Toxicity and Clinical Results after Proton Therapy for Pediatric Medulloblastoma: A Multi-Centric Retrospective Study

Medulloblastoma is the most common malignant brain tumor in children. Even if current treatment dramatically improves the prognosis, survivors often develop long-term treatment-related sequelae. The current radiotherapy standard for medulloblastoma is craniospinal irradiation with a boost to the primary tumor site and to any metastatic sites. Proton therapy (PT) has similar efficacy compared to traditional photon-based radiotherapy but might achieve lower toxicity rates. We report on our multi-centric experience with 43 children with medulloblastoma (median age at diagnosis 8.7 years, IQR 6.6, M/F 23/20; 26 high-risk, 14 standard-risk, 3 ex-infant), who received active scanning PT between 2015 and 2021, with a focus on PT-related acute-subacute toxicity, as well as some preliminary data on late toxicity. Most acute toxicities were mild and manageable with supportive therapy. Hematological toxicity was limited, even among HR patients who underwent hematopoietic stem-cell transplantation before PT. Preliminary data on late sequelae were also encouraging, although a longer follow-up is needed

Angiocentric glioma-associated seizures: The possible role of EATT2, pyruvate carboxylase and glutamine synthetase

Purpose: Our purpose was to better understand the pathogenesis of seizures associated with angiocentric glioma. Angiocentric glioma is an indolent and rare low-grade glioma. Its typical clinical presentation is with epileptic seizures. The pathogenesis of tumor-associated seizures is poorly understood. Among the possible pathomechanisms, the increased neurotoxic concentrations of the glutamate has been proposed. Glutamate transporters, pyruvate carboxylase and glutamine synthetase are involved in maintaining the physiological concentration of glutamate in the inter synaptic spaces. Methods: We evaluated the immunohistochemical expression of EAAT2 (the most important glutamate transporter), pyruvate carboxylase and glutamine synthetase in 17 angiocentric gliomas. Results: EAAT2 was never expressed (0%) in the neoplastic cells in none of the cases studied. Pyruvate carboxylase was expressed in the cytoplasm of the neoplastic cells in 16/17 cases (94 %). Glutamine synthetase was expressed in the cytoplasm of the neoplastic cells in 15/17 cases (88 %). Conclusion: The net result of this enzymatic expression, in particular considering the loss of EAAT2, could be an increased glutamate concentration in the synaptic clef, which might increase local network excitability initially involving intratumoral neurons. The observation that the angiocentric glioma-associated epilepsy might be at least in part related to EAAT2 deficiency opens up interesting therapeutic perspectives

Endoscopic endonasal skull base surgery in pediatric patients. A single center experience

Object: At present, a minimally invasive endoscopic endonasal approach is considered an efficient option for lesions affecting the anterior and middle skull base with sellar and parasellar region involvement. In this study we will retrospectively analyze the pediatric patients that we have been treating in the same medical center for the past four years, using an endoscopic approach in the skull base. Methods: We performed a retrospective chart and imaging review of pediatric patients who underwent endoscopic endonasal skull base surgery (ESBS) at the Meyer Children's Hospital, (Azienda Ospedaliero Universitaria Meyer), in Florence, from January 2012 to July 2016. Results: Mean age was 12,5 years; 28 (65%) of the 44 patients were females. Skull base lesions were broadly classified as either bony abnormalities (4 cases) or skull base tumors (40 cases). The postoperative clinical follow-up duration ranged from 2 to 36 months. The two more frequently occurring diseases in our study were: craniopharyngioma and pituitary adenoma. Conclusions: After reviewing the international literature about pediatric endoscopic endonasal approach to skull base, we can affirm that our study is the world's second broadest work for number of records. While compiling this report, we have examined the first 40 consecutive pediatric patients to undergo ESBS at our institute. The appearance of postoperative complications is consistent with other international studies, confirming ESBS being feasible and safe even in the pediatric population. Level of evidence: 4

Management of orbital and brain complications of sinusitis: A practical algorithm.

The aim of this work was to present a practical management algorithm for orbital and brain complications of sinusitis. According to the inclusion criteria, a sample of 68 patients was collected between 2008 and 2018 (39 males and 29 females). Among them, 44 were adults, with a mean age of 50.46 years, and 24 were pediatric patients, with a mean age of 10.33 years. Oral or intravenous antibiotic therapy was administered to all patients. Pharmacological resolution was observed in 14 cases. Early surgical treatment within 48 hours was necessary in 10 cases. Surgery consisted of abscess drainage, associated or not with functional endoscopic sinus surgery. Delayed surgery within 15–30 days was performed in 44 patients. The core procedure was functional endoscopic sinus surgery. Subsidiary procedures were abscess drainage, tooth extraction or cranial base repair. The combination of two or more of these procedures was case selected. Median follow-up was of 46.36 months. Sinusitis complications necessitate rapid diagnosis and prompt treatment. Antibiotic therapy alone is enough for mistreated rhinosinusitis with no anatomical predisposing factor. Surgery is mandatory for altered nasal and paranasal sinus anatomy or odontogenic infections

Multimodal treatment of peritoneal carcinomatosis and sarcomatosis

Peritoneal carcinomatosis and sarcomatosis (PCS) are short-term fatal conditions amenable only to palliative treatment. They are generally considered as a systemic disease at clinical presentation, and are resistant to standard treatments. However, there may be in the natural history a phase of loco-regional tumour spread during which the tumour may still be curable. Surgical treatment alone, or in combination with systemic chemotherapy, has yielded poor results in terms of survival and quality of life. One approach is cytoreductive surgery (CS) combined with the intraperitoneal administration of antiblastic agents. This may diminish any residual tumour following macroscopic excision and may overcome the pharmacokinetic limits of systemic chemotherapy. A further improvement in this multimodal approach may be achieved by the use of hyperthermic intraperitoneal intraoperative chemotherapy (HIIC). Results so far have been encouraging. However, series reported in the literature are relatively small and heterogeneous, and clinical and technical factors which include the selection of patients, optimal drugs dosage and temperature, evaluation of outcome and costs are still under discussion