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- Burman, Pia, et al.
(författare)
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Aggressive Pituitary Tumors and Pituitary Carcinomas : From Pathology to Treatment
- 2023
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Ingår i: Journal of Clinical Endocrinology and Metabolism. - : Endocrine Society. - 0021-972X .- 1945-7197. ; 108:7, s. 1585-1601
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Forskningsöversikt (refereegranskat)abstract
- Aggressive pituitary tumors (APTs) and pituitary carcinomas (PCs) are heterogeneous with regard to clinical presentation, proliferative markers, clinical course, and response to therapy. Half of them show an aggressive course only many years after the first apparently benign presentation. APTs and PCs share several properties, but a Ki67 index greater than or equal to 10% and extensive p53 expression are more prevalent in PCs. Mutations in TP53 and ATRX are the most common genetic alterations; their detection might be of value for early identification of aggressiveness. Treatment requires a multimodal approach including surgery, radiotherapy, and drugs. Temozolomide is the recommended first-line chemotherapy, with response rates of about 40%. Immune checkpoint inhibitors have emerged as second-line treatment in PCs, with currently no evidence for a superior effect of dual therapy compared to monotherapy with PD-1 blockers. Bevacizumab has resulted in partial response (PR) in few patients; tyrosine kinase inhibitors and everolimus have generally not been useful. The effect of peptide receptor radionuclide therapy is limited as well. Management of APT/PC is challenging and should be discussed within an expert team with consideration of clinical and pathological findings, age, and general condition of the patient. Considering that APT/PCs are rare, new therapies should preferably be evaluated in shared standardized protocols. Prognostic and predictive markers to guide treatment decisions are needed and are the scope of ongoing research.
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- Trouillas, Jacqueline, et al.
(författare)
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Initial pathology in aggressive pituitary tumours and carcinomas : 2b or not 2b?-that is the question
- 2023
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Ingår i: European Journal of Endocrinology. - : Oxford University Press (OUP). - 1479-683X .- 0804-4643. ; 188:4, s. 5-8
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Tidskriftsartikel (refereegranskat)abstract
- From a cohort of 171 patients comprising 121 aggressive pituitary tumours (APT) and 50 pituitary carcinomas (PC), the clinicopathological or five-tiered classification based on tumour invasion and proliferation evaluated by at least 2 proliferative markers over the cut-offs (Ki-67 ≥3% or ≥10%, p53 positive or expressed in %, mitotic count >2%), could be applied on 43 tumours: 20 PC and 23 APT. At the initial surgery, 29/43 tumours (67.4%) were grade 2b (invasive and proliferative) of which 44.8% developed metastases during follow-up (PC, grade 3). Out of these 29 tumours, 55.1% had a Ki-67 ≥10%, and were classified grade 2b* (invasive and highly proliferative). There was one tumour grade 1b* (non-invasive and highly proliferative) which metastazed. Out of the 43 tumours, 30.2 % were grade 2a (invasive and non-proliferative). The sensitivity and the specificity of grade 2b for the diagnosis of APT at the initial surgery, were 68% and 90% respectively. The comparison of the high percentage (67.4%) of grade 2b tumours in this selected cohort of APT/PC with the low percentage (8.8%) in a surgical cohort of unselected tumours shows that the initial pathological diagnosis of grade 2b tumour may be considered, in the clinic, as representing a diagnosis of APT. In addition, a significant subgroup of tumours, which will develop metastases supports the proposal that an aggressive grade 2b tumour is “a tumour with malignant potential” or “a malignant tumour without metastases”. So, the clinician may take into account the pathological diagnosis, at the initial surgery, to propose a strict follow-up and to consider earlier use of radiotherapy and/or of temozolomide in the presence of tumours with aggressive behaviour.
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