Aggressive Pituitary Tumors and Pituitary Carcinomas: A Narrative Review of Clinical Features and Management.

Abstract

Background: Aggressive pituitary tumors (APTs) are commonly defined as invasive neoplasms refractory to treatment with early recurrences, while pituitary carcinomas (PCs) represent a subset of APTs that have developed metastases. However, the diagnostic criteria for APTs remain poorly standardized, and many different classifications of these tumors currently exist. Early and accurate identification of APTs is essential, as these tumors frequently recur and are associated with significant therapeutic challenges.

Summary: In this review we provide an overview of different classifications of APTs and summarize the key characteristics and treatment methods for these tumors. Proposed criteria of APTs include histopathological features (such as high Ki-67 index, increased mitotic activity, aggressive histopathological subtypes), genetic alterations (most commonly involving TP53 and ATRX), radiological findings (like invasive behavior, multilobulated morphology, microcystic changes, low signal intensity on T2-weighted magnetic resonance imaging), and clinical characteristics (including rapid growth, early recurrences, resistance to standard therapies). Treatment of APTs typically requires a multimodal approach. Most patients undergo at least one surgical resection, followed by radiotherapy, and in selected cases, pharmacological treatment with temozolomide. Other drugs like immunotherapeutics or cytotoxic agents are also used. In this review we provide an overview of different classifications of APTs and summarize the key characteristics and treatment methods for these tumors.

Key messages: Early identification of APTs and PCs may be achieved by integrating histopathological, genetic, radiological, and clinical features.