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Central nervous system relapse of rhabdomyosarcoma.

ABSTRACT: 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. 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. 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‐two patients died of CNS disease and one of treatment complications, with metastatic disease at other sites. Median survival post-CNS relapse was 5 months (range, 0.1-49 months). 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.

SUBMITTER: De B

PROVIDER: S-EPMC7343338 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature

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Central nervous system relapse of rhabdomyosarcoma.

De Brian B Kinnaman Michael D MD Wexler Leonard H LH Kramer Kim K Wolden Suzanne L SL

Pediatric blood & cancer 20170711 1

<h4>Purpose</h4>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.<h4>Methods</h4>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 prima ...[more]

PMID: 28696016

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Project description:Objective To track the clinical outcomes in patients who initially presented with tumefactive demyelinating lesions (TDLs), we summarized the clinical characteristics of various etiologies, and identified possible relapse risk factors for TDLs. Methods Between 2001 and 2021, 116 patients initially presented with TDLs in our hospital were retrospectively evaluated. Patients were followed for relapse and clinical outcomes, and grouped according to various etiologies. Demographic information, clinical data, imaging data, and laboratory results of patients were obtained and analyzed. The risk factors of relapse were analyzed by the Log-Rank test and the Cox proportional hazard model in multivariate analysis. Result During a median follow-up period of 72 months, 33 patients were diagnosed with multiple sclerosis (MS), 6 patients with Balo, 6 patients with neuromyelitis optica spectrum disorders (NMOSD), 10 patients with myelin oligodendrocyte glycoprotein antibody-associated demyelination (MOGAD), 1 patient with acute disseminated encephalomyelitis (ADEM), and the remaining 60 patients still have no clear etiology. These individuals with an unknown etiology were categorized independently and placed to the other etiology group. In the other etiology group, 13 patients had recurrent demyelinating phases, while 47 patients did not suffer any more clinical events. Approximately 46.6% of TDLs had relapses which were associated with multiple functional system involvement, first-phase Expanded Disability Status Scale score, lesions morphology, number of lesions, and lesions location (P<0.05). And diffuse infiltrative lesions (P=0.003, HR=6.045, 95%CI:1.860-19.652), multiple lesions (P=0.001, HR=3.262, 95%CI:1.654-6.435) and infratentorial involvement (P=0.006, HR=2.289, 95%CI:1.064-3.853) may be independent risk factors for recurrence. Relapse free survival was assessed to be 36 months. Conclusions In clinical practice, around 46.6% of TDLs relapsed, with the MS group showing the highest recurrence rate, and lesions location, diffuse infiltrative lesions, and multiple lesions might be independent risk factors for relapse. Nevertheless, despite extensive diagnostic work and long-term follow-up, the etiology of TDLs in some patients was still unclear. And these patients tend to have monophase course and a low rate of relapse.

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Central nervous system relapse of rhabdomyosarcoma.

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Central nervous system relapse of rhabdomyosarcoma.

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