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Endocrine Abstracts (2023) 90 RC6.3 | DOI: 10.1530/endoabs.90.RC6.3

1Complejo Hospitalario Universitario de Albacete, Endocrinology and Nutrition, Albacete, Spain; 2Virgen del Rocío University Hospital, Endocrinology and Nutrition, Sevilla, Spain; 3Virgen del Rocío University Hospital, Sevilla, Spain; 4Hospital Universitari de la Ribera, Endocrinology and Nutrition, Alzira, Spain; 5Hospital Universitario Vall d’Hebron, Endocrinology and Nutrition, Barcelona, Spain; 6Hospital de Bellvitge, Endocrinology and Nutrition, L’Hospitalet de Llobregat, Spain; 7Ramón y Cajal Hospital, Endocrinology and Nutrition, Madrid, Spain; 8Hospital Clínic de Barcelona, Endocrinology and Nutrition, Barcelona, Spain; 9Puerta de Hierro Majadahonda University Hospital, Endocrinology and Nutrition, Majadahonda, Spain; 10Gregorio Marañón General University Hospital, Endocrinology and Nutrition, Madrid, Spain; 11Hospital Universitari y Politècnic La Fe, Endocrinology and Nutrition, València, Spain


Introduction: Current guidelines propose temozolomide (TMZ) as the first-line chemotherapy for aggressive pituitary neuroendocrine tumors (PitNETs), but no clinical trials have been conducted and clinical experience in this context is limited.

Patients and methods: A retrospective study of patients with aggressive pitNETs treated with TMZ was conducted by the members of the Neuroendocrinology Working Area of the Spanish Society of Endocrinology and Nutrition.

Results: Twenty-eight patients (9 women and 19 men), aged 46.6+16.9, from 10 Spanish Neuroendocrinology Units were included. Visual disturbances were present in 82.1%, headache in 71.4% and hypopituitarism in 67.9%. Four patients had Cushing’s disease (one of them Nelson’s syndrome), 9 prolactinomas and 15 clinically non-functioning pituitary adenomas (NFPAs). Among the 15 NFPA, immunohistochemistry was positive for ACTH in 7 cases, for GH in 3 cases, for prolactin in one case, for gonadotrophins in one case, and 3 tumors were negative for all pituitary hormones. Median size at diagnosis was 10.5 cm3 (IQR 4.7-22.5), with cavernous invasion in 88% and no metastases. Pre-TMZ treatment, these data were 5.2 cm3 (IQR 1.9-12.3), 89.3% and 14.3% (2 intracranial and 2 spinal metastases). Before TMZ treatment all patients had undergone surgery (1-5 surgeries, median 2), 25 (89.3%) had received radiotherapy (7 of them reirradiated) and 13 (46.4%) had received cabergoline. One patient interrupted TMZ prematurely due to intercurrent complications. The other 27 patients received a median of 13 cycles (range 3-66) of TMZ with a median final daily dose of 300 mg (IQR 200-334). Eight patients (29.6%) had a significant reduction (>30%) in tumor volume and 14 (51.9%) attained tumor stabilization. After a median follow-up of 29 months (IR 10-55), 8 out of those 22 progressed (median time from treatment to progression 16.5 months, RI 7-27). Four patients received a new course of TMZ treatment. Seven patients (25%) died (all of them because of tumor progression or complications of treatments) at 77 months (IQR 42-136) from diagnosis and 29 months (IQR 16-55) from TMZ first treatment. Adverse effects occurred in 18 patients (14 mild and 4 moderate or severe).

Conclusions: TMZ is an effective medical treatment for aggressive pitNET (81.5% tumor shrinkage or stabilization) but is sometimes followed by tumor progression. Its safety profile is acceptable. After a median follow-up of 29 months, we observed a 25% mortality.

Volume 90

25th European Congress of Endocrinology

Istanbul, Turkey
13 May 2023 - 16 May 2023

European Society of Endocrinology 

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