Faculty Bibliography

Objectives: To evaluate patterns of relapse and outcome in patients newly-diagnosed with CNS 'secreting' (or Mixed Malignant) GCT treated initially with chemotherapy without irradiation on the International CNS GCT Study III. Methods: A retrospective chart review was conducted using all 25 patients enrolled on the International CNS GCT Study III, with at least 7 years follow-up for all patients. Details of the chemotherapy regimen have been published previously (DaSilva et al: Pediatric Blood & Cancer, 54:337-383, 2010). Results: Thirteen patients at diagnosis had 'secreting' CNS GCT by pathology and tumor markers (n=11) or tumor markers alone (n=2). Twelve were treated with chemotherapy alone, one receiving focal irradiation following chemotherapy prior to relapse. Six patients (46%) relapsed (mean of 30.5 months; range 6 to 59 months), two beyond and 4 within the primary site alone. Three patients relapsed 'early' (between 6 and 23 months from diagnosis), 2 with alpha-fetoprotein (AFP) elevations and one without tumor markers assessed; all 3 expired of progressive disease at 2-10 months following initial relapse. Three patients relapsed 'late' (between 37 and 59 months), all without AFP elevations, one with pathologically-pure germinoma, two with mild beta-human chorionic gonadotropin elevations (<20mIU/mL in serum/cerebro-spinal fluid); these patients survive disease-free at 86+, 94+ and 126+ months following additional chemotherapy and irradiation. Conclusions: Patients with CNS 'secreting' tumors who relapse following chemotherapyonly regimens display two distinct patterns of recurrence and outcome; patients relapsing 'early' appear to possess 'secreting' elements and have a dismal prognosis, while patients relapsing 'late' appear to do so with pure germinomatous elements and have an excellent outcome. Current international cooperative group studies utilizing more localized fields of irradiation should evaluate closely the patterns of relapse and outcome; late recurrences with germinomatou!

Thrombotic thrombocytopenic purpura (TTP) is a type of microangiopathic hemolytic anemia that is uncommon in childhood. Adults with TTP have a high mortality rate unless they are treated with plasma exchange. There are few reports of children with acquired idiopathic TTP, and most of those children received some form of treatment. We describe a child with acquired idiopathic TTP who had severe thrombocytopenia and anemia that resolved over several months without the use of any medications. This case suggests that some children with acquired idiopathic TTP might be safely observed without ill effects.

OBJECTIVE: It is not well appreciated that the clinical presentation of amyloid myopathy can mimic that of polymyositis. By retrospective clinicopathologic analysis we determined distinctive features of amyloid myopathy that differentiate the 2 diseases. METHODS: Two patients with clinical and histologic evidence of an inflammatory myopathy had fatal outcomes despite appropriate treatment for polymyositis. Their clinical course and original pathologic specimens were reviewed. In addition, original tissue samples were obtained and analyzed using Congo red staining and immunoperoxidase. RESULTS: The initial diagnosis of polymyositis was supported in both cases by muscle biopsies showing inflammatory infiltrates and elevations of creatine phosphokinase and by classic electromyography. Retrospective evaluation of the initial muscle biopsies disclosed subtle but incontrovertible evidence of vascular amyloid. Further analysis of the original specimens confirmed the presence of immunoglobin light chain (AL) amyloid. CONCLUSION: Amyloid myopathy can mimic polymyositis. Both can have similar clinical symptoms, as well as inflammatory infiltrates on muscle biopsy. Failure to recognize amyloid myopathy deprives patients of potentially life prolonging treatment. Congo red staining and immunohistochemical analysis of tissue could prevent misdiagnosis.

Twenty three adolescents with a history of cancer, 27 physically abused adolescents, and 23 healthy, nonabused adolescents were administered structured posttraumatic stress disorder (PTSD) interviews and self-report questionnaires regarding family functioning. Thirty five percent of adolescent cancer subjects met criteria for lifetime PTSD as compared to only 7% of the abused adolescents: 17% of the cancer subjects and 11% of the abuse subjects met criteria for current PTSD. Adolescents with cancer viewed their mothers and fathers as significantly more caring and more protective than the comparison and abused adolescents. Cancer subjects who met criteria for lifetime PTSD saw their families as significantly more chaotic than those who did not have PTSD. Eighty three percent of cancer subjects who had lifetime PTSD also had mothers who had PTSD

During the evaluation of a child who had completed treatment for Hodgkin's disease, a PET study strongly suggested recurrent disease in the mediastinum. Biopsies were obtained and revealed normal thymic tissue only, with no evidence of recurrent disease. The ongoing difficulty in establishing accurate disease status in patients treated for Hodgkin's disease is discussed, along with recommendations for treating pediatric patient populations.

Prevalence of posttraumatic stress disorder (PTSD) in 24 mothers of pediatric cancer survivors was compared with its prevalence among 23 mothers of healthy children. Significantly more mothers of pediatric cancer survivors were diagnosed with lifetime PTSD. Significant differences were also found in lifetime arousal, as well as current and lifetime reexperience and avoidance symptom clusters. Significant difference existed in the distribution of the number of prediagnosis high-magnitude events experienced by the mothers diagnosed with current PTSD as compared with the prediagnosis experience of the mothers who were not diagnosed with current PTSD. Illness severity, level of perceived family and extrafamilial social support, and Symptom Checklist-90-Revised global severity index scores did not significantly differ in the PTSD-positive and PTSD-negative groups