ODP294 An Unusual Case of Endocrine Disruption as an Early Expression of Low Grade Intra Sellar Chordoma

• Published in: Journal of the Endocrine Society Vol. 6; no. Supplement_1; pp. A483 - A484
• Main Authors: Babu, Vedesh Kumar, Suravajjala, Devi, Kolli, Swapna, Eranki, Madhuri, Eranki, Vijay
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 01.11.2022
• Subjects: Neuroendocrinology and Pituitary
• ISSN: 2472-1972; 2472-1972
• DOI: 10.1210/jendso/bvac150.1004

Abstract A 53y/o male patient presented to his PCP with fatigue and diagnosed with low testosterone and was started on testosterone. P ituitary labs were not checked then. After 6-8 weeks he was referred to neurology for new onset vision problems, diplopia, and daily headaches. MRI brain then showed a sellar mass of 2.4×2.6×2.7 cm with sphenoid sinus extension and bony thinning alongthe margins of the sella turcica which was nearly imperceptible. On testosterone replacement, biochemical assessment of anterior pituitary function showed total testosterone: 836 (264–916 ng/dl), Free Testosterone: 32.6 (2.5-9.5 ng/ml), LH<0.1 (1.2-8.6 IU/L), FSH 0.2 (1.5-12.4 IU/L), Prolactin 18 (4-26 ng/ml), TSH 0. 03(0.4-4.1 UIU/ml), FT4 0.63 (0.8-1.9ng/dl), IGF 188 (55-186 ng/ml), ACTH<5 (10-65 pg/ml), Cortisol<1 (4.8-19.5 ug/dl). Endoscopic endonasal surgery was performed with complete removal of the tumorand abdominal fat graft harvest. Histopathological findings confirmed well differentiated low grade chordoma. Post operatively he improved, and headaches and visual disturbances resolved. Patient was sent home on Hydrocortisone 10mg am/5mg pm. Then, he presented to our endocrine clinic for initial hormonal evaluation. On steroid replacement, endocrine testing was repeated four weeks post-surgery. Labs showedTSH 2. 070 (0.4-4.1 UIU/ml), FT4 1.16 (0.8-1.9ng/dl), FSH<0.3(1.5-12.4 IU/L), LH<0.3(1.2-8.6 IU/L), Prolactin 26 (4 -26), ACTH 19.6 (10-65 pg/ml), Cortisol 7 (4.8-19.5 ug/dl), IGF-1 129 (55-186 ng/ml). Four months after the surgery, patient passed the cosyntropin stimulation test and was able to wean off the hydrocortisone replacement. Post-op repeat MRI brain showed complete resolution of the clivial mass. Current, he remains stable with regular follow ups. The most common presenting symptoms of a sellar chordoma include visual problems and uncommonly hypopituitarism. Our case shows that non-adenomatous tumors can still challenge us with the original presentation as an endocrine deficit. In a case series, only one of nine cases had presented with pituitary dysfunction. Our patient was noted to have hypogonadism and started on testosterone supplementation without further investigation. It is important to evaluate hormonal status in all pituitary masses and recognize that some cases of sellar chordoma can present with endocrine dysfunction as the initial manifestation. In our case, endocrine function of the patient returned to normal once the pituitary compression from chordoma was eliminated. Presentation: No date and time listed