A clinical overview of pheochromocytomas/paragangliomas and carcinoid tumors

Pheochromocytomas/paragangliomas are rare tumors; most are sporadic. Biochemical proof of disease is better with measurement of plasma metanephrines and less cumbersome than determinations in urine; its implementation is expanding. Anatomical imaging with computed tomography or magnetic resonance imaging should be followed by functional (nuclear medicine) imaging: chromaffin tumor-specific methods are preferred. Treatment is surgical; for nonoperable disease other options are available. Overall 5-year survival is 50%. Carcinoid tumors derive from serotonin-producing enterochromaffin cells in the fore-, mid- or hindgut. Biochemical screening (and follow-up) is done with measurements of 5-hydroxyindoloacetic acid in urine. For most carcinoids, functional imaging is better than other modalities in localizing primary tumors. Surgery is the treatment of choice; nonresectable tumors are treated with somatostatin analogs or chemotherapy. Overall 5-year survival for patients with carcinoids is 67%.     

多层螺旋CT及多平面重组诊断嗜铬细胞瘤的价值

随着多层螺旋CT及三维后处理技术在临床的应用,对肾上腺嗜铬细胞瘤(adrenal pheochromocytomas)的诊断及鉴别诊断有很大的进步。回顾性分析我院自2000年6月~2006年6月期间收集的经手术及病理证实的14例嗜铬细胞瘤患者,探讨多层螺旋CT及多平面重组对嗜铬细胞瘤的诊断价值。1材料与方法1.1一般材料本组14例中,男性11例,女性3例,年龄22~55岁,平均年龄38.5岁。临床表现有腰痛头晕、头痛、心悸、多汗,有阵发性或持续性高血压症状者11例,发作时血压均在190/120~220/170mmHg,无明显临床症状,体检时发现3例。实验室检查24h尿中香草扁桃酸(VMA)1.…     

PET imaging of adrenal cortical tumors with the 11beta-hydroxylase tracer 11C-metomidate.

The purpose of the study was to evaluate PET with the tracer 11C-metomidate as a method to identify adrenal cortical lesions. METHODS: PET with 11C-metomidate was performed in 15 patients with unilateral adrenal mass confirmed by CT. All patients subsequently underwent surgery, except 2 who underwent biopsy only. The lesions were histopathologically examined and diagnosed as adrenal cortical adenoma (n = 6; 3 nonfunctioning), adrenocortical carcinoma (n = 2), and nodular hyperplasia (n = 1). The remaining were noncortical lesions, including 1 pheochromocytoma, 1 myelolipoma, 2 adrenal cysts, and 2 metastases. RESULTS: All cortical lesions were easily identified because of exceedingly high uptake of 11C-metomidate, whereas the noncortical lesions showed very low uptake. High uptake was also seen in normal adrenal glands and in the stomach. The uptake was intermediate in the liver and low in other abdominal organs. Images obtained immediately after tracer injection displayed high uptake in the renal cortex and spleen. The tracer uptake in the cortical lesions increased throughout the examination. For quantitative evaluation of tracer binding in individual lesions, a model with the splenic radioactivity concentration assigned to represent nonspecific uptake was applied. Values derived with this method, however, did show the same specificity as the simpler standardized uptake value concept, with similar difference observed for cortical versus noncortical lesions. CONCLUSION: PET with 11C-metomidate has the potential to be an attractive method for the characterization of adrenal masses with the ability to discriminate lesions of adrenal cortical origin from noncortical lesions.     

原发性肾上腺皮质癌的CT诊断

目的：总结肾上腺皮质癌的ＣＴ表现。材料与方法：分析２０例经手术病理证实的临床和ＣＴ资料。结果：功能性肿瘤占３０％。１例双侧性肿瘤。肿瘤大小范围６～１５ｃｍ，６个＜６ｃｍ。ＣＴ表现为肿瘤中心呈低密度的出血与坏死（１６例），钙化（４例），不规则增强（１５例），低密度的肿瘤周围环绕实质性的增强环（４例），轮廓不规则（１１例），边缘模糊（９例）。肿瘤可以直接侵犯邻近器官或转移到肝、淋巴结、胰腺、小肠和骨。结论：ＣＴ能提示肾上腺皮质癌的恶性性质。     

肾上腺肿瘤的影像学诊断

肾上腺（suprarenal gland）肿瘤是指发生在肾上腺皮质、髓质及间质的良、恶性肿瘤以及转移瘤和淋巴瘤。关于肾上腺肿瘤的发生率，国内外尚无统一报道。肾上腺区肿瘤组织来源复杂，临床和影像学表现多样。随着医学影像技术的发展，对肾上腺肿瘤诊断的准确率有了明显的提高，尤其是高分辨CT、MRI等检查，不但能确定肿瘤的有无，还能了解肿块的性质及血供情况，肿块与周围组织的关系等，为临床治疗提供了可靠的影像依据。     

Magnetic resonance imaging of adrenal cortical carcinoma

Five cases of adrenal cortical carcinoma examined with magnetic resonance (MR) are presented. Clinical histories, computed tomographic (CT) scans, and final pathologic findings were reviewed in each case. All masses were hypointense compared to the liver on T1-weighted images and became hyperintense compared to the liver on T2-weighted images. Signal intensity of adrenal masses, fat, and liver were measured. Adrenal/liver and adrenal/fat signal intensity ratios were then calculated. All the masses were readily identified with MR. The MR also demonstrated displacement or invasion of adjacent organs, as well as liver metastases. The inferior vena cava was also identified in each case. Even though there were no consistent MR findings to diagnose adrenal cortical carcinomas accurately, superior blood vessel identification and multiplanar capabilities may make MR the imaging modality of choice in evaluating the extent of disease and in planning surgical excision.     

Radiation therapy of adrenal cortical carcinoma.

The role of radiation therapy in the management of adrenal cortical carcinoma has had little documentation in the literature. Fourteen patients with this malignancy were given 18 courses of palliative, preoperative or postoperative irradiation. The clinical results are presented. Significant palliation was obtained in all patients along with occasional long term local control of unresectable lesions.     

肾上腺肿瘤的CT和MRI诊断

目的：探讨肾上腺肿瘤的CT和MRI影像学特点。方法：回顾分析经临床、手术病理证实的40例肾上腺肿瘤的CT资料和其中10例。MRI资料，观察其CT和MRI征象，提出诊断和鉴别诊断要点。结果：40例中，腺瘤16例，其中Cushing腺瘤8例，直径2．0～3．0cm的密度均匀的肿块，其信号与肝脏信号类似；Conn腺瘤6例，直径1．0～2．0cm的均一水样密度肿块；2例腺瘤无症状。皮质腺癌7例，呈巨大不规则的不均匀密度或信号肿块。嗜铬细胞瘤7例，较大的不均匀密度或信号肿块，实体部分明显强化。转移瘤8例，双侧或单侧肿块，T2WI明显高信号。髓性脂肪瘤1例，不均质肿块内有显著的脂肪组织密度或信号。淋巴管囊肿1例，不强化均一水样密度或信号的肿块。结论：肾上腺肿瘤CT和MRI表现有一定的特征，结合临床及生化检查，能对其做出定性诊断。