Treatment of two cases with refractory, metastatic intermediate-risk neuroblastoma with isotretenoin alone or observation.

Patients <12 months with favorable biology, metastatic neuroblastoma have >90% overall survival following treatment with chemotherapy and surgery. We report two infants with favorable biology, stage 4 neuroblastoma with refractory disease after standard intermediate-risk chemotherapy and additional retrieval chemotherapy. One patient was treated with six additional cycles of isotretinoin and the other observed. Both remain clinically well with persistent disease but no evidence of tumor progression for 28 and 13 months following completion of cytotoxic treatment. Similar to residual tumor in primary sites, refractory metastatic disease may not portend a poor outcome in patients with favorable biology, intermediate-risk neuroblastoma.

Juvenile dermatomyositis: correlation of MRI at presentation with clinical outcome.

OBJECTIVE: The clinical course of juvenile dermatomyositis (JDMS) is unpredictable. MRI is used to determine muscle biopsy site and to monitor disease activity. It is unknown whether soft-tissue features on MRI obtained at diagnosis correlate with clinical outcome. The purpose of our study is to determine whether initial MRI findings in the pelvis and thighs in children with JDMS can predict clinical disease course. MATERIALS AND METHODS: Forty-five children (31 girls and 14 boys; median age, 6 years; range, 1-18 years) with clinically diagnosed biopsy-proven JDMS and at least 24 months of clinical follow-up were included. Clinical outcome was categorized as limited or chronic disease, according to the established Crowe clinical classification scheme. Pretreatment MRI examinations of the pelvis and thighs were evaluated for signal abnormalities of muscle and fascia and reticulated signal changes in subcutaneous fat; associations with clinical outcome were examined. RESULTS: Twenty-two patients had limited disease and 23 had chronic disease. Signal intensity ranged from normal (n = 3) to floridly increased in all muscle compartments (n = 17). Muscle and fascial involvement were not associated with clinical outcome. Controlling for duration of symptoms, the adjusted odds of progressing to chronic disease were higher for patients with abnormal subcutaneous fat signal than for those with normal fat signal (odds ratio, 9.0; 95% CI, 1.5-53.5; p < 0.02). CONCLUSION: MRI findings of muscle or fascia involvement do not predict clinical outcome in children with newly diagnosed JDMS. Abnormal subcutaneous fat signal appears to have a significant association with a more aggressive chronic disease course.

Medial collateral ligament of the knee on magnetic resonance imaging: does the site of the femoral origin change at different patient ages in children and young adults?

BACKGROUND: The medial patellofemoral ligament (MPFL), a chief medial restraint preventing lateral patellar dislocation, often is reconstructed in children with recurrent dislocation. The femoral MPFL attachment can be difficult to delineate at surgery. Therefore, the origin of the medial collateral ligament (MCL) frequently is used to approximate the reattachment site. The purpose of our study was to compile normative data from MR imaging examinations over different patient ages, to determine the effect of growth on the relationship of the MCL origin site to the distal femoral physis and medial femoral condyle (MFC). SUBJECTS AND METHODS: This HIPAA-compliant study was IRB approved. Informed consent requirement was waived. Three hundred knee MR imaging examinations (143 boys, 157 girls, 0-20 y) were evaluated. MCL origin to femoral physis distance, MFC height, and MCL origin-physis distance: MFC height ratio (MCL:MFC ratio) were calculated. Relationships between these values and age, gender, and physeal patency were assessed using linear regression models. RESULTS: With physeal patency, MCL origin-physis distance was significantly associated with increasing age in boys (P=0.0394), and trended toward significance in girls (P=0.0557). Distance increased 0.01 cm/y in both genders. MFC height increased 0.15 cm/y in boys and 0.13 cm/y in girls (P<0.0001). MCL:MFC ratio decreased 0.01/y (P<0.0001). With physeal closure, no significant change was measured for any variable. CONCLUSIONS: During growth, there are statistically significant, albeit minimal, changes of the MCL origin-physis distance and MFC height. As these changes are essentially negligible, no adjustment for age is needed during restorative MPFL surgery in growing children. CLINICAL RELEVANCE: As there is neglible change in location of the origin of the MCL relative to the distal femoral physis during skeletal growth in both boys and girls, no adjustment for patient age is necessary when using the origin of the MCL as a landmark to locate the site of femoral reattachment of a disrupted MPFL.