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Background and Aims: Nuclear protein of the testis (NUT) carcinoma (NC) is a rare and highly aggressive tumor defined by the presence of a somatic NUTM1 rearrangement, occurring mainly in adolescents and young adults. We analyzed the clinical and biological features of German pediatric patients (≤18 years) with NC., Methods: This study describes the characteristics and outcome of 11 children with NC registered in the German Registry for Rare Pediatric Tumors (STEP)., Results: Eleven patients with a median age of 13.2 years (range 6.6-17.8) were analyzed. Malignant misdiagnoses were made in three patients. Thoracic/mediastinal tumors were found to be the primary in six patients, head/neck in four cases; one patient had multifocal tumor with an unknown primary. All patients presented with regional lymph node involvement, eight patients (72.7%) with distant metastases. Seven patients underwent surgery, eight radiotherapy with curative intent; polychemotherapy was administered in all patients. Novel treatment strategies including immunotherapy, targeted therapies, and virotherapy were applied in three patients. Median event-free survival and overall survival were 1.5 and 6.5 months, respectively., Conclusions: Every undifferentiated or poorly differentiated carcinoma should undergo testing for the specific rearrangement of NUTM1, in order to initiate an intense therapeutic regimen as early as possible. As in adults, only few pediatric patients with NC achieve prolonged survival. Thus, novel therapeutic strategies should be included and tested in clinical trials., (© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Both hemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD) are diagnosed in patients with prolonged resistant fever by using a scoring system. Concurrent manifestation of both conditions has been reported previously. We describe an infant of 7 weeks whose condition fulfilled the criteria of HLH, but who, after clinical response to treatment, suddenly died from a myocardial infarction at 11 weeks. Post-mortem examination revealed a previously unknown coronary arteritis typical for KD. Since it is difficult to distinguish between KD and HLH, both diseases should be considered in young children with overlapping symptoms. Repeated echocardiograms may be helpful in these cases., ((c) 2009 Wiley-Liss, Inc.)

Background: Clinical studies analyzing CNS complications in pediatric oncology systematically are rare., Procedure: In a single center retrospective analysis, CNS complications in 950 subsequent pediatric patients treated between 1992 and 2004 by chemotherapy or hematopoietic stem cell transplantation (HSCT) were studied. Forty-six patients had pre-existing CNS diseases and were excluded. Out of the 904 remaining, 76 (8.4%) had 82 CNS complications., Results: The most common manifestations were seizures (in 50.6% of the CNS episodes), altered states of consciousness, and motor deficits (in 47.5% of the episodes each). CNS complications were caused by infections (26.8%), toxicity (25.6%), neoplasma (13.4%), vascular (10.9%), and metabolic disturbances (8.5%). In 14.6%, the mechanism remained unclear. Patients (23.7%) died from the CNS event. Neoplastic disorders had the worst (50%) while metabolic the best (0% mortality) prognosis. Imaging techniques were the most effective diagnostic measures, followed by laboratory analysis, clinical examination, and CSF analysis. A neuro-psychological (CBCL, CFT-1/-20-testing) examination could be done in 21 of 32 long-term survivors. Seven had a major, 3 minor neurological impairment, 11 were normal in all tests., Conclusions: These data show that there is not one typical CNS complication, but a wide variety. There is no close connection between either underlying disease or symptoms and cause of the complication. Prognosis is variable. About two thirds of the long-term survivors could lead a normal life., ((c) 2007 Wiley-Liss, Inc.)