Synchronous Central Nervous System Atypical Teratoid/ Rhabdoid Tumor and Malignant Rhabdoid Tumor of the Kidney: Case report of a Long-Term Survivor and Review of the Literature.

Synchronous Central Nervous System Atypical Teratoid/ Rhabdoid Tumor and Malignant Rhabdoid Tumor of the Kidney: Case report of a Long-Term Survivor and Review of the Literature.

World Neurosurg. 2017 Dec 06;:

Authors: Abu Arja MH, Patel P, Shah SH, Auletta JJ, Meyer EK, Conley SE, Aldrink JH, Pindrik JA, AbdelBaki MS

Abstract
BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) with synchronous or metachronous extra-CNS disease is a rare childhood malignancy with a dismal prognosis.
CASE DESCRIPTION: We report a seven-week-old female with metastatic AT/RT and synchronous malignant rhabdoid tumor of the kidney who received an intensive multimodal approach combining surgical resection, intrathecal chemotherapy, and high-dose chemotherapy with autologous peripheral blood stem cell transplant (PBSCT). She is currently 24 months of age without any evidence of disease. In addition, we completed an extensive literature review of cases with CNS AT/RT and synchronous or metachronous extra-CNS primary tumors. To date, a total of 31 pediatric cases have been reported, and the median overall-survival was six months post-diagnosis. The only three survivors received autologous PBSCT, and two of these patients had complete resection of their CNS tumor.
CONCLUSIONS: The rarity of CNS AT/RT with extra-CNS primary disease and the lack of standard treatment contribute to its reported dismal prognosis. We report a case of a long-term survivor with metastatic AT/RT and synchronous extra-CNS primary tumor. Maximal surgical resection, intrathecal chemotherapy, and consolidative autologous PBSCT may improve prognosis and avoid radiation.

PMID: 29223518 [PubMed - as supplied by publisher]

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