肾上腺节细胞神经瘤3例诊断与治疗

肾上腺节细胞神经瘤是一种临床罕见的良性肿瘤,好发于交感神经节,而在肾上腺髓质发生较为少见。我院自2003年3月至2006年3月共收治3例,且瘤体均大于10cm,术后经病理证实。现总结如下。1资料与方法1.1临床资料本组肾上腺节细胞神经瘤3例,均为女性,年龄38~42岁,平均年龄39.7岁,病     

腹腔镜及开腹手术切除儿童肾上腺区神经源性肿瘤的对比研究

目的 探讨腹腔镜下切除儿童肾上腺区神经源性肿瘤的可行性及远期疗效.方法 回顾性分析2006年5月至2016年5月行腹腔镜下肾上腺区肿瘤切除,且病理结果为节细胞神经瘤、节细胞神经母细胞瘤、神经母细胞瘤15例患儿(腹腔镜组)的临床资料.在同一时间段内以患儿年龄、性别、肿瘤大小及部、术前肿瘤临床分期、术后病理结果为配对条件,在我院行开放性手术切除肾上腺区神经源性肿瘤患儿中进行抽选,与腹腔镜组1∶1配对,共13例成功配对(开放组),并行统计学分析.结果 腹腔镜组术后中位瘤床引流量为(40 ml)少于开放组(78 ml),组间比较差异有统计学意义(P=0.028).腹腔镜组与开放组在手术时间(140 min vs 80 min)、术中出血量(20 ml vs 30 ml)、术后置管天数(3 d vs4 d)、术后恢复进食时间(24 h vs23 h)及术后住院日(7 dvs7 d)方面,组间比较,差异均无统计学意义(P＞0.05).腹腔镜组中有1例出现术后电解质紊乱,予对症补充电解质后得到及时纠正;1例发生淋巴瘘,通过增加留置引流管时间,充分引流后治愈出院.术后中位随访时间为36个月,两组3年无瘤生存率分别为76.2％和69.9％,5年总体生存率分别为85.7％和80.0％,差异均无统计学意义(P＞0.05).结论 腹腔镜下切除儿童肾上腺区神经源性肿瘤安全可行,且术中对周围组织损伤较开放性手术小,远期效果与开放性手术无明显差异.     

Tumor with watery diarrhoea,hypokalaemia in a 3-year-old girl

Watery diarrhoea, hypokalaemia and achlorhydria (WDHA) syndrome was caused by vasoactive intestinal polypeptide (VIP)-producing tumour. A 3-year-old Chinese girl with watery diarrhoea, abdominal distension and hypokalaemia due to a thoracic paraspinal VIP-secreting ganglioneuroma is reported. The girl coughed, fevering up to 39°C after a flu-like episode. She had eight to ten abundant stools daily which is not improved by dietary treatment, resulting in an important weight loss. She weighed 6.8 kg (nl P50 at 6 months of age) and is 76 cm (nl P50 at 9 months of age) in height. Blood electrolytes showed 129 mmol/L sodium, 2.42 mmol/L potassium, 94 mmol/L chloride and 18.6 mmol/L bicarbonate; urinary catecholamines were normal. Computed tomography scan evidenced a left side paravertebral mass of 4 × 6 cm in the lower thoracic region leading to the blood determination of vasoactive intestinal polypeptide which amounted 830 pmol/L(normal < 25 pmol/L). Surgical removal showed a ganglioneuroma of 160 g and was associated with disappearance of the diarrhoea and normalization of VIP level below 20 pmol/L. Review of the 63 reported cases in children with WDHA showed that many of the cases presented with non-treatable watery diarrhoea, hypokalaemia. Achlorhydria is not necessarily part of the WDHA syndrome. The male to female ratio is 1:1.5. Ganglioneuroblastoma and ganglioneuroma are the commonest tumours. Location of the tumour is variable: abdomen, chest or neck. Abdominal distension, flushing, episodic hypertension and colonic dilatation, constipation and ataxia were the other associated features. Surgical resection is the treatment of choice of VIP-producing tumours.     

Imaging of retroperitoneal ganglioneuroma

The aim of this study was to describe the radiological appearance of retroperitoneal ganglioneuroma. We retrospectively reviewed seven cases of histologically proven retroperitoneal ganglioneuroma. Ultrasound and enhanced CT were obtained in all cases, and MRI in three cases. The masses were well-circumscribed, ranged in size from 5 x 3 x 3 to 10 x 6 x 4 cm. In three cases close relationships between the tumor mass and major blood vessels were noted, resulting in vessel displacement or surrounding, but without compression or occlusion. On ultrasound examination the tumor showed a heterogeneous solid echostructure. Non-enhanced CT showed homogeneous or mildly heterogeneous low attenuation, and a punctate calcification was seen in one case. Contrast uptake was absent (n = 1) or delayed (n = 6). Progressive but incomplete enhancement was observed in three cases. On MRI, T2-weighted images showed a high signal intensity. Dynamic studies depicted the same enhancement pattern as described on CT. Ganglioneuroma is a rare tumor which should nevertheless be included in differential diagnosis of retroperitoneal masses when presenting as a well-delimited tumor with possible tendency to surround or displace major blood vessels, low density on non-enhanced CT, and delayed progressive enhancement on CT and MRI.     

Technetium-99m sestamibi imaging in paediatric neuroblastoma and ganglioneuroma and its relation to P-glycoprotein

Imaging with technetium-99m sestamibi offers a non-invasive approach to detect the presence of functional P-glycoprotein (Pgp), one of the major causes of multidrug resistance, in human malignancies. A clinical role for Pgp has been suggested in the subpopulation of primary neuroblastoma without amplification of the proto-oncogene MYCN. We wanted to evaluate the usefulness of ^99mTc-sestamibi scintigraphy in the screening of neural crest tumours for the presence of Pgp. In ten children suffering from MYCN-negative neuroblastoma, ganglioneuroblastoma or ganglioneuroma, ^99mTc-sestamibi imaging was performed at initial diagnosis. All patients underwent planar imaging 20–30 min and 3.5–4 h after intravenous injection of 740 MBq/1.73 m^{2 99m}Tc-sestamibi. Tumour to normal tissue ratios, as well as washout rates, were determined and compared with in vitro flow cytometric analysis of Pgp expression and function. Pgp expression was analysed flow cytometrically with the monoclonal antibodies 4E3 and MRK16, and Pgp function was evaluated by means of rhodamine 123 uptake and efflux either in the absence or in the presence of the Pgp inhibitor verapamil. In nine of ten patients, we found that the intratumoral ^99mTc-sestamibi activity was comparable to the background activity, which might be suggestive of Pgp presence. This was confirmed flow cytometrically in all but one patient. ^99mTc-sestamibi enhancement was seen in the primary tumour and the bone marrow metastases of one of the ten patients, and this result was concordant with a negative Pgp status. The findings presented suggest that ^99mTc-sestamibi imaging results might correlate with the presence of functional Pgp in neural crest tumours without MYCN amplification. Received 17 October and in revised form 13 December 1998     

Retroperitoneal ganglioneuroma in a 15-year-old boy,an interesting case studied through multimodal imaging

Benign ganglioneuroma contains mature autonomous ganglion cells, including satellite cells and long axonal processes, as well as Schwann cells, which come from neural crest-derived cells that form the adrenal medulla and sympathetic nervous system during embryonic development and is a rare benign tumor which occurs spontaneously and can also occur during radiotherapy or chemotherapy, accounting for 0.72% -1.6% of primary retroperitoneal tumors, commonly found in the posterior mediastinum and retroperitoneum, and affected patients usually have no symptoms due to of its non-functional feature, although several complications can arise if the tumor is large enough to press against adjacent organs.     

Un incidentalome surrenalien inhabituel: le ganglioneurome

Ganglioneuroma is a rare, benign, neurogenic tumor originating from the neural sheath. It is frequently located in the retroperitoneum. The localization in the adrenal gland is rare. We report a case of adrenal ganglioneuroma in a 42 year-old woman who had bronchiectasis and asthma. The tumor was discovered incidentally in the computed tomography indicated in the etiological assessment of her bronchiectasis. Our diagnosis was confirmed by histopathology.     

Resection of the uncinate process of the pancreas due to a ganglioneuroma

A 33-year-old woman who presented with epigastric discomfort and diarrhea underwent an abdominal ultrasound （US）. This investigation and subsequent contrastenhanced computed tomography, magnetic resonance imaging and endoscopic US with fine needle aspiration （FNA） revealed a 40 mm well-circumscribed mass in the uncinate process of the pancreas. Findings were suggestive of a mucinous or solid-cystic pseudopapillary tumor of the pancreas, although other lesions such as a nonfunctioning neuroendocrine tumor could not be ruled out. FNA samples were negative for malignant cells, but of limited value due to poor cellularity. It was decided to surgically remove the tumor because malignancy could not be discounted. Multiple intraoperative biopsies were suggestive of mesenchymal tumor and consequently a conservative resection （uncinatectomy） was performed. The postoperative course was uneventful. The definitive diagnosis was ganglioneuroma. Immunocytochemistry showed positive staining with vimentin, S-100 protein, neurofilament and neuron-specific enolase. Ganglioneuroma is a rare benign tumor that can also present as a pancreatic tumor. Uncinatectomy is feasible, safe and a good surgical technique for the treatment of nonmalignant tumors located in the uncinate process of the pancreas.     

小儿外周性节细胞神经瘤的影像表现与病理对照分析

目的:分析小儿外周性节细胞神经瘤的影像表现特征,以提高诊断准确性。方法:回顾性分析24例小儿外周性节细胞神经瘤影像学资料,并与病理结果进行对照。术前19例CT平扫与增强,7例MRI平扫与增强,其中2例同时做了CT和MRI检查。结果:24例外周性节细胞神经瘤中位于腹膜后10例,肾上腺8例,后纵隔5例,颈部1例。均为单发病灶,边界清楚,圆形或类圆形22例,不规则形2例。病灶平均直径7.9cm,CT平扫为均匀或不均匀低密度,4例肿瘤内出现点状或条状钙化。增强后轻度强化或不均匀条片状强化,4例延迟后进行性增强。T1WI表现为均匀或不均匀低信号,T2WI表现为均匀或不均匀高信号,其中1例内见曲线形低信号灶。强化方式与CT相似。肿瘤推移和包绕血管但不侵犯血管。结论:CT和MRI平扫及增强扫描能较好地显示节细胞神经瘤的内部结构和影像学特点,反映病灶的病理特征,有助于与其它病变的鉴别。