Primary Gastrointestinal Stromal Tumor of the Prostate: Unexpected Guest.

Primary mesenchymal lesions of the prostate are exceptionally rare. They comprise 1% of all prostatic neoplasms. Despite its rare location, the diagnosis of primary gastrointestinal stromal tumors (GISTs) of the prostate gland should never be missed. Such a diagnosis can be made after the rolling out of direct extension from adjacent organs, especially the rectum. GIST diagnosis has a clinical impact on patient treatment and clinical outcomes. They harbor a certain KIT activation mutation that responds to pharmacologic therapy inhibitors. The objective of the current study was to provide a thorough review of GIST arising primarily in the prostate gland along with a comprehensive study of GIST pathogenesis, histologic morphology, immunohistochemistry, and molecular studies' findings, and their importance in differentiating GIST from other prostate mesenchymal tumors. This will emphasize the role of careful spindle cell lesion diagnosis in the prostate gland that can influence the prognostic stratification of clinical management, future follow-up, and disease outcome. Thirteen cases were collected after an extensive and detailed review of the English literature through PubMed, Medknow, Google Scholar, as well as personal experience. The anatomic location of this lesion plays a significant role in the differential diagnosis. It is difficult to establish the accurate primary origin of GIST on core needle tissue biopsy. Thus, clinical, and radiological examinations play a crucial role in rolling out the possibility of rectal GIST secondarily invading and involving the prostate gland. To conclude, primary prostatic GIST is a rare diagnosis. Extraintestinal, particularly rectal, GIST can clinically and radiologically mimic the impression of the prostatic lesion. Before diagnosing primary prostatic spindle cell lesions, such as solitary fibrous tumor (SFT), inflammatory myofibroblastic tumor (IMT), leiomyoma, leiomyosarcoma, or prostatic stromal tumors, one should include CD117/c-Kit in the workup of a prostatic spindle cell lesion. GIST has distinct pathogenesis, and its diagnosis can have a clinical impact on the patient's management plan and clinical outcome.