[Aggressive pituitary adenoma and pituitary carcinoma]. / Agresszív hypophysisadenoma és hypophysiscarcinoma.

Anterior pituitary tumours, once considered benign neoplasms, may rarely have aggressive behaviour and can even metastasize. The current guideline of the European Society of Endocrinology defines aggressive pituitary adenomas as radiologically invasive tumours with an unusually rapid growth rate and frequent relapses despite the optimal use of standard therapies. Currently, there is not any single, well-defined pathological marker of malignancy. Pituitary carcinomas are thus clinically defined by the presence of craniospinal or distant metastases, typically developing several years after the first presentation. Histopathology may predict aggressive behaviour if the Ki67 index and mitotic rate are elevated and in case of positive p53 staining. These patients' short- and long-term therapy should be individualized and regularly discussed by a multidisciplinary pituitary team. Besides standard medical treatment administered in maximally tolerated doses, current recommendations suggest repeated surgery and radiotherapy. If this approach fails, the next choice of treatment is chemotherapy with temozolomide. After that, immune checkpoint inhibitors, bevacizumab, and peptide receptor radiotherapy are emerging therapies that should be used on a case-by-case basis. Orv Hetil. 2023; 164(30): 1167-1175.

[Thyrotropin-secreting pituitary adenoma]. / Tirotropintermelo hypophysisadenoma.

Thyrotropin-secreting pituitary tumors are rare causes of hyperthyroidism and account for less than 1% of all pituitary adenomas. The number of reported cases increased over the last few years as a consequence of the routine use of ultrasensitive immunometric assays for measuring thyrotropin levels. In the clinical practice, thyrotropin secreting adenomas must be considered in case of inappropriately normal to elevated thyrotropin in the presence of elevated free serum thyroid hormone levels. The authors present the case history of a middle aged female patient, who suffered from hyperthyreodism caused by a thyrotropin-secreting pituitary macroadenoma. After transient thyreostatic treatment, radical neurosurgical removal of the tumor was performed. The pituitary surgery was effective in restoring the patient's euthyreodism. The postoperative pituitary function remained intact. During follow-up, the recurrence of the disease was not detected. In our case report, the difficulties in the differential diagnoses are also discussed. Orv Hetil. 2020; 161(12): 474-478.

[Genetic factors in hypopituitarism. The role of transcription factors in pituitary hormone deficiency]. / Genetikai tényezok a hypopituitarismus kialakulásában. A transzkripciós faktorok szerepe az agyalapimirigy-elégtelenség hátterében.

Developmental disorders affecting the hypothalamic-pituitary system can result in pituitary hormone deficiency showing a diverse clinical presentation. A significant majority of these disorders are closely linked to defects in transcription factor genes which play a major role in pituitary development. Those affecting the early phase of organogenesis typically lead to complex conditions affecting the pituitary as well as structures in the central nervous system. Transcription factors involved in the late phase can result in combined but rarely isolated pituitary hormone deficiency without extra-pituitary manifestation. Identifying the defects in these pituitary transcription factor genes may provide a useful tool in predicting disease progression as well as screening family members. Several pituitary transcription factors can be detected in the adult gland as well which is strongly emphasized in the World Health Organization's most recent guideline for pituitary tumor classification. Our review summarizes the current essential knowledge relevant for clinical endocrinologists. Orv Hetil. 2018; 159(7): 278-284.