"Benign" giant cell tumor of bone metastasizing to lung

A case of “benign” metastasizing giant cell tumor is presented. Previous experience shows that this is a relatively favorable lesion not having the grave implications of sarcomatous disease, as evidenced by the good long-term results in our previously reported patient [14] and a number of others reported in the literature. An aggressive surgical approach to pulmonary metastasis of giant cell tumors is warranted because long-term control, if not permanent eradication, can be accomplished by that means.     

Treatment of giant cell tumor of bone: Current concepts

Giant cell tumor (GCT) of bone though one of the commonest bone tumors encountered by an orthopedic surgeon continues to intrigue treating surgeons. Usually benign, they are locally aggressive and may occasionally undergo malignant transformation. The surgeon needs to strike a balance during treatment between reducing the incidence of local recurrence while preserving maximal function.Differing opinions pertaining to the use of adjuvants for extension of curettage, the relative role of bone graft or cement to pack the defect and the management of recurrent lesions are some of the issues that offer topics for eternal debate.Current literature suggests that intralesional curettage strikes the best balance between controlling disease and preserving optimum function in the majority of the cases though there may be occasions where the extent of the disease mandates resection to ensure adequate disease clearance.An accompanying treatment algorithm helps outline the management strategy in GCT.     

Mucin-producing pancreatic tumors: A study of nuclear DNA content by flow cytometry

Nuclear DNA content in eight surgically resected mucin-producing pancreatic tumors (MPPT) consisting of two mucinous intraductal adenocarcinomas (MIDAC), two mucinous intraductal adenomas (MIDA), one mucinous cystadenocarcinoma (MCAC), and three mucinous cystadenomas (MCA) were measured by flow cytometry using paraffin-embedded tissue samples. The technique of Shutte was used for the preparation of paraffin-embedded tissue into single dissociated nuclei, while the method of Vindelov was used for staining the isolated nuclei with propidium iodine. Clinicopathologically, the four patients with MIDAC or MIDA were all male and had cystic lesions with a dilated pancreatic duct at the head of the pancreas, while the four patients with MCAC or MCA were all females and had cystic tumors at either the body or tail of the pancreas. All eight patients with MPPT had no metastasis to the regional lymph nodes and were all still alive without recurrence. In an analysis of nuclear DNA content, seven of eight patients had DNA diploid tumors while one patient with a MIDAC perforating the duodenum and choledochus had a DNA aneuploid tumor. Thus, these findings suggest that DNA diploid patterns in MPPT might be associated with a favorable prognosis in MPPT although some patients whose MPPT invaded the surrounding organs might have DNA aneuploid tumors.     

肩胛骨骨巨细胞瘤1例

骨巨细胞瘤是常见的原发性骨肿瘤之一,起始于骨髓内间叶组织,原发部位几乎都发生在长管状骨干骺端,原发于肩胛骨极为少见。1病例资料患者,男,33岁。因右肩肩胛骨肿块7个月入院。无明显诱因及症状,无家族病史。查体:一般情况良好,发育正常,营养中等,无明显消瘦,双上肢前屈、后伸     

骶骨巨大骨巨细胞瘤1例

1病例资料 患者,女,24岁。腰骶部胀痛不适2年、加重,伴下腹包块进行性增大、坠胀、尿频6个月余,于2008年6月14日入院。发病3个月后出现月经不调,无畏寒发热、尿急尿痛、肉眼血尿等异常。实验室检查：RBC3．1×10^12／L,Hb 95g/L,血沉28mm／1h;AFP、CEA、CA125、CA199均正常;     

Resection-reconstruction arthroplasty for giant cell tumor of distal radius

Background:

Giant cell tumor (GCT) of the distal radius poses problems for reconstruction after resection. Several reconstructive procedures like vascularized and non-vascularized fibular graft, osteo-articular allograft, ceramic prosthesis and megaprosthesis are in use for substitution of the defect in the distal radius following resection. Most authors advocate wrist arthrodesis following resection of distal radius and non vascularized fibular graft. Here we have analyzed the results of aggressive benign GCTs of the distal radius treated by resection and reconstruction arthroplasty using autogenous non-vascularized fibular graft.

Materials and Methods:

Twenty-four cases of giant cell tumor of the distal radius (mean age 32 years, mean follow-up 6.6 years) treated by en-bloc resection and reconstruction arthroplasty using autogenous non-vascularized ipsilateral fibular graft with a minimum followup of two years have been included in this retrospective study. Nineteen cases were of Campanacci grade III and five were of Grade II recurrence. The mean resected length of the radius was 9.5 (8-12) cm. Routine radiographs and clinical assessments regarding pain, instability, recurrence, hand grip strength and functional status were done at regular intervals and functional results were assessed using (musculoskeletal tumor society) MSTS-87 scoring.

Results:

Early radiological union at host-graft junction was achieved at mean 12.5 weeks, (range 12-14 weeks) and solid incorporation with callus formation was observed in mean 29 weeks (range 28-32 weeks) in all the cases. Satisfactory range of motion (mean 63%, range 52-78%) of the wrist was achieved in 18 cases. Grip strength compared to the contralateral hand was found to be 67% (range 58-74%). Functional results were excellent in six cases (25%), good in 14 cases (58.3%) and four (16.7%) cases had fair results. Soft tissue recurrence was seen in one patient. The most commonly encountered complication was fibulo-carpal subluxation (10 cases, 41.7%).

Conclusion:

Resection of the distal radius and reconstruction arthroplasty with non-vascularized proximal fibular graft is useful in preserving the functional movement and stability of the wrist as well as achieving satisfactory range of movement and grip strength.     

16例膝关节骨巨细胞瘤的外科治疗

1992年3月至2000年1月收治膝关节周围骨巨细胞瘤患者16例,采用彻底切刮病灶后氩气束综合电刀反复烧灼残腔,自体髂骨T型或倒T型植骨,L-梯形加压钢板内固定治疗,经6个月至7年随访,取得满意疗效.     

Multicentric giant cell tumor around the knee

A case of multicentric giant cell tumor with synchronous occurrence in all three bones around the knee is reported here in view of its rarity. A 33-year-old average built male reported with complaints of severe pain, gradually increasing swelling around the right knee. A 3 × 2 cm swelling was present on the lateral aspect of the distal end of the right femur and a 3 × 3 cm swelling on the proximal part of the right tibia. Plain X-ray of right knee showed subarticular eccentrically located expansile lytic lesion in the lateral tibia condyle, lateral condyle of femur and patella. Fine needle aspiration cytology and subsequent histology ascertained the diagnosis of giant cell tumor of the bone. The patient was treated successfully with curettage, bone grafting and methyl methacrylate cementing (Sandwich technique).     

刮除并植骨治疗四肢骨巨细胞瘤50例

目的 探讨刮除植骨治疗四肢骨巨细胞瘤的疗效。方法 将50例Ⅰ-Ⅱ级四肢骨巨细胞瘤彻底刮除，用3%碘酒在病损腔内反复涂擦，蒸馏水反复冲洗，然后植入自体骨。结果 经2-13年随访，优良率88%，3例复发，无截肢病例。结论 Ⅰ-Ⅱ级四肢骨巨细胞瘤采用该方法可获得较为满意的疗效。     

Multicentric giant cell tumor involving the same foot: A case report and review of literature

Multicentric giant cell tumour (GCT) is extremely rare; no case has been previously reported where two lesions occurred in the same foot at different sites. We report a case involving the calcaneus and subsequently the 3^rd toe of the same foot and review the reported literature. In established cases of multicentricity, the histopathology has to be properly reviewed and the patient has to be followed up for a longer time with serial whole body assessment to pick up any subsquent lesions. The treatment of the local disease does not differ from a standard GCT.     

肢体骨巨细胞瘤复发特征与治疗

目的 总结肢体骨巨细胞瘤复发特征及治疗措施。方法 随访复发性肢体骨巨细胞瘤30例,对其临床表现、影像学特征、病理及治疗方法进行回顾性分析。结果 复发病例大多为病灶刮除术后,3年内复发占73．33％,其中有症状者26例,以局部疼痛、肿胀为主;无症状者4例。影像学主要表现为局部植骨吸收,出现不规则密度减低区,病变范围扩大。组织学检查27例复发后仍呈良性,3例恶变。复发治疗;早期病例行再次病灶清除植骨术17例,其中再复发3例;行瘤段切除关节置换4例、关节融合术3例、截肢术6例,均未再复发。结论 定期随访可以早期发现病灶;早期病例可采用彻底的病灶清除加植骨术,晚期病例可采用瘤段切除人工关节置换或截肢等手术。     

Excision of high sacral giant cell tumor and reconstruction with bone cement

We report the case of a woman who had a right sacroiliac giant cell tumor with S1 nerve root involvement. No specific functional defects were noted after she underwent combined anterior abdominal and posterior sacral approaches for tumor excision and reconstruction with bone cement. Our goal is to determine whether or not, with carefully planned procedures and delicate nerve protection, the biomechanical stability of the sacrum was affected after the tumor resection. We believe that bone cement impaction can be considered as an alternative reconstructive method instead of high-priced, complicated fixation devices or allografts that are not readily available.     

PTHrP increases RANKL expression by stromal cells from giant cell tumor of bone

Giant cell tumor of bone (GCT) presents with numerous osteoclast-like multinucleated giant cells that are principally responsible for the extensive bone resorption by the tumor. Although the precise etiology of GCT remains uncertain, the accumulation of giant cells is partially due to the high expression of the receptor activator of nuclear factor-κB ligand (RANKL) from the neoplastic stromal cells. Here, we have investigated whether parathyroid hormone-related protein (PTHrP) plays a role in the pathogenesis of GCT. Immunohistochemistry results revealed PTHrP expression in the stromal cells of the tumor, and that its receptor, the parathyroid hormone type 1 receptor (PTH1R), is expressed by both the stromal cells and giant cells. PCR and Western blot analyses confirmed the expression of PTHrP and PTH1R by isolated stromal cells from five patients presenting with GCT. Treatment of GCT stromal cells with varying concentrations of PTHrP (1-34) significantly increased both RANKL gene expression and the number of multinucleated cells formed from RAW 264.7 cells in co-culture experiments, whereas inhibition of PTHrP with a neutralizing antibody decreased RANKL gene expression. These results suggest that PTHrP is expressed within GCT by the stromal cells and can contribute to the abundant RANKL expression and giant cell formation within the tumor.     

Prognostic role of nuclear factor/IB and bone remodeling proteins in metastatic giant cell tumor of bone: A retrospective study

Giant cell tumor of bone (GCTb) represents 5% of bone tumors, and although considered benign, 5% metastasize to the lung. The expression of proteins directly or indirectly associated with osteolysis and tumor growth was studied on 163 samples of GCTb. Of these, 33 patients developed lung metastasis during follow‐up. The impact of tumor–host interaction on clinical aspects was evaluated with the aim of finding specific markers for new biological therapies, thus improving clinical management of GCTb. Protein expression was evaluated by immunohistochemical analysis on Tissue Microarray. The majority of GCTb samples from patients with metastatic disease were strongly positive to RANKL and its receptor RANK as well as to CAII and MMP‐2 and to pro‐survival proteins NFIB and c‐Fos. Kaplan–Meier analysis indicated a significant difference in metastasis free survival curves based on protein staining. Interestingly, the statistical correlation established a strong association between all variables studied with a higher τ coefficient for RANK/RANKL, RANK/NFIB, and RANKL/NFIB pairs. At multivariate analysis co‐overexpression of NFIB, RANK and RANKL significantly increased the risk of metastasis with an odds ratio of 13.59 (95%CI 4.12–44.82; p < 0.0005). In conclusion, the interconnection between matrix remodeling and tumor cell activity may identify tumor–host endpoints for new biological treatments. © 2015 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 33:1205–1211, 2015.     

Bladder wash flow cytometry in transitional cell carcinoma: useful or misleading?

To evaluate the practical usefulness of flow cytometry (FC) applied to bladder wash specimens for the diagnosis of transitional cell carcinoma (TCC), a study was conducted on a series of 101 cases comprising 60 patients with tumor or with past history of TCC, and a control group of 41 patients undergoing cystoscopy for causes other than TCC in which the absence of tumor was confirmed after 1 year of follow-up. When results of the 33 patients with tumor were compared with those of the control group, FC gave low specificity and positive predictive values (54% and 58%, respectively). Conventional cytologic study was superior to FC in this setting. Although the combination of both techniques increased the sensitivity for low-grade tumors, specificity remained lower than that of cytologic study alone. Otherwise, when considering only the cases with a past history of TCC, results of FC were superior to those of cytologic examination, and the combination of both techniques gave high sensitivity and negative predictive values (94% and 93%, respectively). In conclusion, the use of FC in a general diagnostic setting could be misleading, whereas in the follow-up of patients with a history of TCC it becomes a useful adjunct to cytologic study in order to obtain a high diagnostic performance that could allow cystoscopies to be spaced out in these patients.     

Quantitative DNA measurement by flow cytometry and image analysis of human nonseminomatous germ cell testicular tumors

Current clinical staging, which includes the use of serum tumor markers and imaging techniques, fails to identify the 30–40% of clinical stage I (CS I) nonseminomatous germ cell testicular tumor (NSGCT) patients who have occult metastatic disease. Therefore, there is a real clinical need to evaluate new biological parameters of the primary tumor that might be useful as predictors of occult metastatic disease. This study was undertaken to compare quantitative DNA measurements by flow cytometry and image analysis in CS I NSGCT, and to analyze the relevance of these parameters for predicting occult lymph node involvement. Different blocks of formalin-fixed, paraffin-embedded NSGCTs of 62 CS I patients who underwent retroperitoneal lymph node dissection between 1985 and 1989 were prepared according to the Hedley technique, and analyzed by quantitative cytometry. Thirty-six (58.1%) patients had histologically proven lymph node involvement (pathological stage II), whereas 26 (41.9%) patients (pathological stage I) had neither lymph node metastases according to retroperitoneal lymph node dissection (RPLND) specimens nor tumor recurrence during follow-up. Concordant results were found in 76.5% of the samples by both cytometric techniques. For flow cytometry, the percentages of aneuploid cells in the S- and the G2M+S-phase were the most robust predictive parameters for lymph node involvement, whereas for image analysis the 5c exceeding rate (5cER) had the most predictive significance. Based on the experience obtained in this study, both cytometric techniques provide additional information on tumor aggressiveness that might be useful in therapeutic selection of early stage NSGCT patients for either RPLND or surveillance only.     

Osteoclast-like giant cell tumor of the liver: A rare neoplasm with an aggressive clinical course

Osteoclast-like giant cell tumors (OCGTs) usually involve the bone and rarely affect the alimentary tract. Within the gastrointestinal tract the liver has been one of the most infrequently reported locations for this neoplasm to occur. In this article we report the occurrence of an OCGT arising in the liver of a 61-year-old woman. The patient presented with abdominal pain and a rapidly enlarging hepatic mass. Magnetic resonance imaging (MRI) indicated a multilocular solid lesion in the right lobe of the liver. A small extrahepatic lobulation at the lateral aspect of the lesion with penetration of the capsule was visible. Local extension into adjacent organs was not evident. Positron emission tomography (PET) did not indicate a tumor in the pancreas or elsewhere in the body. The tumor was removed by performing a formal right hepatic lobectomy. Histologic and immunohistochemical examinations revealed an OCGT. Within 3 months of the hemihepatectomy, widespread intraabdominal and pulmonary metastasis developed and the patient succumbed to her illness shortly thereafter. This report contributes further evidence to the aggressive biological behavior with regard to this rare neoplasm. The absence of metastatic disease indicated when using magnetic resonance imaging and positron emission tomography does not seem to change the overall dismal prognosis of this tumor.     

Tenosynovial giant cell tumor in the foot and ankle

BackgroundTenosynovial giant cell tumor (TSGCT) originates from the synovial cells of the tendon sheath and is the most common soft tissue tumor of the foot and ankle. Due to the lack of clinical data about TSGCT in the foot and ankle, this study was performed with the aim of investigating the clinical characteristics, and surgical outcomes that might predict the likelihood of recurrence.MethodsClinical data, obtained from the pathology records and the clinic files, along with the tumor subtype, local recurrence, and patient functional status among 26 cases of TSGCT were evaluated with the mean 73 months follow-up period.ResultsThere were 26 patients including 16 males and 10 females with a mean age of 40 years, who underwent surgery. There were 15 localised TSGCT and 11 diffuse TSGCT. The diffuse TSGCT was more likely to be in the hindfoot dorsum (54,5%, 6/11). The localised TSGCT was mostly located in the forefoot (80%, 12/15). The recurrence rate in the diffuse TSGCT was 27,3% (3/11). In the localised TSGCT, recurrence was seen in 6,6% of patients (1/15). The mean AOFAS score was 79.ConclusionDiffuse TSGCT is more likely to occur in the hindfoot and localised TSGCT is more common in the forefoot. Excision with clear margins is an effective treatment for TSGCT, with good oncological and clinical outcomes. But the orthopaedic surgeons should consider the equilibrium between surgical margins and the functional status of the patient.     

食指腱鞘巨细胞瘤1例

1病例资料 患者,男,23岁.右食指末节掌侧肿胀1年余,隐痛3个月,无其他不适,肿胀逐渐加重,食指活动可.体检:右食指末节掌侧明显肿胀,皮肤无红热,掌侧偏尺侧可触及一肿物,约1 cm×0.6 cm×0.6 cm,表面光滑,边界清楚,可活动,质韧,轻度压痛.X线片检查:右食指末节指骨尺侧骨质外压性凹陷(图1),局部软组织肿胀.