ABSTRACT
PURPOSE: To report a case of orbital rhabdomyosarcoma and highlight the treatment approach to the dilemma of a residual mass. OBSERVATIONS: An eleven-year-old boy was diagnosed with Stage 1, Group III embryonal rhabdomyosarcoma in the orbit. After completing a 24-week treatment regimen of chemotherapy and radiation, imaging showed a large persistent mass with erosion through the medial wall. It was uncertain whether the erosion was due to radiation osteonecrosis or to advancing tumor, creating a treatment dilemma for the providers. A repeat biopsy was planned. During the procedure, the mass was completely excised due to ease of removal, and the biopsy showed completely treated tumor. MRI surveillance at four years follow up showed that the patient remains tumor-free. CONCLUSIONS AND IMPORTANCE: Rhabdomyosarcoma was once a disease with a very poor outcome, but advances in imaging, chemotherapy, and radiation therapy have improved the prognosis of these patients. What was once a surgical disease treated with morbid resection is now predominantly a medical disease diagnosed with biopsy and treated with chemotherapy and radiation. However, such patients may have a residual mass after completing treatment. This situation presents a challenge, as it may not be clear whether the persistent mass is active tumor or treated tumor. This report describes the presentation and management of such a case in the orbit and demonstrates that a residual orbital mass may remain and represent completely treated tumor.
ABSTRACT
BACKGROUND: Determine the prognostic impact of magnetic resonance imaging (MRI)-defined diffuse axonal injury (DAI) after traumatic brain injury (TBI) on functional outcomes, quality of life, and 3-year mortality. METHODS: This retrospective single center cohort included adult trauma patients (age > 17 years) admitted from 2006 to 2012 with TBI. Inclusion criteria were positive head computed tomography with brain MRI within 2 weeks of admission. Exclusion criteria included penetrating TBI or prior neurologic condition. Separate ordinal logistic models assessed DAI's prognostic value for the following scores: (1) hospital-discharge Functional Independence Measure, (2) long-term Glasgow Outcome Scale-Extended, and (3) long-term Quality of Life after Brain Injury-Overall Scale. Cox proportional hazards modeling assessed DAI's prognostic value for 3-year survival. Covariates included age, sex, race, insurance status, Injury Severity Score, admission Glasgow Coma Scale Score, Marshall Head computed tomography Class, clinical DAI on MRI (Y/N), research-level anatomic DAI Grades I-III (I, cortical; II, corpus callosum; III, brainstem), ventilator days, time to follow commands, and time to long-term follow-up (for logistic models). RESULTS: Eligibility criteria was met by 311 patients, who had a median age of 40 years (interquartile range [IQR], 23-57 years), Injury Severity Score of 29 (IQR, 22-38), intensive care unit stay of 6 days (IQR, 2-11 days), and follow-up of 5 years (IQR, 3-6 years). Clinical DAI was present on 47% of MRIs. Among 300 readable MRIs, 56% of MRIs had anatomic DAI (25% Grade I, 18% Grade II, 13% Grade III). On regression, only clinical (not anatomic) DAI was predictive of a lower Functional Independence Measure score (odds ratio, 2.5; 95% confidence interval, 1.28-4.76], p = 0.007). Neither clinical nor anatomic DAI were related to survival, Glasgow Outcome Scale-Extended, or Quality of Life after Brain Injury-Overall Scale scores. CONCLUSION: In this longitudinal cohort, clinical evidence of DAI on MRI may only be useful for predicting short-term in-hospital functional outcome. Given no association of DAI and long-term TBI outcomes, providers should be cautious in attributing DAI to future neurologic function, quality of life, and/or survival. LEVEL OF EVIDENCE: Epidemiological, level III; Therapeutic, level IV.
