Atypical teratoid rhabdoid tumor natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Natural History, Complications and Prognosis
Complications
Metastatic spread is noted in approximately one-third of the AT/RT cases at the time of diagnosis and tumors can occur anywhere throughout the CNS. The ASCO study of the 188 documented AT/RT cases prior to 2004 found 30% of the cases had metastasis at diagnosis.[1] Metastatic spread to the meninges (leptomenigeal spread sometimes referred to as sugar coating) is common both initially and with relapse. Average survival times decline with the presence of metastasis. Primary CNS tumors metastasize only within the CNS.
One case of metastatic disease to the abdomen via ventriculoperitoneal shunt has been reported with AT/RT . Metastatic dissemination via this mechanism has been reported with other brain tumors including germinomas, medulloblastomas, astrocytomas, glioblastomas, ependymomas and endodermal sinus tumors. Guler and Sugita separately reported cases of lung metastasis without a shunt.[2][3]
Prognosis
Most patients with AT/RT are less than three years old, the overall prognosis for AT/RT is very poor. Compared to medulloblastoma, AT/RT has a significantly worse prognosis. AT/RT occurs in young children (often younger than three years) who are difficult to evaluate, it is resistant to many current therapies, and its recurrence is fast.
The prognosis for AT/RT is very poor, although there are some indications that an IRSIII-based therapy can produce long-term survival (60 to 72 months). Two-year survival is less than 20%, average survival postoperatively is 11 months, and doctors recommend palliative care, especially with younger children because of the poor outcomes.
Patients with metastasis (disseminated tumor), larger tumors, tumors that could not be fully removed, tumor reoccurrence, and were younger than 36 months had the worse outcomes (i.e., shorter survival times).
A retrospective survey from 36 AT/RT St. Jude Children's Hospital patients from 1984 to 2004 found a less than 10% survival rate in children under three, but a 70% survival rate in older children.[4] A retrospective register at the Cleveland Children's hospital on 42 AT/RT patients found median survival time is 16.25 months and a survival rate around 33%. One-quarter of these cases did not show the mutation in the INI1/hSNF5 gene.
The longest term survivals reported in the literature are:
- (a) Hilden and associates reported a child who was still free from disease at 46 months from diagnosis.[5]
- (b) Olson and associates reported a child who was disease free at five years from diagnosis based on the IRS III protocol. [6]
- (c) In 2003 Hirth reported a case who had been disease free for over six years.[7]
- (d) Zimmerman in 2005 reported 50-to-72 month survival rates on four patients using an IRS III-based protocol. Two of these LT survivors had been treated after an AT/RT reoccurrence.[8]
- (e) A NYU study (Gardner 2004) has 4 of 12 longer term AT/RT survivors; the oldest was alive at 46 months after diagnosis.[9]
- (f) Aurélie Fabre, 2004, reported a 16-year survivor of a soft-tissue rhabdoid tumor.[10]
Cancer treatments in long-term survivors who are children usually cause a series of negative effects on physical well being, fertility, cognition, and learning.[11][12][13][14]
References
- ↑
- ↑ Güler E, Varan A, Söylemezoglu F; et al. (2001). "Extraneural metastasis in a child with atypical teratoid rhabdoid tumor of the central nervous system" (PDF). J. Neurooncol. 54 (1): 53–6. PMID 11763423.
- ↑ Sugita Y, Takahashi Y, Hayashi I, Morimatsu M, Okamoto K, Shigemori M (1999). "Pineal malignant rhabdoid tumor with chondroid formation in an adult". Pathol. Int. 49 (12): 1114–8. PMID 10632935.
- ↑ "Atypical Teratoid / Rhabdoid Tumor (ATRT)". St Jude's Hospital. Retrieved 2007-07-10.
- ↑ Hilden JM, Meerbaum S, Burger P; et al. (2004). "Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry". J. Clin. Oncol. 22 (14): 2877–84. doi:10.1200/JCO.2004.07.073. PMID 15254056. Retrieved 2007-05-23.
- ↑ Olson TA, Bayar E, Kosnic E (1995). "Successful treatment of disseminated central nervous system malignant rhabdoid tumors". J Pediatr Hematol Oncol. 17: 71–75. PMID 7743242.
- ↑ Hirth A, Pedersen P-H, Wester K; et al. (2003). "Cerebral Atypical Teratoid/Rhabdoid Tumor of Infancy: Long-Term Survival after Multimodal Treatment, also Including Triple Intrathecal Chemotherapy and Gamma Knife Radiosurgery--Case Report (Abstract)". Pediatric Hematology and Oncology 2003. 20 (4): 327–332. doi:10.1080/713842315.
- ↑ Zimmerman MA, Goumnerova LC, Proctor M; et al. (2005). "Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor". J. Neurooncol. 72 (1): 77–84. doi:10.1007/s11060-004-3115-y. PMID 15803379. Retrieved 2007-05-20.
- ↑ Gardner S, Diez B, Green A; et al. (June 13–16). "THER 27. INTENSIVE INDUCTION CHEMOTHERAPY FOLLOWED BY HIGH-DOSE CHEMOTHERAPY WITH AUTOLOGOUS STEM CELL RESCUE (ASCR) IN YOUNG CHILDREN NEWLY DIAGNOSED WITH CENTRAL NERVOUS SYSTEM (CNS) ATYPICAL TERATOID RHABDOID TUMORS (ATT/RT)—THE "HEAD START" REGIMENS" (PDF). Abstracts from the Eleventh International Symposium on Pediatric Neuro-Oncology. Retrieved 2007-06-03. Check date values in:
|date=, |year= / |date= mismatch(help) - ↑ Fabre A, Eyden B, Ali HH (January 2004). "Soft-Tissue Extrarenal Rhabdoid Tumor with a Unique Long-Term Survival". Ultrastructural Pathology. 28 (1): 49–52. doi:10.1080/01913120490275259. Retrieved 2007-05-28.
- ↑
Fouladi M, Gilger E, Kocak M; et al. (October 1, 2005). "Intellectual and Functional Outcome of Children 3 Years Old or Younger Who Have CNS Malignancies". Journal of Clinical Oncology. 23 (28): 7152–60. doi:10.1200/JCO.2005.01.214. Check date values in:
|date=(help) - ↑
Monteleone P, Meadows AT (June 6, 2006). "Late Effects of Childhood Cancer and Treatment". eMedicine from WebMD. Check date values in:
|date=(help) - ↑ Foreman NK, Faestel PM, Pearson J; et al. (January 1999). "Health Status in 52 Long-term Survivors of Pediatric Brain Tumors". Journal of Neuro-Oncology. 41 (1): 47–52. doi:10.1023/A:1006145724500.
- ↑ Meyers EA, Kieran MW (2002). "Brief Report Psychological adjustment of surgery-only pediatric neuro-oncology patients: a retrospective analysis". Psycho-Oncology. John Wiley & Sons, Ltd. 11 (1): 74–79. doi:10.1002/pon.553.