Mucormycosis is an uncommon but severe fungal infection, typically observed in immunocompromized patients. We report a case of acute lymphoblastic leukemia complicated by rhino-oculo-cerebral mucormycosis in a pediatric patient. Combination lipid polyeneechinocandin therapy, along with surgical debridement appeared to be effective. Nevertheless, a severe relapse occurred during posaconazole prophylaxis; antifungal therapy, hemimaxillectomy and suspension of chemotherapy were performed. Although mucormycosis is a frequently lethal infection, prompt diagnosis and aggressive treatment can be successful even in cases of relapse.

Central precocious puberty (CPP) is a condition that causes early gonadotropin-dependent sexual development; CPP is idiopathic in girls in most cases, whereas more than 50% of boys have an identifiable etiology. We conducted a qualitative systematic review following the ENTREQ (enhancing transparency in reporting the synthesis of qualitative research) framework. A search was made in MEDLINE/Pubmed and MeSH Database using the terms “precocious puberty” AND “brain tumor” OR “posterior fossa tumor” OR “cerebellar tumor” OR “infratentorial tumor”, identifying five cases of pediatric patients with infratentorial tumors and CPP and a case of cerebellar ganglioglioma without hypothalamic−pituitary−gonadal axis involvement and/or intracranial hypertension. Our work highlights the importance of a multidisciplinary approach and extensive central nervous system imaging for patients presenting with CPP in order to detect possible tumor association. Moreover, we believe that this manuscript could contribute to stimulate other research because the exact mechanism of CPP in infratentorial brain lesions has not been understood yet.

Background/Objectives: Selumetinib, a MEK1/2 inhibitor, is commonly used for treating neurofibromatosis type 1 (NF1) and is associated with cutaneous side effects such as paronychia and periungual granulomas. These complications can be painful and difficult to manage, often leading to the discontinuation of treatment. The objective of this study was to evaluate the effectiveness of photodynamic therapy (PDT) as a novel treatment for MEKi-induced paronychia in a patient with NF1. Methods: We present a case report of an 18-year-old patient with NF1 who developed painful periungual granulomas on the toenails after 12 months of Selumetinib therapy. PDT was administered using methyl aminolevulinate (METVIX^®) as the photosensitizing agent, followed by treatment with a red LED light source (630 nm, 37 J/cm² for 8 min and 30 s). The patient was followed up for two months post-treatment and then at two years. Results: After a single PDT session, the patient exhibited complete clinical remission of the periungual granulomas and associated pain. No recurrence of the lesions was noted during the two-year follow-up. The patient tolerated the procedure well, reporting only mild discomfort during treatment. Conclusions: PDT appears to be an effective, minimally invasive treatment for Selumetinib-induced paronychia and periungual granulomas. This case demonstrates that PDT can provide a complete resolution of symptoms with a single treatment session, offering an alternative to more invasive procedures. Further studies with larger cohorts are needed to establish PDT as a standard treatment option for this condition.

Li-Fraumeni syndrome (LFS) is a rare high-penetrance and autosomal-dominant pathological condition caused by the germline mutation of the TP53 gene, predisposing to the development of tumors from pediatric age. We conducted a qualitative systematic review following the ENTREQ (Enhancing Transparency in Reporting the Synthesis of Qualitative Research) framework. A search was made in MEDLINE/Pubmed and MeSH Database using the terms “Li-Fraumeni” AND “pediatric high-grade glioma (HGG)”, identifying six cases of HGGs in pediatric patients with LFS. We added a further case with peculiar features such as no familiar history of LFS, association of embryonal rhabdomyosarcoma and bithalamic HGG, whose immunohistochemical profile was accurately defined by Next Generation Sequencing. Knowledge synthesis and case analysis grounded the discussion about challenges in the management of this pathology in pediatric age.

The purpose of the study was to examine safety and efficacy of non-activated Protein C (PC) supplementation in our cohort of septic pediatric hematological patients. We conducted a retrospective study of 22 septic patients receiving human plasma-derived PC concentrate from 2008 to 2015 at our Pediatric Oncology Center (Bari, Italy). The Surviving sepsis campaign definitions for sepsis, severe sepsis and septic shock were used to define the patients’ septic status. For each patient, we calculated Lansky performance status scale (LPSS) and a risk score defined the Hematologic risk score (HRS) that we created in 2007. Patients were defined as High risk for severe sepsis/septic shock in case of HRS>3. HRS10% PC concentration decrease within 12 hours from the first evaluation. All patients received 80 U/kg/day PC, intravenously, every twenty-four hours. No drug-related adverse event was observed. The observed sepsis-related mortality rate in our cohort was 9%. PC supplementation in our cohort appeared to be safe, and, probably due to prompt PC administration, we observed an overall mortality that was much lower than expected mortality in cancer severe septic patients.

Background: Serum albumin is crucial for critically ill patients. To date, several reports have focused on the influence of lower albumin levels on poorer prognosis and disease outcome in different subsets of critical clinical conditions varying from sepsis, to cirrhosis, renal failure, and cancer. In the last few years, investigators reported the role of serum albumin levels in predicting the thrombotic risk in patients with nephrotic syndrome, and, in particular, the degree of hypoalbuminemia seemed to influence the risk of thromboembolism. Decreased serum albumin has been associated with the risk of venous thromboembolism and mortality in adult cancer patients after ending chemotherapy for different malignancies. Aims: We aimed to investigate the role of serum albumin in a cohort of children diagnosed as having VTE (venous thromboembolism) during their treatment for acute lymphoblastic leukemia (ALL) compared to ALL children who did not experience VTE. Methods: A nested case-control study was conducted at the Pediatric Oncology and Hematology Department, University Hospital of Bari. A total of 167 patients were diagnosed as having ALL and treated according to AIEOP-BFM ALL 2000-R2006 protocol. Among these, 12 cases of VTE were recorded and matched to 31 controls, for a total of 43 ALL patients (30 males, aged 1.2–16.6 years) enrolled in the present study. Serum albumin level was collected at diagnosis—before the start of any treatment—(time point 0) and at the moment of the VTE or corresponding time point of the protocol (time point 1). Information on inherited thrombophilia genotype were also recorded. Results: Patients presenting VTE showed a marked reduction of average albumin levels as compared to the control children: t0–t1 1.1 IC (95%) = (0.55, 1.65) vs. 0.31 IC (95%) = (0.08, 0.55); p < 0.005. Conclusions: The reduction of serum albumin levels in our cohort might be an expression of altered vascular and endothelial homeostasis, likely predisposing to VTE. This important clinical observation warrants further larger studies.