Oncological Superiority of Hilar En Bloc Resection for the Treatment of Hilar Cholangiocarcinoma

Purpose

Long-term results after liver resection for hilar cholangiocarcinoma are still not satisfactory. Previously, we described a survival advantage of patients who undergo combined right trisectionectomy and portal vein resection, a procedure termed “hilar en bloc resection.” The present study was conducted to analyze its oncological effectiveness compared to conventional hepatectomy.

Patients

During hilar en bloc resection, the extrahepatic bile ducts were resected en bloc with the portal vein bifurcation, the right hepatic artery, and liver segments 1 and 4 to 8. With this “no-touch” technique, preparation of the hilar vessels in the vicinity of the tumor was avoided. The long-term outcome of 50 consecutive patients who underwent curative (R0) hilar en bloc resection between 1990 and 2004 was compared to that of 50 consecutive patients who received curative conventional major hepatectomy for hilar cholangiocarcinoma (perioperative deaths excluded).

Results

The 1-, 3-, and 5-year survival rates after hilar en bloc resection were 87%, 70%, and 58%, respectively, which was significantly higher than after conventional major hepatectomy. In the latter group, 1-, 3-, and 5-year survival rates were 79%, 40%, and 29%, respectively (P = 0.021). Tumor characteristics were comparable in both groups. A high number of pT3 and pT4 tumors and patients with positive regional lymph nodes were present in both groups. Multivariate analysis identified hilar en bloc resection as an independent prognostic factor for long-term survival (P = 0.036).

Conclusions

In patients with central bile duct carcinomas, hilar en bloc resection is oncologically superior to conventional major hepatectomy, providing a chance of long-term survival even in advanced tumors.

     

Changing treatment and outcome of children with hepatoblastoma: analysis of a single center experience over the last 20 years

Background/PurposeThe aim of the study was to analyze changing management and survival of children with hepatoblastoma (HBL) treated in one center.Materials and MethodsOver the last 20 years, 51 children with HBL were treated. Surgery was performed in 48 children (94.1%), conventional liver resection in 38 (of those, 2 received a rescue liver transplantation [LTx] for relapse), and total hepatectomy and primary LTx in 10 patients. The remaining 3 patients received only palliative treatment. Patient data were analyzed for survival with respect to PRETreatment EXTent of disease (PRETEXT), metastases, histopathology, conventional resection, and LTx.ResultsSurvival of children with HBL treated with liver resection is 71% and 80% for primary LTx. Favorable prognostic factors for patient survival was tumor histology as epithelial-fetal subtype and mixed epithelial and mesenchymal type, without teratoid features, and good response to chemotherapy (necrosis, fibrosis). Unfavorable prognostic factors were small cells undifferentiated, transitional liver cell tumor, α-fetoprotein level above 1,000,000 IU/mL and below 100 IU/mL at diagnosis, lung metastases, and local recurrence after initial resection. Survival was related to PRETEXT stage. However, among patients with PRETEXT III and IV, LTx resulted in better survival.ConclusionLiver transplantation is a good option for children with advanced HBL. Early referral of children with potentially unresectable tumors to centers where combined treatment (chemotherapy, surgery including LTx) is available is crucial.     

Extensive Hepatectomy as an Alternative to Liver Transplant in Advanced Hepatoblastoma: A New Protocol Used in a Pediatric Liver Transplantation Center

BackgroundSurgery is a key factor in the treatment of hepatoblastoma, but choosing between an aggressive resection and liver transplant may be an extremely difficult task. The aim of this study was to describe the outcomes of patients with advanced hepatoblastoma: pretreatment extent of disease (PRETEXT)/post-treatment extent of disease (POST-TEXT) III and IV undergoing aggressive resections or living donor liver transplant in cases involving the entire liver. Based on this experience, a new protocol for the treatment of these patients was proposed.MethodsA retrospective study included patients with advanced hepatoblastoma (POST-TEXT III and IV) who were referred for a liver transplant from 2010 to 2017.ResultsA total of 24 children were included: 13 (54.2%) were male, with a median age at diagnosis of 42 months (range, 15–120 months), and a history of prematurity was identified in 20.8% of the patients. Ten cases (41.7%) were staged as PRETEXT/POST-TEXT III, and 12 cases (50.0%) were staged as PRETEXT/POST-TEXT IV. Two patients were referred after posthepatectomy recurrence. Five patients underwent a liver transplant, with recurrence and death in 2 patients (40.0%) within a mean period of 6 months. In the extensive hepatectomy group, there was recurrence in 6 patients (31.6%), with disease-free outcomes and overall survival in 63.2% and 94.7% of patients, respectively.ConclusionIn cases of advanced hepatoblastoma, an extensive surgical approach is a valuable option. The fact that the team was fully prepared to proceed with living donor liver transplant allowed the surgeon to be more aggressive and to switch to transplantation when resection was not possible.     

Survival after liver transplantation for hepatoblastoma: a 2-center experience

Purpose

Complete resection with adjuvant chemotherapy is the accepted treatment for hepatoblastoma. The aim of this study is to evaluate our results of liver transplantation (LT) for tumors still unresectable after adequate chemotherapy.

Methods

All patients transplanted for hepatoblastoma from 2 institutions between 1990 and 2004 were included. Variables reviewed to determine impact on survival included the following: previous tumor resection, metastatic disease at diagnosis, microscopic vascular invasion, α-fetoprotein (AFP) levels at diagnosis and at transplant, tumor histology, and administration of posttransplantation chemotherapy. Effectiveness of pretransplantation chemotherapy was defined as a drop of more than 99% in peak AFP levels.

Results

Fourteen patients were transplanted: 9 boys and 5 girls (age range, 18 months-13 years; mean age, 57 ± 48 months). Patients were transplanted a mean of 4 ± 1 months after diagnosis. Overall survival was 71% (10/14) with a mean follow-up of 46 months. All deaths were secondary to recurrent tumor. Of 10 patients who underwent a primary LT, 9 survived compared to only 1 of 4 transplanted for unresectable tumor recurrence after primary resection (90% vs 25%; P = .02). Decline in peak AFP of more than 99% was also associated with better survival (100% vs 56%; P = .08). Similarly, patients who received posttransplantation chemotherapy had 100% survival compared with 56% without chemotherapy (P = .08). Other variables had little effect on survival.

Conclusions

Liver transplantation is a successful treatment option for children with unresectable hepatoblastoma with a 90% survival rate for primary transplantation. Rescue LT for recurrent hepatoblastoma after previous resection has a poor survival outcome and should be considered a relative contraindication. Posttransplantation chemotherapy improves survival. A prospective multicenter collaboration to validate these findings with a larger patient population is necessary. Until that time, patients who receive rescue transplants should receive posttransplantation chemotherapy.     

Free disease long-term survival in primary thoracic spine leiomyosarcoma after total en bloc spondylectomy: A case report

IntroductionTo describe an unusual primary vertebral leiomyosarcoma in thoracic spine.Presentation of caseAn isolated lesion of the T11 vertebra in a 62-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade malignant lesion. TC-guided biopsy failed thus open incisional biopsy was needed. A diagnosis of low-intermediate mesenchymal sarcoma was made. A total en bloc spondylectomy of T11 was performed with three-column reconstruction. The histology and immunostaining showed the appearance of leiomyosarcoma. After diagnosis, post-operative radiation therapy was performed. Metastatic lesion was ruled out by CT scans of the chest, abdomen and pelvis, in addition to total body radionuclide scanning and 18-F-FDG-PET. After five years of follow-up, no signs of local recurrence, metastasis or distant lesions suggesting a primary lesion were observed.DiscussionVertebral primary leiomyosarcoma is exceedingly rare. Primary vertebral leiomyosarcoma diagnosis must be performed when the metastatic origin is excluded. For the treatment of primary tumors, total en bloc spondylectomy (TES) is the technique of choice to achieve marginal or wide tumor resection, decrease the risk of local recurrence and remote lesions and increase survival.ConclusionsA well-planned pre-operative study and a wide surgical excision can result in local tumor control and long-term survival. This case presents the longest disease-free survival period of a primary leiomyosarcoma in spinal location after total en bloc spondylectomy.     

Surgery for recurrent medulloblastoma: A review

IntroductionMedulloblastoma (MB) is the most common malignant brain tumour in children. Despite significant progress in its management, a proportion of children relapse; tumour recurrence still carries a poor prognosis. While surgery is a mainstay of the management of primary MB, its role in recurrent MB is unclear. The objective of this literature review is to explore current practice and potential benefits of surgery in recurrent MB.Material and methodsWe reviewed all articles published in PubMed and Scholar from 1990 to 2018 with the following terms: “medulloblastoma” AND “recurrence” AND “neurosurgical procedures”. Among 69 articles, 12 were directly relevant.ResultsA total of 581 cases of recurrent MB were identified from published series. Median time from diagnosis to relapse was 20.4 months. The majority of relapses involved disseminated craniospinal disease and only one-fifth relapses was located in the posterior fossa. The outcome was consistently poor, with a median survival of 12.4% and a median survival time after relapse of 18.5 months. In the HIP-SIOP-PNET4 study, surgery at relapse was performed in 25% of cases and was associated with improved prognosis in solitary posterior fossa recurrence.ConclusionRecurrent medulloblastoma is often fatal in children who have previously received radiotherapy. The role of surgery in improving survival is unclear, but there is some evidence that resection of a focal single posterior fossa recurrence can bring survival benefit. The value of biopsy lies in the optimisation and selection of appropriate targeted therapy and in excluding a second malignancy.     

Unusual presentation in Haiti of a recurrent giant cell tumor of bone affecting the distal radius: A case report

IntroductionGiant cell tumor of bone (GCTB) is a benign-aggressive tumor that has a high-rate of recurrence with curettage resection alone. Patients with GCTB in underserved regions of the world can have progression of the tumor with significant disability due to a lack of specialty care. We present a case of an en bloc resection of an aggressive, recurrent GCTB of the radius with excellent function and no evidence of tumor recurrence two years after surgery.Presentation of caseA 22-year-old right-hand dominant female in Haiti developed an aggressive recurrence of a giant cell tumor of bone (GCTB) of the distal radius. Treatment consisted of en bloc resection of the distal radius with the proximal row of the carpus and centralization of the ulna. At two-year follow-up, the patient maintained good functional capacity with no clinical or radiological evidence of recurrence.DiscussionGCTB can cause significant destruction of the bone and articular surface if not treated adequately. Treatment options should be considered carefully in underserved regions of the world based on the resources available. This case exemplifies that complex limb-salvage surgery is possible when coordination of care between international and local surgeons is provided with an emphasis on continuity of care post-operatively.ConclusionEn bloc resection with centralization of the ulna remains a viable technique to address aggressive GCTB of the distal radius and can be appropriate in resource-limited settings.     

Hepatoblastoma in childhood,long term survival achieved: 2 case reports and literature review

IntroductionHepatoblastoma is the most common primary liver tumor for children under 5 years of age. It usually presents as an abdominal mass, symptomatic only when large enough to cause mass effect on nearby organs. Symptoms such as early satiety, anorexia, abdominal pain or weight loss are the most common. Diagnosis depends on imaging studies, AFP levels and percutaneous biopsy. Treatment modality is usually surgical with neoadjuvant chemotherapy.CasesIn this article, we present 2 cases of hepatoblastoma treated 15 years ago by neoadjuvant chemotherapy and surgery, and are presenting for long term follow-up with complete disease remission.DiscussionComplete resection and remission can be achieved as demonstrated below by our 2 cases of hepatoblastoma, especially when performing a true anatomical hepatectomy, along with a neoadjuvant chemotherapy regimen. Although one of the cases did not respond to chemotherapy very well a complete resection was achieved and therefore a disease free survival of 15 years.ConclusionHepatoblastoma are rare tumors of the pediatric age group. Management depends highly on combined surgical and pediatric oncological knowledge. A complete disease remission can be achieved when both modalities are treatment are optimal. Therefore, hepatoblastoma cases should be referred to specialized centers for management.     

Hepatocellular carcinoma with right atrial tumor thrombus: Report of a case

Hepatocellular carcinoma (HCC) with a tumor thrombus (TT) extending into the right atrium is generally regarded as a terminal-stage condition. We report a case of long-term survival following treatment of this complication with en bloc hepatectomy and resection of the thrombus under cardiopulmonary bypass. Our review of 19 similar cases reported in the literature found the following: that lung metastasis, the most critical prognostic factor, occurred in only 5 (27.8%) patients; that postoperative survival ranged from 18 days to 56 months, with a median survival of 11 months; and that 7 (38.9%) patients showed no signs of recurrence, with 4 (21.1%) surviving longer than 2 years. Thus, to prevent sudden death and extend the survival of patients with HCC and TT extending into the right atrium, we advocate simultaneous en bloc resection performed under cardiopulmonary bypass, provided distant metastasis and recurrence in the remnant liver are controlled.     

Hepatoblastoma of the adult with pericardial metastasis: A case report

BackgroundHepatoblastoma is the most frequent liver tumor in children, but very rare in the adult and associated with an unfavorable prognosis. The diagnosis is always postoperative or post mortem and biopsy is not useful. Surgery is the only accepted treatment.Case presentationOur patient underwent surgery in the suspect of liver metastasis from a previous gastric cancer. Surgery consisted in left lobectomy with partial diaphragm resection and partial pericardiectomy for a pericardial lesion, found after the opening of the thorax. The diaphragm defect was corrected with a biological mesh.ResultsThe histopathological examination indicated hepatoblastoma of the adult with pericardial metastases. The patient was asymptomatic and without recurrence after 21 months of follow up.ConclusionThe hepatoblastoma of the adult is related to a poor prognosis with median survival time less than 5 months. Surgery is the only curative treatment, but in many cases tumor resection requires complex operations. Vascular and thoracic expertise could be useful in the management of hepatoblastoma.     

Liver Transplant for Adult Recurrent Hepatic Mesenchymal Hamartoma and a Feasible Treatment Modality: A Case Report and Literature Review

BackgroundAdult hepatic mesenchymal hamartoma (HMH) is an extremely rare hepatic tumor. Recurrence following complete resection is uncommon. Liver transplantation (LT) is described as a possible treatment option in nonresectable HMH.We conducted a systematic review investigating LT in adult HMH followed by a case report describing evidence of extensive recurrence following complete resection of large right-sided HMH requiring LT.Case ReportA 46-year-old woman with symptomatic large right-hepatic HMH underwent right hemi-hepatectomy with histologic evidence of complete resection. Two and a half years postresection, she presented with abdominal pain and distension; imaging revealed large multi-septated hepatic cystic lesions within the liver suggestive of extensive recurrence of disease with concerns of malignant sarcomatous transformation. After a multidisciplinary team discussion, the lesion was deemed unresectable and the patient was referred for LT. Findings on transplantation included giant multiple hepatic cystic lesions occupying the entire abdomen and histopathological analysis confirmed recurrent HMH with no malignancy.The 6-month follow-up was unremarkable with no signs of postoperative complications or rejection.ConclusionWe identified only 3 reported adult unresectable HMH cases in the English literature requiring LT, with good clinical outcome and no rejection on a 1-year follow-up. To our knowledge, we report the first recurrent HMH that required LT in the English literature. Current evidence suggests possible malignant sarcomatous transformation of those lesions.No guidelines exist on postresection surveillance for HMH; however, given their malignant potential, we suggest a benefit of imaging-based surveillance following HMH resection. Offering LT for nonresectable or recurrent HMH is a feasible treatment modality with a reported good outcome.     

Surgical resection for liver metastasis and local recurrence of pheochromocytoma 16 years after primary surgery: A case report

Introduction and importancePheochromocytomas arise from the adrenal medulla and are rare. Pheochromocytomas metastasize to bone, lung and liver, and surgery might be the curative treatment. However, few cases are detected when they are resectable lesions because of difficulty in diagnosis and rapid growth. We herein report a patient who underwent resection of liver metastasis and local recurrence of pheochromocytoma.Case presentationA 74-year-old woman visited our hospital for treatment for liver and retroperitoneal tumors. She had undergone left adrenal gland resection for pheochromocytoma 16 years earlier. Eleven years after primary surgery, breast cancer was diagnosed and resected. During the breast cancer follow-up, a liver tumor was identified with computed tomography. Breast cancer recurrence and metastasis were considered, so chemotherapy was administered first. However, the liver tumor gradually enlarged, and another lesion appeared in the retroperitoneum. The tumors were diagnosed as pheochromocytoma recurrence using ¹²³I-metaiodobenzylguanidine scintigraphy, and she underwent resection of the local recurrence and liver metastasis. She was discharged on postoperative day 25 without complications, and no evidence of recurrence occurred more than 3.5 years postoperatively.Clinical discussionAll pheochromocytomas have metastatic potential; however, there are no reliable markers to predict malignancy. Early detection of recurrence by regular imaging and complete resection are important in the treatment. If the recurrence was oligometastasis and tumor growth is slow, surgical resection may be eligible.ConclusionA favorable outcome resulted from complete resection for liver metastasis and local recurrence of pheochromocytoma.     

全脊椎整块切除术--一种治疗原发性恶性脊柱肿瘤的新手术方法

[目的]介绍一种整体切除脊柱原发性恶性肿瘤并使肿瘤治愈成为可能的新手术技术“全脊椎整块切除术”。背景资料：传统切除脊柱原发性恶性肿瘤的方法,是从肿瘤病灶内分块进行逐步切除。而经组织病理学证实,手术切缘较宽或较窄的病灶外整块切除的报告却鲜为有之。[方法]全脊椎整块切除术是在前路椎间支撑器植入,后路脊柱器械固定的情况下进行整块椎板切除和整块椎体切除的新手术技术。有5例息有原发性恶性肿瘤的患者及2侧患有巨细胞瘤的患者施行了这种手术。除l例患者在术后7个月时因肿瘤转移到纵隔而死亡外,其余所有患者的观察期为2～6．5a。[结果]除1例病人之外的所有患者在手术之后均获得了满意的临床效果,并且没有严重的并发症发生。经组织学研究证实,除椎弓根外,均获得了较宽或较窄的手术边缘,但在病变影响到椎管和脊柱的后方时,则需采用病灶内切除的传统方式。1例死于肿瘤转移的患者,其死亡原因与手术没有直接的相关性,且肿瘤切除的部位也没有局部复发的迹象。[结论]全脊椎整块切除术的优势在于,它是将整个椎体分两次予以完全切除,也就是一次切除椎体的一半,这种技术远胜于分块逐步切除的模式,并且是一次性完成整个手术的全过程。“全脊椎整块切除术”为脊柱原发性恶性肿瘤的治疗提供了一个更具有挑战性的手术方法。     

Rescue liver transplantation after post-hepatectomy acute liver failure: A systematic review and pooled analysis

BackgroundPost-hepatectomy liver failure is a severe complication after major liver resection and is associated with a high mortality rate. Nevertheless, there is no effective treatment for severe liver failure. In such a setting, rescue liver transplantation (LT) is used only in extraordinary cases with unclear results. This systematic review aims to define indication of LT in post-hepatectomy liver failure and post-LT outcomes, in terms of patient and disease-free survivals, to assess the procedure's feasibility and effectiveness.MethodsA systematic review of all English language full-text articles published until September 2022 was conducted. Inclusion criteria were articles describing patients undergoing LT for post-hepatectomy liver failure after liver resection, which specified at least one outcome of interest regarding patient/graft survival, postoperative complications, tumour recurrence and cause of death. A pseudo-individual participant data meta-analysis was performed to analyse data. Study quality was assessed with MINORS system. PROSPERO CRD42022349358.ResultsPostoperative complication rate was 53.6%. All patients transplanted for benign indications survived. For malignant tumours, 1-, 3- and 5-year overall survival was 94.7%, 82.1% and 74.6%, respectively. The causes of death were tumour recurrence in 83.3% of cases and infection-related in 16.7% of LT recipients. At Cox regression, being transplanted for unconventional malignant indications (colorectal liver metastasis, cholangiocarcinoma) was a risk factor for death HR = 8.93 (95%CI = 1.04–76.63; P-value = 0.046). Disease-free survival differs according to different malignant tumours (P-value = 0.045).ConclusionsPost-hepatectomy liver failure is an emergent indication for rescue LT, but it is not universally accepted. In selected patients, LT can be a life-saving procedure with low short-term risks. However, special attention must be given to long-term oncological prognosis before proceeding with rescue LT in an urgent setting, considering the severity of liver malignancy, organ scarcity, the country's organ allocation policies and the resource of living-related donation.     

Ante situm liver resection with inferior vena cava replacement under hypothermic cardiopolmunary bypass for hepatoblastoma: Report of a case and review of the literature

IntroductionHepatoblastoma with tumour thrombi extending into inferior-vena-cava and right atrium are often unresectable with an extremely poor prognosis. The surgical approach is technically challenging and might require major liver resection with vascular reconstruction and extracorporeal circulation. However, which is the best surgical technique is yet unclear.Presentation of caseA 11-months-old boy was referred for a right hepatic lobe mass(90 × 78 mm) suspicious of hepatoblastoma with tumoral thrombi extending into the inferior-vena-cava and the right atrium, bilateral lung lesions and serum alpha-fetoprotein level of 50.795 IU/mL. After 8 months of chemotherapy (SIOPEL 2004-high-risk-Protocol), the lung lesions were no longer clearly visible and the hepatoblastoma size decreased to 61 × 64 mm. Thus, ante situm liver resection was planned: after hepatic parenchymal transection, hypothermic cardiopulmonary bypass was started and en bloc resection of the extended-right hepatic lobe, the retro/suprahepatic cava and the tumoral trombi was performed with concomitant cold perfusion of the remnant liver. The inferior-vena-cava was replaced with an aortic graft from a blood-group compatible cadaveric donor. The post-operative course was uneventful and after 8 months of follow-up the child has normal liver function and an alpha-fetoprotein level and is free of disease recurrence with patent vascular graft.ConclusionsWe report for the first time a case of ante situ liver resection and inferior-vena-cava replacement associated with hypothermic cardiopulmonary bypass in a child with hepatoblastoma. Herein, we extensively review the literature for hepatoblastoma with thumoral thrombi and we describe the technical aspects of ante situm approach, which is a realistic option in otherwise unresectable hepatoblastoma.     

How to safely perform laparoscopic liver resection for children: A case series of 19 patients

PurposeThe purpose of this study was to determine the feasibility and outcome of laparoscopic hepatectomy in pediatric patients with liver tumors and introduce surgical techniques to minimize complications.MethodsThis was a retrospective study of 19 patients less than the age of 18 years old who underwent LLR for liver tumors between November 2005 and May 2017.ResultsTen females and nine males with a median age of 26 months and a median body weight of 14.7 kg were enrolled. Diseases of these patients were hepatoblastoma, neuroblastoma, biliary atresia, and liver abscess. The numbers of resected hepatic segments were more than two in thirteen patients and one in six patients. One patient required conversion to laparotomy to control bleeding caused by injury to the left hepatic vein. Nine patients received transfusions perioperatively. Median duration of operation was 230 min, and median postoperative hospital stay was 7.0 days. There was no postoperative complication. Only one patient showed nonhepatic recurrence of neuroblastoma which caused mortality.ConclusionsLLR for benign and malignant liver tumors showed acceptable amount of bleeding and low complication rate. Therefore, LLR would be a safe and feasible option for liver tumors in children with proper technical efforts and selection of patients.Level of evidenceLevel III.     

The outcome of surgical treatment for recurrent giant cell tumor in the appendicular skeleton

BackgroundThe treatment for recurrent giant cell tumor (GCT) remains controversial. In this study, we evaluated the outcome of surgical intervention for recurrent GCT.MethodTwenty-seven patients (14 males and 13 females) with recurrent GCT were recruited. Their primary GCTs were all treated with intralesional surgery. Among these recurrent GCTs, 9 grade III and 1 grade II tumors were treated with en bloc resection and endoprosthetic replacement, whereas 16 grade II and 1 grade III tumors were treated with intralesional curettage and PMMA bone cement filling.ResultsThe mean interval between initial surgery and first recurrence was 28.8 months (range 7–97 months). About 70 % of first recurrences affected bones around the knee, 44 % in the proximal tibia and 26 % in the distal femur. Of 27 patients, 3 women treated with intralesional procedures suffered second recurrences in the proximal tibia. No second recurrence was found in patients with en bloc resection. Two grade III re-recurrence GCTs were treated with en bloc resection, and 1 grade II was treated with an intralesional procedure. One patient with en bloc resection developed tumor metastasis in both lungs. Compared to patients with intralesional treatment, the functional score was significantly decreased in patients with en bloc resection (p < 0.01).ConclusionThe re-recurrence risk of grade III GCTs can be significantly decreased by wide en bloc resection and endoprosthetic replacement. However, intralesional treatment is a good option for less aggressive (相似文献   

Staging and treatment of osteoblastoma in the mobile spine: a review of 51 cases

Purpose

To define the role of Enneking staging system and of the consequent different treatment options on the outcome of osteoblastoma (OBL) of the spine.

Methods

A retrospective review of 51 patients with OBL of the mobile spine conducted to compare the outcomes among the different types of treatments at long term follow-up (25–229 months, av.90). These 51 patients were previously staged according to Enneking staging system and treatment selected accordingly. 10 stage two (st.2) OBLs were treated with intralesional excision and 41 stage three (st.3) OBLs were treated either by intralesional excision or en bloc resection. The intralesional excision group was divided considering the use or not of radiation therapy after surgery. The recurrence rate was compared among these groups and also considering previous open surgery (“non intact” vs. “intact”). The statistical significance was defined using the Fisher Exact test.

Results

No local recurrence occurred in the st.2 patients treated by intralesional excision. Considering the st.3 patients, 2 local recurrences out of 13 patients occurred in the en bloc resection (15.4 %) group. All occurred in “non intact” cases (67 %). In the intralesional group, 5 local recurrences out of 27 patients occurred (18 %) being none in the group that received radiation therapy after surgery. Two occurred in the “intact” (7 %) and three in the “non intact” group (75 %). Considering all patients, the difference between the recurrence rate between “intact” and “non intact” groups was statistically significant (p < 0.002).

Conclusions

Intralesional excision proved to be effective in st.2 lesions and en bloc resection in st.3. Radiotherapy seems to be an effective adjuvant treatment when en bloc resection is not feasible or requires unacceptable functional sacrifices. The first treatment significantly affects the prognosis as previously treated patients have worse prognosis.     

Liver transplantation for unresectable hepatocellular carcinoma in patients without liver cirrhosis

Summary Hepatocellular carcinoma (HCC) arising in noncirrhotic and nonfibrotic liver (NC‐HCC) is a rare type of malignancy frequently found in healthy young individuals. Partial liver resection is the treatment of choice with expected 5‐year survival rates between 40% and 70%. As a result of absence of any symptom, a considerable number of patients are diagnosed when the malignancy has progressed to an advanced stage and the tumor has turned already unresectable. Some other patients suffer from intrahepatic recurrence after previous liver resection that cannot be re‐resected or locally ablated. In these situations, liver transplantation (LT) may be the only potentially curative treatment. The indication for LT in NC‐HCC patients, however, is not well established. The preliminary results of recent analysis of the European Liver Transplant Registry (ELTR) together with a literature review identified over 150 patients transplanted for NC‐HCC during the last 15 years. In contrast to the historical data, these studies showed 5‐year survival rates at 50–70% in well‐selected patients. Important determinants of poor outcome are macrovascular invasion, lymph node involvement, and time interval of <12 months when LT is used as rescue therapy for intrahepatic recurrence after a previous partial liver resection. Interestingly, outcomes after both liver resection and LT for NC‐HCC are much less influenced by tumor size than is the case with cirrhotic HCC. A large tumor size per se should, therefore, not to be seen as a strict contraindication for performing LT in patients with NC‐HCC.