Summary. This study aimed to ascertain whether extracorporeal photochemotherapy (ECP) is an effective treatment for paediatric patients with refractory graft-versus-host disease (GVHD). From January 1992 to December 2000, 77 children (median age 8AE6 years) with either acute (n ¼ 33) or chronic (n ¼ 44) GVHD, resistant to conventional immunosuppressive therapy, were treated with ECP in four Italian paediatric hospitals. After ECP, acute GVHD involving skin, liver and gut responded completely in 76%, 60% and 75% of patients respectively. The 5-year overall survival was 69% for responding patients vs 12% for nonresponders (P ¼ 0AE001). Among the 44 children with chronic GVHD, 15 (44%) showed a complete response and 10 (29%) a significant improvement after ECP. The 5-year overall survival was 96% for responders vs 58% for nonresponders (P ¼ 0AE04). Our results suggest that ECP is an effective treatment that may be useful in paediatric patients with either acute or chronic GVHD who have failed to respond to standard immunosuppressive therapy.

We investigated the incidence, risk factors and outcome of haemorrhagic cystitis (HC) in paediatric patients undergoing HSCT and the predictive value of BK viruria and viraemia for developing HC. Over a period of 54 months, 74 patients were recruited. The cumulative incidence of HC was 22%. Among 15 patients prospectively monitored for BK viruria and viraemia, four patients developed HC of grade XII. This group, which had two consecutive BK positive samples, showed a sensitivity of 100%, a specificity of 82%, a positive predictive value of 67%, and negative predictive value of 100% for developing HC. Analysed by a receiver-operator characteristic curve (ROC), a urine BK load 49 Â 10 6 genomic copies/ml had a sensitivity of 95% and specificity of 90%; while a blood BK load 41 Â 10 3 genomic copies/ml had a sensitivity of 40% and a specificity of 93% for HC, respectively. In univariate analysis, BK positivity was the only factor significantly associated with HC. After a median follow-up of 1.8 years, patients with HC showed a lower overall survival, 40 vs 65%, P 0.01, and a lower event-free survival, 42 vs 62%, P 0.03, compared to patients without HC. We conclude that BK detection in urine and/or plasma is a specific predictor for developing HC.

Summary:The purpose of this multicenter study was to assess the incidence and the treatment of hemorrhagic cystitis (HC) in 1218 pediatric patients, with a mean age of 10.8 years, who underwent hematopoietic stem cell transplantation (HSCT). In all, 44 patients (3.6%) developed HC a median 23 days after HSCT. The incidence of HC was higher in allogeneic than in autologous HSCT recipients (P ¼ 0.0001). Of the 44 patients, 37 (84%) recovered from HC in a median 30 days (range 3-100); the other seven children died while still suffering from HC. Hyperbaric oxygen therapy (HOT) achieved significantly better results than prostaglandin therapy (P ¼ 0.02) in the treatment of grade II-III HC. By multivariate analysis, age o96 months and allogeneic HSCT were significantly associated with the occurrence of HC: P ¼ 0.008 and 0.013, respectively. After a median follow-up of 5.75 years, the 5-year survival of patients who did or did not develop HC was: 43 vs 52%, P ¼ 0.03, respectively. This study indicates that age and type of HSCT are factors predisposing to HC in children given HSCT and demonstrates the promising role of HOT in a conservative approach to HC treatment. Bone Marrow Transplantation (2003) 32, 925-931.

BackgroundThe aim of the study was to evaluate the clinical characteristics and the long‐term outcome of chronic hepatitis C in a cohort of Caucasian children cured of pediatric malignancy.ProcedureThe study population included 83 consecutive patients, referred to our Center with a diagnosis of leukemia/lymphoma (50) or solid tumors (33) between 1977 and 1989 and infected with hepatitis C virus (HCV) during chemotherapy.ResultsAt enrollment 77 subjects were HCV‐RNA positive. After a median follow‐up of 21 years (range 13–36), a sustained virological response (SVR) was obtained in 3 of 29 patients (10%) treated with interferon (IFN), in 1 of 3 patients (33%) treated with IFN and ribavirin, and in 5 of 11 patients (42%) treated with pegylated‐IFN and ribavirin (P = 0.03). Forty‐two patients remained untreated and only one (2.5%) cleared viremia. Four of 77 patients (5%) developed cirrhosis while other 4 patients died of causes not related to liver. At last follow‐up, 72% of HCV‐RNA positive patients had abnormal ALT.ConclusionsIn patients cured of pediatric malignancy chronic hepatitis C tends to run an indolent course during childhood and adolescence but more than 70% of treated and more than 80% of untreated cases children maintained HCV viremia. Moreover, after 2–3 decades of observation, 60% of HCV‐RNA positive patients had abnormal ALT and 5% had developed cirrhosis. Among treated patients, IFN or pegylated‐IFN and ribavirin obtained the higher rate of HCV‐RNA clearance. Pediatr Blood Cancer 2010;55:108–112. © 2010 Wiley‐Liss, Inc.

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Myositis ossificans circumscripta (MOC) is a benign disease characterized by localized heterotopic bone formation within muscles or soft tissue, usually interesting great muscles of extremities. We report a rare case of unusual location in the neck not associated with previous trauma, mimicking a solid tumor, with well-documented diagnostic imaging features. During COVID-19 pandemic outbreak in Italy, in May 2020, a 14-year-old boy developed a progressive and persistent neck pain on the right side, without known history of trauma. Initial therapy with non-steroid anti-inflammatory drugs and physiokinetic therapy gave only a slight improvement. A neck ultrasound showed an inhomogeneous right neck mass, with posterior shadowing due to calcifications. Computed tomography and magnetic resonance imaging confirmed a huge right neck mass, located in the paravertebral space with peripheral calcifications and mild central contrast enhancement. After surgical excision of the lesion, pathology revealed the presence of muscular tissue mixed with fibroblastic/myofibroblastic proliferation and ossification areas consistent with myositis ossificans. A careful analysis of clinical and radiological features is very important to manage young patients showing progressive pain and swelling of the neck, since MOC can mimic soft tissue or bone tumors, and it should be suspected even in the absence of a known history of trauma.