Atypical Teratoid/Rhabdoid Tumors in Adults: A Case Report and Treatment-Focused Review

Division of Oncology and Hematology, University of Nebraska Medical Center, 987680 Nebraska Medical Center, Omaha NE 68198-7680, USA.
Journal of Clinical Medicine Research 04/2011; 3(2):85-92. DOI: 10.4021/jocmr535w
Source: PubMed


Atypical teratoid/rhabdoid tumor is predominantly a childhood tumor and has only been rarely reported in adults; therefore, treatment regimens are often extrapolated from the pediatric experience. Typically, children are treated with craniospinal radiation therapy which is often followed by systemic chemotherapy. Employing pediatric regimens to treat this tumor in adult patients poses a particular risk for myelosuppression, as the prescribed doses in pediatric protocols exceed those tolerated by adults, and conventional craniospinal radiation can be associated with prolonged myelotoxicity and a depletion of the bone marrow reserve in vertebrae of adults. Here we present a case of a woman with a pineal region atypical teratoid/rhabdoid tumor, an unusual adult cancer presenting in an atypical location. This is followed by a review of the disease in adult patients with an emphasis on treatment and suggestions to minimize myelotoxicity.

Atypical rhabdoid tumor; AT/RT; Pineal tumor; Adult

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Available from: Shauna Choi, Nov 17, 2014
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    • "AT/RT is characterized by an aggressive clinical behavior in most pediatric patients, who usually die within approximately one year after the diagnosis despite aggressive therapy, while adult patients are reportedly better with some longer-term survivors.[36] A recent report suggest that cerebral location of an AT/RT renders the tumor amenable to gross total resection as well as aggressive adjuvant chemoradiation therapy available in adults, which would not be tolerated by small children.[35] Therefore, immunohistochemical verification of this entity is very important for a proper treatment and better prognosis and AT/RT must be considered in the diagnosis of a high-grade tumor regardless of age. "
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    ABSTRACT: Background: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system tumor composed of primitive rhabdoid cells that may differentiate along neuroectodermal, mesenchymal and epithelial lineages. AT/RT in adults is rare but not completely exceptional. It generally arises from the posterior fossa of infants, but the broad majority of the reported AT/RT in adults manifested supratentorially with the exception of four cases that arose in the cerebellum and two that arose in the spinal cord. Case Description: A 44-year-old female complained of visual disturbance. We performed craniotomies twice and removed partially for each time, but any malignant cells were not found in the specimens. Finally, we determined histological diagnosis from the extended lesion. She died of respiratory failure 17 months after the initial treatment. Conclusion: AT/RT should be considered in the differential diagnosis of a sellar lesion in adult patients. However AT/RT is rare in adults, the appropriate immunohistochemical evaluation should be performed to diagnose this rare entity.
    Full-text · Article · May 2014 · Surgical Neurology International
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    • "In adults, however, the behavior of the tumor seems to differ somewhat from pediatric cases in that survival time, based on the literature review, appears to be longer. Of the 37 adult patients with pure AT/RT in which survival data could be retrieved, the average overall survival was 25.5 months, although it ranged widely from 4 weeks to over 17 years [1]. The longest survivals were 17, 9, 6, and 5 years, in 2 males and 2 females with a relatively younger age than the average age of the group (27 years), all occurring in the cerebral hemispheres. "

    Full-text · Article · Feb 2014 · Clinical neuropathology
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    • "A tumor composed of rhabdoid cells in the central nervous system (CNS), was first reported in 1985. The name atypical teratoid/rhabdoid tumor (AT/RT) exemplifies the tumors disparate mixtures of rhabdoid, primitive neuroepithelial, mesenchymal, and epithelial components, although the tumors may be composed entirely or partly of rhabdoid cells.1–4 The histologic and immunohistochemical features of this neoplasm have been extensively described.5–10 "
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    ABSTRACT: Here we present the case of a 60-year-old woman with a rare sellar region atypical teratoid/rhabdoid tumor (AT/RT), complicated by lung metastasis and treated with neurosurgery, radiotherapy, and chemotherapy. The patient had recurrent headache associated with left cavernous sinus syndrome after a previous endonasal transsphenoidal resection for a presumptive pituitary macroadenoma. Pituitary magnetic resonance imaging showed a tumor regrowth in the original location with a haemorrhagic component involving the left cavernous sinus. A near complete transsphenoidal resection of the sellar mass was performed followed by 3 months of stereotactic radiotherapy. Because of a worsening of the general clinical conditions, respiratory failure, and asthenia, the patient underwent a contrast enhanced computer tomography of the whole body which showed the presence of lung metastasis. The histopathological diagnosis on samples from pituitary and lung tissues was AT/RT. The patient survived 30 months after diagnosis regardless chemotherapy. In the adult, the AT/RT should be considered as a possible rare, aggressive, and malignant neoplasm localized in the sella turcica.
    Full-text · Article · Nov 2013 · Clinical Medicine Insights: Case Reports
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