Hepatic mesenchymal hamartoma and infantile hemangioma: a rare association

Background/PurposeThe association between liver infantile hemangioma and mesenchymal hamartoma is rare. This communication reports the clinical, radiologic, and pathologic features of 3 infants with concurrent liver hemangioma and mesenchymal hamartoma.MethodsWe conducted a comprehensive search of the database of our Vascular Anomalies Center over the past 12 years (1999-2010) for the keywords mesenchymal hamartoma, liver, and hemangioma in a large cohort of patients with liver hemangioma. Medical records and imaging studies of multiple modalities were reviewed. The search included infants with histopathologically proven liver hemangioma and mesenchymal hamartoma confirmed by the clinical and imaging findings.ResultsThere were 112 children with the diagnosis of liver hemangioma; 3 of them (0.027%) had concurrent mesenchymal hamartoma. Mesenchymal hamartoma predates the appearance of multifocal liver hemangiomas in 1 patient.ConclusionsThe rare coexistence of liver infantile hemangioma and mesenchymal hamartoma could be a true association.     

Spectrum of hepatic hemangiomas: management and outcome

Purpose

Infants with multiple cutaneous hemangiomas often present with hepatic hemangiomas. They can follow a benign clinical course or require complex management. We reviewed our experience in the management of hepatic hemangiomas.

Methods

We performed a retrospective review of patients (1996-2007) with hepatic hemangiomas treated in our institution.

Results

Twenty-six patients were diagnosed with hepatic hemangiomas as follows: 8 focal, 12 multiple, and 6 diffuse lesions. Nineteen (73%) patients had associated cutaneous hemangiomas. Sixteen patients had multiple and 3 patients had single cutaneous hemangiomas. All patients with multiple or diffuse liver lesions were screened for heart failure and hypothyroidism. Congestive heart failure developed in 4 patients, 3/4 of these patients had diffuse lesions. Two patients required thyroid replacement because of elevated thyroid-stimulating hormone. Because of progression of disease, 9 patients required steroid treatment. Two patients were treated with vincristine and 3 patients received α-interferon because of poor response to steroid treatment. Two patients went on to surgical resection for failed response to medical management and worsening heart failure (left lobectomy, liver transplant). Both patients had uncomplicated postoperative courses. Five patients had a previously undescribed constellation of rapidly involuting cutaneous hemangiomas (gone by 3 months, glut-1-negative) with associated liver lesions also resolving at a faster pace (mean resolution of cutaneous hemangiomas, 1.9 vs 7.9 months; P = .001; liver, 5.8 vs 25.3 months; P = .004). All patients in our series survived.

Conclusion

Patients with multiple cutaneous hemangiomas should be screened for hepatic lesions. Patients with diffuse or multifocal liver hemangiomas should be screened for congestive heart failure and hypothyroidism. A subgroup of rapidly involuting cutaneous hemangiomas have a significantly shorter time for involution of hepatic lesions. The status of cutaneous lesions can be used as indicators for the liver hemangiomas.     

Interleukin 8 correlates with intestinal involvement in surgically treated infants with necrotizing enterocolitis

PurposeThe aim of this study was to test the predictive value of interleukin (IL) 8 in the assessment of intestinal involvement in necrotizing enterocolitis (NEC).MethodsForty infants with surgically treated NEC were classified into 3 groups based on intestinal involvement during laparotomy: focal (n = 11), multifocal (n = 16), and panintestinal (n = 13). Preoperatively obtained serum levels of IL-8, C-reactive protein, white blood cell count, and platelet count were correlated with intestinal involvement using logistic regression models.ResultsInterleukin 8 correlated significantly with intestinal involvement in infants with surgically treated NEC (odds ratio, 1.74; confidence interval, 1.27-2.39; P < .001). An exploratory IL-8 cutoff value of 449 pg/mL provided a specificity of 81.8% and sensitivity of 82.8% to discriminate focal from multifocal and panintestinal disease. An IL-8 cutoff value of 1388 pg/mL provided a specificity of 77.8% and a sensitivity of 76.9% to discriminate panintestinal disease from focal and multifocal disease.ConclusionsTo our knowledge, this is the first study to demonstrate a significant correlation of IL-8 with intestinal involvement in advanced NEC in a large patient population. Our results indicate that IL-8 may be a promising biomarker for assessing intestinal involvement in infants with advanced NEC.     

Dynamic Contrast Enhanced Magnetic Resonance Imaging of the Breast Is Superior to Triple Assessment for the Pre-Operative Detection of Multifocal Breast Cancer

Background: Inadequately treated multifocal and multicentric disease results in increased local recurrence following breast-conserving surgery. The accurate preoperative diagnosis of multifocal/centric breast cancer would facilitate the planning of appropriate surgery and prevent reoperation for residual disease. While triple assessment remains the established diagnostic technique, its sensitivity for the diagnosis of multifocal disease remains poor. Dynamic contrast enhanced Magnetic Resonance Imaging (DCE-MRI) of the breast represents an alternative emerging diagnostic modality that has been shown to be highly sensitive for the delineation of primary breast cancer. The aim of this study was to prospectively compare the diagnostic accuracy of DCE-MRI of the breast with conventional triple assessment for the preoperative diagnosis of multifocal/centric breast cancer.Methods: Patients were recruited from the symptomatic breast clinics. All patients underwent standard triple assessment and DCE-MRI. The MRI scans were reported by a single radiologist blinded to the results of the triple assessment. Surgery was then planned accordingly to all available scan results and the specimens examined by a single pathologist. All patients who did not undergo surgery have been followed up for a minimum of 18 months.Results: A total of 334 women were recruited. There were 178 (52%) cancers that were histologically confirmed and multifocal/centric breast cancer was diagnosed provisionally by the preoperative investigations in 68 (38%); multifocal n = 33, multicentric n = 35, of these patients. In this group, subsequent histology confirmed multifocal/centric disease in 50 (73.5%): multifocal n = 15, multicentric n = 35. Unifocal cancer was found in 15 (22%) and benign disease in 3 (4.4%). The resultant sensitivity, specificity, positive, and negative predictive values were 18%, 100%, 100%, and 76% for triple assessment and 100%, 86%, 73%, and 100% for DCE-MRI.Conclusion: DCE-MRI identified a subgroup of breast cancer patients with multifocal/centric disease not evident on standard triple assessment. MRI of the breast should be considered for the preoperative planning of surgery for primary breast cancer.Presented at the 1999 Annual Meeting of the Society of Surgical Oncology, Orlando, Florida, March 4–7, 1999.     

PSMA expression in Schwannoma: a potential clinical mimicker of metastatic prostate carcinoma

ObjectivesRadioimmunoscintigraphy using a radiolabeled antibody against prostate-specific membrane antigen (PSMA) is frequently used to detect prostate carcinoma (PCa) recurrence and metastasis to lymph nodes, soft tissues, and bone. PSMA expression has been shown in occasional nonprostatic neoplasms (e.g., urothelial adenocarcinoma) and in the vasculatures of other malignancies. PSMA expression has not been described in benign neoplasms. Recently, during evaluation of a prostatic carcinoma patient, we encountered a false positive PSMA radioimmunoscintigraphy scan in a pathologically confirmed Schwannoma (SCH) lesion. The current study further evaluates PSMA expression in Schwannomas.MethodsEleven SCH were retrieved from our surgical pathology archives. Representative sections were immunostained with monoclonal antibody for PSMA. PSMA expression was evaluated in tumor cells and lesional vessels. Extent of staining was calculated as percent of positive cells in highest areas of expression. Positive staining was considered focal, multifocal, or diffuse based on the percent of positive cells: <5%, 5% to 75%, and >75%, respectively.ResultsAll 11 SCH showed tumoral and or vascular staining; 7 (7/11) displayed both vascular and tumoral cell staining; the remaining 4 had only vascular staining (2/11) or tumor cell staining (2/11). The extent of tumoral cell and vascular staining varied widely among lesions (tumor cells: focal in 8 and diffuse in 1; vascular: focal in 7, multifocal in 1, and diffuse in 1 lesion).ConclusionThis is the first report of PSMA expression in a benign neoplasm. Given our finding of frequent expression of PSMA in Schwannomas, they should be clinically considered in the differential diagnosis of a lesion that is positive on PSMA radioimmunoscintigraphy study performed during a metastatic work-up of PCa patient.     

基于Faster R-CNN及增强CT图像自动检测肝血管瘤

目的 基于快速区域卷积神经网络（Faster R-CNN）构建肝血管瘤自动检测系统,观察其检出增强CT图像中的肝血管瘤的效能。方法 收集经腹部增强CT诊断的128例肝血管瘤患者、共2 304幅增强CT图像,按8∶2比例将其分为训练集（n=102）和测试集（n=26）,分别含1 836幅及468幅增强CT图像。利用Faster R-CNN、针对增强CT图像构建自动检测肝血管瘤系统,基于迁移学习方案,采用Resnet50预训练分类网络作为提取特征模块的基础骨架,以区域提议网络提取训练集增强CT图像特征,以边界框分类回归模块输出预测边框的精确位置坐标和类别的概率分数。训练过程中绘制Loss曲线,评估模型对训练集的训练效果及其稳定性;采用随机梯度下降法作为优化器对参数进行调整,以提升模型性能。通过平均精度均值（mAP）评估系统检出测试集增强CT图像中的肝血管瘤的效能。结果 训练集训练过程损失函数Loss曲线中,自动检测系统呈快速下降趋势,提示模型学习能力良好,预测性能稳定。mAP曲线显示,迭代次数epoch为40~80时,系统对测试集468幅增强CT图像检出肝血管瘤的mAP为0.962~0.973,波动小,提示模型已收敛,自动检测效果良好。结论 基于Faster R-CNN的增强CT图像自动检测系统可有效检出肝血管瘤。     

弥漫型和局限型子宫腺肌病超声消融安全性对比分析

目的评价超声消融治疗弥漫型子宫腺肌病的安全性。方法对308例子宫腺肌病患者行高强度聚焦超声(HIFU)消融治疗,并于术后1天行MR检查,其中弥漫型子宫腺肌病184例(弥漫组),局限型子宫腺肌病124例(局限组)。评估并比较两组的消融治疗效果、术中及术后不良反应及并发症情况。结果对308例患者均顺利完成治疗,消融发生率为99.35%(306/308),弥漫组消融率[(25.47±13.29)%]低于局限组[(45.00±20.44)%],差异有统计学意义(P0.01)。HIFU术中弥漫组较局限组发生皮肤烫的风险增加1.80倍,局限组较弥漫组发生腹股沟区痛的风险增加2.12倍;术后局限组较弥漫组发生下肢感觉异常的风险增加6.28倍。308例中,65例(65/308,21.10%)因并发症接受简单治疗[国际放射治疗协会(SIR)-B级],其中弥漫组与局限组分别为39例(39/184,21.20%)和26例(26/124,20.97%),差异无统计学意义(P0.05)。两组均未发生SIR-C~SIR-F级并发症。结论在严格控制消融范围的情况下,对弥漫型与局限型子宫腺肌病同样行HIFU治疗是安全、可行的。     

HIFU消融治疗弥漫型和局限型子宫腺肌病的近远期疗效

目的比较HIFU治疗弥漫型和局限型子宫腺肌病的近远期疗效。方法收集308例接受HIFU治疗的子宫腺肌病患者,依据术前MRI将其分为弥漫组和局限组,计算消融率及消融发生率,并评估2组患者术前、术后痛经及月经量情况。结果共297例患者进行了有效随访,随访时间1~50个月,其中弥漫组177例,局限组120例。297例患者消融发生率为99.33%(295/297),弥漫组及局限组消融率分别为(26.00±13.36)%、(44.32±19.93)%。2组患者术后痛经及月经量评分与术前比较均有明显降低(P均0.05)。痛经症状总缓解率在术后3、6、12、24及36个月分别为92.96%(264/284)、86.18%(237/275)、73.51%(197/268)、60.71%(136/224)及46.83%(59/126);各随访时段局限组痛经缓解率均高于弥漫组,且在术后6、24及36个月差异有统计学意义(P0.05)。月经量过多症状总缓解率在术后3、6、12、24及36个月分别为87.38%(187/214)、83.09%(172/207)、68.63%(140/204)、63.64%(105/165)及45.92%(45/98);各随访时段局限组均高于弥漫组,但差异均无统计学意义(P均0.05)。结论 HIFU治疗子宫腺肌病疗效显著,局限型与弥漫型近期疗效相当,远期疗效局限型优于弥漫型。     

Risk factors for biochemical recurrence following radical perineal prostatectomy in a large contemporary series: A detailed assessment of margin extent and location

ObjectivesThe implications of positive surgical margin (PSM) extent and location during radical perineal prostatectomy (RPP) have not been assessed in a contemporary series. We aimed to examine the incidence, location, and extent of PSM as well as their impact on biochemical recurrence (BCR) following RPP.Materials and methodsA total of 794 patients underwent RPP by a single surgeon between June 1993 and August 2010. Covariates included age, pathologic T stage, pathologic Gleason sum, preoperative PSA, prostate volume, PSM extent, and location. Life table, Kaplan-Meier, and Cox regression analyses assessed predictors of BCR following RPP.ResultsPSM were recorded in 162 patients (20.4%); of these, 83 (51.2%) were focal (≤1 mm) whereas 79 (48.8%) were broad (>1 mm). Location of PSM was anterior 10.5%, posterior or lateral 14.8%, bladder neck 23.5%, apical 32.1%, and multifocal 19.1%. At a median follow-up of 54 months, the 5-year BCR-free probability was 90.8% in patients with negative margins, 77.5% in patients with focal PSM, and 47.5% in patients with broad PSM. On multivariable analyses adjusted for age, pathologic T stage, pathologic Gleason sum, preoperative PSA, and prostate volume, broad PSM, (HR = 3.49, P < 0.001) as well as anterior (HR = 3.77, P = 0.003), bladder neck (HR = 2.25, P = 0.01) and multifocal (HR = 3.55, P < 0.001) PSM were independent predictors of BCR.ConclusionsIn this study, we present oncologic outcomes following RPP in a large contemporary cohort of patients undergoing RPP. In adjusted analyses, broad and anterior PSM carried the highest risk of recurrence after RPP.     

Laparoscopic transcystic common bile duct exploration versus transgastric endoscopic retrograde cholangiography during cholecystectomy after Roux-en-Y gastric bypass

BackgroundTreatment of common bile duct (CBD) stones after Roux-en-Y gastric bypass (RYGB) poses a particular challenge given the altered anatomy and inability to perform a standard endoscopic retrograde cholangiogram (ERC). The optimal treatment strategy for intraoperatively encountered CBD stones in post-RYGB patients has not been established.ObjectivesTo compare outcomes following laparoscopic transcystic common bile duct exploration (LTCBDE) and laparoscopy-assisted transgastric ERC for CBDs during cholecystectomy in RYGB-operated patients.SettingSwedish nationwide multi-registry study.MethodsThe Swedish Registry for Gallstone Surgery and ERCs, GallRiks (n = 215,670), and the Scandinavian Obesity Surgery Registry (SOReg) (n = 60,479) were cross-matched for cholecystectomies with intraoperatively encountered CBD stones in patients with previous RYGB surgery between 2011 and 2020.ResultsRegistry cross-matching found 550 patients. Both LTCBDE (n = 132) and transgastric ERC (n = 145) were comparable in terms of low rates of intraoperative adverse events (1% versus 2%) and postoperative adverse events within 30 days (16% versus 18%). LTCBDE required significantly shorter operating time (P = .005) by on average 31 minutes, 95% confidence interval (CI) [10.3–52.6], and was more often used for smaller stones <4 mm in size (30% versus 17%, P = .010). However, transgastric ERC was more often used in acute surgery (78% versus 63%, P = .006) and for larger stones >8 mm in size (25% versus 8%, P < .001).ConclusionsLTCBDE and transgastric ERC have similarly low complication rates for clearance of intraoperatively encountered CBD stones in RYGB-operated patients, but LTCBDE is faster while transgastric ERC is more often used in conjunction with larger bile duct stones.     

肝切除联合术中热消融治疗肝硬化背景下多灶性肝癌

目的探讨肝切除联合术中热消融治疗肝硬化背景下多灶性肝癌的有效性和安全性。方法回顾性分析63例接受肝切除联合术中热消融治疗肝硬化背景下多灶性肝癌患者的临床资料,分析患者的治疗效果及并发症。结果 63例患者共切除病灶85个,病灶平均最大直径(4.66±3.82)cm,热消融治疗病灶146个,病灶平均最大直径(1.48±0.66)cm。146个病灶完全消融率达100%(146/146),局部复发率4.11%(6/146)。患者1、2、3、4年无瘤生存率分别为54.82%、31.28%、12.03%、6.02%;1、3、5年累积生存率分别为83.51%、56.83%、45.20%。未出现治疗相关的死亡病例。肝切除相关严重并发症8例(8/63,12.70%)。未出现热消融相关严重并发症。结论肝切除联合术中热消融治疗肝硬化背景下多灶性肝癌安全、有效,扩大了肝切除术的适应证。     

Classification and prognosis of intrahepatic biliary stricture after liver transplantation.

Intrahepatic biliary stricture (IHBS) after liver transplantation (LT) may develop in patients with hepatic artery thrombosis, chronic rejection, or ABO incompatibility, as well as in patients with prolonged warm ischemia in non-heart-beating donor (NHBD) LT. However, the clinical course and methods of management have not been well defined for IHBSs to date. Thus, the purpose of this study was to provide a classification of post-LT IHBS and to investigate patient prognosis. Forty-four patients who developed IHBS after NHBD LT were enrolled. On the basis of the cholangiographic appearance, patients were classified into 4 groups: unilateral focal (UF, n=8), confluence (CO, n=10), bilateral multifocal (BM, n=21), and diffuse necrosis (DN, n=5). The UF type was defined as cases with stricture only in the segmental branch of the unilateral hemiliver; the CO type in cases with several strictures at confluence level; and the BM type in cases with multiple strictures bilaterally. Cases with diffuse obliteration of peripheral ducts or destruction of the central architectural integrity, over a long segment, were classified as the DN type. Five patients with the CO type required several interventions requiring biliary dilatation, yet all patients with the UF or CO type had a good prognosis. Among the patients with the BM type, 3 patients (14.3%) died or underwent retransplantation due to biliary complications, and 7 (33.3%) required repeated interventions for >1 year without improvement. Moreover, among 5 patients classified as the DN type, 1 (20%) died of biliary sepsis, 2 (40%) underwent retransplantation, and the remaining 2 (40%) did not recover from persistent jaundice and life-threatening cholangitis despite multiple interventions. In conclusion, all patients classified as UF or CO had a good outcome with or without additional interventions. However, all patients with the DN type and about half the patients with the BM type did not recover from life-threatening complications, despite repeated aggressive interventions; early retransplantation was therefore the only treatment option for these patients.     

Interventional treatment of infantile hepatic hemangioendothelioma

Background/Purpose: Hemangioendothelioma is the most frequent liver tumor in infancy. Untreated symptomatic patients with heart failure have a high mortality rate. Symptomatic forms may request nonoperative treatment, because surgery is burdened with high risks in patients with heart failure. The authors report their experience with interventional coil occlusion of infantile hepatic hemangioendothelioma (IHE).Methods: Four patients (age range, 2 to 146 days; mean, 53 days) suffering from IHE associated with heart failure were treated by endovascular coil occlusion of arterial feeders. Catheter intervention was performed via an arterial (n = 2) or venous (n = 2) approach.Results: Signs of heart failure resolved within 2 to 8 days after occlusion in 3 patients. Tumor regression could be observed sonographically within 4 weeks postinterventionally. In 3 children, tumor size was reduced from a mean of 544 mL (65 to 1,350) to a mean of 4 mL (2 to 6); Mean systolic peak velocity in the hepatic artery was decreased from 170 cm/s (140 to 200) before occlusion to 45 cm/s (36 to 70) during follow-up. In the fourth patient, endovascular intervention could not control a rapidly progressing hemangioendotheliomatosis, and finally a liver transplantation had to be performed.Conclusions: Interventional occlusion of feeding arteries in symptomatic IHE is a safe and effective alternative to early open surgery. The efficacy of endovascular intervention in multifocal tumors seems questionable.     

Multifocal hepatoblastoma: What is the risk of recurrent disease in the remnant liver?

PurposeMultifocal hepatoblastoma (HB) is often treated with total hepatectomy and transplantation owing to concerns of surgical resectability, local recurrence, and/or metachronous tumor in the remnant liver. We aimed to review HB patients to determine the risk of local recurrence in multifocal disease.MethodsWe undertook retrospective cohort analysis of all HB patients at a single tertiary referral center between 2001 and 2015. Demographics, diagnostic features, operative details, and outcomes were analyzed.ResultsSixty patients underwent surgical management of HB. 39 had unifocal, and 21 had multifocal disease. Of multifocal patients, 9 underwent liver transplantation, 10 anatomic resections, and 2 nonanatomic resections. Overall, two patients had recurrence in the remnant liver — both from the unifocal group. There were equivalent distant (lung) recurrences between the groups (8% for unifocal versus 14% for multifocal), p = 0.89. At a mean of 75 months of follow-up, overall survival was 97% for unifocal patients and 86% for multifocal patients, p = 0.12.ConclusionMultifocal HB was not associated with increased local recurrence in the setting of R0 resection and chemotherapy. These data do not support the contention that all patients with multifocal HB require a total hepatectomy and transplantation to reduce the incidence of local recurrence and/or metachronous tumor development.Level of evidenceLevel III — Limited cohort analysis.     

Fetal and neonatal hepatic tumors

Background/Purpose

Although hepatic tumors are uncommon in the perinatal period they are associated with significant morbidity and mortality in affected patients. The purpose of this review is to focus on the fetus and neonate in an attempt to determine the various ways liver tumors differ clinically and pathologically from those found in the older child and adult and to show that certain types of tumors have a better prognosis than others.

Methods

The author conducted a retrospective review of perinatal hepatic tumors reported in the literature and of patients treated and followed up at Children's Hospital San Diego and Children's Hospital Los Angeles. Only fetuses and infants younger than 2 months with adequate clinical and pathologic data ere accepted for review. The period of patient accrual was from 1970 to 2005. Length of follow-up varied from 1 week to more than 5 years. Elevated α-fetoprotein level was defined as one significantly higher than that of the reporting institution's normal level for age group; laboratory values for this protein vary from one institution to the next and therefore it was not possible to assign one figure as a standard reference number. Discussion of the differential diagnosis and pathologic findings of hepatic tumors in the fetus and neonate are described elsewhere and will not be discussed here in detail (Perspect Pediatr Pathol 1978;4:217; Weinberg AG, Finegold MJ. Primary hepatic tumors in childhood. In: Finegold M, editor. Pathology of neoplasia in children and adolescents. Philadelphia, PA: WB Saunders, 1986; Am J Surg Pathol 1982;6:693; Pediatr Pathol 1983;1:245; Arch Surg 1990;125:598; Semin Neonatol 2003;8:403; Pediatr Pathol 1985;3:165; Isaacs H Jr. Liver tumors. In: Isaacs H Jr, editor. Tumors of the fetus and newborn. Philadelphia, PA: WB Saunders, 1997; Isaacs H Jr. Liver tumors. In: Isaacs H Jr, editor. Tumors of the fetus and infant: an atlas. Philadelphia, PA: WB Saunders, 2002).

Results

One hundred ninety-four fetuses and neonates presented with hepatic tumors diagnosed prenatally (n = 56) and in the neonatal period (n = 138). The study consisted of 3 main tumors: hemangioma (117 cases, 60.3%), mesenchymal hamartoma (45 cases, 23.2%), and hepatoblastoma (32 cases, 16.5%). The most common initial finding was a mass found either by antenatal sonography or by physical examination during the neonatal period. Overall, hydramnios was next followed by fetal hydrops, respiratory distress, and congestive heart failure, which were often related to the cause of death. Half of the fetuses and neonates with hepatoblastoma had abnormally elevated serum α-fetoprotein levels compared with 16 (14%) of 117 of those with hemangioma and 1 neonate with mesenchymal hamartoma. There were 76 (65%) examples of solitary (unifocal) hemangiomas and 41 (35%) of multifocal (which included the entity diffuse hemangiomatosis) with 86% and 71% survival rates, respectively. Of 45 patients with mesenchymal hamartoma, of the 29 (64%) who had surgical resections, 23 (79%) survived. Patients with hepatoblastoma had the worst outcome of the group, for only 8 (25%) of 32 were alive. Half of patients with either stage 1 or 3 hepatoblastoma died; no patient with stage 4 survived. There was some relationship between histologic type and prognosis. For example, half of the patients with the pure fetal hepatoblastoma histology survived compared with those with fetal and embryonal histology where 30% survived. Fifteen of 32 hepatoblastoma patients received surgical resection with or without chemotherapy, resulting in 7 (47%) of 15 cures. The 56 fetuses and 138 neonates with hepatic tumors (hemangioma, mesenchymal hamartoma, and hepatoblastoma) had survival rates of 75%, 64%, and 25%, respectively. The overall survival of the entire group consisting of 194 tumors was 125 or 64%.

Conclusions

The study shows that clinical findings in fetuses and neonates with hepatic tumors are less well defined than in older children. Survival rates are much lower as well. When the clinical course is complicated by associated conditions such as stillbirth, fetal hydrops, congestive heart failure, severe anemia, or thrombocytopenia, the mortality rate is much greater. If the patient is mature enough and in a clinical condition where he or she can be operated on, survival figures approach those of the older child. Some hepatic tumors have a better prognosis than others. Neonates with focal (solitary) hepatic hemangiomas have the best outcome and fetuses with hepatoblastoma the worst. Although infantile hemangioma undergoes spontaneous regression, it may be life threatening when congestive heart failure and/or consumptive coagulopathy occur. Mesenchymal hamartoma is a benign lesion best treated by surgical resection, which usually results in cure. However, there are fatal complications associated with this tumor, ie, fetal hydrops, respiratory distress, and circulatory problems owing to a large space occupying abdominal lesion and sometimes stillbirth, all contributing to the death rate. Hepatoblastoma, the major malignancy of the fetus and neonate, is treated primarily by surgical resection. Pre- or postoperative chemotherapy is reserved for those patients with unresectable tumors or metastatic disease. The survival rate is much lower than that reported by multigroup prospective trials. Patients die from the mass effect caused by the tumor, which lead to abdominal distension, vascular compromise, anemia, hydrops, respiratory distress, and stillbirth. Metastases to the abdominal cavity, lungs, and placenta are other causes of death. Because of the danger of labor-induced rupture of the tumor and potentially fatal intraabdominal hemorrhage, cesarean delivery is recommended when a hepatic tumor is found on prenatal ultrasound.     

Combined Resection and Radiofrequency Ablation for Advanced Hepatic Malignancies: Results in 172 Patients

Background: Resection combined with radiofrequency ablation (RFA) is a novel approach in patients who are otherwise unresectable. The objective of this study was to investigate the safety and efficacy of hepatic resection combined with RFA.Methods: Patients with multifocal hepatic malignancies were treated with surgical resection combined with RFA. All patients were followed prospectively to assess complications, treatment response, and recurrence.Results: Seven hundred thirty seven tumors in 172 patients were treated (124 with colorectal metastases; 48 with noncolorectal metastases). RFA was used to treat 350 tumors. Combined modality treatment was well tolerated with low operative times and minimal blood loss. The postoperative complication rate was 19.8% with a mortality rate of 2.3%. At a median follow-up of 21.3 months, tumors had recurred in 98 patients (56.9%). Failure at the RFA site was uncommon (2.3%). A combined total number of tumors treated with resection and RFA >10 was associated with a faster time to recurrence (P = .02). The median actuarial survival time was 45.5 months. Patients with noncolorectal metastases and those with less operative blood loss had an improved survival (P = .03 and P = .04, respectively), whereas radiofrequency ablating a lesion >3 cm adversely impacted survival (HR = 1.85, P = .04).Conclusions: Resection combined with RFA provides a surgical option to a group of patients with liver metastases who traditionally are unresectable, and may increase long-term survival.     

应用覆膜血管内支架植入术治疗DeBakey Ⅲ型主动脉夹层

主动脉夹层在急性期死亡率极高,血肿压迫主动脉分支血管时,相应脏器出现缺血症状。传统手术治疗需要阻断主动脉血流,导致内脏或脊髓缺血时间较长,加上人造血管吻合口渗血、术后切口易出现并发症及需要长时间的重症监护等因素,使其应用受到限制。自从1994年Dake     

The predictive value of preoperative fluorine-18-L-3,4-dihydroxyphenylalanine positron emission tomography-computed tomography scans in children with congenital hyperinsulinism of infancy

Background/Purpose

In congenital hyperinsulinism (CHI) of infancy, the use of preoperative fluorine-18-l-3,4-dihydroxyphenylalanine-positron emission tomography-computed tomography (¹⁸F-DOPA-PET-CT) scan has recently been reported. The aim of this study was to evaluate the accuracy of this technique in discriminating between diffuse and focal CHI and the anatomical localization of focal lesions.

Methods

Between 2006 and 2010, ¹⁸F-DOPA-PET scan was performed in 19 children with CHI (median age, 2 months; range, 1-12 months) who were not responding to medical therapy and underwent laparoscopic or open surgery. The findings of ¹⁸F-DOPA-PET scan were correlated with histology.

Results

In 5 children, ¹⁸F-DOPA-PET scan showed diffuse pancreatic uptake, confirmed at histology and supporting the genetic suspicion of diffuse disease. In 14 children, ¹⁸F-DOPA-PET scan indicated focal pancreatic uptake, which corresponded to histology. However, in 5 patients (36%), ¹⁸F-DOPA-PET scan was inaccurate in defining the location of the lesion (n = 3), size of the lesion (n = 1), or both location and size (n = 1), leading to an inaccurate pancreatic resection.

Conclusions

Fluorine-18-l-3,4-dihydroxyphenylalanine-positron emission tomography-computed tomography scan discriminates between diffuse and focal forms of CHI. In focal forms, ¹⁸F-DOPA-PET scan is useful in 2/3 of patients in defining the site and dimension of the focal lesion. Intraoperative histologic confirmation of complete focal lesion resection is needed.     

Hepatic Resection for Metastatic Melanoma: Distinct Patterns of Recurrence and Prognosis for Ocular Versus Cutaneous Disease

Background Resection of melanoma metastatic to the liver remains controversial. We evaluated the efficacy of hepatic resection in patients with metastatic ocular and cutaneous melanoma and assessed factors that could affect survival after resection. Methods Forty patients with hepatic melanoma metastasis underwent resection at four major hepatobiliary centers. Clinicopathologic factors were evaluated with regard to recurrence and survival by using χ² and log-rank tests. Results The primary tumor was ocular in 16 patients and cutaneous in 24. The median disease-free interval from the time of primary tumor treatment to hepatic metastasis was the same for both groups (ocular, 62.9 months; cutaneous, 63.1 months; P = .94). Most patients underwent either an extended hepatic resection (37.5%) or hemihepatectomy (22.5%). Twenty-six patients (65%) received perioperative systemic therapy. Thirty (75.0%) of 40 patients developed tumor recurrence. The median time to recurrence after hepatic resection was 8.3 months (ocular, 8.8 months; cutaneous, 4.7 months; P = .3). Patients with primary ocular melanoma were more likely to experience recurrence within the liver (53.3% vs. 17.4%; P = .015), whereas patients with a cutaneous primary tumor more often developed extrahepatic involvement. The 5-year survival rate for patients with a primary ocular melanoma was 20.5%, whereas there were no 5-year survivors for patients with cutaneous melanoma (P = .03). Conclusions Patterns of recurrence and prognosis after resection of hepatic melanoma metastasis differ depending on whether the primary melanoma is ocular or cutaneous. Resection should be performed as part of a multidisciplinary approach, because recurrence is common.     

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children

Background

Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI.