Τετάρτη 12 Φεβρουαρίου 2020

Parameningeal Alveolar Rhabdomyosarcoma.Rhabdomyosarcoma, albeit rare in adults, is the most common soft-tissue sarcoma in children. Rhabdomyosarcoma of the head and neck can be categorized as parameningeal (originating in close anatomic relationship to the meninges), nonparameningeal, or orbital.

Patterns of Failure in Parameningeal Alveolar Rhabdomyosarcoma.:

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Patterns of Failure in Parameningeal Alveolar Rhabdomyosarcoma.

Int J Radiat Oncol Biol Phys. 2020 Feb 07;:

Authors: Bradley JA, Indelicato DJ, Uezono H, Morris CG, Sandler E, de Soto H, Mailhot Vega RB, Rotondo R

Abstract

PURPOSE: To determine patterns of failure, clinical outcomes, and prognostic factors among pediatric patients treated with radiotherapy for parameningeal alveolar rhabdomyosarcoma (ARMS).

METHODS: We evaluated clinical and treatment planning records of children aged ≤21 years with parameningeal ARMS treated with definitive or adjuvant radiotherapy 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 radiotherapy with a median dose of 50.4GyRBE (range, 41.4-59.4) at a median 13 weeks, following 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=0.002 and 0.02, respectively). Female sex portended better local control (p=0.05). 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 <5cm were associated with a statistically significant improvement in freedom from distant metastases.

CONCLUSION: While 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.

PMID: 32044412 [PubMed - as supplied by publisher]



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