Composite paraganglioma‐ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with multiple endocrine neoplasia type 2B: A case report

Multiple endocrine neoplasia type 2 (MEN2) is an autosomal‐dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2‐associated extra‐adrenal pheochromocytomas (paraganglioma) are rare. A 59‐year‐old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10‐mm left adrenal mass and a 30‐mm retroperitoneal mass. He had paroxysmal elevations in blood pressure and in urinary metanephrine and vanillylmandelic acid values. Laparoscopic excision of the left adrenal gland and retroperitoneal mass was performed. We experienced an extremely rare case of composite paraganglioma‐ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with MEN2B.     

椎管内节细胞神经瘤MRI征象

目的 分析椎管内节细胞神经瘤的MRI征象,以提高术前诊断能力。方法 回顾性分析9例经手术病理证实、累及椎管的节细胞神经瘤MRI资料,观察肿瘤位置、形态、大小、信号和强化程度等。结果 共纳入9个病灶,其中位于颈椎4个,胸椎2个,腰骶椎3个;7个累及椎管内外,呈哑铃状,2个位于椎间孔区,呈结节状;9个病灶T2WI均呈均匀或不均匀高信号,1个病灶内见囊变坏死。8个接受增强扫描的病灶中,3个呈明显强化,5个呈轻中度强化,4个病灶内见条状强化。所有病灶均伴有受累椎间孔扩大,但骨质未见破坏。结论 椎管内节细胞神经瘤MRI表现具有一定特征性,有助于术前准确诊断。     

后纵隔节细胞神经瘤的CT、MR表现

目的：提高对后纵隔节细胞神经瘤（Posterior mediastinal ganglioneuroma,PMGN）的认识。材料和方法：搜集10例经手术病理证实为PMGN患者的临床、影像学资料（5例行CT检查,1例行MR检查,4例同时行CT及MR检查）,并对其CT、MR表现进行分析、总结。结果：影像学上,PMGN瘤体上下径大于前后径及左右径,且紧贴脊椎生长。我们将本组PMGN根据其是否累及邻近的椎管分为椎管相关性节细胞神经瘤（GN）（n=1）和非椎管相关性GN（n=9）,另外根据肿瘤的生长方式又分为单侧型（n=7）和跨中线型（n=3）。CT平扫表现为均匀或不均匀性低密度,1例内可见散在点状钙化,1例内可见片絮状脂肪密度影;增强扫描表现为无强化或轻中度不均匀或均匀强化。MR上,T1WI病灶均表现为均匀低信号,T2WI病灶均表现为不均匀稍高信号,T1WI增强扫描1例表现为不均匀混杂强化,其余表现为无强化。结论：PMGN在CT及MR上具有一定特点,对其特征性表现提高认识,有助于做出正确的诊断。     

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.     

肾上腺节细胞神经瘤的影像诊断学表现

目的分析肾上腺节细胞神经瘤的影像学特点,提高对该病的认识及诊断。方法回顾性分析经手术和病理证实的12例肾上腺节细胞神经瘤的影像学表现,研究其影像表现与病理的相关性。结果 11例发生在右侧肾上腺,1例发生在左侧肾上腺。10例行B超检查,均表现为肾上腺低回声肿块,其中4例肿块内部见强回声,伴后方衰减,病理显示为钙化;10例CT检查示肿瘤呈类圆形或椭圆形,少数呈不规则分叶状,轮廓光整,平扫呈均匀稍低密度,CT值28~46 Hu,6例伴散在细点状钙化,增强后肿瘤呈不均匀轻至中度强化;2例磁共振成像检查示肿瘤呈类圆形,T1WI呈均匀稍低信号,T2WI呈不均匀稍高信号。结论肾上腺节细胞神经瘤影像学表现具有一定特征性,可在术前作出诊断及鉴别诊断。     

节细胞神经瘤的CT表现及病理分析

目的探讨节细胞神经瘤的CT平扫与动态增强扫描的特征性表现。方法回顾性分析10例经手术与病理证实节细胞神经瘤的CT表现,所有病例均行CT平扫,动脉期、静脉期及延迟期动态增强扫描。结果肿块位于纵隔7例,肾上腺2例,腹膜后1例。肿块边界清晰,呈椭圆形2例,不规则形8例,肿块大小为1.2cm×2.2cm×3.0cm～6.5cm×6.5cm×7.5cm,4例肿块有嵌入性生长特点。CT平扫肿块密度低于肌肉,密度均匀者9例,密度不均匀者1例,其中3例伴有颗粒状、条状钙化。增强扫描后,肿块表现为不同的强化型式,其中,1例肿块无强化;7例肿块动脉期无明显强化,静脉期及延迟期逐渐轻度强化;2例肿块动脉期轻度强化,静脉期及延迟期逐渐明显强化。结论节细胞神经瘤有一些特征性影像表现及强化型式,CT动态增强扫描有助于其诊断及鉴别诊断,可清晰显示病变同周围血管及器官之间的关系,并为手术治疗提供更多参考信息。     

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21例临床分析

回顾性分析我院治疗的21例肾上腺节细胞神经瘤的临床表现、影像学、内分泌检查等临床资料.9例腰部不适,4例阵发性头晕乏力（其中1例高血压）,1例向心性肥胖,7例为体格检查发现.5例术前诊为肾上腺节细胞神经瘤,其余诊为肾上腺占位病变.术后病理检查证实均为肾上腺节细胞神经瘤.随访1～7年,1例仍有头晕,血压150/95 mm Hg（20.0/12.6 kPa）,余患者均康复.提示肾上腺节细胞神经瘤的诊断主要靠影像学检查和病理检查诊断,手术是主要治疗手段,大部分患者可完全康复.     

节细胞神经瘤的 CT、MRI 影像表现与病理对照研究

目的：研究节细胞神经瘤的CT和MRI表现并进行病理学对照。方法对经手术病理证实的12例节细胞神经瘤患者的临床病理资料进行回顾性分析,所有患者均进行CT和MRI检查,并与病理学结果进行对照分析。结果病灶大小为（4．7±1．2）cm,CT平扫为不均匀或均匀的低密度;MRI平扫中,T1WI均呈现不均匀的低信号,T2 WI均呈现不均匀的等高信号,中间有部分条状的低信号。12例进行MRI增强扫描时,不强化的有4例,轻度强化的有3例,均匀渐进性强度强化的有2例,不均匀渐进性强化的有3例,边缘环状强化的有1例,与CT增强扫描结果一致。结论 CT和MRI作为检测节细胞神经瘤的影像学手段,其表现具有一定的特征性,并在很大程度上能够反映出肿瘤的形态、大小及组织成分,与病理中肿瘤所富含的大量黏液基质具有一定的关系。