Patterns of Failure in Parameningeal Alveolar Rhabdomyosarcoma.

Abstract

PURPOSE:

To determine patterns of failure, clinical outcomes, and prognostic factors among pediatric patients treated with radiation therapy for parameningeal alveolar rhabdomyosarcoma.

METHODS AND MATERIALS:

We evaluated clinical and treatment planning records of patients aged ≤21 years with parameningeal alveolar rhabdomyosarcoma treated with definitive or adjuvant radiation therapy at our institution. The Kaplan-Meier product limit method assessed disease control and survival; the log-rank test was used to evaluate prognostic impact.

RESULTS:

We identified 24 patients with a median age of 3.5 years (range, 1-20) treated between 2009 and 2016. The median follow-up was 2.4 years for all (range, 0.3-5.6) and 3.2 years for living patients (range, 0.7-5.6). Most patients had group III (96%), node-negative (67%), positive FOX fusion status (63%) disease, and intracranial extension (54%). The paranasal sinus was the most common subsite (29%). All patients were treated with concurrent chemotherapy and proton radiation therapy with a median dose of 50.4 Gy relative biological effectiveness (range, 41.4-59.4) at a median 13 weeks after induction chemotherapy (range, 3-25). The 3-year local control, regional control, disease-free survival, and overall survival rates were 66%, 94%, 40%, and 58%, respectively. Median time to any failure was 0.5 years (range, 0.2-2.1). N1 disease and intracranial extension (ICE) portended inferior overall survival (P = .002 and .02, respectively). Female sex portended better local control (P = .05). All 7 patients with distant metastases as the first site of recurrence had central nervous system metastases. Age <4 years, absence of ICE, N0 disease, and primary tumor <5 cm were associated with a statistically significant improvement in freedom from distant metastases.

CONCLUSIONS:

Although regional nodal failures were rare, in-field local recurrences and leptomeiningeal progression in those with ICE suggest the need for modification of local and central nervous system therapies.

• Authors: Julie A Bradley, Daniel J Indelicato, Haruka Uezono, Christopher G Morris, Eric Sandler, Hernando de Soto, Raymond B Mailhot Vega, Ronny Rotondo
• Journal: International journal of radiation oncology, biology, physics (Int J Radiat Oncol Biol Phys) Vol. 107 Issue 2 Pg. 325-333 (06 01 2020) ISSN: 1879-355X [Electronic] United States
• PMID: 32044412 (Publication Type: Clinical Trial, Journal Article)
• Copyright: Copyright © 2020 Elsevier Inc. All rights reserved.

Topics

• Adolescent
• Child
• Child, Preschool
• Female
• Humans
• Infant
• Kaplan-Meier Estimate
• Male
• Meningeal Neoplasms (radiotherapy)
• Radiotherapy, Adjuvant
• Rhabdomyosarcoma, Alveolar (radiotherapy)
• Treatment Failure