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Cranial nerve palsies in childhood parameningeal rhabdomyosarcoma
Author(s) -
Zorzi Alexandra P.,
Grant Ronald,
Gupta Abha A.,
Hodgson David C.,
Nathan Paul C.
Publication year - 2012
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24128
Subject(s) - medicine , rhabdomyosarcoma , sarcoma , pathology
Background Children with parameningeal rhabdomyosarcoma (PM RMS) and cranial nerve palsy (CNP) are at risk for permanent neurologic dysfunction. Clinicians often consider the use of emergent therapies such as expedited radiation and/or corticosteroids; however, there is a paucity of information describing the natural history of CNP in PM RMS. We sought to describe the clinical features of patients with PM RMS plus associated CNP and to evaluate the patient, disease, and treatment‐related factors that impacted neurologic recovery. Methods We conducted a retrospective review of PM RMS cases treated at the Hospital for Sick Children between 1985 and 2010. Results Thirty‐five children were treated for PM RMS, 19 (54%) of whom presented with CNP. Children with CNP were nine times more likely to have other high‐risk features (cranial base bony erosion and/or intracranial extension) at the time of presentation than children without CNP (OR 9.6, 95% CI 1.69, 54.79, P = 0.013). In addition to commencing chemotherapy, 13 patients (68%) received expedited RT and corticosteroids, four (21%) corticosteroids alone, and two (11%) received only standard chemotherapy and RT. At last follow up of the 11 survivors, neurologic recovery was complete in five (45%), partial in five (45%), and absent in one (9%). Conclusions In our cohort, recovery of PM RMS associated CNP was often incomplete despite multi‐modal therapy. A larger cohort of patients is required to determine the utility of emergent initiation of radiation or corticosteroids. This study will facilitate the counseling of future families on the long‐term neurologic recovery CNP in PM RMS. Pediatr Blood Cancer 2012; 59: 1211–1214. © 2012 Wiley Periodicals, Inc.