Endocrine Abstracts

Background: Radiation-induced sarcoma (RIS) in the sella is a rare but serious complication following radiotherapy for pituitary tumors.

Clinical Case: A 43-year-old patient with recurrent extra-sellar pituitary macroadenomas underwent trans-sphenoidal surgery (TSS) in 2006 and 2016 due to optic chiasm compression. Initial histology showed gonadotroph staining with a KI index of 2%. After a residual tumor was found in the right cavernous sinus, the patient received adjuvant stereotactic radiosurgery (SRS) of 12.5 Gy in 2016. Post-operative tests revealed suboptimal growth hormone (GH) and testosterone levels, leading to treatment with Genotropin SC and Tostran 2% gel. Seven years later, in 2023, the patient presented with headache, double vision, and right third nerve palsy. MRI a few months earlier had shown empty sella with a small residuum. Repeat MRI indicated a large sellar and suprasellar mass compressing the optic chiasm, causing bony erosions and extending into the right cavernous sinus, distally displacing the internal carotid artery. Given the stability of the disease for seven years, the aggressive nature of the new mass was unexpected, especially in the light of the earlier routine follow-up MRI scan. An emergency endoscopic TSS was performed for decompression and maximal resection. Histology revealed spindle cell sarcoma. CT TAP excluded metastases. Post-operative MRI indicated a residual mass measuring 12 x 17 x 29 mm in the pituitary fossa. The multidisciplinary team (MDT) initiated adjuvant chemotherapy, with three cycles of Doxorubicin and Ifosfamide so far. Follow-up MRIs show continuous reduction in residual tumor size with persistent suboptimal cortisol, testosterone, thyroid, and GH responses requiring hydrocortisone, Thyroxine, and Nebido.

Conclusion: This case exemplifies secondary sarcoma following pituitary irradiation. Strict follow-up and high suspicion after previous irradiation treatment is recommended for early diagnosis and management.