Type of Cancers

Ewing's Sarcoma
Ewing's sarcoma / Peripheral Primitive Neuroectodermal Tumors (PNET) of bone is a type of cancer usually found in children and young adults. The peak incidence is between ages 10 and 20, it is less common in children under 5 or in adults over 30. Ewing's s can occur in any bone in the body; the most common sites are the pelvis, thigh, lower leg, upper arm, and rib. The tumor is composed of small round blue cells. Ewing's sarcoma can also arise in soft tissue (extra-skeletal).

Osteosarcoma
Osteogenic Sarcoma (osteosarcoma) is a bone forming cancer. It is the most frequent type of bone tumor and is most common between the ages of 15 to 25. Over 90% of tumors are located in the metaphysis (the growing ends of the bone), the most common sites are the bones around the knee which account for 80% of cases. Osteosarcomas vary greatly in radiological and pathological features and therefore needs careful diagnosis to differentiate this from other bone tumors. Most are high grade intramedullary osteosarcomas, about 5% are low grade lesions, some are secondary osteosarcomas (for example those caused by radiation therapy).

Childhood Germ Cell Tumors
These tumors develop from germ cells. In the developing embryo germ cells migrate to the ovaries or testicles and form the ova (egg cells) or sperm cells. Germ cell tumors occur where these cells become cancerous. These tumors typically express high levels of alpha-fetoprotein (AFP). Germ cell tumors are most common in children and young adults, there are different sub-types including endodermal tumors (mostly found in children), dysgerminoma, teratoma, and seminoma (young men). The most common location of these tumors are the ovaries (in females) or testicles (in males). However, they can also develop in other parts of the body such as the sacrococcygeal region, brain, abdomen and other sites - this may occur when some of the germ cells in the embryo did not migrate properly. Germ cell tumors in children tend to be quite different to those in adults.

Langerhan's Cell Histiocytosis
Langerhan's Cell Histiocytosis (also known as Histiocytosis X) is a rare disease. It is not really a cancer, though it may behave like one in some respects. Histiocytes are normal cells found throughout the body, in this disease abnormally large numbers are found. LCH is more common in children but it is often seen in adults too. The disease will be classed as either single-system or multi-system depending on how many of the body's systems are effected. In a study of 314 people treated for LCH at the Mayo Clinic (Howarth et al, Cancer 1999;85:2278-90) 69% had single-system disease and 31% had multi-system LCH. Approximately half of those treated at Mayo were under 25 years old at diagnosis. Of people with single-system disease the system involved was bone (52%), pulmonary (lung) (40%), skin/mucous membrane (7%) and other sites (1%). Bone involvement was more common in younger patients while pulmonary involvement was mostly seen in those over 15 years old at diagnosis.

Childhood Leukemia
Leukemia is the most common cancer of childhood. The body produces lymphocytes to protect the body from infection, in leukemia these cells do not mature properly and become too numerous in the blood and bone marrow. Leukemia may be acute or chronic. The most common type is acute lymphoblastic leukemia (ALL). There are a number of other less common acute types which may be grouped together as acute non-lymphoblastic leukemia (ANLL), this includes acute myeloid leukemia (AML).

Childhood Liver Cancer
Childhood liver tumors are rare. There are two main types of liver cancer; hepatoblastoma and hepatocellular carcinoma. Liver cancer can be found in children of all ages; Hepatoblastomas are more common in patients aged under 3 years, while hepatocellular carcinomas are usually found in patients aged under 4 or between the ages of 12 and 15.

Hodgkin's Disease and Non-Hodgkin's Lymphoma
The lymphatic system helps the body fight infection. There are two main types of cancer associated with the lymphatic system: Hodgkin's Disease and Non-Hodgkin's Lymphoma (NHL). Both are rare in children aged under 3, and are more common in older children and adults. More boys than girls have childhood Hodgkin's disease.

Childhood Non-Hodgkin's Lymphoma
The lymphatic system helps the body fight infection. There are two main types of cancer associated with the lymphatic system: Hodgkin's Disease and Non-Hodgkin's Lymphoma (NHL). Both are rare in children aged under 3, and are more common in older children and adults. More boys than girls have childhood Hodgkin's disease. Childhood non-Hodgkin's lymphoma is a disease in which cancer (malignant) cells are found in the lymphatic system.

Neuroblastoma
Neuroblastoma is one of the most common solid tumors of early childhood usually found in babies or young children. The disease originates in the adrenal medulla or other sites of sympathetic nervous tissue. The most common site is the abdomen (near the adrenal gland) but can also be found in the chest, neck, pelvis, or other sites. Most patients have widespread disease at diagnosis.

Retinoblastoma
Retinoblastoma is a rare tumor of the eye which develops in the cells of the retina, most patients are under 5 years old. Sometimes only one eye is affected (unilateral-retinoblastoma ), but in about two fifths of patients both eyes have the disease (bilateral-retinoblastoma ). Some cases are known to be hereditary.

Childhood Soft Tissue Sarcoma
Childhood soft tissue sarcomas account for approximately 10% of all childhood cancers. About half of all childhood soft tissue sarcomas are rhabdomyosarcoma, which arises from skeletal muscle, these are most common between the ages of 2 and 6. The other soft tissue sarcomas of childhood include a wide range of different histologies including fibro sarcoma, leiomyosarcoma, liposarcoma, schwannoma, soft tissue Ewing's / peripheral neuroectodermal tumors, synovial sarcoma and many other types. These non-rhabdo sarcomas are more common in adults, but these tumors usually behave quite differently in children compared to the same tumors in adults.

Wilms' Tumor
Wilms' tumor is a cancer of the kidney which is very different to adult kidney cancer. Most patients are under 5 years of age at diagnosis, though Wilms' tumor is sometimes seen in older children and occasionally in young adults. In most cases only one kidney has disease (unilateral-Wilms' Tumor); but in some cases both kidneys are affected (bilateral-Wilms' tumor). A small minority of cases are known to be hereditary. Other less common kidney cancers in children include malignant rhabdoid tumors and clear cell sarcoma. Treatment for these is usually similar to that for Wilms' tumor.

Other Cancers / Related diseases
There are a number of other less common types of cancer found in children. Many of these are more common in adults and only effect a small number of children. These cancers include renal carcinoma (kidneys), chondrosarcoma (cartilage), adrenocortical carcinoma, thyroid carcinoma, nasopharyngeal carcinoma, and melanoma. There are many other less common types of cancer, some may be diagnosed with out an identifiable primary site, others may have difficult histologies that are hard to categorize.

What is Fanconi Anemia ?
Fanconi Anemia is a rare disorder found in children that involves the blood and bone marrow. The symptoms include severe aplastic anemia, hypoplasia of the bone marrow, and patchy discoloration of the skin. This is an autosomal recessive condition, affected children usually develop severe aplastic anemia by age 8 to 9 years. Treatment usually consists of bone marrow transplant. Fanconi Anemia is not a cancer, though recent research has shown an association between Fanconi Anemia and leukemia. There are 8 types of Fanconi Anemia; known as complementation groups A through to H.

Malignant Rhabdoid Tumor of Kidney

Malignant Rhabdoid Tumor (MRT) of kidney is an aggressive sarcomatous neoplasm with a histology distinct from Wilms' tumor. In a review of 111 cases of MRT of kidney by the National Wilms' Tumor Study (Weeks, 1989) the median age at diagnosis was 11 months (range 0 - 106 months) and the male: female ratio was 1.5:1. Treatment for MRT of kidney is often similar to that for Wilms' tumor (MRT starting in the brain or other non-renal sites have different treatments). A common cytogenetic feature of MRT is deletion of material from chromosome 22q11.2 including the SMARCB1 (SNF5/INI1) gene.

Mary Jane Glass

Chief Officer

 

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