What is a atypical teratoid rhabdoid tumor?

What is a atypical teratoid rhabdoid tumor?

Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT) is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children aged three years and younger, although it can occur in older children and adults. About half of these tumors form in the cerebellum or brain stem.

What is rhabdoid tumor cancer?

Rhabdoid tumor (RT) is an aggressive pediatric soft tissue sarcoma that arises in the kidney, the liver, the peripheral nerves and all miscellaneous soft-parts throughout the body. RT involving the central nervous system (CNS) is called atypical teratoid rhabdoid tumor. RT usually occurs in infancy or childhood.

What type of tumor is ATRT?

Atypical teratoid rhabdoid tumors (ATRT) are rare, aggressive tumors of the central nervous system. They occur primarily in the cerebellum (the part of the brain that controls movement and balance) and the brain stem (the part of the brain that controls basic body functions).

What is a Teratoid?

teratoid in American English (ˈtɛrəˌtɔɪd ) adjective. Biology. resembling a monster; malformed or abnormal.

Can ATRT spread?

Do ATRTs spread? ATRTs can be very fast-growing. They often spread to other areas of the CNS through cerebrospinal fluid (CSF).

What is Teratoid tumor?

Atypical teratoid rhabdoid tumor (ATRT) is a rare and fast-growing cancerous tumor of the brain and spinal cord. About half of these tumors begin in the cerebellum or brain stem: The cerebellum, located at the base of the brain, controls movement, balance and posture.

Has any child survived ATRT?

0001. No other clinical characteristics were predictive of survival. Three of four patients 3 years or older with progressive disease were successfully rescued with ifosfamide, carboplatin, and etoposide therapy. Conclusion: Children presenting with ATRT before the age of 3 years have a dismal prognosis.

What is an atypical teratoid rhabdoid tumor?

MRI of an Atypical Teratoid Rhabdoid Tumor (ATRT) in the brain. ATRT is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord. To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue. What is the grade of ATRTs?

What is an rhabdoid tumor of the kidney?

Rhabdoid tumors are large and heterogeneous, usually located centrally within the kidney. They are lobulated with individual lobules separated by intervening areas of decreased attenuation, relating to either previous hemorrhage or necrosis 2.

What is the prevalence of rhabdoid tumors in children?

Rhabdoid tumors occur exclusively in children, with 60% occurring before the age of 1 year of age, and 80% before the age of 2 years (average age of presentation = 11 months). Approximately 2% of pediatric renal malignancies are rhabdoid tumors 1. Rhabdoid tumors are very aggressive and initial manifestation may be due to metastatic disease.

What are the signs and symptoms of rhabdoid tumors?

Rhabdoid tumors are very aggressive and initial manifestation may be due to metastatic disease. Local symptoms and signs include hematuria and loin/flank mass. Additionally, patients may develop hypercalcemia secondary to elevated parathormone levels 1 .