粘液性肾上腺皮脂腺瘤——中国的第一例报道

Myxoid adrenal cortical adenoma is a rare tumor and till now only 9 cases have been presented in the world. We here report another case of myxoid adenoma of the adrenal gland in a 45-year-old Chinese man who was admitted to hospital because of the right adrenal mass and mild hypertension. At surgery, the mass was well-circumscribed, measured 3.3 cm in diameter. Light-microscopic findings showed most of the tumor region with myxoid stroma, and the tumor cells were benign-looking. Immunohistochemical study showed the tumor had the positivity for vimentin, synaptophysin, neuron specific endolase but negative with cytokeratin and epithelial membrane antigen. Moreover, it was negative with alpha-inhibin that is not in accordance with those reported. There was no finding corresponding to malignancy.     

Myxoid adrenal cortical adenoma—the first case reported in China

Myxoid neoplasms of the adrenal cortex are extremely rare[1]. To our knowledge, there were only 19 cases that have been reported thus far, including 10 carcinomas and 9 adenomas. As for the later, it was typical to notice the lack of atypia, more or less the myxoid component and heterogeneous growth pattern[2]. We here present another case of myxoid adrenal cortical adenoma that was the first case reported in China. Histologically, it was alike those reported that the benign-looking tumor cel…     

Prolonged survival following six pulmonary resections for metastatic adrenal cortical carcinoma: A case report

A case is presented of prolonged survival following multiple thoracotomies performed for metastatic adrenal cortical carcinoma that was refractory to other treatment modalities. In selected patients with isolated metastases to the lungs, repeated surgical resection may offer the only possibility for cure or extended palliation.     

667例肾上腺偶发瘤的临床分析

  目的  分析肾上腺偶发瘤的临床特点,探讨其诊治经验。  方法  回顾性分析2001年1月至2013年1月667例中山大学附属肿瘤医院和附属第一医院肾上腺肿瘤患者的临床资料。  结果  667例患者中病理确诊的肾上腺偶发瘤为511例。最常见的病理类型为皮质腺瘤240例,占47%（240/511）;嗜铬细胞瘤90例,占18%（90/511）;皮质腺癌41例,占8%（41/511）。511例患者中肿瘤直径≤4 cm为266例,良性占98%（260/266）,183例行腹腔镜下肾上腺肿瘤切除术;肿瘤直径>6 cm为245例,恶性占37%（91/ 245）,162例行开放性肾上腺肿瘤切除术。  结论  诊断肾上腺偶发瘤时肿瘤直径4 cm为参考临界值。腹腔镜肾上腺切除术是直径≤4 cm的肾上腺偶发瘤患者的首选治疗。      

肾上腺皮质腺癌20例临床分析

目的分析原发性肾上腺皮质腺癌的治疗及预后。方法回顾1965年至1998年间20例肾上腺皮质腺癌术后±局部放疗或化疗的存活情况。结果18例行根治性手术,2例行姑息性手术;术后6例接受放疗,3例接受化疗。随访至1998年12月,仅1例存28年。结论肾上腺皮质腺癌应早期手术。术后辅以米托坦治疗有望改善存活。     

原发性无功能肾上腺皮质癌的诊断治疗

目的：探讨原发性无功能肾上腺皮质癌的诊断治疗。方法：回顾性分析16例经影像学检查、手术及病理证实的原发性无功能肾上腺皮质癌临床资料。结果：本组16例患者,男6例,女10例,年龄平均47岁,肿瘤直径平均9.5cm。16例患者相关内分泌检查均提示无异常。B超显示肿瘤内回声和形态多不规则,MRI,CT总体表现为肿瘤形状不规则,边界不清晰,肿瘤内部密度不均匀,增强后周边明显强化。临床分期Ⅰ期1例;Ⅱ期6例;Ⅲ期6例;Ⅳ期3例。16例患者均行手术治疗。术后病理均证实为肾上腺皮质腺癌。术后随访4例至今存活。结论：B超、CT、MRI可帮助诊断原发性无功能肾上腺皮质癌。积极手术是确切的治疗手段。50岁以上人群应以B超等影像检查监测肾上腺,以便早期发现。     

原发性无功能肾上腺皮质癌的诊断治疗

目的:探讨原发性无功能肾上腺皮质癌的诊断治疗。方法:回顾性分析16例经影像学检查、手术及病理证实的原发性无功能肾上腺皮质癌临床资料。结果:本组16例患者,男6例,女10例,年龄平均47岁,肿瘤直径平均9.5cm。16例患者相关内分泌检查均提示无异常。B超显示肿瘤内回声和形态多不规则,MRI,CT总体表现为肿瘤形状不规则,边界不清晰,肿瘤内部密度不均匀,增强后周边明显强化。临床分期Ⅰ期1例;Ⅱ期6例;Ⅲ期6例;Ⅳ期3例。16例患者均行手术治疗。术后病理均证实为肾上腺皮质腺癌。术后随访4例至今存活。结论:B超、CT、MRI可帮助诊断原发性无功能肾上腺皮质癌。积极手术是确切的治疗手段。50岁以上人群应以B超等影像检查监测肾上腺,以便早期发现。     

Adrenal carcinosarcoma

The clinical and pathologic features of a case of adrenal carcinosarcoma are reported. Although synchronous malignancy of the adrenal gland has been described, no case of an adrenal tumor combining both carcinomatous and sarcomatous elements has been previously documented. This neoplasm is extremely aggressive with distant metastasis arising from the sarcomatous component, and rapid progression despite multimodal therapy.     

Extended survival in a patient with recurrent and metastatic adrenal cortical carcinoma by aggressive transarterial embolization--a case report

The prognosis of inoperable recurrent or metastatic adrenal cortical carcinoma is poor due to lack of effective treatment modalities. We report a case of recurrent and metastatic adrenal cortical carcinoma in which prolonged survival of 58 months was achieved with aggressive three sequential transarterial embolization. It is probably the first reported case with the longest survival by transarterial embolization in the literature to date. A 60-year-old man received operation for left adrenal cortical carcinoma. Liver metastases, tumor bed recurrence, and spleen metastasis were noted during follow-up. Three sequential transarterial embolization for metastatic liver tumors, tumor bed recurrence, and metastatic spleen tumor were performed and resulted in relief of symptoms and prolonged survival of 58 months after recurrence verified. Aggressive transarterial embolization seems to be a safe and effective procedure for symptoms relief, and may prolong survival in the management of inoperable adrenal cortical carcinoma. It can be considered in any patient with inoperable adrenal cortical carcinoma if not contraindicated.     

肾上腺皮质腺癌的分子诊治研究进展

检索关于肾上腺皮质肿瘤分子生物学的相关报道,进行分析汇总。搜索出大量相关文献,分别在差异基因、甲基化、基因突变、microRNAs、LncRNAs等方面获得不同的研究进展。通过肿瘤分子生物学,可对疾病进行早期鉴别诊断与治疗,改善并提高肾上腺皮质腺癌患者的预后与生存质量。     

Conditional survival among patients with adrenal cortical carcinoma determined using a national population-based surveillance,epidemiology, and end results registry

Surgical excision is essential for management of the rare and aggressive neoplasm adrenal cortical carcinoma (ACC). Five-year overall survival (OS) after surgery for ACC is dependent on disease stage, but for all stages the risk of death declines with time after surgery. We calculated the effect of post-surgical duration on conditional survival (CS) among ACC patients. A total of 641 patients with M0 ACC were selected from the Surveillance, Epidemiology, and End Results (SEER) registry (1988–2012). OS for the entire cohort at 1, 2, 3, 4, 5 and 6 years was 81.4%, 66.8%, 56.3%, 50.3%, 47.2% and 44.3%, respectively. CS for an additional year given prior survival for 0, 1, 2, 3, 4 or 5 years was 81.4%, 81.1%, 83.0%, 87.5%, 93.4% and 93.4%, respectively. Age, tumor stage, tumor grade and marital status affected OS and CS. Increases in 1-year CS over time were more pronounced in patients with poorer prognostic factors. With longer follow-up, tumor stage- and grade-dependent differences in CS decreased or even disappeared. CS may provide more meaningful life expectancy predictions for survivors of ACC than conventional survival analysis.     

Pediatric Adrenal Cortical Carcinoma: Brain Metastases and Relationship to NF-1, Case Reports and Review of the Literature

Summary Adrenal cortical carcinoma (ACC) is a rare childhood neoplasm that seldom manifests brain metastases; hence few papers in the literature focus on neurological manifestations associated with ACC. Although ACC is known to be a signature tumor type in several inherited cancer predisposition syndromes, particularly Li Fraumeni, ACC has not been previously associated with neurofibromatosis, type 1 (NF-1), an inherited disorder with frequent CNS lesions that might prompt concern for metastatic disease by neuroimaging studies. We present two pediatric patients with ACC and unusual CNS findings. The first child developed metastasis to the brain 4 years after resection of his adrenal primary and 2 and 1 years, respectively, after metastases to the liver and lungs. Soon after our experience with this patient, a girl with known NF-1 presented with virilization; adrenalectomy disclosed an ACC and systemic metastases were found within months. Disseminated disease prompted concern that her complex intracranial lesions identified by neuroimaging studies might represent brain metastases, but this proved to be NF1-related hamartomatous lesions. We review the literature on ACCs in pediatric patients regarding brain metastases and previous associations with NF-1.     

恶性血液病合并粒细胞缺乏患者并发深部真菌感染的研究

目的：研究恶性血液病合并粒细胞缺乏患者并发深部真菌感染的临床特点。方法：回顾性分析30例粒细胞缺乏合并DFI患者的资料。结果：接受骨髓移植、多次化疗、反复应用激素及免疫抑制剂等治疗的粒细胞缺乏患者,细胞及体液免疫功能明显降低,同时长期应用广谱抗生素破坏了宿主正常菌群生态平衡,成为真菌感染的高危人群。医院内DFI的病原菌主要为白色念珠菌,占61.8%;应用rhG-CSF等药物能缩短粒缺的时间,有利于DFI的治疗。结论：粒细胞缺乏的血液病患者极易并发深部真菌感染,应尽早行保护性隔离。应及时应用rhG-CSF,缩短白细胞减少持续时间,合理使用抗生素,对高度怀疑真菌感染病灶存在时应预防性应用抗真菌治疗,适时据培养结果选用有效抗菌药物。     

恶性血液病合并粒细胞缺乏患者并发深部真菌感染的研究

目的:研究恶性血液病合并粒细胞缺乏患者并发深部真菌感染的临床特点.方法:回顾性分析30例粒细胞缺乏合并DFI患者的资料.结果:接受骨髓移植、多次化疗、反复应用激素及免疫抑制剂等治疗的粒细胞缺乏患者,细胞及体液免疫功能明显降低,同时长期应用广谱抗生素破坏了宿主正常菌群生态平衡,成为真菌感染的高危人群.医院内DFI的病原菌主要为白色念珠菌,占61.8%;应用rhG-CSF等药物能缩短粒缺的时间,有利于DFI的治疗.结论:粒细胞缺乏的血液病患者极易并发深部真菌感染,应尽早行保护性隔离.应及时应用rhG-CSF,缩短白细胞减少持续时间,合理使用抗生素,对高度怀疑真菌感染病灶存在时应预防性应用抗真菌治疗,适时据培养结果选用有效抗菌药物.     

Bilateral adrenal lesions

Bilateral adrenal lesions include a spectrum of disorders: neoplastic disorders (metastases, lymphoma, bilateral phaeochromocytoma, adrenocortical carcinoma and myelolipoma); longstanding congenital adrenal hyperplasia and macronodular adrenal hyperplasia; infections such as tuberculosis, histoplasmosis and blastomycosis; adrenal haemorrhage; adrenals in hypoperfusion complex and paediatric masses. Bilateral distribution in addition to other imaging characteristics can help narrow differential diagnoses in case of adrenal masses. We present a pictorial review highlighting the adrenal lesions that can present bilaterally.     

Huge adrenal haemangioma

Adrenal tumours are either functioning or non-functioning. Non-functioning adrenal tumours are generally asymptomatic and usually of enormous proportions at the time of presentation. A case is presented here of a patient with a huge right adrenal haemangioma which was successfully treated surgically. This unusual tumour was 25 cm in diameter, was well encapsulated and weighed 4 kg. The literature pertaining to this interesting case is reviewed.     

Imaging features of unusual adrenal masses

The widespread use of cross‐sectional imaging techniques has resulted in increased detection of adrenal masses. The imaging characteristics of common adrenal masses such as adenoma, phaeochromocytoma and metastasis are well established. In this pictorial essay, the imaging features of unusual adrenal masses including cyst, lymphoma, adrenal carcinoma, myelolipoma, haemangioma, smooth muscle tumour, haematoma, and infection (acute tuberculous adrenitis and histoplasmosis) are presented. Computed tomography permits a diagnosis of cyst, acute or subacute haematoma and myelolipoma. Although the imaging features of other lesions are non‐specific, imaging has an important role in the detection of these unusual adrenal masses, and in aiding their diagnosis and management.     

Cavernous hemangioma of the adrenal gland

A case report is presented of a huge cavernous adrenal hemangioma which was the second largest reported among the 17 which have appeared in the literature. With the advent of modern and sophisticated diagnostic techniques in arteriography, ultrasound, and computerized axial tomography, preoperative recognition and diagnosis should be simplified and surgical removal carried out to rule out possible malignancy, preclude traumatic rupture, or relieve symptoms of the mass lesion.