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Diffuse midline glioma (DMG) of the pons remains the leading cause of death among pediatric patients with brain tumors, despite numerous attempts to intensify treatment. While standard treatment includes 54Gy in 30 fractions of radiation over six weeks of time, nearly all patients progress within the treatment field, and many experience symptomatic radionecrosis with steroid dependence. Symptom improvement typically begins after 20Gy to the tumor. Both hypofractionation and reirradiation after recurrence have been found to be safe for patients with DMG. Our study aims to enroll patients with newly diagnosed pontine DMG aged >1 year (no maximum age), and collect molecular information about DMG via stereotactic biopsy, followed by a short 2 week course of 25Gy in 10 fractions of radiation, with volumes guided by MRI with tractography reconstruction and FDOPA PET radiotracer uptake. Patients are followed closely and can complete the 25Gy in 10 fraction radiation course up to 3 times total for meeting radiographic and clinical progression criteria. Our primary endpoint is to estimate the time to progression from diagnosis to after receiving the second 25Gy course, and to compare this to the historical 7 month standard from diagnosis to progression after one 54Gy course. We aim to improve the time interval where patients are asymptomatic at home while minimizing time receiving daily treatments. Other endpoints will include the patient quality of life, caregiver quality of life, PFS intervals after each course, overall survival, and toxicity.

Objectives Sialoblastoma is a rare congenital salivary gland tumor of epithelial origin. The objectives of this study are to review the literature regarding clinical presentation of sialoblastoma, evaluate the effectiveness of various treatment methods, and present guidelines for evaluation and management in the pediatric population. Data sources: Case presentation and literature review. Review methods A comprehensive search was conducted to identify cases of pediatric sialoblastoma in the English-language literature. The presentation, evaluation, and management of reported cases were analyzed. We also report an invasive and recurrent case in a pediatric patient to highlight the aggressive nature of these lesions. Results Sixty-two cases of pediatric sialoblastoma were reviewed. The age at initial presentation ranged from before birth to 15 years. The parotid gland was the most common location (n = 47). Surgical excision was the primary treatment in all patients. Nine patients developed metastatic disease of the lung, lymph nodes, or bone. Almost a third of patients had recurrence and over two thirds of patients were tumor-free for at least 1 year following their last treatment intervention. Conclusion Prompt and complete surgical excision should be recommended to prevent local and systemic recurrence of pediatric sialoblastoma. Chemotherapy has also shown promise in several cases, and clinical genomics may shed light on more therapy options. Patients should be closely followed for at least 12 months following diagnosis, or longer depending on the histopathological staging of the tumor.

Masses intrinsic to the pineal gland are uncommon and caused by a small number of tumor types, most of which are similar in imaging appearance. As a result, it is difficult to provide a specific diagnosis based on the imaging characteristics alone. Nonetheless, it is possible to narrow potential diagnostic considerations to a limited number of pathologic entities based on a careful analysis of the imaging. In this chapter we emphasize this systematic approach to pineal region masses by reviewing the anatomy and function of the pineal gland followed by a review of the epidemiology of pineal lesions, a discussion of imaging features of pineal region tumors, and last, an overview of treatment and prognosis of these lesions.