tumors.
Malignant Rhabdoid Tumors.
Nasopharyngeal Carcinoma.
Neuroendocrine Tumors.
NUT Midline Carcinoma.
Pancreatic Tumors.
Pleuropulmonary Blastoma (PPB).
Retinoblastoma.
Sex Cord-Stromal Tumors.
Thyroid Tumors.

1) Adrenocortical Carcinoma:

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

The First Stage - Tumor is not more than 5 cm in length, and it is confined to the adrenal gland.
The Second Stage - Tumor is confined to the adrenal gland but larger than 5 cm.
The Third Stage - Tumor spreads to the lymph nodes but not to the surrounding organs.
The Fourth Stage - Tumor has grown around the adrenal gland and invaded the lymph nodes and organs. Large tumors press against the organs in the abdomen, causing pain and stomach fullness. The most common sites of metastasis are lungs, retroperitoneal lymph nodes, and bones.

Incidence:

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.

Features:

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.

Treatment:

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,

Pheochromocytoma.
Neuroblastoma.

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.

Incidence:

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.

Features:

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.

Treatment:

Chemotherapy.
Radiation.
Surgery (little role).

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:

Type I - Purely cystic with subtle malignant changes.
Type Ir (regressed) - Purely cystic with no malignant components.
Type II - The mixed cystic and solid.
Type III - Purely solid malignant neoplasm.

Features:

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.

Treatment:

Chemotherapy reduces the recurrence risk.
Surgery + VAC chemotherapy for Type 1.
Surgery + IVADo chemotherapy for Type 2 and 3.
Consider radiation for incomplete resection.
Also, close observation is essential.

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.

Incidence:

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.

Features:

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.

Treatment:

It needs a multidisciplinary approach with treatment options such as,

Enucleation.
Systemic chemotherapy.
Local chemotherapies-

Arterial chemotherapy.
Vitreal or subconjunctival chemotherapy.

Other local therapies-

Laser therapy.
Cryotherapy.

Radiation.

Conclusion:

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.

Frequently Asked Questions

1. Name a Few Rarest Cancers in Children?

Rare cancers in children make up fewer than one in 30 of all childhood cancers and can broadly be grouped as rare cancers that only affect children, such as pancreatoblastoma, malignant rhabdoid tumors, and melanotic neuroectodermal tumors of infancy. Cancers that usually only affect adults are cancers of the digestive system, the thyroid, and the adrenal gland.
- Rare cancers in the head and neck area, such as nasopharyngeal cancer.
- Rare hormonal/endocrine cancers, such as pheochromocytoma.
- Rare brain tumors, such as meningioma.
- Rare skin cancers, such as melanomas.

2. Which Is the Most Common Solid Tumor in Children?

The most common types of solid tumors in children include brain tumors, neuroblastoma, rhabdomyosarcoma, Wilms' tumor, and osteosarcoma. This article reviews the etiology and treatment of these diseases and discusses the differences between adult and pediatric solid tumors.

3. Which Kind of Cancer Is Hardest to Find?

Pancreatic cancer is hard to find early. The pancreas is deep inside the body, so early tumors cannot be seen or felt by healthcare providers during routine physical exams. People usually have no symptoms until the cancer has become very large or has already spread to other organs.

4. Which Type of Cancer Is Termed as Number One Rarest Type? How Common Is Epithelioid Hemangioendothelioma?

EHE is very rare, with only one in every one million people diagnosed with this cancer worldwide. Because EHE can be hard to diagnose, the actual number of people with EHE may be higher.

5. What Is the Reason to Overlook Childhood Caries?

Many childhood cancers have much higher survival rates when they are diagnosed in the early stages of the disease. Unfortunately, childhood cancers are sometimes overlooked or misdiagnosed because early symptoms are mistakenly attributed to more common injuries or illnesses.

6. Can Childhood Cancers Be Cured?

The average five-year survival rate for childhood cancers, when considered as a whole, is 86 percent. Cancer survival rates vary not only depending on the type of cancer but also upon individual factors attributable to each child. The average five-year survival rate, not including children with ALL, is 80 percent.

7. What Is the Origin of Childhood Caries?

About five percent of all cancers in children are caused by an inherited mutation (a genetic mutation that can be passed from parents to their children). Most cancers in children, like those in adults, are thought to develop as a result of mutations in genes that lead to uncontrolled cell growth and, eventually, cancer. Childhood cancers often happen or begin in the stem cells, which are cells capable of producing other types of specialized cells that the body needs. A sporadic (happens by chance) cell change or mutation is usually what causes childhood cancer.

8. What Will Be the Life Expectancy for Patients With Adrenal Cancer?

When the tumor is found at the early stage and can be removed surgically, the five-year survival rate is 50 to 60 percent. The prognosis for adrenal cancers that have spread to nearby or distant organs is much less favorable, with only ten to 20 percent surviving five years.

9. How to Diagnose Adrenocortical Carcinoma?

MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). An MRI of the abdomen is done to diagnose adrenocortical carcinoma.

10. Is There Any Treatment Which Cures Adrenocortical Carcinoma?

Initial surgery — complete surgical resection is the only potentially curative treatment for adrenocortical carcinoma (ACC) [1,2]. For patients with potentially resectable stage I to III disease who are surgical candidates, the doctors recommend complete surgical resection as initial therapy

11. Is There Any Treatment to Cure Adrenal Cancer?

When adrenal cancer is found early, there is a chance for cure. But if the cancer has spread to areas beyond the adrenal glands, a cure becomes less likely. Treatment can be used to delay progression or recurrence. Most growths that form in the adrenal glands are noncancerous (benign). Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. For adrenal cancers that cannot be removed with surgery or that return after initial treatments, chemotherapy may be an option to slow the progression of the cancer.

12. Name the Main Cause of Colon Cancer?

- Lack of regular physical activity.
- A diet low in fruit and vegetables.
- A low-fiber and high-fat diet, or a diet high in processed meats.
- Overweight and obesity.
Researchers have found that eating 50 grams of processed meat every day (which is equivalent to one hot dog or four strips of bacon) may increase the risk of colon cancer by 18 percent. While a person necessarily has to give up processed and red meat entirely.

13. Can Colon Cancer Be Cured?

Cancer of the colon is a highly treatable and often curable disease when localized to the bowel. Surgery is the primary form of treatment and results in a cure in approximately 50 percent of patients. However, recurrence following surgery is a major problem and is often the ultimate cause of death.

14. Can Colon Cancer Spread Faster?

Colon cancer most often spreads to the liver, but it can also spread to other places like the lungs, brain, peritoneum (the lining of the abdominal cavity), or distant lymph nodes. In most cases, surgery is unlikely to cure these cancers. In most cases, colon and rectal cancers grow slowly over many years. Most of those cancers start as a growth called a polyp.

15. What Is the Life Expectancy for Colon Cancer Patients?

If the cancer has spread to surrounding tissues or organs and/or the regional lymph nodes, the five-year survival rate is 72 percent. If colon cancer has spread to distant parts of the body, the five-year survival rate is 14 percent. For rectal cancer, the overall five-year survival rate for people is 67 percent. Most of the time, when a person is diagnosed with colon cancer, it is already spreading to other parts of the body. Fact: This is simply not true. The majority of patients diagnosed with colon cancer can be treated and will go on to live normal lives.

Sources

Dr. Naresh Chelakalapelli

Pediatrics

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