Department of Pathology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105, AZ Amsterdam, The Netherlands.
BMC Endocr Disord. 2013 Nov 25;13:55. doi: 10.1186/1472-6823-13-55.
Around 10 per cent of catecholamine-secreting tumours can be found outside the adrenal medulla (paraganglioma). We report a case of a functional sporadic paraganglioma that was localized lateral to the prostate without causing lower urinary tract symptoms.
A 76-year old male with an extensive history of cardiovascular disease suffered from hypertension and an unexplained hypochromic microcytic anaemia for years before the coincidental discovery of a 2.5 × 3.5 cm periprostatic mass upon abdominal contrast-enhanced CT scanning. Transrectal biopsies revealed a paraganglioma. The urinary levels of the catecholamine metabolites were found increased. The paraganglioma showed uptake of iodine-123-metaiodobenzylguanidine by SPECT scanning, indicating a solitary lesion. Successful preperitoneal endoscopic resection of the tumour was performed, which resulted in a decrease in blood pressure and a normalization of the urinary catecholamine metabolites. None of the to date known genetic mutations that have been shown to relate to the existence of paragangliomas were identified in the current case.
An intra- or periprostatic localization of a paraganglioma is very rare. We reviewed the literature and found 6 other cases. Three of the described cases presented with lower urinary tract symptoms. In these three patients, the tumour had a size of 4 cm or larger and in 67 per cent of these cases the paragangliomas were situated within the prostate. The periprostatic region might be considered as a possible location for paragangliomas, especially in the presence of lower urinary tract symptoms even though they were absent in the current case.
约 10%的儿茶酚胺分泌肿瘤可发生在肾上腺髓质以外(副神经节瘤)。我们报告一例功能性散发性副神经节瘤,定位于前列腺外侧,无下尿路症状。
一名 76 岁男性,有广泛的心血管病史,多年来患有高血压和不明原因的低色素小细胞性贫血,在腹部增强 CT 扫描时偶然发现前列腺旁 2.5×3.5cm 的肿块。直肠活检显示为副神经节瘤。尿儿茶酚胺代谢物水平升高。SPECT 扫描显示副神经节瘤摄取碘-123-间碘苄胍,提示为单发病变。成功地进行了腹膜前内镜肿瘤切除术,使血压下降,尿儿茶酚胺代谢物正常化。目前的病例中未发现与副神经节瘤存在相关的已知基因突变。
前列腺内或前列腺周围的副神经节瘤非常罕见。我们回顾了文献,发现了其他 6 个病例。描述的 3 个病例均有下尿路症状。这 3 个患者的肿瘤大小为 4cm 或更大,在这些患者中,67%的副神经节瘤位于前列腺内。在存在下尿路症状的情况下,特别是在没有下尿路症状的情况下,前列腺周围区域可能被认为是副神经节瘤的可能部位。