Non-epithelial neoplasms of the prostate

Non-epithelial prostatic neoplasms are infrequent and cover a broad array of entities that include both benign and highly aggressive tumours. Because they are very infrequent, there is often limited understanding of them, and the recognition of these entities, when encountered, may pose a diagnostic challenge, owing to histological overlap between them or their rarity. Most lesions in this category are mesenchymal in origin, such as prostatic stromal tumours arising from specialized prostatic stroma, smooth muscle tumours, both benign and malignant, and solitary fibrous tumours. Less commonly occurring tumours include neural, germ cell and melanocytic tumours that may be derived from cells not normally present in the prostate. Some tumours have well-established extraprostatic counterparts and, when encountered, are more commonly extraprostatic/secondary in origin; these include gastrointestinal stromal tumours and most haematopoietic tumours. The majority of tumours are characterized by a spindle cell pattern with significant overlap in morphological features. In this setting, appropriate use of immunohistochemistry and molecular studies are often necessary for accurate diagnosis, prognosis, or prediction for therapy. This review addresses and updates the clinicopathological features of the entire spectrum of non-epithelial tumours with an approach to the histological diagnosis.