The challenging  management of aggressive, giant silent corticotroph PiTNET in a young male patient

A. Boguslawska*^a (Dr), A. Gilis-Januszewska1^a (Prof), M. Godlewska^a (Dr), L. Kluczyński^a (Dr), E. Rzepka^a (Dr), M. Minasyan^a (Dr), A. Hubalewska-Dydejczyk^a (Prof)

^a Chair and Department of Endocrinology, Jagiellonian University, Medical College, Cracow, Poland, Cracow, POLAND

* boguslawskaania@gmail.com

Silent corticotroph pituitary neuroendocrine tumours (PiTNETs) are a subtype of nonfunctioning PiTNETs, that present positive immunostaining for adrenocorticotropin (ACTH) and/or show the expression of the transcription factor T-PIT without clinical signs of hypercortisolemia.

We present a 33-year-old male with an aggressive course of silent corticotroph PitNET. The patient was admitted to Emergency Department due to severe headaches and vomiting. Headaches (8-9/10 using numbering rating score (NRS)) and worsening vision loss were present one year before the surgery. In computer tomography, a sellar tumour mass (39x33x55 mm) was found. Additionally, features of cerebral oedema were described. The emergency external ventricular drainage was performed due to obstructive hydrocephalus, two days later, debulking transsphenoidal surgery (TSS) was performed. Histopathology results showed silent adenoma subtype 1 (densely granulated), Ki67<1%. However, 3 months later, magnetic resonance imaging (MRI) showed progression of PitNET (40x39x30 mm) with increasing hydrocephalus. Subsequently, a second TSS was performed. Biochemically, persistent multiple pituitary hormone deficiencies and diabetes insipidus were diagnosed. Clinically, severe headaches (9-10/10 using NRS) without improvement after analgesic and worsening vision loss were observed. The patient was consulted by a multidisciplinary pituitary tumour board and radiotherapy was planned. Pasireotide (10mg) monthly and 0.5 mg of cabergoline weekly were scheduled, however, due to the rapid progression of the tumour and the compression of the optic chiasm, emergency TSS (05.2021) with the decompression of the optic nerves was performed. After surgery, chemotherapy with temozolomide was introduced. After the first cycle, an adjuvant stereotactic fractionated was performed. Temozolomide at the dose of 200mg/m² for 5 days every 4 weeks was continued. Severe headaches (9-10/10 using NRS) without improvement after analgesic were still present. Pasireotide (increasing dose from 10 to 40 mg/month) was reimplemented, a decrease of headaches from (initially 9-10 to none /10 using NRS) has been observed. In last MRI, after 5 cycles of temozolomide, and during pasireotide and cabergoline treatment, regression of the pituitary tumour (current measurements: 20x30x29 mm) was observed. Additionally, the patient is in a very good general condition and reports no headaches.

The author has declared no conflict of interest.