Endocrine Abstracts

Craniopharyngiomas are rare, benign tumors, typically found in childhood or early adulthood, that can cause a wide range of symptoms such as visual impairment, headaches, nausea and endocrine disturbances. Ewing sarcoma, on the other hand, is a rare and aggressive tumor that arise from primitive neuroectodermal cells and represents about 10% of all pediatric osseous primary tumors. We present the case of a 14-year-old patient who was admitted to our clinic in March 2022 for obesity, secondary amenorrhea and visual impairment. The patient was diagnosed at the age of 11 with Ewing sarcoma of the lumbar spine with left intracanal extension L5-S1. She had received concomitant local radiotherapy (45Gy per 25 fractions) and chemotherapy (8 cycles of Vincristine, Ifosfamide, Doxorubicin, Etoposide) according to Ewing Protocol from 2008. The patient experienced her first menarche 2 months after the first cure of chemotherapy, followed by one additional menstrual cycle at a two-month interval, with secondary amenorrhea since. In July 2020, she experienced a seizure and visual disturbances. Cerebral MRI showed a suprasellar cystic mass measuring 1.6/1.3/1.5cm, causing optic chiasm compression, first diagnosed as a Rathke cleft cyst. The patient began anticonvulsant treatment and continued to receive regular follow-up care. During the patient’s first presentation in our clinic, laboratory evaluation revealed elevated follicle-stimulating hormone levels (35.30mIU/ml) with low estradiol levels (13.18 pg/ml) and AMH levels (0.01ng/ml) suggesting primary ovarian failure due to radiotherapy and chemotherapy. Thyroid function was also modified, suggesting hypothyroidism due to autoimmune thyroiditis. Ophthalmologic evaluation revealed right homonymous hemianopia. The 2-year follow-up MRI showed unchanged dimensions of the suprasellar mass, impingement on the optic chiasm. Unfortunately, according to the neurosurgeon the mass was highly suggestive for a craniopharyngioma requiring surgery if the visual impairment intensified. Taking in consideration the fact that our patient had a history of epilepsy, we initiated sex hormone replacement therapy with low doses of estrogens, progestin and levothyroxine. In conclusion, we report a rare association between Ewing sarcoma, an aggressive bone and soft tissue tumor and craniopharyngioma, a benign, intracranial tumor, both requiring complex and challenging management by a multidisciplinary team due to significant long-term morbidities caused by their treatment and the loco-regional tumor growth. A lifetime follow-up is necessary to address multiple endocrine deficits, that may adversely affect fertility, growth or thyroid function, highly influencing the quality of life.