Central nervous system relapse of rhabdomyosarcoma Journal Article


Authors: De, B.; Kinnaman, M. D.; Wexler, L. H.; Kramer, K.; Wolden, S. L.
Article Title: Central nervous system relapse of rhabdomyosarcoma
Abstract: Purpose: The optimal management of central nervous system (CNS) relapse of rhabdomyosarcoma (RMS) is unclear. We examined diagnosis, management, and outcomes of patients with RMS developing CNS relapse. Methods: Records of 23 patients diagnosed with CNS relapse between 1999 and 2016 were reviewed. Median age at presentation of CNS relapse was 15 years (range, 1–34 years). High-risk features at initial presentation were as follows: 16 alveolar patients, 13 Stage IV, and 13 with primary tumor in parameningeal locations. Results: CNS relapse occurred at a median 12 months (range, 1–23 months) from diagnosis and most common presenting symptoms were headache (n = 9), nausea/vomiting (n = 8), visual difficulty (n = 5), and none (n = 5). Leptomeningeal metastases were detected in 21 patients while only 2 developed parenchymal metastases without leptomeningeal involvement. Fifteen patients received CNS-directed radiation therapy (RT), including craniospinal irradiation to a median 36 Gy (range, 18–36 Gy) and/or whole brain radiotherapy to a median 30 Gy (range, 6–41.4 Gy). Three patients received concurrent chemotherapy. Follow-up magnetic resonance imaging was conducted in 13 patients after RT initiation with 8 demonstrating improvement, 2 with stable disease, and 3 with progression. Twelve patients were tested for reactivity to I-131-labeled monoclonal antibody 8H9, and three tested positive and received at least one intra-Ommaya dose; all three lived >12 months post-CNS relapse. Twenty-one patients died of CNS disease and two of metastatic disease at other sites. Median survival post-CNS relapse was 5 months (range, 0.1–49 months). Conclusions: The prognosis for patients with RMS developing CNS relapse remains poor. Treatment including CNS-directed RT should be considered and investigation into preventative therapies is warranted. © 2017 Wiley Periodicals, Inc.
Keywords: adolescent; adult; cancer chemotherapy; cancer survival; child; clinical article; preschool child; school child; child, preschool; disease-free survival; primary tumor; survival rate; retrospective studies; young adult; unclassified drug; mortality; cancer recurrence; doxorubicin; cancer growth; pathophysiology; disease free survival; temozolomide; cancer staging; nuclear magnetic resonance imaging; follow up; multiple cycle treatment; neoplasm recurrence, local; etoposide; nausea; vomiting; cohort analysis; cyclophosphamide; vincristine; pathology; retrospective study; ifosfamide; irinotecan; monoclonal antibody; temsirolimus; immunotherapy; infant; meningeal neoplasms; tumor recurrence; dactinomycin; radiation therapy; headache; symptomatology; meningioma; tracer; rhabdomyosarcoma; visual disorder; meningeal metastasis; central nervous system metastasis; whole brain radiotherapy; craniospinal irradiation; clinical outcome; monoclonal antibody 8h9 i 131; cancer prognosis; humans; human; male; female; priority journal; article; cns relapse; median survival time; central nervous system cancer; vinorelbine tartrate
Journal Title: Pediatric Blood and Cancer
Volume: 65
Issue: 1
ISSN: 1545-5009
Publisher: Wiley Periodicals, Inc  
Date Published: 2018-01-01
Start Page: e26710
Language: English
DOI: 10.1002/pbc.26710
PUBMED: 28696016
PROVIDER: scopus
PMCID: PMC7343338
DOI/URL:
Notes: Article -- Export Date: 2 January 2018 -- Source: Scopus
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MSK Authors
  1. Kim Kramer
    236 Kramer
  2. Suzanne L Wolden
    560 Wolden
  3. Leonard H Wexler
    191 Wexler
  4. Brian Sandeep De
    10 De