Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord.

One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal.

AT/RT was only recognized as an entity in 1996 and added to the World Health Organization Brain Tumor Classification in 2000 (Grade IV).

[6] Current research is focusing on using chemotherapy protocols that are effective against rhabdomyosarcoma in combination with surgery and radiation therapy.

[8] Survival rates can be significantly improved when the correct genetic diagnosis is made at the outset, followed with specific multimodal treatment.

A case of a seven-month-old child with a primarily spinal tumor that presented with progressive paraplegia and abnormal feeling in the legs was reported.

There is not enough known about the function of INI1, either as an independent modulator of gene expression or through its association with the SWI/SNF complex, to be able to use specific targeted biological agents for treatment.

Prospective clinical and biologic trials are greatly needed to understand the efficacy of therapeutic interventions, as well as the role of the gene.

In importance of the hSNF5/INI1 gene located on chromosomal band 22q11.2 is highlighted, as the mutation's presence is sufficient to change the diagnosis from a medulloblastoma or PNET to the more aggressive AT/RT classification.

The standard work-up for AT/RT includes:[citation needed] The initial diagnosis of a tumor is made with a radiographic study (MRI[22] or CT-).

Increasingly it is recommended that a genetic analysis be performed on the brain tumor, especially to find if a deletion in the INI1/hSNF5 gene is involved (appears to account for over 80% of the cases).

[citation needed] AT/RT may be related to malignant rhabdoid tumor (MRT), which occurs outside the CNS, usually in the kidney.

[citation needed] AT/RTs can occur at any sites within the CNS; however, about 60% are located in the posterior fossa or cerebellar area.

[3] The tumors' appearance on CT and MRI are not specific, tending towards large size, calcifications, necrosis (tissue death), and hemorrhage (bleeding).

Solid parts of the tumor often enhance with contrast MRI finding on T1 and T2 weighted images are variable.

In addition, 30% of the AT/RTs are located supratentorially and a predilection exists for the cerebellopontine angle,[26] which makes surgical resection difficult.

Various chemotherapeutic agents have been used against AT/RTs, which are also used against other CNS tumors including cisplatinum, carboplatinum, cyclophosphamide, vincristine, and etoposide.

However, the long-term outcomes of AT/RT are so poor that some protocols call for upfront radiation therapy, often in spite of young age.

Proton beam radiation was only offered at Massachusetts General Hospital in Boston and at Loma Linda, California, as of 2002.

[citation needed] Recently, a protocol used by a multicenter trial reported in the Journal of Clinical Oncology resulted in a 70% survival rate at 2–3 years, with most relapses occurring within months, leading to hope that a point exists beyond which patients can be considered cured.

The longest-term survivals reported in the literature are: Cancer treatments in long-term survivors who are children usually cause a series of negative effects on physical well-being, fertility, cognition, and learning.

[44][45][46][47] Metastatic spread is noted in roughly one-third of the AT/RT cases at the time of diagnosis, and tumors can occur anywhere throughout the CNS.

[citation needed] By 1995, AT/RT had become regarded as a newly defined aggressive, biologically unique class of primarily brain and spinal tumors, usually affecting infants and young children.

[50] In January 2001, the U.S. National Cancer Institute and Office of Rare Diseases hosted a Workshop on Childhood Atypical Teratoid/Rhabdoid Tumors of the Central Nervous System.

This observation is not surprising because rhabdoid tumors at both locations possess similar histologic, clinical, and demographic features.

Atypical teratoid rhabdoid tumor is rare, and no therapy has been proven to deliver long-term survival, nor a set of protocols made standard.

In February 2012, she spent the day in person with Justin Bieber, her pretend husband, after a Facebook campaign to meet her idol.

Joel continued to survive for four more years after developing seven additional tumors and eventually dying from cancer on March 13, 2014.

Ryan Green wanted to provide the experiences of raising Joel in the form of a video game to help the player to understand the difficulties and realities with which they had to deal during this time.

[55][56] After Joel's death, the game was reworked to instead act as a tribute to Ryan and Amy's five short years with their third child.