Background Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high‐risk patients. Methods Retrospective mono‐institutional clinical, radiological, and histological review of patients with uni‐ or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed. Results Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11‐96). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10/11 patients—by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high‐dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5‐19.4]). Conclusion Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre‐operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.

We present a teenager, in maintenance chemotherapy for leukemia, who was admitted for digestive symptoms related to a parasitic infection and required nutritional support with parenteral nutrition. After 6 weeks, his condition worsened with refractory shock of presumed septic origin, necessitating extracorporeal membrane oxygenation. Despite hemodynamic stabilization, his lactic acidosis worsened until thiamine supplementation was started. Lactate normalized within 12 hours. Thiamine is an essential coenzyme in aerobic glycolysis, and deficiency leads to lactate accumulation through anaerobic glycolysis. Thiamine deficiency is uncommon in the pediatric population. However, it should be considered in patients at risk of nutritional deficiencies with lactic acidosis of unknown origin.

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Delivering optimal cancer care to children, adolescents and adults with ASD has recently become a healthcare priority and represents a major challenge for all providers involved. In this review, and after consideration of the available evidence, we concisely deliver key information on this heterogenous group of neurodevelopmental disorders, as well as recommendations and concrete tools for the enhanced oncological care of this vulnerable population of patients.

10551 Background: To evaluate treatment and outcome of patients with uni- or bilateral retinoblastoma (RB) with radiologic optic nerve invasion (RONI) at diagnosis. Methods: Retrospective clinical, radiological and histological review of patients with uni- or bilateral RB with RONI at diagnosis treated in the Institut Curie. Results: Between 1997 and 2014, 936 patients with RB were treated in the Institut Curie. Eleven patients had detectable RONI confirmed by Computed Tomography and/or Magnetic Resonance Imaging. RB was unilateral in 10/11 patients, bilateral in 1. Median age at diagnosis was 29 months (range 12-96). The patient with the bilateral RB had a unilateral RONI. Nine patients had ON enhancement and 3 had meningeal sheath enhancement. Nine received neoadjuvant chemotherapy (CT) and 2 had a primary enucleation. Partial response to neoadjuvant CT was obtained for all the patients. Enucleation was performed in 10/11 patients, by anterior approach in 3 patients, by anterior and subfrontal approach in 7 patients. Three patients had positive ON margin and among them, 2 were primary enucleated. All enucleated patients received adjuvant treatment (conventional CT: 10, High Dose CT: 7 and radiotherapy: 5). Three patients died of meningeal progression (2 during treatment and 1 during the first year after treatment). The patient with the bilateral RB was lost to follow up just after a meningeal progression during treatment. Seven are still alive (median follow up: 8 years, range : 1.5-17.5). Conclusions: Neoadjuvant CT has an important place in the management of unilateral RB with RONI at diagnosis. Pretreatment accurate staging by orbital and brain MRI is mandatory, as well as preoperative reassessment. Surgery should be performed by experienced ophthalmologists and if necessary neurosurgical team in order to obtain the best conditions for a tumor-free resection margin in patients with RONI.