成人肾上腺神经母细胞瘤1例报告并文献复习

目的探讨成人肾上腺神经母细胞瘤的临床、病理特点,提高该病的诊疗水平。方法文献复习并报道我院收治的1例成人肾上腺神经母细胞瘤患者。结果本例患者行后腹腔镜左肾上腺肿瘤切除术,完整切除肾上腺肿物,经病理学检查及免疫组化证实为成人肾上腺神经母细胞瘤。结论成人肾上腺神经母细胞瘤极为罕见,恶性程度高,预后极差,手术为主、辅助性放疗化疗的综合治疗具有一定的疗效,但有效的新疗法仍有待探索。     

成人肾上腺神经母细胞瘤1例

患者 ,女 ,41岁。因右上腹隐痛 2个月余于1 996年 9月 2日入院。无异常临床表现 ,内分泌定性检查均为阴性。CT示右肾上腺有一 4cm× 3cm囊实性肿块 ,平扫值为 2 1 .7～ 5 5 .4Hu,边缘清晰 ,增强后见边缘稍有强化 ,块影内无明显加强 ,肿块与下腔静脉分界不清 ,右肾受压 ,肝、脾及腹腔内散在小点状钙化影。于 9月 1 3日在全麻下行右肾上腺探查术 ,术中见肿瘤如鸡蛋大小 ,有包膜 ,肿瘤上附着部分肾上腺。行肿瘤切除术 ,术中血压无明显波动。术后病理检查 :有包膜肿瘤 ,6.0 cm× 3.5 cm× 3.0 cm,切面灰白色 ,中部有一囊腔 ,腔周暗褐色 ,质软…     

儿童巨大肾上腺神经母细胞瘤1例

<正>患儿,男,3岁,发现腹部肿块入院于2010年4月30日就诊。查体:腹部膨隆,右侧稍明显,触诊右侧腹可及包块,活动度差,越过中线约3cm,下界越过脐水平约1cm,双下肢无水肿。辅检:常规血生化、胸片、心电图等未见异常。腹部CT平扫+增强报告示:(右肾上腺区)巨大占位,大小16.8cm×13.0cm×13.0cm,压迫肾脏,强化不明显,瘤体内可见不均匀坏死(图1)。请儿科、肿瘤科及     

左侧肾上腺巨大血管瘤1例并文献复习

目的提高肾上腺血管瘤的诊治水平。方法回顾分析我院收治的1例肾上腺海绵状血管瘤病例的临术资料,总结归纳其临床特征及影像学CT和MRI表现,结合相关文献综合分析。结果患者,体检发现左侧肾上腺区肿物。查体无明显阳性体征。生化、内分泌等实验室检查均在正常范围。CT表现为向心性增强、斑片状边缘强化、弥漫性钙化的特点。在全麻下成功行左侧肾上腺血管瘤切除术,肿瘤最大径10cm,呈圆形、实性、有完整包膜。手术时间2.5h,出血约200mL,术后1周出院。术后随访1个月,未见肿瘤复发及转移。结论肾上腺血管瘤临床罕见,无特异性临床表现,但其CT和MRI的特征性表现有助于对该病的诊断。     

肾上腺神经母细胞瘤（附5例报告并文献复习）

本文报告我院１９８７～１９９２年收治的经病理确诊的５例肾上腺神经母细胞瘤的诊断和治疗，结合文献复习，对本病的临床分期及预后、新的诊断技术及治疗作了讨论。认为：肿瘤局限者以手术切除为主，远处转移应作综合性化疗；放疗有利于缩小肿瘤切除不净而残留较大的肿瘤组织和增加手术切除机会。     

肾上腺区巨大淋巴结增生症1例报道并文献复习

<正>巨大淋巴结增生症又称Castleman’s Disease或血管滤泡性淋巴组织增生,1954年由Castleman首先报道。临床较少见,文献报道好发生于胸部纵膈,发生于腹膜后则极为少见。现报道1例腹腔镜微创手术治疗腹膜后局灶型巨大淋巴结增生症,并复习相关文献以加深对该病的认识,提高诊出率。     

肾上腺神经母细胞瘤并瘤内出血1例

<正>临床资料男性,16岁,因发现"右上腹包块10年、异常增大1个月"于2010年2月27日入院。查体:右上腹稍膨隆,右侧肋缘下可触及一包块,大小约10 cm×6 cm,质稍硬,边界清,轻触痛,活动度不明显,右肋脊角叩击痛(+)。实验室检查:血常规白细胞12.05×109/L;立卧位高血压三项:肾素、血管紧张素偏高,醛固酮正     

肾上腺节细胞神经瘤1例报告并文献复习

节细胞神经瘤是少见的起源于交感神经节细胞的良性肿瘤,组织学上由神经节细胞、雪旺细胞以及神经纤维构成[1].肾上腺髓质起源于神经嵴交感神经胚细胞,因此节细胞神经瘤也可发生于肾上腺. 患者,女,22岁,因"反复左腰痛1年半,加重1d"入院.患者无高血压、无四肢乏力及向心性肥胖.B超:左肾上腺低回声占位,类圆形,大小约80mm×60mm.CT:左肾上腺区有一约82mm×61mm的类圆形占位,平扫CT值26~37HU,强化不明显,病灶内可见斑点状高密度影,考虑腺瘤可能性大.见图1.入院后完善血常规、血生化、肝肾功能及肾上腺功能相关检查.0am、8am、4pm和8pm4个时间点血皮质醇浓度均在正常范围(分别为3.26、19.56、7.31和6.12μg/dl),皮质醇昼夜分泌节律无紊乱;血肾素[卧位:1.10ng/(ml·h);立位:1.49ng/(ml·h)]、醛固酮(卧位:85.09pg/ml;立位:208.03pg/ml)、钾离子(4.23mmol/L)、肾上腺素(26.23pg/ml)、去甲肾上腺素(150.55pg/ml)、多巴胺(54.89pg/ml)及24h尿香草扁桃酸(VMA)(5.2mg/24h)均未见异常,考虑为无功能肿瘤.采取经腹入路,左侧肋缘下斜切口,完整切除肿瘤.术中患者血压无明显波动,出血量约10ml,手术时间69min,手术过程顺利.切除肿瘤的大小约80mm×60mm,类圆形,表面光滑,包膜完整,质地均匀,切面呈苍白色;瘤细胞呈梭形,束状排列,边缘可见神经节细胞.见图2.病理报告"符合肾上腺节细胞神经瘤".术后患者康复顺利,术后第7天拆线,第8天痊愈出院.左腰痛症状消失,随访3年,症状无复发,复查CT病灶无复发.     

肾上腺神经母细胞瘤1例

患者，男，年龄9个月。因体检时超声发现左肾上腺区肿块，于2008年9月收入院。体检：一般情况正常，尿3-甲基-4-羟基苦杏仁酸（VMA）正常，胸片未见异常。超声检查示左肾上腺实质性肿块，大小37mm×34mm，边界清楚，形态规整，内部回声欠均匀，肝、胆、胰、肾未见异常。CT检查示左肾上腺区可见36mm×37mm大小软组织密度影，CT值39．1Hu；边缘清晰，     

成人腹膜后节细胞神经母细胞瘤2例报告并文献复习

目的：探讨成人节细胞神经母细胞瘤的临床和病理特点。方法：回顾分析2例成人节细胞神经母细胞瘤的临床资料和病理资料。结果：2例均为体检时发现，无自觉症状，实验室检查均无异常。肿瘤大小分别为7cm×5cm和5cm×4cm，包膜完整，均与周围组织有粘连，切面呈鱼肉状，免疫组化NSE，Syn，CgA，S-100及vimentin均为（＋）。1例术后随访17个月死亡，1例随访6个月无转移。结论：成人节细胞神经母细胞瘤临床少见，多为体检时发现。治疗最有效的方法是手术切除，可辅以放、化疗，     

An Adult Adrenal Neuroblastoma: A Case Report

Gastric cancer usually affects people older than sixty years. This type of cancer is very rare in adults under thirty years of age. In addition, the prognosis in this part of population is grave due to the high incidence of undifferentiated tumours and advanced stage at time of diagnosis. Radical surgery affords the only chance for long term survival, but even this option is often limited upon finding locally advanced disease or peritoneal seeding. The following are case studies of three young adults from a group of 45 patients, who were treated between January 1^st, 2000 and December 31^st, 2003.     

Adrenal Neuroblastoma in an Adult: Report of a Case

Neuroblastomas comprise a major part of adrenal tumors in children. However, they are seldom reported in adults. In this report we present the case of a 30-year-old woman who was operated on for an incidentaloma. Following an adrenalectomy, she was put on a chemoradiotherapy regimen. A histopathologic assessment of the specimen revealed Homer-Wright rosettes against a background of fibrillary matrix, which indicated a diagnosis of a neuroblastoma. In spite of a low incidence in adulthood, it should be emphasized that all adrenal tumors without any particular characteristics may be a neuroblastoma, which tends to show a very poor prognosis. Hence, such patients should undergo postoperative chemoradiotherapy, which may improve both the remission rates and survival.     

A Giant Adrenal Pseudocyst: Case Report and Review of the Literature

A 45-year-old woman whose MR images revealed a lobulated, complicated cyst with septations on the superior pole of left kidney underwent retroperitoneoscopic cyst marsupialization. In pathologic examination beneath the atrophic adrenal gland, hyalinized, calcification foci cyst wall without any surrounding epithelium was seen microscopically. It was reported to be “adrenal pseudocyst”.     

肾上腺钙化性纤维性肿瘤1例报告及文献复习

探讨肾上腺钙化性纤维性肿瘤（CFT）的临床表现、病理学变化及鉴别诊断方法,并讨论其生物学行为。方法：回顾性分析1例肾上腺CFT患者的临床资料,并结合国内外文献对本病的临床表现、病理学特点、鉴别诊断及治疗方法和预后进行讨论。结果：患者出现腰痛及反复肉眼血尿;病理形态学的主要特点为肿瘤境界清楚,细胞成分稀少,肿瘤主要由胶原化纤维构成,可见散在钙化或砂粒体及小团淋巴细胞、浆细胞浸润。免疫组织化学显示梭形细胞vimentin阳性,desmin、ALK、CD34、MSA、S-100均阴性。结论：肾上腺CFT需与其它肾上腺梭形细胞肿瘤相鉴别。本瘤生物学行为良性,局部肿瘤切除即可治愈。     

Right Adrenal Metastases of Hepatocarcinoma After Liver Transplantation: Case Report and Literature Review

Background

Hepatocellular carcinoma (HCC) represents one of the most common malignancies globally, accounting for nearly one million new cases per year. Although the treatment of extrahepatic metastases from primary liver tumors is essentially palliative, a solitary metastasis from such tumors offers a possibility of cure by surgical resection. The adrenal gland is an uncommon site for metastasis from primary liver tumors.

Methods

We report a liver transplantation case of HCC and hepatitis B virus in a 23-year-old man with an excellent postoperative result. However, because an increased alpha-fetoprotein was evident and complete radiologic and blood tests were performed, all of which were normal. Three years posttransplantation, a right adrenal mass was identified by CT. PAAF was performed as well as adrenalectomy for a solitary adrenal metastasis from hepatocellular carcinoma.

Results

The patient underwent adrenalectomy for the right adrenal metastasis at 3 years following liver transplantation for HCC. He is presently alive and disease-free 24 months after adrenalectomy.

Conclusion

Carefully selected patients with solitary metastasis from HCC may be considered for resection.     

肾上腺小细胞神经内分泌癌1例报告并文献复习

目的：探讨肾上腺小细胞神经内分泌癌的起源、诊断要点和治疗方法,以提高对该病的认识。方法：回顾性分析我院收治的一例肾上腺小细胞神经内分泌癌患者,结合国内外文献对本病进行复习总结。结果：本病起源于神经内分泌细胞,临床表现不典型,免疫组化NSE、CgA和Syn可呈阳性,诊断前需排除转移癌,早期发现并手术治疗可能收到良好效果。结论：肾上腺小细胞神经内分泌癌是一种罕见的高度恶性肿瘤,临床表现缺乏特异性,组织学特点和免疫组化神经内分泌标志物是诊断的主要依据,需与肾上腺转移癌相鉴别。可借鉴肺小细胞癌的治疗模式,早期诊断治疗可能是提高其疗效的关键。     

Erythropoietic Protoporphyria: A Case Report and Literature Review

Erythropoietic protoporphyria is considered a rare disease overall, but in children is the most common form of porphyria, and certainly the most common type of erythropoietic porphyria. Despite this fact, erythropoietic protoporphyria is a disease that has been known to evade or at least delay diagnosis, leading to unnecessary suffering by the patient. Given the distress it may cause a patient and his or her family as well as the potential complications of this disease, the importance of maintaining a heightened awareness when presented with a child complaining of photosensitivity cannot be overstated. This case report will review the important clinical indicators, pathogenesis, histology, diagnosis, management, and treatment of this disease, so that affected children will no longer have to play “hide and seek” when diagnosed with this sun-sensitive disease.There are several types of cutaneous porphyrias that are grouped into one of two categories: hepatic porphyrias and erythropoietic porphyrias. In erythropoietic porphyrias, the excess of porphyrins is mainly found in the red cells.¹ Erythropoietic protoporphyria (EPP) is a type of erythropoietic porphyria and is the most common porphyria found in children.² EPP was first clearly defined in 1961 by I.A. Magnus et al³ and is clinically characterized by photosensitivity to visible light with subsequent physical cutaneous signs in the skin exposed to sun. Photosensitivity with cutaneous lesions usually presents in infancy or early childhood. However, there have been cases of delayed diagnosis resulting from either a late onset of symptoms, patients with only mild symptoms, or simply from a failure to diagnose. As a result, dermatologists must employ a keen awareness for EPP in children with subjective photosensitivity even without associated clinical cutaneous findings.     

A Giant Lymphatic Cyst of the Adrenal Gland: Report of a Rare Case and Review of the Literature

Lymphatic type of adrenal cysts is most common; however, this type of endothelial cyst is quite rare in excessively large adrenal cysts. A 37-year-old Japanese woman was admitted to our institution with distension of her left flank and the upper quadrant of her abdomen. Abdominal ultrasonography revealed a cystic lesion with a homogenous anechoic texture, and measuring 21 cm in diameter. Computed tomography and magnetic resonance imaging displayed a giant cystic lesion adjacent to the liver, pancreas, kidney, and spleen. The origin of the cyst was not identified. We were not able to make a preoperative diagnosis; therefore, the patient underwent resection of the mass by open laparotomy for therapeutic diagnosis. Intraoperatively, the mass was identified to be cystic and adhered to the left adrenal gland. It was filled with more than 2000 mL of serous brown-red fluid. The content of the cyst contained no atypical cells on cytological examination. The wall of the cyst was composed of a lining of a single layer of lymphatic vessel–derived cells, and the cyst was pathologically classified as a true cyst. No abdominal symptoms were observed and a postoperative radiological work-up showed no evidence of recurrence during a 6-year follow-up period. We describe a case of a patient with a giant lymphatic cyst of the adrenal gland. The preset data suggest that surgeons should decide treatment strategy for large adrenal cysts in consideration of hormonal function, degree of size, and possibility of malignancy.Key words: Giant adrenal cyst, Adrenal gland, Lymphatic cyst, Endothelial cystCystic lesions of the adrenal gland are relatively rare. In 1903, Doran suggested that the first case of an adrenal cyst was described by Greiselius in 1670.¹ In an autopsy series, the incidence of adrenal cysts was reported to be 0.06 to 0.18%.² The actual frequency remains unknown; however, the detection rate of adrenal cysts has risen dramatically due to frequent application of new imaging modalities such as computed tomography (CT) and magnetic resonance imaging (MRI), which accounts for the identification of approximately 5% of incidental adrenal lesions.³ As patients with small adrenal cysts seldom have any symptom, these cysts are rarely recognized. Small adrenal cysts are usually clinically silent and incidental,⁴ whereas large adrenal cysts—especially greater than 10 cm in diameter—are associated with symptoms because of the mass effect and compression of adjacent organs.⁵ Cases of excessively large adrenal cysts have been reported sporadically, only few dozens of adrenal cysts over 20 cm in diameter have been found.^5–28 Moreover, the lymphatic type of endothelial cyst of the adrenal gland is quite rare in excessively large adrenal cysts.^20,22 Here, we report a rare case of a giant lymphatic cyst of the adrenal gland in a middle-aged woman and summarize the current clinicopathological information on excessively large adrenal cysts and lymphatic type of endothelial cysts.     

Intrasalivary Thymic Carcinoma: A Case Report and Literature Review

Ectopic thymic carcinomas are rarely diagnosed in the thyroid gland, let alone in extrathyroid tissues. In the currently available literature, only five cases of extrathyroidal malignancies with thymic differentiation have been reported as arising in the major salivary glands. A 69-year-old female presented with a slow-growing palpable mass in the left parotid gland. Fine needle aspiration biopsy suggested metastatic cancer, whereas core needle biopsy revealed high-grade squamous cell carcinoma. The patient underwent left radical parotidectomy with selective ipsilateral lymph node dissection and subsequent radiation therapy. The surgical specimen was taken for histopathological examination. Microscopically, the tumor resembled thymic carcinoma. It was composed of large nests of squamoid cells with smooth contours, focally with a syncytial growth pattern, and accompanied by abundant lymphocytes with reactive lymphoid follicles. This appearance resembled a micronodular thymic carcinoma with lymphoid hyperplasia. Moreover, the tumor displayed expression of squamous markers (p40 and p63) and markers of thymic carcinoma (CD5 and CD117). Therefore, the final diagnosis of intrasalivary thymic carcinoma was rendered. The molecular analysis including next-generation sequencing demonstrated no variants of the strong, potential, or unknown clinical significance. The patient remains disease-free at 1-year follow-up. In the current case, we comprehensively present a clinical, microscopic, molecular, and radiological picture of CD5-positive squamous cell carcinoma of the parotid. We postulate that similar cases should be designated as intrasalivary thymic carcinoma analogically to similar thyroid tumors. Our case and the limited literature data indicate they should be distinguished from conventional squamous cell carcinoma of major salivary glands due to their rather favorable prognosis.