This case report describes a patient with a rare occurrence of primary spinal intramedullary Ewing's sarcoma (ES) in the cervical and thoracic spine. The older age of disease occurrence, uncommon location in the cervical and thoracic spine, and EWSR1 gene fusion as the basis of diagnosis are unique features of this case. There is no clear protocol for treatment of primary extraskeletal ES of the spine, with controversy between evidence for pursuing surgery versus a combination of radiation and chemotherapy. Our patient was treated with temozolomide chemotherapy for recurrent metastatic disease of primary ES of the spine.
Background/Aim: Prolonged use of glucocorticoids (GC) in glioma treatment can lead to adrenal insufficiency (AI) and subsequent steroid dependence due to suppression of the hypothalamic-pituitary-adrenal (HPA) axis. This is challenging to diagnose due to its nonspecific clinical symptoms erroneously ascribed to treatment. This study aimed to evaluate the risk factors predisposing patients with gliomas to develop AI. Patients and Methods: Charts in the neurooncology clinic from July 2018 to March 2019 were reviewed. Inclusion criteria included >18 y/o with WHO Grade II-IV gliomas, and secondary AI. Demographic profile, tumor characteristics, and treatment profile were compared. Results: The majority of patients were started on high dose dexamethasone at >8 mg daily, and were on dexamethasone for 4-8 months. The minimum dose needed to prevent symptoms was 0.5 mg to 2 mg daily. The majority received standard radiation doses ranging from 54-60 Gy. Most patients had radiation exposure to the HPA axis within the prescription isodose levels. Conclusion: Prolonged steroid dependency can result from chronic GC use in patients with glioma. Dose and duration of GC are risk factors for its development. Radiation exposure to the HPA axis may also be a contributing factor.Glucocorticoids (GC) are a mainstay in the treatment of primary brain tumors. GC have been used for reducing vasogenic edema and improving symptoms related to swelling including: lethargy, headache, and nausea, among others. Dexamethasone is the commonly used GC for the treatment of central nervous system (CNS) tumors due to its potent anti-cerebral edema effects, long half-life, and low mineralocorticoid activity, hence minimizing fluid retention.Despite their benefits, prolonged use of GC can have a number of negative consequences. One of the most commonly unrecognized symptoms is the development of adrenal insufficiency (AI) and subsequent steroid dependence due to the suppression of the hypothalamicpituitary-adrenal (HPA) axis. Clinically, this can present with a wide range of signs and symptoms, including weakness/fatigue, malaise, nausea, vomiting, diarrhea, abdominal pain, headaches, fever, anorexia/weight loss, myalgia, arthralgia, as well as psychiatric symptoms (1). Screening for AI includes measuring early morning cortisol at 8:00 AM after GC dose has been tapered to a physiologic dose, and holding any oral GCs the evening and morning prior to the test. If the morning cortisol is normal but clinical suspicion for AI is high, an adrenocorticotrophic stimulating hormone (ACTH), cosyntropin stimulation, test can be performed to clarify the diagnosis (1).The challenge in diagnosing AI in glioma patients may stem from its nonspecific clinical presentation, and thus the tumor itself or the treatments (e.g. chemotherapy, radiation therapy) may be erroneously ascribed as the cause of these symptoms. Hence, GC use is often extended unnecessarily. In addition, there are no guidelines on the dosing, duration, and tapering of GC in neuro-oncologic patients, a...
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