Endocrine Abstracts

JOINT1667

Introduction: Treatment of aggressive corticotropinomas is a challenge due to the frequent invasive growth of the tumor which complicates radical surgery and can increase the risk of recurrence, thus together with tumor resistance to radiotherapy leads to persistence of the disease.

Clinical case: In August 2023 a Cushing disease was diagnosed in a woman of 48 years of age and according to MRI in the cavity of the sella turcica, in the suprasellar cistern, in the left cavernous sinus (Knosp IV), in the sphenoid sinuses and in the cells of the ethmoid labyrinth on the left was determined tumor tissue measuring 28x25x39 mm. Transsphenoidal adenomectomy was performed (morphology: pituitary tumor of solid structure composed of cells with amphophilic cytoplasm, 1 pathological mitosis; immunohistochemistry: expression of synaptophysin, CAM 5.2 and ACTH, Ki-67-13.4%, no expression of somatostatin receptors subtype 5). Due to clinical manifestation of adrenal insufficiency in the postoperative period was initiated hydrocortisone therapy, which was cancelled in February 2024 due to the persistence of endogenous hypercortisolism and MRI noted tumor tissue in the right part of the adenohypophysis measuring 14.5x13x12 mm and in the left cavernous sinus measuring 17x16x33 mm. In March 2024 patient was treated by repeated transsphenoidal adenomectomy. A month later due to manifestation of neuro-ophthalmologic symptoms and the spread of tumor tissue measuring 26x31x41 mm into the orbit according to MRI, another transnasal transsphenoidal adenomectomy was performed (morphology: fibrous tissue with deformed tumor cells with hyperchromic nuclei; immunohistochemistry: ACTH expression in 100% of cells, CAM 5.2 in 80% of cells, Ki-67-10%, high reaction with VEGF and PD-L1 expression in 5% of cells, no expression of LH, FSH, prolactin and somatostatin receptors subtypes 2A and 5). In June-August 2024 the patient underwent stereotactic radiation therapy (TrueBeam, 55.8 Grey). In September 2024 was confirmed persistence of the disease and MRI described tumor tissue in the left cavernous sinus measuring 10x14x14 mm, endo-suprasellar on the right measuring 16x14x14 mm and along the upper, right and lower contours of the left optic nerve. The patient was consulted by a neurosurgeon, dynamic observation was recommended.

Conclusion: The clinical case demonstrates the problems in treatment of aggressive corticotropinomas. Anti-VEGF therapy and immune checkpoint inhibitors immunotherapy in some studies has shown effectiveness in the treatment of aggressive corticotropinomas. Thus, the expression of VEGF and PD-L1 in tumor cells detected during the immunohistochemistry in this patient may provide alternative treatment options for achieving remission.