Intracranial germ cell tumors: a single-institution experience

Intracranial germ cell tumors [GCTs] are not a common disease. We reviewed the experience of a single institution to determine the variables that affect treatment outcome. A retrospective review of patients with the diagnosis of intracranial germ cell tumors treated in a single institution [KFSHRC] during the period from March 1985 to December 2007. Fifty-seven patients with the diagnosis of intracranial GCT were recorded in the KFSHRC Tumor Registry during the period from 1985 to 2007. Seven patients with a pineal region tumor treated as germinomas in the earlier years without a tissue diagnosis were excluded. This retrospective study was restricted to the remaining 50 patients with a tissue or marker diagnosis: 31 germinomas and 19 non-germinomatous germ cell tumors [NGGCTs]. The 10-year overall survival [OS], event-free survival [EFS] and relapse-free survival [RFS] were 87%, 88% and 96% for patients with germinoma, with a median follow-up of 4.5 [range 2-17] years, compared with 26%, 29% and 46% for patients with NGGCT with a median follow-up of 3 [range 1.5-13] years. For NGGCT, variables favorably influencing OS were younger age [< 16 y vs >/= 16 y, P=.01], higher radiation dose [>50 Gy vs 1990 vs <1990 P=.002]. Tissue diagnosis of GCTs is mandatory prior to treatment except for patients with elevated markers. In germinoma, localized radiotherapy [RT] for M0 patients may be adequate. Long-term follow-up is needed to define the benefit of adding chemotherapy. For NGGCT, the use of combined modality treatment and RT dose >50 Gy are important factors that influence the outcome. Second-look surgery and resection of residual/refractory tumors is always recommended

Use of tranexamic acid in pediatric Kaposiform hemangio-endothelioma complicated with Kasabach-merritt syndrome

Kasabach-Merritt syndrome is an infrequent combination of Kaposiform hemangio-endothelioma and severe thrombocytopenia, which may be life- threatening with an overall mortality rate of 20-30% and for which there are no definitive methods of treatment. A retrospective data collection of a single institute [KFSHRC]* reporting three cases of Kasabach-Merritt syndrome, showing a very good response to tranexamic acid with the reversal of the hematological disorder, decrease in steroid requirement and diminution of the size of the lesion in few months duration. The early use of tranexamic acid in the treatment of Kasabach-Merritt syndrome, especially the non-resectable vascular tumors, was recommended to be combined with interferon-alpha or steroid therapy for children