肾上腺区域和肾周Castleman病5例报道

目的分析5例肾周和肾上腺区域Castleman病(Castleman disease,CD)的临床特点,提高对CD诊治的认识。方法结合文献复习,回顾分析本院2006年5月至2014年6月5例肾周和肾上腺区域CD的临床表现、影像学特点、治疗和预后。结果本组5例,男3例,女2例,中位年龄54岁(33～58岁)。CD病灶位于肾上腺区域3例,肾周2例。其中体检发现2例,因腰腹痛就诊2例,因发热和肌痛就诊1例。肿块最大径2.8～9.6cm,平均5.3cm。术前诊断为肾上腺良性肿瘤1例,肾上腺或肾恶性肿瘤2例,淋巴瘤1例,肾周Castleman病1例。影像学表现多无特异性,超声检查CD病灶多表现为界限清晰的低回声肿,较大的肿瘤中心部位可见点状钙化;CT平扫为均质肿块,可伴有微小星点状钙化,增强扫描呈明显强化的均质富血供肿块;MRI检查肿块呈T_1WI呈低或等信号,T_2WI呈均质等或高信号,增强扫描可明显强化。体积较大的肿块在CT和MRI动态增强扫描时,表现为从周边强化开始,逐渐向中间弥散的均质肿块。实验室检查除1例有发热等全身症状者C反应蛋白和血沉升高外,其余4例均未见异常。5例患者中,1例行右肾上腺及右肾切除,2例行腹腔镜左肾上腺及后腹膜淋巴结切除,1例行右肾根治+腹膜后淋巴结切除,1例行左肾门旁肿块切除。术后病理均为Castleman病,其中透明血管型4例,术后中位随访时间59.5个月(6～103个月)均未复发;浆细胞型1例,术后54个月腹膜后复发并骨转移。根据病理、临床特点及影像学临床最终诊断为局灶型Castleman病(LCD)4例,多中心型Castleman病(MCD)1例。结论手术切除是肾周和肾上腺区域LCD的首选治疗,MCD手术效果不佳。因病灶部位的特殊性,以及临床和影像学表现的非特异性,该区域的CD易误诊为肾和肾上腺恶性肿瘤,导致不必要的脏器切除。对于该区域的均质、血供丰富,特别是伴有微小点状钙化、从周边开始弥散增强的肿块,均应怀疑CD。术中活检再次评估,或可避免切除范围的扩大。

Castleman's Disease in the Retroperitoneal Space Mimicking Adrenal or Renal Neoplasm:Report of 5 Case and Literature Review

Objective 5 cases of pararenal and adrenal region Castleman disease (CD )were reviewed. Methods The clinical characteristic,treatment,and outcome of 5 patients with pararenal and adrenal region CD from May 2006 to June 2014 were analyzed,and literature in recent years were reviewed. Results 2 females and 3 males were diagnosed as pararenal or adrenal region CD were included. The median age was 54 years old (33~58 years). The lesion was located in adrenal region in 3 cases,in pararenal region in 2 cases. In these pa-tients,2 cases were asymptomatic and diagnosed as adrenal or renal tuomor during a health check,2 cases with chief complaint of abdominal and back discomfort for the past 2~6 months,and the another one had been suf-fered from a fever since four months ago. The median diameter of mass was 5. 3cm (2. 8~9. 6cm). Except for one patient was made a definite diagnosis as pararenal CD,all the other 4 cases were misdiagnosed as adrenal or renal tumor. Radiological finding were not specific. Ultrasonography usually demonstrated a well-circumscribed hypoechoic and homogenous mass with central areas of sharp acoustic shadowing due to calcification. Pre-con-trast CT scan show a solid,homogenous mass with star-shaped microcalcifications,and post-contrast CT re-vealed a well-defined hypervascularity homogenous enhancing mass;MRI characteristics of lesion were hypo-dense on the T1 WI and hyperdense on the T2 WI. When the contrast or Gadolinium injection in CT or MRI,an enhancement appear in periphery to become diffuse to the centre. The laboratory anomalies include elevated e-rythrocyte sedimentation rate and C-reactive protein concentration was only found in 1 case. Adrenalectomy and nephrectomy were done in 1 case,laparoscopic adrenalectomy in 2 cases,radical nephrectomy and retroperitone-al mass resection in 1 case,and pararenal mass resection in 1 case. All these patients were diagnosed as Castle-man disease by pathomorphological examination,including 4 hyaline-vascular subtype and 1 plasma cell sub-type. There were no recurrence after median 59. 5 months (6-103 months)follow-up in 4 cases which were diagnosed as localized Castleman disease (LCD ),1 case was diagnosed as multicentric Castleman disease (MCD)which suffered from retroperitoneal recurrence and bone metastases after 54 months. Conclutions Re-sective surgery is the gold standard of treatment of LCD when resection is complete and en-bloc. The diagnosis of pararenal and adrenal region CD is difficult because it had no specific clinical and radiological presentation, and the mass usually adherences to adjacent organ. Misdiagnosis could cause unnecessary resection of adjacent organ. Pararenal and adrenal region mass which is homogenous and harbor calcifications and hypervascularisation associated with typical flush,the diagnosis of CD should be suspected. Reevaluate during surgery by biopsy is conducive to avoid unnecessary resection in case of CD suspicion.