肾上腺区神经节细胞瘤(附18例报告)

目的较全面的了解神经节细胞瘤的临床特点及治疗方法。方法回顾性分析18例病理确诊的神经节细胞瘤患者临床表现、实验室检查、影像学检查和治疗效果。结果15例是无临床症状的影像学偶然发现病例,内分泌检查均在正常范围;另外3例以阵发性高血压为主要临床表现,其中1例尿儿茶酚胺升高,肾上腺髄质显像(MIBG)阳性。18例B型超声检查均为低回声;13例CT平扫见散在点状高密度影,12例CT增强扫描肿瘤周边强化明显。手术前诊断为嗜铬细胞瘤12例,神经节细胞瘤4例,肾上腺无功能腺瘤2例。18例行肿瘤切除,随访6个月~7年,高血压症状消失,2例肿瘤复发。结论肾上腺区神经节细胞瘤与肾上腺髄质肿瘤难以鉴别,综合各项影像学检查对诊断有较高的参考价值。手术治疗是唯一的有效方法。     

后腹膜神经节细胞瘤的CT和MR诊断(附1例报告与文献复习)

目的分析后腹膜神经节细胞瘤的CT和MR表现。方法回顾性分析1例经手术证实的后腹膜神经节细胞瘤患者的肿瘤的CT和MR表现,并作文献复习。结果肿块边界清楚,CT平扫表现为均一的低密度,并且其中可以看见小点状钙化。增强扫描延迟期可见进行性不均一的强化。MRT2WI上呈不均一的高信号,T1WI上表现为低信号。增强MR扫描呈轻度强化。结论在后腹膜肿瘤的鉴别诊断中神经节细胞瘤是一种较少见的肿瘤,它常常表现为边界清的肿块,并且具有包绕周围大血管生长的倾向。CT平扫为低密度,增强扫描在CT和MR上均表现为延迟期进行性的增强。     

肾上腺节细胞神经瘤8例的诊断和治疗

目的：回顾分析8例肾上腺节细胞神经瘤的临床资料,以提高其诊断的水平,方法：8例病例行CT平扫肾上腺区,其中4例加行CT增强扫描,2例加做肾区磁共振成像MRI检查,对其影像学特点进行总结。结果：8例经病理证实的肿瘤中,7例CT平扫呈密度均匀,低于肌肉密度的肿块,增强扫描4例呈密度均匀或销不均匀肿块,低于或等于肌肉强化密度,MRI2例在冠状位T1加权像表现为均匀低信号,轴位T2加权介呈不均匀高信号（相对于正常肝脏信号）。结论：（1）CT和MRI检查在肾上腺节细胞神经瘤术前诊断中有重要价值。（2）治疗主要领先外科手术。     

颈段椎管内外哑铃形节细胞神经瘤1例报告并文献复习

目的探讨颈段椎管内外哑铃形节细胞神经瘤的临床表现、诊断及治疗。方法报告1例颈段椎管内外哑铃形节细胞神经瘤,结合文献复习,回顾分析其临床表现、诊断及治疗。结果颈段椎管内外节细胞神经瘤影像学表现常为哑铃形,易误诊为神经鞘瘤;可直接起源于椎管内,也可经椎管外长入椎管内,这与脊髓及脊神经根症状出现的早晚有关;病理表现为肿瘤组织中散在分布分化成熟的神经节细胞;主要依靠显微外科手术治疗。结论颈段椎管内外哑铃形节细胞神经瘤易误诊为神经鞘瘤,主要依靠术后病理检查确诊,一期显微外科手术切除效果好。     

Unusual secondary tumors after childhood lymphoid malignancy

Second malignant neoplasms (SMN) in individuals who survived childhood cancer have been reported with increasing frequency during the last decades. The overall probability of developing second malignancy for children treated for cancer was estimated at about 2–5% at 25 years. In children, the tumors most often associated with the development of SMN are retinoblastoma and Hodgkin's disease. We report two cases of unusual second tumors in two patients cured of lymphoid malignancy: one boy cured of acute lymphoblastic leukemia developed mediastinal ganglioneuroma nine years later and one girl had gastric carcinoma seven years after Hodgkin's disease. Both developed a tumor in nonirradiated areas. Gastric carcinoma and ganglioneuroma are not reported as recurrent SMN in survivors after childhood cancer, with one single case of gastric carcinoma and one of ganglioneuroblastoma having been reported as second tumor in survivors after childhood cancer. © 1995 Wi1ey-Liss Inc.     

Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy

We report a 56-year-old man in whom an olfactory neuroblastoma with epithelial and endocrine differentiation transformed into a mature ganglioneuroma after chemoradiotherapy. The tumor arising from the sphenoidal and maxillary sinuses showed rapid growth into the frontal lobe and metastasis to the cervical lymph nodes. The patient showed signs of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A radical craniofacial resection of the primary tumor was performed after 16 Gy of local irradiation and systemic chemotherapy. Three months after the operation, the patient died of mediastinal metastasis. The biopsy before chemoradiotherapy showed a neuroblastoma with Homer-Wright rosettes, fibrillary matrix, Flexner-Wintersteiner rosettes and antidiuretic hormone production. After chemoradiotherapy, the histology changed to that of a ganglioneuroma consisting of large ganglion cells and Schwann cells without immature neuroblastoma components. Although transformation to ganglioneuroma in an adrenal neuroblastoma is common, an olfactory neuroblastoma showing ganglioneuronal maturation after chemoradiotherapy has not been reported. The pluripotent progenitor cells of the olfactory neurons may be the origin and their existence explains why various neoplasms with neuronal and epithelial differentiation arise from the olfactory mucosa.     

Differential diagnosis and laparoscopic treatment of adrenal pheochromocytoma and ganglioneuroma

Background Adrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions. Methods Clinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and ^131I-metaiodobenzylguanidine （MIBG） were performed. Neither ^131I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance. Results The mean age of patients in the adrenal ganglioneuroma group was 41.2 years （16-67 year） and in the adrenal pheochromocytoma patients 38 years （17-74 year）. Contrast enhanced CT showed that the foci were intensified in 5 cases （27.8%） of adrenal ganglioneuroma and there were obvious contrast indications in 30 （83.3%） of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 （100%） cases with adrenal pheochromocytoma. ^131I-MIBG nuclear scan showed negative results in 4 patients （100%） with adrenal ganglioneuroma and positive results in 25 （96.2%） with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of （43±6） months, and all cases survived well. Conclusions CT, urinary catecholamine and ^131I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.     

Neuropeptide Y in neuroblastoma: Increased concentration in metastasis,release during surgery,and characterization of plasma and tumor extracts

Five children with neural crest tumors (two ganglioneuromas, one ganglioneuroblastoma, and two neuroblastomas) were investigated regarding neuropeptide Y-like immunoreactivity (NPY-Ll) in tumor tissue and plasma at diagnosis and during surgery. Radioimmunoassay of extracted plasma revealed higher NPY-Ll at diagnosis of neuroblastoma (640 and 230 pmol/L resp) than ganglioneuroblastoma or ganglioneuroma (74, 45, and 26 pmol/L resp). During surgery of neuroblastoma plasma NPY-L2 increased two- to four-fold while no peroperative increase was seen in the other children. NPY-Ll was considerably higher in neuroblastoma tissue (220 pmol/g and 144 pmol/g) than in ganglioneuroblastoma (40.2 pmol/g), ganglioneuroma (0.6 and 4.4 pmol/g), or healthy adrenal tissue (5.5 pmol/g). The highest NPY-Ll concentration was found in neuroblastoma metastasis, 3,091 pmol/g. Gel-permeation chromatography of a neuro-blastoma tumor showed that a majority of NPY-Ll was representing intact NPY (NPY 1–36) while metastasis and plasma from the same child mainly contained smaller immunoreactive fragments. High concentrations of systemic NPY in neuroblastoma patients are of tumoral origin. Plasma levels of NPY and its fragments can be useful in diagnosing and monitoring neuroblastoma, and for early detection of relapse or metastatic disease. A possible involvement of NPY in neuroblastoma tumor growth and spread deserves further investigation. © 1993 Wiley-Liss, Inc.     

以咳嗽为主要症状的胸椎椎管内神经节细胞瘤1例

神经节细胞瘤是一种起源于交感神经节细胞的良性肿瘤,常发生于后纵隔、腹膜后、肾上腺髓质。椎管内神经节细胞瘤临床上较为罕见,好发于颈椎和胸腰椎,以手术治疗为主。河北省沧州市人民院收治1例以咳嗽为主要症状的患者,术前行胸椎核磁检查,手术行后正中入路肿瘤全切术,经病理诊断为位于胸3~4左侧椎间孔区、椎管内的神经节细胞瘤,HE染色可见基质内散在生长的成熟的神经节细胞,无核分裂像。术后3个月复查未见肿瘤复发。