THU099 The Challenging Management Of Silent Corticotroph PitNET—From Very Aggressive To Mild Course Of Disease During Long Follow Up

Disclosure: A. Bogusławska: None. &. Kluczyński: None. A. Hubalewska-Dydejczyk: None. A. Gilis-Januszewska: None. We present a case series of silent corticotroph PitNETs with various clinical outcomes. Case 1. A 33-year-old male admitted to Emergency Department (ED) due to severe headaches and vomiting- giant PitNET (39x33x55 mm) was visualized-debulking transsphenoidal surgery (TSS) was performed. Histopathology results showed silent adenoma subtype 1 (densely granulated), Ki67<1%. Due to rapid progression of PitNET- patient underwent two emergency TSS and stereotactic fractionated radiotherapy. Treatment with temozolomide, pasireotide, cabergoline, radiotherapy was introduced. After 18 months of combined therapy stable disease was observed. Implementation of pasireotide resulted in spectacular decrease of headaches (initially 9-10 to none /10 using NRS). Case 2. A 31-year-old male presented in our clinic due to headaches and bitemporal hemianopsia. In MRI PitNET was found (22x19x23mm) and active Cushing disease (CD) was diagnosed. TSS was performed in 2013. Histopathology results showed corticotroph PitNET with Crook cells. Postoperatively, PitNET progression (17x26x13mm) was observed with no overt CD. In 2022, due to worsening of headaches and vision loss, second TSS was performed. Histopathologists described corticotroph tumor with Crook cells with ATRX mutation of uncertain significance. Pasireotide implementation is planned. Case 3. A 47-yeard-old female was admitted to ED due to severe headache and nausea. In MRI pituitary tumor (20x17x20mm) with local invasion was found. The patient was operated and histopathology showed densely granulated corticotroph PitNET; ACTH(+), GH(+), Ki-67% was 3%, p53<5%, with single Crook cells. In follow-up, progression of PitNET was noted (31x32x30 mm) and the patient underwent proton therapy. Case 4. A 56-yeard old female after three TSS (2012,2017,2018) and craniotomy (2020) due to aggressive corticotroph PitNET with Ki67 <1%. In 2009 a pituitary tumour (9x10x13 mm) was found. Before last craniotomy, the tumour was 24x24x28mm. After neurosurgery, paresis of right III nerve and left hemiparesis occurred. Pasireotide implementation is planned. Case 5. A 70-year old female presented in 2015 with severe headaches/nausea. In MR PitNET (32x24x31mm) was found. The patient underwent TSS and histopathology showed corticotroph PitNET with Ki67<1%. Due to PitNET progression, second TSS and fractionated radiotherapy was performed (2017) however in last MRI (2022), tumor relapse was noted. Case 6/7: A 46-year-old and 50 year-old females were operated due to local symptoms of PitNET. Histopathology revealed silent corticotroph PitNET. In follow-up MRI after one year showed no progression of pituitary tumor. The management of silent corticotroph PitNETs is very challenging and individualized approach is required. Further multicenter studies to understand the pathophysiology and the course of silent corticotroph PitNETs are needed. Presentation: Thursday, June 15, 2023