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BACKGROUND: IGTS is a rare phenomenon of paradoxical germ cell tumor (GCT) growth during or following treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of IGTS in patients in 21 North-American and Australian institutions. METHODS: Patients with IGTS diagnosed from 2000-2017 were retrospectively evaluated. RESULTS: Out of 739 GCT diagnoses, IGTS was identified in 33 patients (4.5%). IGTS occurred in 9/191 (4.7%) mixed-malignant GCTs, 4/22 (18.2%) immature teratomas (ITs), 3/472 (0.6%) germinomas/germinomas with mature teratoma, and in 17 secreting non-biopsied tumours. Median age at GCT diagnosis was 10.9 years (range 1.8-19.4). Male gender (84%) and pineal location (88%) predominated. Of 27 patients with elevated markers, median serum AFP and Beta-HCG were 70 ng/mL (range 9.2-932) and 44 IU/L (range 4.2-493), respectively. IGTS occurred at a median time of 2 months (range 0.5-32) from diagnosis, during chemotherapy in 85%, radiation in 3%, and after treatment completion in 12%. Surgical resection was attempted in all, leading to gross total resection in 76%. Most patients (79%) resumed GCT chemotherapy/radiation after surgery. At a median follow-up of 5.3 years (range 0.3-12), all but 2 patients are alive (1 succumbed to progressive disease, 1 to malignant transformation of GCT). CONCLUSION: IGTS occurred in less than 5% of patients with GCT and most commonly after initiation of chemotherapy. IGTS was more common in patients with IT-only on biopsy than with mixed-malignant GCT. Surgical resection is a principal treatment modality. Survival outcomes for patients who developed IGTS are favourable.
BACKGROUND: The use of high-dose chemotherapy (HDC) and autologous hematopoietic stem cell transplant (HSCT) has been used in certain pediatric patients with brain tumours to delay/spare radiotherapy. We aimed to study factors predicting a successful stem cell collection (SCC) and correlate stem cell dose infused with HSCT outcomes. METHODS: A retrospective chart review was undertaken for pediatric patients with brain tumours treated at our centre with HDC/HSCT between 2004-2016. RESULTS: Fifty-five patients were identified (32 male) with median age of 6.3 years at time of SCC (range 0.4-18.7). Patients' diagnoses were medulloblastoma (62%), ATRT (20%), and PNET (18%). Most patients (82%) underwent a single/1-day SCC, while the remaining required 2 SCC procedures. Peripheral blood stem cells were the source in most collections (95%). Successful SCC (CD34 collected greater-than-or-equal-to 2 x10^6/kg/transplant) and ideal SCC (greater-than-or-equal-to 5 x10^6/kg/transplant) was achieved in 85% and 45% of patients, respectively. Use of mobilizing chemotherapy with G-CSF was the only factor associated with achieving an ideal collection, while gender, age, stem cell source, and pre-apheresis peripheral blood CD34 count were not significant. Higher CD34/kg infused was associated with faster neutrophil engraftment in the first 3 courses of HDC/HSCT and platelet engraftment in the first course. CONCLUSIONS: The majority of SCC for autologous HSCT can be successfully completed with a single apheresis session. Mobilization with both chemotherapy and G-CSF yields higher CD34 compared to G-CSF alone. Higher dose of CD34/kg infused was associated with faster neutrophil and to a more limited scale platelet recovery post-HSCT.
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