A ganglioneuroma of the sigmoid colon presenting as leading point of intussusception in a child: a case report

We present a case of intestinal ganglioneuroma (GN) of the sigmoid colon in a 5-year-old girl, which caused intermittent colocolic intussusception. Ganglioneuromas are rare benign tumors of the autonomic nervous system composed of mature ganglion cells and satellite cells. Colonic GNs are uncommon. The unusual intramural proliferation of neural elements in this case resembled the diffuse intestinal ganglioneuromatosis, which is known to be associated with multiple endocrine neoplasia type 2B. However, the specific mutations of multiple endocrine neoplasia type 2B were not found by genetic sequencing. This is the first pediatric case described in the literature of a solitary polypoid GN presenting as a colocolic intussusception. We present a brief overview of intestinal ganglioneuromatous lesions and associated conditions.     

Adrenal ganglioneuroma: report of a new case

Although adrenal ganglioneuroma (GN) is a rare tumor originating from the neural crest tissue of the sympathetic nervous system, detection of this tumor has increased, as imaging procedures such as ultrasonography (US) and computed tomography (CT) have become prevalent. The clinical presentation for most patients is asymptomatic, and most of those tumors are hormone silent. We describe a case of adrenal GN incidentally diagnosed in a 68-year-old female patient. Physical examination, routine laboratory studies, and hormonal tests were within normal ranges. Abdominal CT and magnetic resonance imaging showed a solid oval tumor approximately 6 × 4 cm in the left adrenal gland without remarkable signs of malignancy. Left adrenalectomy was performed for treatment purposes. Histological diagnosis of the tumor was a ganglioneuroma originating from the adrenal medulla. Adrenal GN occurs rarely in adults and preoperative diagnosis is difficult, especially in asymptomatic cases. It needs careful evaluation and surgical treatment. According to our knowledge, this is the fifth case of adrenal GN in an adult patient from Turkey in English literature.     

Coincidental adrenal ganglioneuroma – a case presenting with enuresis nocturna

A 9-year-old boy presenting with primary enuresis nocturna was discovered to harbour a large left adrenal mass which produced no hormonal abnormalities. He was undergone an exploration and the mass was removed completely. It was found to be an adrenal ganglioneuroma weighing 240 g. Though no cause-and-effect relationship is established, the case no long suffered from enuresis soon after surgery and free of recurrence after 5 years.     

节细胞神经瘤的CT表现

目的　探讨节细胞神经瘤的特征性CT表现。材料与方法　对 11例经病理证实的节细胞神经瘤的CT表现进行回顾性分析。结果　 8例位于右侧肾上腺 ,3例位于腹膜后 ,3例中 1例同时合并双侧肾上腺嗜铬细胞瘤。肿瘤最大直径 3～ 6cm ,平均4.4cm。呈椭圆形者 9例 ,2例呈圆形。肿瘤边界均清晰 ,无周围组织和血管侵犯征象。平扫呈均匀低密度 ,平均 2 7.8± 4.7HU ,内有点状钙化者 3例 ( 2 7% )。增强扫描有轻度强化 ,平均 3 6.3± 9.4HU。结论　节细胞神经瘤有一些特征性CT表现 ,但确诊仍依赖组织学检查。     

Laparoscopic resection of a large nonadrenal ganglioneuroma adhered to the aorta and inferior mesenteric artery: A case report and literature review

IntroductionGanglioneuromas are well-differentiated benign tumors that arise from sympathetic ganglion cells. In ganglioneuromas of the retroperitoneum, nonadrenal cases are resected by laparotomy due to the proximity to major vessels. There have been few reports of laparoscopic resection for retroperitoneal paraaortic ganglioneuromas. We experienced a case in which laparoscopic resection was required for a 90-mm ganglioneuroma adhered to the aorta and inferior mesenteric artery.Presentation of caseA 49-year-old female patient presented with epigastric pain. Computed tomography showed a 90 mm retroperitoneal tumor, partially located between the aorta and inferior mesenteric artery. A definitive diagnosis was not obtained, and laparoscopic excision of the retroperitoneal tumor was performed transabdominally. The patient recovered without postoperative complications and left the hospital on postoperative day 8. Postoperative pathological findings revealed a ganglioneuroma from the abdominal periaortic plexus.DiscussionWe searched the literature for nonadrenal ganglioneuromas resected laparoscopically using a transabdominal approach and summarized the tumor locations. The median age was 33 years, and the median tumor size was 50 mm. Regarding the surgical results, the median operative time was 170.5 min, median blood loss was 21.5 mL, and median postoperative stay was 7 days.ConclusionLaparoscopic resection of nonadrenal ganglioneuromas is feasible even when a tumor adheres to major blood vessels.     

Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report

INTRODUCTIONGanglioneuromas are rare benign neoplasms arising from the sympathetic neuroendocrine system. These tumors usually occur in the abdomen and tend to grow around major blood vessels making often their complete excision challenging and demanding.PRESENTATION OF CASEThe authors present the challenging surgical management of a sizable retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, portal triad, celiac axis and superior mesenteric artery in a 23-year-old female patient. The tumor was safely and completely excised in toto with preservation of all neighboring vital anatomical structures using a midi laparotomy access.DISCUSSIONGanglioneuromas should be included in the differential diagnosis of any retroperitoneal mass. Their management involves total surgical excision however, in some instances; it can be challenging and demanding because of their tendency to engage neighboring vital anatomical structures.CONCLUSIONA surgical strategy including meticulous operative dissection guided by the quality principles of surgical oncology although challenging and demanding can result to a safe and complete tumor excision, which is directly correlated with an improved patients’ postoperative outcome and excellent prognosis.     

肾上腺节细胞神经瘤的诊断及治疗

目的 探讨肾上腺节细胞神经瘤的临床特点及诊疗方法. 方法 回顾性分析1997年1月至2011年2月我院15例病理确诊为肾上腺节细胞神经瘤患者的临床资料.男5例,年龄24 ～45岁,平均29岁;女10例,年龄25 ～ 69岁,平均31岁.肿瘤位于右肾上腺10例,左肾上腺5例.体检时B超检查发现8例;因阵发性腹部疼痛就诊4例;因阵发性高血压就诊3例,血压160 ～ 190/100 ～ 110 mm Hg(1 mm Hg =0.133 kPa).1例血清醛固酮112 pmol/L;1例血清去甲肾上腺素496nmol、肾上腺素210 nmol.15例B超检查肾上腺肿瘤均为低回声,5例B超示肿瘤内部有钙化的强光斑,边界清晰,2例肿瘤与下腔静脉界限不清.15例CT平扫可见密度稍低的肿块.11例行CT增强扫描,10例无明显强化.1例行MRI检查示T1WI呈低信号,T2WI为不均匀高信号.肿瘤最大径1.5 ～11.2 cm,平均6.1 cm. 结果 行经腹部、11肋间肾上腺肿瘤切除术12例,经后腹腔镜肾上腺肿瘤切除手术3例.肿瘤完整切除14例,部分切除1例.15例手术均顺利,无出血、肾上腺危象等并发症发生.随访13 ～74个月,平均35个月,3例高血压患者,血压降至正常;1例肿瘤复发,予以观察,随访55个月无转移. 结论 肾上腺节细胞神经瘤与肾上腺髓质肿瘤术前难以鉴别,术前影像学检查可获提示性诊断.手术治疗是惟一有效的方法.预后良好.     

肾上腺节细胞神经瘤的超声特征与病理分析

目的探讨肾上腺节细胞神经瘤超声表现及病理特征以提高对该病诊断水平。方法回顾分析经手术病理证实的12例肾上腺节细胞神经瘤患者的超声及病理资料。重点观察病灶的部位、形态、大小、回声特点、有无钙化、彩色血流信号以及病灶与周围组织的关系。结果 12例中,9例发生在右侧肾上腺,3例发生在左侧肾上腺,均为单发,肿瘤直径1.8～12cm,多呈类圆形或椭圆形,部分呈不规则形或＂嵌入式＂生长,肿瘤均呈低回声,5例内部见强或高回声,病理证实为钙化,3例肿瘤可见少量点状彩色血流信号,12例肿瘤均与周围结构清晰。病理上肿瘤主要由神经节细胞及施旺细胞组成。结论肾上腺节细胞神经瘤的超声具有一定特征性,观察肿瘤生长部位、形态及回声特点,对该肿瘤的诊断具有重要价值。     

An ultrastructural study of two cases of adrenal ganglioneuroma

Tissue samples of adrenal ganglioneuromas were obtained from two patients: 35- and 47-year-old males. Light microscopic studies showed that these tumors contained Schwann cells and ganglion cells. Electron microscopic examinations revealed numerous unmyelinated and myelinated axons surrounded by Schwann cells. The ganglion cells in the tumors had abundant organelles, such as well-developed cisternae of the rough endoplasmic reticulum, many profiles of the Golgi apparatus, mitochondria, lysosomes, microtubules and neurofilaments. Electron dense cored granules resembling catecholamine granules were present in the ganglion cell bodies and neural processes. These features resmebled those of normal sympathetic ganglion cells. It is concluded that adrenal ganglioneuroma originates from sympathetic ganglion tissue. This study was presented in part at the 26th Annual Meeting of the Clinical Electron Microscopy Society of Japan, Kochi, October 5–7, 1994.