Difficulties in the management of an Askin tumor in a pediatric patient with cystic fibrosis: case report and literature review.

Treating Ewing's Sarcoma of the thorax (Askin's tumor) with antineoplastic therapy in a malnourished cystic fibrosis patient colonized with Pseudomonas aeruginosa and Staphylococcus aureus may carry a significant potential for complications. We present the case of a known cystic fibrosis patient, diagnosed with Askin's tumor 5 years ago. Despite facing severe neutropenia, exacerbations of cystic fibrosis with Pseudomonas aeruginosa infections, and challenges in maintaining adequate caloric intake during the oncological treatment, the patient's outcome has been favorable. Chemotherapy doses had to be adjusted, and continuous antibiotic treatment was introduced throughout the course of therapy to reduce the frequency and intensity of exacerbations. Approximately 5 years after the cancer diagnosis, with no signs of relapse, the patient was started on CFTR (Cystic fibrosis transmembrane conductance regulator) modulator treatment. This intervention has successfully corrected the weight deficit. The coincidence of Ewing's sarcoma of the chest wall and cystic fibrosis in a single patient is 2.857 × 10-5% and to the best of our knowledge, this scenario has not been documented before.

Unusual extramedullary relapses in a case of common B-cell acute lymphoblastic leukemia. Case report and review of literature.

BACKGROUND: Acute lymphoblastic leukemia (ALL) represents the most common malignancy in children with an overall cure rate of 85%. Relapses occur in 20% of the cases. Commonly, extramedullary relapses (EMRs) involve central nervous system (CNS) or testes. Unusual EMRs in ALL are relatively rare reported. CASE PRESENTATION: The authors present a 24-year-old woman with ALL, who experienced three unusual EMRs. In 2007, she was diagnosed with B-cell precursor (BCP)-ALL - high-risk (HR) group, and she was treated according to ALL Intercontinental Berlin-Frankfurt-Münster (IC-BFM) 2002∕HR Protocol. She entered complete remission (CR). In 2012, a vaginal wall solid mass infiltrate occurs. Biopsy concluded for EMR of ALL. Chemotherapy was restarted; the patient responded again with CR. Magnetic resonance imaging (MRI) and positron emission tomography∕computed tomography (PET∕CT) examinations during follow-up revealed supraclavicular, mesenteric, and retroperitoneal lymphadenopathies (2014). Pathological examination of the supraclavicular lymph node showed a benign pattern: schwannoma. The patient's evolution worsened, imposing a biopsy from the retroperitoneal tumor which revealed a second EMR of ALL. Again, ALL-REZ BFM 2002 Protocol was started, followed by haploidentical mother-to-child peripheral blood hematopoietic stem cell transplantation (HSCT). After suffering a few managed complications related to the transplant, our patient achieved CR again. In 2017, 10 years after the initial diagnosis, the patient presented for the third time an EMR (gastric wall) and eventually died due to progression of the disease. CONCLUSIONS: The patient presented an extremely aggressive type of ALL with three unusual EMRs: vaginal, retroperitoneal and gastric.

Current Trend of Invasive Orthopaedic Interventions for People with Haemophilia in Romania: Single Centre Experience.

OBJECTIVE: In countries with low factor concentrate consumption, disabling joint disease remains the major morbidity in patients with haemophilia. The objective of the present analysis is to express the trend and profile of invasive orthopaedic interventions in our country with low usage of factor replacement, lacking the prophylaxis program until recent years. PATIENTS AND METHODS: This retrospective descriptive study was conducted in our university centre in Timisoara with long-lasting experience in haemophilia care, which succeeded in developing an exceptionally valuable genuine comprehensive inter-institutional cooperation. This study refers to 115 invasive interventions performed on 97 patients: 83 with haemophilia A, 10 with haemophilia B and 4 with von Willebrand disease in the period of 2001 to 2017; 17 of them had inhibitors, 5 developing inhibitors after the intervention. RESULTS AND DISCUSSION: The cohort of patients consisted mostly of young adults, aged between 7 and 49 years. The vast majority of them, 91.3% of the patients, had the burden of severe chronic arthropathy. This was the reason for 95 interventions, with programmed solution. In 20 cases the cause of invasive interventions was emergency. Knee and hip replacement represented 28% of the major interventions. The complications we encountered were excessive bleeding (12.2%), infection (13%) and inhibitor development (4.3%). CONCLUSION: Surgery is a demanding intervention in patients with haemophilia, which unfortunately cannot be ignored in our country. Hopefully, the improved availability and accessibility to replacement therapy will eliminate the necessity of these interventions, optimizing the outcomes for the next generations.