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Rhabdoid Tumor

Rhabdoid Tumor Causes

The cell origin of rhabdoid tumors is currently unknown. However, recent research indicates a genetic basis for the tumor regardless of location. The disease has also been associated with abnormalities or mutations in chromosome 22.

Rhabdoid Tumor Definition

Rhabdoid tumor pertains to a very rare and extremely aggressive tumor commonly occurring in children ages 2 to 13 years. Initially, rhabdoid tumor was believed to be a variant of Wilms' tumor. However, recent studies have shown that rhabdoid tumor is an entirely separate entity. The location of this highly malignant tumor may be intraventicular, supratentorial, or infratentorial.

Rhabdoid Tumor Diagnosis

A rhabdoid tumor is commonly detected through magnetic resonance imaging or MRI. This imaging technique usually provides greater anatomical detail, allowing physicians to distinguish a tumor from healthy tissue. Additionally, magnetic resonance spectroscopy or MRS may be helpful in identifying specific organic compounds produced metabolically within the sample tissue, thus differentiating between glial tumors and neuronal tumors.

Rhabdoid Tumor Symptoms and Signs

Rhabdoid tumors have varied symptoms, which depend largely on the age of the affected child as well as the location of the tumor. In documented cases, common symptoms include seizures, enlargement of the skull combined with increased pressure on the brain (hydrocephalus), as well as enlarged head size (fontanels) in infants. Affected children suffer from raised intracranial pressure because the rhabdoid tumor obstructs the normal flow of the cerebrospinal fluid, causing an overproduction of fluid. The abnormal accumulation of fluids in turn leads to vomiting, headaches, lethargy, and movement restrictions.

Rhabdoid Tumor Treatment

Treatment for rhabdoid tumor varies according to the affected child's age, health and medical history, as well as the tumor's location, type, and size. Often, a multidisciplinary approach is employed, ranging from neurology to oncology. A standard mode of treatment is surgical removal of the malignant tumor, followed by consistent chemotherapy. In addition, radiation therapy may be done on children over 3 years of age. For younger children, focal radiation therapy may be incorporated with caution.

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