SFEIES24 Poster Presentations Neuroendocrinology (30 abstracts)
Multidisciplinary management of aggressive corticotroph pitnets. a case series
Serban Radian 1,2 , Dan Alexandru Niculescu 1,2 , Simona Galoiu 1,2 , Liviu Stoleru 3 , Monalisa Preda-Ivascu 2 , Andreea Nuta 1 , Cristina Iancu 2 , Vasile Ciubotaru 4 , Ionut Gobej 5 , Alexandru Cristescu 6,7 & Catalina Poiana 1,2
1Department of Endocrinology, C. Davila University of Medicine and Pharmacy, Bucharest, Romania; 2C.I. Parhon National Institute of Endocrinology, Bucharest, Romania; 3Department of Radiotherapy, Neolife, Bucharest, Romania; 4Department of Neurosurgery, Bagdasar-Arseni Emergency Hospital, Bucharest, Romania; 5Department of Neurosurgery, Neurohope, Bucharest, Romania; 6Department of Neurosurgery, Colentina Emergency Hospital, Bucharest, Romania; 7Department of Neurosurgery, Ponderas Academic Hospital, Bucharest, Romania
Background: Aggressive PitNETs require multidisciplinary management. Cushing’s disease (CD) poses specific challenges regarding biochemical control and management of complications.
Aim: To describe management of a series of aggressive corticotroph PitNETs.
Patients: Six patients (2F/4M, 46-75 yo), managed through the pituitary MDT.
Results: At diagnosis all patients had overt CD due to macroPitNets (2-3.8 cm diameter), with suprasellar extension (2), cavernous (4) and sphenoid sinus (3) invasion. Initial manifestions included diabetes mellitus (100%), hypertension (83%), obesity (100%), optochiasmatic syndrome (83%), thyrotroph (83%) and gonadotroph (83%) failure. 4 patients had oculomotor palsy: 3 at onset (1-tumour invasion, 2-apoplexy), 1 later, through cavernous invasion. 3 patients had pituitary apoplexy: 2 at onset (one recurred), 1 during follow-up. 2 patients presented nonfatal pulmonary thrombembolism. Median follow-up was 31 months (0-191). 2 patients died: M, 75 yo, before PitNET treament, due to respiratory sepsis and M, 67 yo, treated multimodally, 31 months after diagnosis, due to septic lower limb ulcers.
Treatment: Transphenoidal surgery: 4 patients (1-3 interventions/patient), subsequently irradiated: 3 fractionated radiotherapy (RXT), 1 gammaknife. The patient with recurrent apoplexy underwent RXT and medical treatment only. Pathology (n = 4): ACTH+, Ki-67=10-80%, small cell tumours (2), frequent mitoses (2), necrosis (1), Crooke cell tumour (1). 5 patients achieved tumour control initially, 4 escaped, after 15-132 months. Presently 2 patients have tumour control, the 2 others are undergoing radiotherapy (1) and chemotherapy (1). 3 patients achieved biochemical/clinical control, following surgery and RXT (1), metyrapone (1, postoperatively) and relacorilant (1). 1 patient with rapid tumour regrowth following 3 operations and RXT (last Ki-67=80%) is receiving temozolomide and capecitabine, uncontrolled biochemically on 1000 mg/day ketoconazole. No patient developed PitNET metastasis.
Conclusions: Aggressive corticotroph PitNETs require complex multidisciplinary management and close follow-up to detect relapse, which is frequent. Pituitary apoplexy is frequent and oculomotor palsy portends poor prognosis.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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