Skull metastasis from clear cell chondrosarcoma

A 67-year-old man presented with left lower cranial nerve paresis and dysfunction of the left cerebellar hemisphere 4 years after amputation of the left lower leg because of clear cell chondrosarcoma (CCC). Neuroimaging studies showed an osteolytic extradural mass with homogeneous enhancement in the left posterior fossa. Bone scintigraphy disclosed a single high-uptake lesion at the same site. The tumor was removed totally via a left suboccipital craniotomy. Histological examination found mainly clear cells arranged in a microlobular pattern separated by thin fibrovascular stroma. The nuclei were regular with few mitotic figures. Immunohistochemical staining showed the tumor cells reacted intensely for both S-100 protein and vimentin. Osteoclast-like multinucleated giant cells were found at the periphery of the lobules. The primary tumor showed the same findings and the metastatic tumor manifested no malignant change. The histological diagnosis was metastatic CCC. CCC is a very rare neoplasm with slow growth and low-grade malignancy. Distant metastasis is rare but can occur in the skull base bone despite radical resection of the primary tumor. Osteolytic findings of homogeneous enhancement on magnetic resonance imaging and a high uptake on bone scintigraphy are indicative of metastatic tumor from previous CCC.     

Runx2在普通软骨肉瘤及去分化软骨肉瘤中的差异表达

目的 检测Runx2在人普通软骨肉瘤及去分化软骨肉瘤的差异表达,探讨Runx2在软骨肉瘤去分化过程中的作用及意义.方法 应用逆转录-聚合酶链反应(RT-PCR)和Western blot方法,检测普通软骨肉瘤及去分化软骨肉瘤标本中Runx2的差异表达;并利用免疫组织化学SP法检测42例软骨肉瘤标本(普通软骨肉瘤20例,去分化软骨肉瘤22例)中Runx2的表达.结果 RT-PCR和Western blot结果显示Runx2在去分化软骨肉瘤中高表达,而在普通软骨肉瘤中低表达;免疫组织化学实验结果表明普通软骨肉瘤中Runx2阳性表达率为10.O%,去分化软骨肉瘤中Runx2阳性表达率为95.5%,两者差异有统计学意义(P＜0.05).结论 Runx2在去分化软骨肉瘤中的高表达涉及了去分化软骨肉瘤的发生和发展.

Abstract：

Objective To detect the differential expression of Runx2 in the conventional chondrosarcoma and dedifferentiated chondrosarcoma, and study the role and significance of Runx2 in the dedifferentiation of chondrosarcoma. Methods The expression of Runx2 in 42 cases of chondrosarcoma (20 cases of conventional chondrosarcoma, and 22 cases of dedifferentiated chondrosarcoma) was detected by using immunohistochemistry SP method. Results The positive expression rate of Runx2 in conventional chondrosarcoma was 10%, which was significantly lower than in dedifferentiated chondrosarcoma (95.5%). Conclusion Runx2 is up-regulated in dedifferentiated chondrosarcomas, suggesting Runx2 may involve in the occurrence and development of dedifferentiated chondrosarcoma.     

Hepatectomy for Peripheral Cholangiocarcinoma in Elderly Patients

Background Peripheral cholangiocarcinoma (CCC) is less common than hepatocellular carcinoma. Little is known about CCC patients older than 70 years who have undergone hepatectomy.Methods Between 1977 and 2004, the clinical features of 33 elderly CCC patients (>70 years old) undergoing hepatectomy were reviewed, and 185 CCC patients younger than 70 years (younger CCC) were used for comparison.Results A total of 218 CCC patients undergoing hepatectomy were investigated with ages ranging from 28 to 93 years (median, 59.0 years). The elderly and younger CCC groups had a similar sex ratio and a similar positive rate of carcinoembryonic antigen (CEA) and CA19-9. A similar rate of hepatolithiasis, mucobilia, papillary pattern, stage distribution, curative hepatectomy, surgical morbidity, and mortality for CCC were also observed between the two groups. During a follow-up duration ranging from 1.1 to 145.0 months (median, 11.7 months), elderly CCC and younger CCC patients had similar prognoses after hepatectomy (P = .827). Elderly CCC patients with a low CEA level, an intraductal papillary growth pattern, curative hepatectomy, and postoperative chemotherapy tended to have favorable survival. However, elderly CCC patients with a low CEA level independently showed favorable survival.Conclusions Hepatectomy is feasible for selected elderly CCC patients. Elderly CCC patients undergoing hepatectomy had clinicopathologic features and prognoses similar to those of patients younger than 70 years undergoing hepatectomy. Elderly CCC patients with a low CEA level, intraductal papillary growth pattern, curative hepatectomy, and postoperative chemotherapy tended to have favorable survival. However, elderly CCC patients with low CEA level independently showed favorable survival.     

基质金属蛋白酶-7基因及蛋白表达与软骨肉瘤的相关性研究

目的　基质金属蛋白酶在肿瘤侵袭和转移中发挥重要作用 ,本研究探讨MMP 7基因表达与软骨肉瘤发生的可能关系。方法　应用逆转录 聚合酶链反应 (RT PCR)和免疫组织化学(S P)方法 ,对 2 4例软骨肉瘤患者的肿瘤和瘤旁正常组织的MMP 7基因转录本和蛋白表达进行检测。结果　应用RT PCR方法检测 2 4例软骨肉瘤组织中MMP 7基因mRNA表达情况 ,结果显示MMP 7在软骨肉瘤和瘤周正常组织中的表达差异有非常显著性 (P <0 .0 1) ,MMP 7mRNA在软骨肉瘤组织中表达明显增高。软骨肉瘤中MMP 7蛋白表达阳性百分比为 66.7% (16/2 4) ,瘤周正常组织为 2 9.2 % (7/2 4) ,差异有非常显著性 (P <0 .0 1) ,MMP 7蛋白在软骨肉瘤中表达增高。结论　MMP 7基因表达改变可能与软骨肉瘤的发生机制有关     

Development of chondrosarcoma animal models for assessment of adjuvant therapy

Chondrosarcoma is a primary cancer of bone causing significant morbidity due to local recurrence and limited treatment options. Relatively few chondrosarcoma animal models have been developed, and the only orthotopic model is technically demanding and has limited clinical relevance. The aim of this review is to assess the features of current animal chondrosarcoma models for the purpose of developing new models in which to test adjuvant chondrosarcoma therapy. The available literature on this topic was identified using the PubMed database, and then analysed for relevance to the human chondrosarcoma disease and feasibility in testing new therapeutic agents. Animal‐derived chondrosarcoma models comprise predominantly allograft tumour transplanted into the rat (Swarm rat chondrosarcoma) or the hamster. These types of models are less relevant to the human disease and have been more useful for evaluation of chondrosarcoma growth and histology than in developing novel therapeutic agents. The athymic nude mouse has enabled reliable human xenograft transplantation. A number of human chondrosarcoma cell lines have been successfully used to generate tumours in this species, including OUMS‐27 and HCS‐2/A. Although effective in demonstrating anti‐tumour effects of a number of agents, the lack of a representative orthotopic model diminishes overall clinical relevance. More clinically relevant models of human chondrosarcoma progression are required either through transgenic mice or orthotopic human xenograft models.     

先天性胆总管囊肿85例临床分析

目的 探讨先天性胆总管囊肿的临床特点，选择合适的手术治疗方法。方法 对85例胆总管囊肿的临床资料进行回顾性分析。结果 85例中发生癌变12例，癌变率14％。影像学检查显示胆总管囊肿位于胰胆管结合部者29例，其中26例有胰胆管合流异常。手术治疗83例，囊肿切除、肝管空肠Roux-en-Y吻合术为主要术式，其中4例囊肿巨大，伴有严重感染者先期行外引流术，待一般情况好转后二次行根治眭手术。囊肿切除的60例中47例获得随访，疗效优良者45例；而仅行内引流术的6例中疗效优良者仅1例。结论 对囊肿伴严重感染者先期行外引流术可有效控制感染。囊肿全切除、肝管空肠Roux-en-Y吻合术应作为胆总管囊肿的首选术式。     

Extraskeletal myxoid chondrosarcoma of the falx.

Intracranial cartilaginous tumors are unusual lesions, of which myxoid chondrosarcoma is the rarest. We describe this tumor arising from the falx in a 28-year-old woman treated at recurrence with a second operation and a radiation implant. The behavior of classic chondrosarcoma and mesenchymal chondrosarcoma is also reviewed.     

Conjunctival and corneal calcificationin is associated with abdominal aortic calcification in maintenance haemodialysis patients

Objective To determine the condition of conjunctival and corneal calcificationin in maintenance haemodialysis patients, and explore the relationship between conjunctival and corneal calcificationin (CCC) and abdominal aortic calcification (AAC). Methods CCC was evaluated by slit-lamp eye photographs, and was graded and scored according to Porter’s classification system in the literature. Abdomen 1ateral X-ray examination were used as a criteria to determine the abdominal aortic calcification. The abdominal aortic calcification (AAC) score was calculated. Spearman correlation coefficient was used to analyze the relationship between CCC and AAC. Logistic regression analysis was used to determine the risk factor of CCC in MHD patients. Results Ninety-eight MHD patients were recruited. Their average age and dialysis vintage were (61.89±12.54) years and 51.67 (3.00～192.00) months, ninety-seven patients had conjunctival and corneal calcificationin, and seventy-two patients had abdominal aortic calcification, The median CCC was 8 (0, 17), the median AAC was 6 (0, 20), and the CCC was positively correlated with AAC (r=0.376, P＜0.001). Compared with the patients of CCC score≤5, the patients of CCC score＞10 had a higher level of phosphate, calcium-phosphate product, iPTH, hs-CRP, and longer dialysis vintage, as well as a higher score of AAC (all P＜0.05). Multivariate logistic regression analysis showed that the higher score of AAC and the longer dialysis vintage were independent risk factors for severe corneal calcificationin calcification. Conclusions Conjunctival and corneal calcificationin is common in MHD patients, and CCC is positively associated with AAC, the risk of CCC rises as a result of a severer AAC and a longer dialysis vintage.     

Poor Outcomes After Liver Transplantation in Patients With Incidental Cholangiocarcinoma Irrespective of Tumor Localization

Introduction

After liver transplantation for cholangiocarcinoma (CCC), patients have a poor prognosis without use of specific therapeutic strategies. Accordingly, recipients with incidental CCC might have the highest risk of recurrent disease; however, sparse data on the long-term outcome of unselected patients with incidental CCC have been published. The aim of this study was to evaluate the post-transplantation outcomes of patients with incidental CCC with special focus on tumor localization.

Material and Methods

There were 11 primary liver transplantations in patients with incidental CCC of 1310 liver transplantation procedures performed between December 1994 and August 2013. All patients with incidental CCC received a chemotherapy regiment including gemcitabine/5 fluorouracil, doxorubicin, and mitomycin. The patients were switched from calcineurin inhibitors to mammalian target of rapamycin inhibitor−based immunosuppression shortly after CCC diagnosis.

Results

Intra- and extrahepatic tumors were found in 6 and 5 patients, respectively. At median follow-up examination of 26.3 months there were 8 CCC recurrences and 7 patient deaths. Overall survival after liver transplantation for incidental CCC was 88.9% at 1 year, 44.4% at 2 years, and 14.8% at 3 years. The corresponding rates of recurrence-free survival were 45.7%, 45.7%, and 0.0%, respectively. Post-transplantation CCC recurrences were universal with 0% 3-year recurrence-free survival both in patients with intra- and extrahepatic tumors (P = .475).

Conclusions

Incidental CCC in liver transplantation is associated with poor outcomes irrespective of tumor localization. Introduction of new adjuvant multimodal treatment concepts is necessary to improve the prognosis for this subgroup of patients.     

Sternal chondrosarcoma: a case report

Primary chest wall tumors are uncommon and constitute 0.2–2% of all tumors. Most common primary malignant bone tumor of sternum is chondrosarcoma. Adequate surgical excision remains the most important aspect of the management of chondrosarcoma. We herein describe a case of sternal chondrosarcoma in adult female.     

Chondrosarcoma of spermatic cord

A case of extraosseous chondrosarcoma arising from the spermatic cord is reported and the literature reviewed. Chondrosarcoma arising from nonosseous tissue is rare, and only a handful of cases have been reported. However, unlike chondrosarcoma of bone, extraosseous myxoid chondrosarcoma behaves in a less aggressive fashion making the distinction between the two entities prognostically significant.     

Mediastinal chondrosarcoma. Case report

A 34-year-old man and a 71-year-old woman underwent radical removal of mediastinally sited chondrosarcoma, presumably originating in the periosteum of the vertebral body. The man (with mesenchymal chondrosarcoma) died of remote metastasis 6 years postoperatively. The woman (poorly differentiated chondrosarcoma, grade 2-3) is still alive 2 years after the operation.     

Primary cardiac chondrosarcoma

Primary cardiac chondrosarcoma is extremely rare, and its clinical characteristics and management are not clear. Only eight cases of primary cardiac chondrosarcoma in the left heart have been reported in the English literature. In this report, we describe a case of primary chondrosarcoma of the left atrium. The management of these rare tumors is the subject of this report.     

Chondrosarcoma of the hand secondary to multiple enchondromatosis; report of two cases

Although malignant transformation to chondrosarcoma may occur in some patients with multiple enchondromatosis, this event rarely occurs in the hand. We encountered two patients with chondrosarcoma of the hand secondary to multiple enchondromatosis. One patient was a 27-year-old man and the other, a 76-year-old man. Both patients manifested multiple osteolytic lesions in the hand on the plain radiographs. Severe bone destruction associated with a large soft-tissue swelling of the proximal and middle phalanges of the little finger was seen in case 1. In case 2, tremendous expansion and bone destruction of the middle phalanx of the ring finger was seen. Magnetic resonance images of the tumour in both patients showed low signal intensity on T1-weighted and high signal intensity on T2-weighted images. Amputation was performed in each patient. Histological examination revealed that the tumour was a grade 2 chondrosarcoma in case 1 and a grade 1 chondrosarcoma in case 2 accompanied by enchondromata. From these findings, the diagnosis of chondrosarcoma secondary to multiple enchondromatosis was made. Because quite a few patients with multiple enchondromatosis develop secondary chondrosarcoma, although rarely in the hand, the enchondromata should be curetted, unless impractical, before malignant transformation occurs.     

A randomized controlled trial comparing cholecystocholangiography with cystic duct cholangiography during laparoscopic cholecystectomy

BACKGROUND: The rate of intraoperative cholangiography fell after the advent of laparoscopic cholecystectomy due to the perceived difficulty of cystic duct cannulation. It is suggested that cholecystocholangiography (CCC) is a valid and easier alternative. The present study compares cystic duct cholangiography (CDC) to CCC with evaluation of procedural time, success rate, image quality, cost and radiation exposure. METHODS: Patients undergoing laparoscopic cholecystectomy were randomized to CCC (n = 40) or CDC (n = 36). Details of operative times, radiation exposure, and use of disposable equipment were recorded prospectively. Cholangiograms were performed using image intensification and were scored from 0 to 6 according to adequacy of images. Data were analysed on an intention-to-treat basis with the chi-squared test, t-test or Fisher's exact test. RESULTS: The success rate for CDC was 100% and for CCC it was 72% (P = 0.0005). Patients with a failed CCC went on to have CDC for a success rate in the CCC arm of 92.5%. Comparing CDC to CCC, there was no significant difference in cost ($30.16 vs $33.36: P = 0.11), operative time (1 h 13 min vs 1 h 3 min; P = 0.19) or cholangiogram time (8 vs 9 min: P = 0.39). There was a significant difference in screening time (0:41 vs 1:33 min; P < 0.0001), adequate image quality (100 vs 72.5%, P = 0.0005) and procedure-related complications (0 vs 5; P = 0.03). CONCLUSIONS: A significant number of CCC fail. Successful CCC provides inferior image quality and greater radiation exposure. It provides no benefit in time or cost and cannot be recommended for operative cholangiography.     

P~(75 NGFR)在先天性胆总管囊肿的表达意义初探

目的　探讨神经生长因子受体 (P75NGFR)在先天性胆总管囊肿 (CCC)的分布情况及其意义。方法　应用免疫组化方法对 1 8例先天性胆总管囊肿患儿及 9例对照组的胆总管进行检测。结果　丰富的P75NGFR染色阳性神经纤维分布于正常胆总管外膜下 ,神经节细胞表现为P75NGFR染色强阳性 ;先天性胆总管囊肿的P75NGFR染色阳性纤维明显减少。结论　P75NGFR在先天性胆总管囊肿的异常分布可能与CCC的发病机理有关。     

Prognostic impact of IDH mutations in chondrosarcoma

BackgroundMutant isocitrate dehydrogenase (IDH) in chondrosarcoma produces the oncometabolite 2-hydroxyglutarate (2-HG) and contributes to malignant progression, and is therefore a potential therapeutic target for chondrosarcoma. Robust historical control data are important in clinical trials of rare cancers such as chondrosarcoma in order to show a clear benefit of new drugs. However, it remains controversial whether IDH mutation status is associated with the clinical outcome of chondrosarcoma, and this hinders the development of mutant IDH inhibitors in clinical trials.backgroundMethodsWe investigated the relationship between IDH gene status and clinicopathological data in 38 chondrosarcoma patients from whom frozen tumor samples were obtained at the time of biopsy or surgery. Targeted next-generation sequencing was also performed to compare genetic alterations between patients with and without IDH mutations.methodsResultsThe results revealed 15 cases (40%) of heterozygous IDH1 mutations and five cases (13%) of IDH2 mutations. IDH-mutant chondrosarcoma was associated with worse overall survival than IDH–wild-type chondrosarcoma (IDH1/2 Mut vs. IDH Wt, P = 0.006; IDH1 Mut vs. IDH Wt, P = 0.030; IDH2 Mut vs. IDH Wt, P < 0.0001). IDH mutation was also a significant poor prognostic factor both in univariate (P = 0.026) and multivariate (P = 0.048) analyses. Targeted next-generation sequencing revealed that characteristic mutations in chondrosarcoma, including TP53 and COL2A1, were more common in the IDH-mutant group than in the IDH–wild-type group.resultsConclusionThis study is the first to report in detail the characteristics and clinical courses of IDH-mutant chondrosarcoma patients in Japan. Our data suggested that IDH-mutant chondrosarcomas might have a worse prognosis than that of IDH-wild-type chondrosarcoma, possibly through the more aggressive characters after metastasis. This information will be useful for designing clinical trials of mutant IDH inhibitors for treatment of advanced chondrosarcoma.conclusion     

Primary mesenchymal chondrosarcoma of the lung

Mesenchymal chondrosarcoma has been well documented in the somatic soft tissue and bone. It is a rare subtype of chondrosarcoma characterized by the presence of islands of chondroid or by less osteoid tissue enmeshed within dense sheets of primitive small blue mesenchymal cells with hemangiopericytoma-like vessels, or by both. The vast majority of previously published pulmonary mesenchymal chondrosarcoma was metastatic. To the best of our knowledge, only one case of primary pulmonary mesenchymal chondrosarcoma has been described in the literature. Herein, we report the second case of primary mesenchymal chondrosarcoma of the lung and emphasize that biopsy may yield only nonspecific small blue cells, whereas a detailed evaluation of the resected specimen allows definite diagnosis of this rare lung tumor.