Lee Hee-Jeong, Hyun Jin-Soo, Jang Hoe-Soo, Sul Hyoung, Park Sang-Gon
Department of Internal Medicine, Chosun University Hospital, Gwangju 501-717, Republic of Korea.
Oncol Lett. 2014 Nov;8(5):1986-1992. doi: 10.3892/ol.2014.2452. Epub 2014 Aug 18.
Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy with a poor outcome that occurs in adolescents and young adults; <200 cases of DSRCT have been reported. Renin-producing tumors are also rare and cases of extrarenal renin-producing tumors are even rarer. The present study describes the case of a 20-year-old male that was diagnosed with DSRCT and presented with severe hypertension and hypokalemia, as well as metabolic alkalosis. The plasma renin activity (PRA) level was identified to be markedly elevated (normal range in standing and supine positions, 1.3-4.0 ng/ml/h and 0.15-2.33 ng/ml/h, respectively) and the plasma aldosterone level was also increased (normal range in standing and supine positions, 4.0-31.0 ng/dl and 1.0-1.6 ng/dl, respectively). The symptoms of the patient were consistent with the renin-secreting tumor triad, which comprises hypertension, hypokalemia and elevated PRA. Paraneoplastic syndromes must always be considered in cancer patients exhibiting unusual clinical findings, despite their rarity. The current patient was diagnosed with paraneoplastic secondary hypertension due to the presence of disseminated renin-secreting DSRCT. The patient was treated with the VAC/IE regimen (vincristine, adriamycin, cyclophosphamide, ifosfamide and etoposide) for six cycles. Following this treatment, the serum renin and aldosterone levels fell to within the normal range and the patient's blood pressure was normalized without antihypertensive medication. Although an immunohistochemical evaluation of renin was not conducted as the sample size was inadequate, the present study demonstrated that the tumor had produced renin. The biosynthesis of renin was identified by the presence of mRNA that coded for the renin precursor, which was observed in the ascites of the patient. The current study describes, to the best of our knowledge, the first reported case of paraneoplastic secondary hypertension in a patient presenting with a renin-producing DSRCT.
促纤维组织增生性小圆细胞肿瘤（DSRCT）是一种罕见且侵袭性强的恶性肿瘤，预后较差，好发于青少年和青年成人；报道的DSRCT病例不足200例。产肾素肿瘤也很罕见，肾外产肾素肿瘤病例更为罕见。本研究描述了一名20岁男性病例，该患者被诊断为DSRCT，并伴有严重高血压、低钾血症以及代谢性碱中毒。血浆肾素活性（PRA）水平显著升高（站立位和仰卧位正常范围分别为1.3 - 4.0 ng/ml/h和0.15 - 2.33 ng/ml/h），血浆醛固酮水平也升高（站立位和仰卧位正常范围分别为4.0 - 31.0 ng/dl和1.0 - 1.6 ng/dl）。患者的症状与肾素分泌肿瘤三联征相符，该三联征包括高血压、低钾血症和PRA升高。尽管副肿瘤综合征罕见，但对于出现异常临床表现的癌症患者，必须始终予以考虑。该患者因存在播散性分泌肾素的DSRCT而被诊断为副肿瘤性继发性高血压。患者接受了VAC/IE方案（长春新碱、阿霉素、环磷酰胺、异环磷酰胺和依托泊苷）六个周期的治疗。治疗后，血清肾素和醛固酮水平降至正常范围，患者血压在未使用抗高血压药物的情况下恢复正常。尽管由于样本量不足未进行肾素的免疫组化评估，但本研究表明肿瘤产生了肾素。通过在患者腹水中观察到编码肾素前体的mRNA的存在，确定了肾素的生物合成。据我们所知，本研究描述了首例伴有分泌肾素的DSRCT患者的副肿瘤性继发性高血压病例。
