长骨骨巨细胞瘤相关复发原因分析

分析长骨骨巨细胞瘤相关复发因素,为其临床治疗及降低复发率提供参考依据。方法：分析２１例长骨复发性骨巨细胞瘤一般资料、影像学检查资料,首次手术方式、肿瘤所在部位、病理骨折与否、Ｊａｆｆｅ’ｓ病理分级、Ｃａｍｐａｎｉｃｃｉ’ｓ放射线分级等,总结相关的复发因素。结果：本组４１例肿瘤中复发者２１例。２１例中Ｊａｆｆｅ’ｓ病理诊断与首次比较,１７例无变化;按Ｃａｍｐａｎｉｃｃｉ’ｓ放射诊断标准,首次１９例为     

骨巨细胞瘤的局部复发与治疗

目的：防止骨巨细胞瘤的局部复发。方法：观察本院１９８１￣１９９８年４０例骨巨细胞瘤患者的术后疗效。结果：２８例骨巨细胞瘤病灶刮除骨水沁填充后,只有１例复发、６例肿瘤病灶刮除自体骨移植后,４例复发。结论：肿瘤病灶刮除后骨水泥填充能减少局部肿瘤复发;对于Ｘ线ＣａｍｐａｎａｃｃｉⅢ级,或者Ｅｎｎｅｃｋｉｎｇ外科分期Ⅰｂ或Ⅱｂ,或者Ｊａｆｆｅ病理分期Ⅲ级患者,应扩大切除肿瘤病灶,近关节处肿瘤可扩大切除术后     

Giant Cell Tumor of Bone

This article provides an overview of giant cell tumor, including the typical clinical, radiographic, and pathologic findings, as well as some unusual features, such as multifocality and metastases. The article addresses recent advances in the molecular biology of giant cell tumor, particularly receptor activator of nuclear factor kappa B (RANK)-ligand signaling, in addition to novel anti–RANK-ligand therapy, the use of which seems promising for unresectable and metastatic tumors.     

Giant Cell Tumor of the Sphenoid Bone

Objective and Importance: Giant cell tumor of the sphenoid bone is extremely unusual. This tumor is usually located in long bones and is rarely located in the cranial vault. It must be differentiated from reparative granuloma and the Brown tumor of hyperparathyroidism. This case report is only the second involving a patient in the first decade.

Clinical Presentation: A 9-year-old girl presented with cephalgia and diplopia. Radiographic imaging revealed an erosive mass in the clivus.

Intervention: The patient underwent an extended transphenoidal resection of the clivus. This was followed with 57.6 CGE in 32 fractionated doses.

Conclusion: At 1 year follow-up, the tumor remains stable. Based on anecdotal evidence, attempted resection with adjuvant radiation therapy allows for long-term survival in these patients.

     

Histogenetic Characterization of Giant Cell Tumor of Bone

The unpredictable behavior of giant cell tumor (GCT) parallels its controversial histogenesis. Multinucleated giant cells, stromal cells, and CD68⁺ monocytes/macrophages are the three elements that interact in GCT. We compared the ability of stromal cells and normal mesenchymal stromal cells to differentiate into osteoblasts. Stromal cells and mesenchymal cells had similar proliferation rates and lifespans. Although stromal cells expressed early osteogenic markers, they were unable to differentiate into osteoblasts but they did express intracellular adhesion molecule-1, a marker of bone-lining cells. They were unable to form clones in a semisolid medium and unable to promote osteoclast differentiation, although they exerted a strong chemotactic effect on osteoclast precursors. Stromal cells may be either immature proliferating osteogenic elements or specialized osteoblast-like cells that fail to show neoplastic features but can induce the differentiation of osteoclast precursors. They might be secondarily induced to proliferate by a paracrine effect induced by monocyte-macrophages and/or giant cells. The increased number of giant cells in GCT may be secondary to an autocrine circuit mediated by the receptor activator of nuclear factor kB. One or more of the authors (NB) has received funding from the Italian Association for Cancer Research. Each author certifies that his or her institution has approved the human protocol for this investigation, that all investigations were conducted in conformity with ethical principles of research, and that informed consent for participation in the study was obtained.     

Modern Interpretation of Giant Cell Tumor of Bone: Predominantly Osteoclastogenic Stromal Tumor

Owing to striking features of numerous multinucleated cells and bone destruction, giant cell tumor (GCT) of bone, often called as osteoclastoma, has drawn major attractions from orthopaedic surgeons, pathologists, and radiologists. The name GCT or osteoclastoma gives a false impression of a tumor comprising of proliferating osteoclasts or osteoclast precursors. The underlying mechanisms for excessive osteoclastogenesis are intriguing and GCT has served as an exciting disease model representing a paradigm of osteoclastogenesis for bone biologists. The modern interpretation of GCT is predominantly osteoclastogenic stromal cell tumors of mesenchymal origin. A diverse array of inflammatory cytokines and chemokines disrupts osteoblastic differentiation and promotes the formation of excessive multi-nucleated osteoclastic cells. Pro-osteoclastogenic cytokines such as receptor activator of nuclear factor kappa-B ligand (RANKL), interleukin (IL)-6, and tumor necrosis factor (TNF) as well as monocyte-recruiting chemokines such as stromal cell-derived factor-1 (SDF-1) and monocyte chemoattractant protein (MCP)-1 participate in unfavorable osteoclastogenesis and bone destruction. This model represents a self-sufficient osteoclastogenic paracrine loop in a localized area. Consistent with this paradigm, a recombinant RANK-Fc protein and bisphosphonates are currently being tried for GCT treatment in addition to surgical excision and conventional topical adjuvant therapies.     

Recurrence After and Complications Associated With Adjuvant Treatments for Sacral Giant Cell Tumor

Background  

The best treatment of giant cell tumor of the sacrum is controversial. It is unclear whether adjuvant treatment with intralesional surgery reduces recurrences or increases morbidity.     

Bisphosphonates Induce Apoptosis of Stromal Tumor Cells in Giant Cell Tumor of Bone

Giant cell tumour of bone (GCT) is an aggressive primary neoplasm that results in the production of osteolytic lesions. Stromal cells, which form the main neoplastic component of this tumor, regulate the formation of osleoclast-like giant cells that are ultimately responsible for bone destruction. Bisphosphonates prevent bone resorption by inhibiting osteoclast activity and promoting osteoclast apoptosis, and they have been known to induce apoptosis of primary neoplastic cells such as those in breast and prostate cancers. We hypothesized that in bisphosphonates may induce apoptosis not only in osteoclast-like giant cells but also in neoplastic stromal cells of GCT both in vitro and in vivo. Twelve patients with GCT were treated with weekly injections of pamidronate for a period of 6 weeks prior to surgery. GCT specimens were collected at the time of biopsy and during definitive surgery. TUNEL assay was used to evaluate apoptotic DNA fragmentation in cells. In addition, twelve GCT primary cultures from these patients were treated with zoledronate, pamidronate, or alendronate for 48 hours at different doses (3, 30, or 150 M) and subjected to apoptosis assay by flow cytometry following fluorescent Annexin-V labeling. The results showed that pamidronate significantly induced apoptosis in both osteoclast-like giant cells and stromal tumor cells, in vivo. All three bisphosphonates caused substantial apoptosis of stromal tumor cells in cultures. Zoledronate was the most potent reagent, resulting in an average cell death of 27.41% at 150 M, followed by pamidronate (22.23%) and alendronate (15.3%). Our observations suggest that these drugs may be considered as potential adjuvants in the treatment of GCT.Both authors (Y.Y. Cheng and L. Huang) contributed equally to this work.     

Giant Cell Tumor (Osteoclastoma) of the Petrous Bone: Case Report

A case of a basal middle fossa giant cell tumor occurring in a 46-year-old man is described. The lesion appeared at the computed tomography (CT) scan examination as an hypodense mass with a peripheral “ring-like” enhancement, and no evident erosion of the skull base. The tumor, which infiltrated the basal temporal parenchyma, was removed via a temporal transzygomatic craniotomy, and extensive drilling of the petrous bone. Despite the occurrence, of significant postoperative complications, the patient ultimately showed a good clinical outcome, with no signs of recurrence at the 1-year follow-up CT scanning. The clinical and diageostic aspects and the management policy, of this rare lesion are discussed.     

Abdominoperineal Resection for Squamous Cell Anal Carcinoma: Survival and Risk Factors for Recurrence

Background

Despite the results of combined chemoradiation therapy for anal canal squamous cell carcinoma (SCC), up to 30?% of patients will undergo abdominoperineal resection (APR). The aim of this study was to evaluate oncologic outcomes, survival, and recurrence, following APR for anal canal SCC performed in a single center over a 13-year period.

Methods

All patients who underwent APR for anal canal SCC between 1996 and 2009 were retrospectively included. Demographic data, details on treatments, pathological report, and follow-up were noted. Survival curves were plotted using the Kaplan?CMeier method and potential prognostic factors were evaluated using Cox proportional hazards models.

Results

A total of 105 patients (77 women) were included. Indications for APR included tumor persistence (n?=?42; 40?%), recurrence (n?=?55; 52.4?%), or a contraindication to radiotherapy (n?=?8; 7.6?%). Median follow-up was 33.3?months (range, 1.5?C174.3?months). Overall survival and disease-free survival were, respectively, 61 and 48?% at 5?years. In multivariate analysis, tumor stage (T3 or T4), positive margin on pathologic examination and existence of distant metastases at the time of the surgery were associated with a poor prognosis. The indication for APR (persistent vs recurrent disease), gender, concurrent HIV infection, or performance of a VRAM flap did not influence OS or DFS. Overall recurrence rate was 42.6?% (n?=?43 of 101). The type of recurrence did not exert a significant effect on survival (p?=?.4571).

Conclusion

This study describes the largest single series of APR for anal carcinoma. Major prognostic factors for survival and recurrence were T status and involved margin. The 5-year overall survival was 60?%.     

Clinical Significance of Preoperative CT and MR Imaging Findings in the Prediction of Postoperative Recurrence of Spinal Giant Cell Tumor of Bone

ObjectivesTo explore the predictive value of preoperative imaging in patients with spinal giant cell tumor of bone (GCTB) for postoperative recurrence and risk stratification.MethodsClinical data for 62 cases of spinal GCTB diagnosed and treated at our hospital from 2008 to 2018 were identified. All patients were followed up for more than 2 years according to the clinical guidelines after surgery. Medical history data including baseline demographic and clinical characteristics, computed tomography (CT) and magnetic resonance imaging (MRI) findings of recurrent and non‐recurrent patients were compared. Two musculoskeletal radiologists read the images and were blinded to the clinical data. The imaging features associated with postoperative recurrence were analyzed by multivariate logistic regression, and receiver operating characteristic (ROC) curve analysis was performed to determine the optimal cutoff value of the largest lesion diameter predicting recurrence after surgery.ResultsAccording to whether the disease recurred within the follow‐up period, patients were divided into the recurrence group and the non‐recurrence group. Of 62 patients (29 males and 33 females), 17 had recurrence and 45 did not. The recurrence rate was 27.4%. The mean follow‐up time was 73.66 (± 32.92) months. The three major treatments were total en bloc spondylectomy (n = 26), intralesional spondylectomy (n = 20), and curettage(n = 16). A total of 16 CT and MRI features were analyzed. A univariate analysis showed no significant difference in age, sex, treatment, multi‐vertebral body involvement, location, boundary, expansile mass, residual bone crest, paravertebral soft tissue mass, CT value, and MRI signal on T1‐weighted imaging (WI), T2‐WI, and T2‐WI fat suppression (FS) sequences (P > 0.05). The largest lesion diameter [(4.68 ± 1.79) vs (5.92 ± 2.17) cm, t = 2.287, P = 0.026] and the vertebral compression fracture (51% vs 82%, χ ²  = 5.005, P = 0.025) were significantly different between the non‐recurrence and recurrence groups. Logistic regression analysis showed that both largest lesion diameter (odds ratio [OR], 1.584; 95% confidence interval [CI], 1.108–2.264; P = 0.012) and compression fracture (OR, 8.073; 95%CI, 1.481–11.003; P = 0.016) were independent predictors of postoperative recurrence. When we set the cutoff value for the largest lesion diameter at 4.2 cm, the sensitivity and specificity for distinguishing the recurrence and non‐recurrence of GCTB were 94.1% and 42.2%, respectively, and the area under the curve (AUC) was 0.671. The combined model achieved a sensitivity, specificity and accuracy of 47.1%, 97.8% and 83.9%, respectively.ConclusionsIn spinal GCTB, maximum lesion diameter and the vertebral compression fracture are associated with tumor recurrence after surgery, which may provide helpful information for planning personalized treatment.     

青春期及儿童骨巨细胞瘤14例报告

目的：总结青春期及儿童骨巨细胞瘤（ＧｉａｎｔＣｅｌＴｕｍｏｒｏｆＢｏｎｅ,ＧＣＴ）的临床表现、放射学特点及治疗。方法：１４例青春期及儿童ＧＣＴ选自１９５７～１９９５年我科收治的２１６例ＧＣＴ病人,随访２～１２年,总结其临床表现,放射学特点,治疗及随访结果。结果：青春期及儿童ＧＣＴ占全部ＧＣＴ的６５％,１３／１４发生于１５～１８岁,临床表现以疼痛、肿胀为主,并发病理骨折者较成人ＧＣＴ发生率高;放射学改变为溶骨性改变,位于干骺端者较成年组多见。临床分期２期以下（１１／１４）,行病灶刮除、５０％氯化锌水溶液烧灼瘤腔壁＋植骨,３期行瘤段切除或截肢,全部病人无复发或转移,功能优良率达９１７％。结论：青春期及儿童ＧＣＴ较多位于干骺端,绝大多数属于临床２期,行刮除＋５０％氯化锌烧灼＋植骨的方法可以达到较理想的疗效     

骨巨细胞瘤P21蛋白的表达及意义

目的 探讨骨巨细胞瘤组织中Ras癌基因的蛋白产物P21的表达及生物学意义。方法 采用ABC免疫组织化学方法对69例骨巨细胞瘤组织中P21蛋白表达进行研究。结果 骨巨细胞瘤组织中P21蛋白阳性表达率为48％,其中病理分级Ⅰ、Ⅱ、Ⅲ级P21蛋白阳性表达率分别为43％、44％、86％,Ⅲ级阳性表达率明显高于Ⅰ级和Ⅱ级(P< 0.05);Enneking外科分期1、2、3期阳性表达率分别为33％、36％和73％,3期阳性表达率极显著高于1期和2期(P<0.01)。结论 Ras癌基因产物P21蛋白表达活性在良恶性骨巨细胞瘤中的表达有明显差异,可作为恶性骨巨细胞瘤的标志物;Enneking外科分期3期P21蛋白阳性表达率明显高于1期和2期,说明P21蛋白阳性表达与骨巨细胞瘤的侵袭性有密切关系。     

The Influence of Adjuvants on Local Recurrence Rate in Giant Cell Tumour of the Bone

Introduction: Intralesional surgery of giant cell tumour of the bone (GCT) may result in a high rate of local recurrence. The introduction of local adjuvants, such as cementation, cryosurgery or phenolization, has proved to be successful in the reduction of recurrence rates. This study presents the results of a single institution in surgery of GCT with an evolution in treatment strategies.

Material & Methods: Forty primary and 25 recurrent surgical procedures in 46 patients with GCT of the bone with a median follow-up of 72 months were reviewed retrospectively. The mean age was 32.6 years (range 13.6–57.9 years). Forty-seven curettages and 18 resections were performed. For the curettages, a large bone window was cut followed by high speed burring and bone grafting or cementation. In 34 of 47 curettages and 7 of 18 resections, phenol was additionally applied.

Results: Two patients showed pulmonary metastasis, one died due to metastatic disease. In total, a third of the patients developed local recurrence (32.3%). This was evenly spread among primary and recurrent diesease (32.5% vs. 32%). Seven of 13 curettages without adjuvant recurred (53.9%), compared to 11 of 34 curettages with adjuvant phenol (32.4%). Three of 18 resections developed a recurrence (16.7%). No complications in respect to the use of phenol were seen. Discussion: Phenolization is a safe local adjuvant therapy for GCT. Although the recurrence rate was lower with the use of phenol, this drop was not significant. The comparable high recurrence rate in our study, even if phenol was used, might be due to the fact that curettage was our favoured treatment, even in cases with an extensive juxta-articular tumour. We recommend adjuvant phenolization in the treatment of GCT of the bone after thorough curettage in applicable cases, including where cementation is used for defect filling.     

Stimulated Type I Collagen Turnover in Patients With Giant Cell Tumor of Bone

The aim of this study was to determine type I collagen turnover in giant cell tumor of bone (GCT) by biochemical markers of type I procollagen aminoterminal propeptide (PINP) and type I collagen carboxyterminal telopeptide (ICTP) as synthesis and degradation markers, respectively. The serum concentrations of PINP and ICTP were measured in 11 patients with GCT using radioimmunoassay, and analyzed by the correlation to the grades of GCT progression described by Campanacci. Serum of the 11 healthy subjects was available for comparison. The serum concentration of PINP was significantly higher in patients with GCT (82.4 ± 46.2 ng/ml) than in controls (40.8 ± 12.1 ng/ml) (P < 0.01), and that of ICTP was also significantly higher in GCT (5.3 ± 2.0 ng/ml) than in controls (3.2 ± 0.8 ng/ml) (P < 0.01). In GCT, the PINP concentration of grade 3 (127.6 ± 38.8 ng/ml) was higher than that in grade 1 patients (46.9 ± 4.8 ng/ml) (P < 0.01). ICTP concentration of both grades 2 (7.1 ± 1.4 ng/ml) and 3 (5.8 ± 1.8 ng/ml) patients was significantly higher than that of grade 1 (3.5 ± 0.6 ng/ml) patients (P < 0.01, P < 0.05, respectively). Two cases of serum concentration of PINP and ICTP after resection of GCT demonstrated that these biomarkers decreased to the control levels in the absence of GCT. Our results indicated that type I collagen turnover evaluated by ICTP and PINP was stimulated in the presence of GCT. Moreover, this enhanced metabolic turnover reflects the grade of GCT.     

Cryosurgery and Impaction Subchondral Bone Graft for the Treatment of Giant Cell Tumor Around the Knee

Giant cell tumors are neoplasms of mesenchymal stromal cells with varied manifestations. There is no uniform accepted treatment protocol for these tumors. Curettage, although an accepted method of treatment, carries a high local recurrence rate. Adjuvant therapies including high-speed burr debridement, cryotherapy, and phenol treatment have been advocated to reduce local recurrence. We have used these adjuvants to determine if improved cure rate with improved outcomes could be attained with regard to local tumor control and functional outcome. Twenty-eight cases of proven giant cell tumors of the distal femur and proximal tibia were included in this prospective case series. The lesions were at the upper tibia in 14 cases and the lower femur in 14 patients. The patients were evaluated clinically, radiologically, and by histological examination. Companacci grading and Enneking staging were determined. The treatment was done in the following steps: Curettage and further debridement with a high-speed burr, cryotherapy, impaction of the cavity with subchondral iliac crest bone graft, and, finally, cementation with or without internal fixation. Functional evaluation was done by Enneking’s system. The follow-up time was between 24–40 months with a mean of 34 months. The functional results of the procedure were rated as good to excellent with a mean of 93.9%. This technique has the advantages of joint preservation, excellent functional outcome, and low recurrence rate when compared with other treatment modalities. For these reasons, it is recommended as an adjuvant to curettage for most giant cell tumors of bone.