Which among the following is the Most Common tumour associated with Neurofibromatosis in a child ?
Juvenile myelomonocytic leukemia (JMML) is a rare form of blood cancer. JMML predominately affects young children, most often developing in children under the age of 4. The male/female ratio of affected children is 2-3:1. The disorder is characterized by the uncontrolled growth of an immature form of a specific type of white blood cell known as monocytes. These abnormal cells accumulate in the bone marrow crowding out other healthy cells. The bone marrow produces specialized cells (hematopoietic stem cells) that grow and eventually develop into red blood cells, white blood cells and platelets. These cells are released into the bloodstream to travel throughout the body and perform specific functions. Red blood cells deliver oxygen to the body; white blood cells help the body to fight infection; and platelets allow the body to form clots to stop bleeding. When abnormal cells accumulate in the bone marrow, they can affect the production and function of these healthy blood cells. JMML cells can accumulate in other organs of the body including the lungs, skin, liver, and intestines. JMML is an aggressive and difficult to treat disease but is currently cured in about 50% of cases. Currently, the only effective treatment for most patients is allogeneic hematopoietic stem cell transplantation (HSCT). The exact, underlying causes of JMML are unknown, but nearly all patients will have a mutation that changes the DNA inside their blood cells in a RAS gene or in a gene that controls the activation levels of Ras proteins. With an incidence of 1 in 3000, NF1 is the most common familial cancer syndrome. Affected persons develop multiple noncancerous (benign) tumors of the nerves and skin (neurofibromas) and areas of decreased or increased coloration of the skin. Cafe-au-lait spots are common in neurofibromatosis type 1. However, many of these findings may not be present in very young children. Reference: GHAI Essential pediatrics, 8th edition
Juvenile myelomonocytic leukemia
Acute lymphoblastic leukemia
Acute monocytic leukemia
Acute myeloid leukemia
Central Nervous system