Giant Adrenal Myelolipoma: Report of a Case

Adrenal myelolipomas are rare benign tumors composed of mature adipose tissue and hematopoietic elements that resemble bone marrow. They are usually asymptomatic, and most cases are incidentally found at radiological examination or autopsy. Symptoms such as abdominal pain and increasing girth occur only when the tumor grows large. We report the case of a giant adrenal myelolipoma in a 51-year old man who presented with a huge abdominal mass and abdominal pain. The resected tumor weighed 6000g and could represent the largest such tumor ever documented in the literature. We discuss the diagnosis and treatment of this unusual tumor.     

肾上腺髓性脂肪瘤的诊断与治疗

目的：探讨肾上腺髓性脂肪瘤的影像学特点和诊治方法.方法：肾上腺肿瘤患者12例.男5例,女7例.平均年龄48（33～64）岁.左侧4例,右侧8例.血压高2例.腰腹部疼痛1例,无特异性症状9例.行B超、CT及MRI检查.术前提示为肾上腺占位,其中肾上腺髓性脂肪瘤10例.嗜铬细胞瘤1例,肾上腺肿瘤1例.12例均行肾上腺肿瘤切除术,其中开放手术7例.腹腔镜手术5例.结果：12例手术均顺利完成.平均手术时间1.5 h,平均术中出血量50 ml.术后无明显外科并发症,平均7天顺利出院.切除肿瘤最小3.0 cm×3.5 cm×4.0 cm.最大6.5 cm×7.5 cm×8.0 cm.病理诊断均为肾上腺髓件脂肪瘤.术后随访0.5～8年（平均2年）.所有患者临床症状消失,B超及CT检查末见肿瘤复发及恶性变.结论：肾上腺髓件脂肪瘤多无特异性临床表现,术前诊断主要靠影像学检查,确诊依赖于病理检查 手术切除预后良好,其中腹腔镜手术创伤小,疗效满意,为治疗本病的理想方法.     

The Natural History and Treatment of Adrenal Myelolipoma

Purpose

We determined the natural history and clarified the treatment of adrenal myelolipoma.

Materials and Methods

A retrospective review of medical records and radiographic imaging studies of 20 patients diagnosed with adrenal myelolipoma was performed.

Results

Of 20 patients 4 presented with abdominal pain and 1 had Cushing's syndrome. The remaining tumors were discovered incidentally. Four patients underwent surgery because of abdominal pain in 2, adrenal hyperfunction (Cushing's syndrome) in 1 and a tumor 10.5 cm. in largest dimension in 1. Of 15 patients (16 adrenal myelolipomas) followed without surgical intervention for an average of 3.2 years (range 0.3 to 10.8) 13 remained asymptomatic and 2 experienced persistent, vague abdominal discomfort. One patient was lost to followup. A total of 13 tumors from 12 patients was serially imaged, with tumor size increasing in 6, decreasing in 2 and remaining unchanged in 5.

Conclusions

These data suggest that the majority of adrenal myelolipomas can be treated conservatively. While tumors can become enlarged, they also exhibit variable growth, and size and growth rate do not necessarily correlate with symptoms. Computerized tomography can be used for diagnosis.     

肾上腺转移瘤(附7例报告)

报告7例肾上腺转移肿瘤的诊断和治疗,原发肿瘤肾癌2例,其中1例为在左侧肾癌右肾上腺发生转移瘤。肺肿瘤3例,肺腺癌1例,肺腺鳞癌1例,肺母细胞1例。乳腺癌双肾上腺转移1例,十二指肠乏特氏壶腹癌1例。转移瘤无特异的临床症状,结合病史和影像检查结果作出诊断,6例行转移瘤切除治疗,转移瘤病理与原发肿瘤相符。转移瘤切除有利于提高患者的生活质量,延长生存期。     

Adrenal Tumors in Adults

Although most adrenal tumors are not diagnostic dilemmas, there are cases that are challenging. This may be due to the tissue provided, for example fragmented tissue received in the setting of morcellation, or it may be due to inherently challenging histology, such as in cases with equivocal features of malignancy. Additionally, much has been learned about the molecular alterations of adrenal tumors, especially pheochromocytomas. Many of these alterations represent germline mutations with significant clinical implications for patients and their families. The aim of this review is to provide an overview of the most common adrenal tumors in adults so that pathologists can tackle these interesting tumors.     

肾上腺髓样脂肪瘤6例报告及文献复习

目的：探讨肾上腺髓样脂肪瘤的诊断、治疗。方法：收治6例肾上腺髓样脂肪瘤的患者。4例行B超和MRI检查,3例行CT检查。3例行肾素-血管紧张素-醛固酮和皮质醇的测定;2例行儿茶酚胺的测定。6例患者全部接受手术治疗,其中腹腔镜手术切除4例,开放手术2例。结果：6例手术均获得成功。术后病理均诊断为肾上腺髓样脂肪瘤。术前具有的相关症状,术后均消失。5例术后获得随访时间为3个月～3年,均未见复发。结论：肾上腺髓样脂肪瘤术前诊断主要依赖于影像学检查;在治疗上有症状者宜手术切除,无症状且肿瘤直径大于4cm者可考虑手术,手术方式以腹腔镜手术为首选。     

心脏肿瘤181例外科治疗的临床分析

目的 探讨心脏肿瘤的外科治疗方法,分析其临床治疗效果.方法 回顾性分析1980年1月至2010年12月在第三军医大学新桥医院接受外科治疗181例心脏肿瘤患者的临床资料,其中男79例,女102例;年龄10个月～76岁(45.22±18.21)岁.179例患者在体外循环下行180次瘤体摘除术;2例恶性肿瘤仅施行开胸探查,瘤体未能切除,瘤体切除后进行病理分析. 结果 全组原发性心脏肿瘤169例(93.4％),其中粘液瘤144例(79.6％),其它原发性良性心脏肿瘤20例(11.0％),原发性恶性心脏肿瘤5例(2.8％);继发性心脏肿瘤12例(6.6％).原发性心脏肿瘤患者围手术期死亡2例(1.1％),1例死于低心排血量综合征,1例死于术后多器官功能衰竭;5例原发性恶性肿瘤患者术后12个月内均死亡;良性肿瘤患者随访6个月～15年(2.41±1.08)年,无复发.继发性心脏肿瘤患者围手术期死亡1例,死亡原因为术后多器官功能衰竭;2例在术后1年内死亡,3例术后第3年死亡. 结论 粘液瘤是最常见的心脏肿瘤;外科手术彻底摘除瘤体是治疗心脏肿瘤的最佳方案;良性肿瘤的手术疗效明显好于恶性肿瘤,原发性恶性心脏肿瘤外科治疗效果较差.     

小肠肿瘤244例临床分析

为探讨小肠肿瘤的临床表现及诊断治疗方法,对244例小肠肿瘤的临床资料进行回顾性分析。结果显示。244例中临床表现为腹痛88例,腹部肿块99例,出血32例,肠梗阻25例。所有患者均经手术证实。肿瘤位于十二指肠20例,空肠126例,回肠98例,术前诊断符合率52％,恶性肿瘤163例根治切除率为54．6％（89／163）,其中150例获随访,死亡82例,平均存活（36±16）个月。结果表明,辅助检查对小肠肿瘤诊断符合率低,患者膝胸位和侧卧位腹部检查发现可移动性腹部肿块是诊断小肠肿瘤的一个重要手段。小肠肿瘤临床表现极不典型,由于受检查手段的限制早期诊断极其困难,医师加强对本病的重视和认识是改善患者预后的关键。     

Central Bisegmentectomy for Malignant Liver Tumors: Experience in 8 Patients

Central bisegmentectomy (CBS) of the liver is an en bloc hepatic resection of Couiaud segments 4, 5, and 8. The indications for CBS include benign and malignant tumors occupying both the left medial and right anterior segments. However, CBS has rarely been reported. Here, we investigate CBS in patients with suboptimal liver function for whom an extended lobectomy is not an optimal solution. Each case was 1 of 8 patients who underwent CBS for hepatocellular carcinoma (HCC) or colorectal cancer liver metastasis (CRLM) at the Department of Surgery, Jikei University Hospital. Indications for CBS consisted of CRLM in 3 patients and HCC in 5 patients. The median duration of operation was 552 minutes, and median blood loss was 2263 g. No postoperative nor in-hospital mortalities occurred. In this study, 1-, 2-, and 3-year disease-free survival rates were 62.5%, 12.5%, and 12.5%, respectively, and 1-, 2-, and 3-year overall survival rates were 100%, 100%, and 85.7%, respectively. CBS is advocated for central liver tumors in patients with suboptimal liver function for whom extended lobectomy could result in less than optimal remnant liver volume and function.Key words: Central bisegmentectomy, Colorectal cancer liver metastasis, Hepatocellular carcinomaCentral bisegmentectomy (CBS) of the liver is an en bloc hepatic resection of Couiaud segments 4, 5, and 8.¹ McBride and Wallace² first reported this procedure in 1972. The indications for CBS include benign and malignant tumors occupying both the left medial and right anterior segments. The traditional procedure for such tumors is extended right or left lobectomy or trisegmentectomy. Because of the possibility of conservation of remnant liver parenchymal volume, CBS may be superior to extended lobectomy or trisegmentectomy, especially for patients with low residual liver function due to viral hepatitis or adjuvant chemotherapy. However, CBS has rarely been reported. We herein report our experience with 8 patients who underwent CBS for colorectal cancer liver metastasis (CRLM) or hepatocellular carcinoma (HCC).     

后腹腔镜手术治疗肾上腺髓质脂肪瘤的疗效观察(附12例报告)

目的:探讨后腹腔镜手术治疗肾上腺髓质脂肪瘤的疗效及临床意义。方法:回顾性分析12例肾上腺髓质脂肪瘤行后腹腔镜手术患者的临床资料。患者年龄33～72岁,平均53岁;左侧3例,右侧9例;偶发10例,因阵发性高血压、腰疼检查发现各1例。术前均诊断为肾上腺髓质脂肪瘤,肿瘤直径2～8 cm,平均4.9 cm;其中8例直径大于3.5 cm,4例小于3.5 cm。12例患者均在全麻下行后腹腔镜肾上腺肿物切除术。结果:12例患者手术均获成功,手术时间60～270 min,平均130 min。出血量10～660 ml,平均116 ml。术中无并发症。术后住院4～10天,平均5.6天。随访时间6个月～5年,平均2年,未出现肿瘤复发。结论:采用后腹腔镜手术治疗肾上腺髓质脂肪瘤安全有效,具有创伤小、出血少、住院时间短等优点,可以作为肾上腺髓质脂肪瘤的主要手术方法。     

Laparoscopic Resection of Large Adrenal Tumors

Background:

Laparoscopic adrenalectomy has rapidly replaced open adrenalectomy as the procedure of choice for benign adrenal tumors. It still remains to be clarified whether the laparoscopic resection of large (≥8cm) or potentially malignant tumors is appropriate or not due to technical difficulties and concern about local recurrence. The aim of this study was to evaluate the short- and long-term outcome of 174 consecutive laparoscopic and open adrenalectomies performed in our surgical unit.

Methods:

Our data come from a retrospective analysis of 174 consecutive adrenalectomies performed on 166 patients from May 1997 to December 2008. Fifteen patients with tumors ≥8cm underwent laparoscopic adrenalectomy. Sixty-five patients were men and 101 were women, aged 16 years to 80 years. Nine patients underwent either synchronous or metachronous bilateral adrenalectomy. Tumor size ranged from 3.2cm to 27cm. The largest laparoscopically excised tumors were a ganglioneuroma with a mean diameter of 13cm and a myelolipoma of 14cm.

Results:

In 135 patients, a laparoscopic procedure was completed successfully, whereas in 14 patients the laparoscopic procedure was converted to open. Seventeen patients were treated with an open approach from the start. There were no conversions in the group of patients with tumors >8cm. Operative time for laparoscopic adrenalectomies ranged from 65 minutes to 240 minutes. In the large adrenal tumor group, operative time for laparoscopic resection ranged from 150 minutes to 240 minutes. The postoperative hospital stay for laparoscopic adrenalectomy ranged from 1 day to 2 days (mean, 1.5) and from 5 days to 20 days for patients undergoing the open or converted procedure. The mean postoperative stay was 2 days for the group with large tumors resected by laparoscopy.

Conclusion:

Laparoscopic resection of large (≥8cm) adrenal tumors is feasible and safe. Short- and long-term results did not differ in the 2 groups.     

腹腔镜下巨大肾上腺肿瘤切除术7例报告

目的：探讨腹腔镜下肾上腺巨大肿瘤（直径≥6 cm）切除术的技术方法和应用价值.方法：7例肾上腺肿瘤患者均行腹腔镜下肾上腺肿瘤切除术,在瘤体表面相对无血管区处切开包膜,沿包膜与瘤体之间迅速分离,切除肿瘤,填塞纱布止血.结果：5例腹腔镜下肾上腺肿瘤切除术顺利完成,1例手助腹腔镜下完成,1例粘连明显转开放手术.平均手术时间120 min;平均术中出血量200 ml.术后1～4天肠功能恢复,3～5天拔除引流管;无明显外科并发症,术后住院平均10天.术后病理诊断嗜铬细胞瘤4例,肾上腺囊肿1例,神经节细胞瘤1例,畸胎瘤1例.术后随访6（3～18）个月,临床症状消失,复查彩超及SCT未见肿瘤复发及恶性变.结论：腹腔镜下肾上腺肿瘤切除术创伤小、恢复快、效果满意,是治疗巨大肾上腺肿瘤的一种理想术式.     

肾上腺恶性肿瘤的诊治（附34例报告）

目的：探讨肾上腺恶性肿瘤的诊断和治疗方法。方法：回顾性分析34例肾上腺恶性肿瘤的临床资料。其中肾上腺皮质癌13例;恶性嗜铬细胞瘤8例;肾上腺转移癌8例;皮质癌并发同侧肾盂癌1例;节神经母细胞瘤1例;恶性纤维组织瘤1例;脂肪肉瘤1例;肾上腺髓外浆细胞瘤1例。全部34例患者中,有临床症状者23例,有内分泌功能改变者22例。结果：所有患者均接受手术治疗,其中29例肿瘤全切,4例肿瘤小部分或包膜残留,1例仅探查取活检。32例获得随访,时间为2个月-5年。死亡11例,死亡患者生存期为5-29个月,平均21个月。结论：对于肾上腺恶性肿瘤患者。手术切除是首选治疗方法,并且术后的随访很重要。肾上腺皮质癌患者总体预后较差,化疗和放疗可作为术后辅助治疗。恶性嗜铬细胞瘤患者预后较好,MIBG和酚苄明可改善患者生存。对于转移性肾上腺肿瘤患者,手术切除孤立的转移灶可明显提高患者的5年生存率。     

Gip-2 Codon 179 Oncogene Mutations: Absent in Adrenal Cortical Tumors

p < 0.05, Fishers’ exact) previously reported but agrees with the report showing 0 of 56 mutations. We conclude a mutation at position 179 of Gi ₂ α is not important in the pathogenesis of most adrenal cortical tumors.     

Robotic Versus Laparoscopic Resection of Large Adrenal Tumors

Background

Although recent studies have shown the feasibility and safety of robotic adrenalectomy, an advantage over the laparoscopic approach has not been demonstrated. Our hypothesis was that the use of the robot would facilitate minimally invasive resection of large adrenal tumors.

Methods

Adrenal tumors ??5?cm resected robotically were compared with those removed laparoscopically from a prospective institutional review board-approved adrenal database. Clinical and perioperative parameters were analyzed using t and chi-square tests. All data are expressed as mean?±?standard error of mean.

Results

There were 24 patients with 25 tumors in the robotic group and 38 patients with 38 tumors in the laparoscopic group. Tumor size was similar in both groups (6.5?±?0.4 [robotic] vs 6.2?±?0.3?cm [laparoscopic], P?=?.661). Operative time was shorter for the robotic versus laparoscopic group (159.4?±?13.4 vs 187.2?±?8.3 min, respectively, P?=?.043), while estimated blood loss was similar (P?=?.147). The conversion to open rate was less in the robotic (4%) versus the laparoscopic (11%) group; P?=?.043. Hospital stay was shorter for the robotic group (1.4?±?0.2 vs 1.9?±?0.1?days, respectively, P?=?.009). The 30-day morbidity was 0 in robotic and 2.7% in laparoscopic group. Pathology was similar between groups.

Conclusions

Our study shows that the use of the robot could shorten operative time and decrease the rate of conversion to open for adrenal tumors larger than 5?cm. Based on our favorable experience, robotic adrenalectomy has become our preferred minimally invasive surgical approach for removing large adrenal tumors.