后腹腔镜手术切除肾上腺节细胞神经瘤疗效观察

目的 :探讨后腹腔镜微创手术治疗肾上腺节细胞神经瘤的适应证和可行性。方法 :采用后腹腔镜手术治疗肾上腺节细胞神经瘤患者 5例 ,其中左侧肾上腺节细胞神经瘤 2例 ,右侧 3例。结果 :5例后腹腔镜手术全部获得成功 ,4例肾上腺肿瘤为单发 ,1例为多发 (4个肿瘤 ) ;肿瘤最大直径 2 .5～ 8.0 (4 .2± 1.8)cm ;手术时间35～ 10 5 (5 9± 2 7)min ,估计出血量 10～ 30 (19± 7)ml,术后镇痛剂吗啡用量 0～ 2 0 (8± 8)mg ,2例未用镇痛剂 ;排气、恢复进食时间 1～ 3(1.4± 0 .5 )d ;术后住院时间 4～ 7(5 .4± 1.5 )d。无围手术期并发症发生。结论 :后腹腔镜手术切除肾上腺节细胞神经瘤是安全可行的 ,能充分体现腹腔镜手术创伤小、恢复快的优点。肾上腺节细胞神经瘤是腹腔镜手术很好的适应证。     

肾上腺节细胞神经瘤的CT表现

目的:探讨肾上腺节细胞神经瘤的CT表现。方法:回顾性分析经手术病理证实的临床材料完整的4例肾上腺节细胞神经瘤。结果:4例肾上腺节细胞神经瘤中,右侧3例,左侧1例。肿瘤直径4~8.9cm,平均6.5cm。肿瘤大部分呈卵圆形,边界清晰,无周围组织和血管侵犯,平扫密度低于肌肉,内可见小点状钙化,增强呈轻中度不均匀强化。结论:肾上腺节细胞神经瘤有一定特征性CT表现,但确诊应根据病理学检查。     

Neurogenic polyps

A neoplastic proliferation of peripheral nerve sheath cells (Schwann cells, fibroblasts and perineurial cells) and ganglion cells in the colorectum may give rise to the mucosal or submucosal polyps. Depending upon the predominant cell types, these neurogenic polyps can be classified as schwannomas, granular cell tumours, neurofibromas, perineuriomas, mixed nerve sheath tumours, ganglioneuromas or paragangliomas. Morphologically, the neoplastic cells repeat or mimic the corresponding nerve sheath cells or neurons in terms of growth pattern, histology and immunoreactivity. They are uncommon, but the polyps can occur in any age group, although the vast majority of patients are adults. The polyps can be either solitary (most peripheral nerve sheath tumours) or multiple, especially if associated with systemic diseases (i.e. syndromes involving the peripheral nerve tissue). They are usually incidental findings or may be accompanied by gastrointestinal symptoms. Almost all colorectal neurogenic polyps are benign, and they rarely undergo malignant transformation unless they are part of a syndromatic manifestation. However, these polyps may cause a diagnostic problem during screening for colorectal cancer. An accurate diagnosis of these entities will help clinicians to make appropriate management decisions.     

CT在三叉神经瘤诊断中的价值

目的探讨CT在三叉神经瘤诊断中的价值。方法搜集经手术和病理学证实的三叉神经瘤12例,行CT平扫及静脉注射60％泛影葡胺或优维显增强扫描。结果12例中表现为特征性哑铃形3例,类哑铃形6例,类圆形3例。肿瘤呈混杂密度、均匀密度或等密度,均无钙化及水肿。结论CT对三叉神经瘤的定位、定性及岩骨和颅底骨的异常改变具有重要的诊断价值。     

Colonic manifestations of PTEN hamartoma tumor syndrome: Case series and systematic review

AIM:To investigate our clinical experience with the colonic manifestations of phosphatase and tensin homolog on chromosome ten(PTEN)hamartoma tumor syndrome(PHTS)and to perform a systematic literature review regarding the same.METHODS:This study was approved by the appropriate institutional review board prior to initiation.A clinical genetics database was searched for patients with PHTS or a component syndrome that received gastrointestinal endoscopy or pathology interpretation at our center.These patient’s records were retrospectively reviewed for clinical characteristics(including family history and genetic testing),endoscopy results and pathology findings.We also performed a systematic review of the literature for case series of PHTS or component syndromes that reported gastrointestinal manifestations and investigations published after consensus diagnostic criteria were established in 1996.These results were compiled and reported.RESULTS:Eight patients from our institution met initial inclusion criteria.Of these,5 patients underwent4.2 colonoscopies at mean age 45.8±10.8 years.All were found to have colon polyps during their clinical course and polyp histology included adenoma,hyperplastic,ganglioneuroma and juvenile.No malignant lesions were identified.Two had multiple histologic types.One patient underwent colectomy due to innumerable polyps and concern for future malignant potential.Systematic literature review of PHTS patients undergoing endoscopy revealed 107 patients receiving colonoscopy at mean age 37.4 years.Colon polyps were noted in92.5%and multiple colon polyp histologies were reported in 53.6%.Common polyp histologies included hyperplastic(43.6%),adenoma(40.4%),hamartoma(38.3%),ganglioneuroma(33%)and inflammatory(24.5%)polyps.Twelve(11.2%)patients had colorectal cancer at mean age 46.7 years(range 35-62).Clinical outcomes secondary to colon polyposis and malignancy were not commonly reported.CONCLUSION:PHTS has a high prevalence of colon polyposis with multiple histologic types.It should be considered a mixed polyposis syndrome.Systematic review found an increased prevalence of colorectal cancer and we recommend initiating colonoscopy for colorectal cancer surveillance at age 35 years.     

32例肾上腺区节细胞神经瘤的CT与MRI诊断

目的 探讨肾上腺区节细胞神经瘤的CT、MRI表现及其诊断价值.方法 回顾性分析经手术病理证实的32例肾上腺区节细胞神经瘤的CT(29例)、MRI(10例)表现.结果 32例患者共33个病灶,双侧1例,单侧31例,肾上腺25个,肾上腺周围8个.肿瘤长径为1.2～18 cm,平均7.5 cm.3例病灶随访有增大,其中1例在短期内迅速增大.除2个长径＞12 cm呈嵌入样生长者外其余病灶均有较完整的包膜,4个较小的病灶呈圆形、类圆形,较大的29个病灶呈分叶、伪足形,大部分呈钻孔样生长.10个病灶有斑点、结节状钙化灶,1个病灶有小囊变,3个病灶有明显分隔.CT平扫:23个病灶呈低密度,3个呈混杂密度,3个呈中等密度;CT增强扫描:21个病灶轻度强化,8个病灶中度强化.MRI平扫:T1 WI呈较均匀低信号6例,不均匀低信号2例,混杂信号2例,T2WI呈较均匀高信号3例,不均匀高信号5例,混杂信号2例;MRI增强扫描:轻度强化3例,中度强化7例.结论 CT、MRI能很好地显示肾上腺区节细胞神经瘤的影像学特点,有助于其诊断和鉴别诊断.CT、MRI平扫和增强扫描能较好地显示肾上腺区节细胞神经瘤的内部成分和影像学特点,反映病灶的血供特点和病理特征,并可观察病变与周围血管及器官的关系.     

Thoracoscopic removal of neurogenic mediastinal tumors: technical aspects

Background: Thoracoscopy is fast becoming the standard approach for the removal of neurogenic mediastinal tumors. However, there are risks for adjacent nervous structures (stellate ganglion, spinal cord). The aim of this study was to review the technical features of this approach.Methods: Between December 1999 and January 2003, nine patients underwent thoracoscopic resection of a mediastinal neurogenic tumor at our hospital. Five of these patients were asymptomatic with incidentally found tumor; the other four patients had compression-related syndromes. Two tumors had developed in the superior sulcus, and one had a spinal canal component (dumbell-type tumor).Results: Thoracoscopic dissection was possible in all cases. In one patient, resection of the tumor was performed via a combined neurosurgical and thoracoscopic approach. Seven tumors were benign nerve sheath tumors (schwannoma), and 2 were nerve cell tumors (ganglioneuroma). The postoperative course was uncomplicated in all patients.Conclusion: The thoracoscopic resection of mediastinal neurogenic tumors is technically easy, except for bulky tumors of the superior sulcus and dumbbell tumors, which require a combined thoracoscopic and neurosurgical approach.     

Low occurrence of familial neuroblastomas and ganglioneuromas in five consecutive GPOH neuroblastoma treatment studies

Familial neuroblastoma is of special interest in view of the oncogenesis of this tumour with its early manifestation in childhood. The inheritance seems to follow an autosomal-dominant Mendelian trait with incomplete penetrance. Familial neuroblastomas and ganglioneuromas have not been reported in detail within large treatment studies. A retrospective clinicopathological survey of patients reported to the German neuroblastoma treatment studies over 24 years was performed. Among 2863 patients (2752 neuroblastomas, 111 ganglioneuromas) included in five consecutive trials, only 22 hereditary cases in ten families were observed. Neuroblastomas were found in 18 patients and ganglioneuromas in four, accounting for less than one percent of all cases. Six patients with neuroblastomas had localised disease, seven had stage 4, three had stage 4S, and stage was unknown in two patients. In four families, two generations were affected, with ganglioneuromas occurring in the parental generation in two families. Two families had three affected patients. Contrary to previous reports, age distribution and number of primary tumours in patients with familial tumours were not significantly different from patients with sporadic tumours. The outcome of both groups was comparable. These data confirm the low prevalence of familial neuroblastoma and may help in counselling the affected families.     

Trieeminal ganglioneuroma

We present the case of an 8-year-old girl with a ganglioneuroma in the left cerebellopontine angle region. The tumor originated from the sensory root of the trigeminal nerve. Histopathologically, it was composed of neoplastic ganglion cells and Schwann cells, leading us to the diagnosis of ganglioneuroma. Intracranial ganglioneuroma is very rare. To our knowledge, this is the first report of a trigeminal ganglioneuroma. The nature and origin of this tumor are discussed and the literature reviewed.     

Ganglioneuroma of small bowel mesentery presenting as acute abdomen

Ganglioneuroma is a rare benign tumor, usually seen in children and young adults, arising in the central nervous system. Ganglioneuroma of the mesentery is extremely rare; only one case has been recorded. We report the second case of a primary mesentery ganglioneuroma. The patient underwent surgical intervention for diagnostic and therapeutic purposes.