Schöffski Patrick, Sciot Raf, Debiec-Rychter Maria, Van Ongeval Johan, D'Hoore André, Merckx Luc, Joniau Steven
Department of General Medical Oncology, University Hospitals Leuven, Leuven Cancer Institute, Leuven, Belgium.
Department of Pathology, University Hospitals Leuven, Leuven Cancer Institute, Leuven, Belgium.
Case Rep Oncol. 2019 Feb 20;12(1):183-191. doi: 10.1159/000496686. eCollection 2019 Jan-Apr.
We report a very uncommon case of a primary, non-metastatic gastrointestinal stromal tumor (GIST) arising in the prostate gland in a 60-year-old patient. The morphology and immunohistochemical profile of the disease resembled GIST of gastrointestinal origin, and the molecular driver of this malignancy was a double mutation in exons 11 and 13 of the KIT gene. The tumor was proliferating slowly, did respond to neoadjuvant therapy with the KIT-inhibiting agent imatinib and was cured by radical, retro-pubic prostatectomy followed by adjuvant imatinib treatment. We postulate that primary GIST tumors of the prostate can arise from prostatic interstitial cells, which are the pacemakers of smooth muscle contractility in the gland, and possibly share a common precursor with typical GIST and the interstitial cells of Cajal in the gastrointestinal tract.
我们报告了一例非常罕见的病例，一名60岁患者的前列腺出现原发性、非转移性胃肠道间质瘤（GIST）。该疾病的形态和免疫组化特征类似于胃肠道起源的GIST，这种恶性肿瘤的分子驱动因素是KIT基因第11和13外显子的双重突变。肿瘤生长缓慢，对KIT抑制剂伊马替尼的新辅助治疗有反应，并通过根治性耻骨后前列腺切除术及辅助伊马替尼治疗得以治愈。我们推测前列腺原发性GIST肿瘤可能起源于前列腺间质细胞，这些细胞是前列腺平滑肌收缩的起搏细胞，并且可能与典型GIST以及胃肠道的 Cajal间质细胞有共同的前体。
