Begnign bone tumors of childhood

The diagnosis of a bone tumor in a child can be a source of great anxiety for the patient, the parents, and the treating physician. Fortunately, most bone tumors in children are benign. Although there are a variety of benign bone tumors that affect skeletally immature patients, most have such characteristic clinical and radiographic presentations that the diagnosis can be made with reasonable accuracy without a biopsy. However, some benign bone tumors can simulate a malignant process and may be best handled by referral to a person trained in orthopaedic oncology for additional evaluation. Treatment alternatives are in part related to the Musculoskeletal Tumor Society stage of the lesion. Recurrences of certain lesions, such as aneurysmal bone cysts and osteoblastomas, can be problematic. By becoming familiar with the presentation of the more common benign bone tumors in children, physicians will be able to alleviate fears, establish a diagnosis, and make treatment recommendations in the most effective manner.     

Aneurysmal bone cysts of the sacrum

BACKGROUND: Aneurysmal bone cysts, first described by Jaffe and Lichtenstein in 1942, are benign lesions that may easily be mistaken for a malignant tumor both radiographically and pathologically. These diagnostic problems are due to their rapid growth, extensive destruction of bone, wide extraosseous tumor masses, and marked cellular exuberance. The differential diagnosis of aneurysmal bone cysts including giant cell tumor, calcified solitary bone cysts, low-grade osteosarcoma, and teleangiectatic osteosarcoma becomes even more complicated when the lesion arises at sites other than the long bones and presents with extensive extraosseous, soft-tissue tumor masses. The latter cases--especially when they occur as sacral or presacral tumors--present challenges with respect to successful treatment, which should combine surgical removal of the entire lesion following oncological criteria to prevent recurrences and osteosynthesis to guarantee the biomechanical stability of the spinal-pelvic junction. Here we report on the clinical case of a female patient with an aneurysmal bone cyst of the sacrum and extensive extraosseous tumor masses. The report includes the diagnostic challenges, the surgical options of sacral and/or presacral tumors, the histopathological findings, and long-term clinical and radiographic surveillance. METHODS: The patient was treated by a combination of preoperative adjuvant selective arterial embolization, radical surgical excision through an anterior approach followed by subsequent osteosynthesis and stabilization through a posterior approach. RESULTS: Clinical and radiographic follow-up for 2 years was uneventful, and the patient is still free of recurrence or any complaints. CONCLUSION: The current report documents the diagnostic and surgical challenge of a gigantic aneurysmal bone cyst of the sacrum and its successful management.     

Fractures due to primary tumors and tumor-like changes

The incidence of pathological fractures resulting from primary bone tumors and tumor-like lesions and various treatment options for such fractures are discussed. In addition to the benign/malignant nature, site, and extent of the tumorous lesion, the patient's age and activity level have to be considered if optimal treatment is to be selected. The treatment is completely different for a pathological fracture resulting from a benign tumor or a tumor-like lesion than for a fracture resulting from a primary malignant bone tumor. As a rule, benign tumors and tumor-like lesions require wide curettage when a fracture occurs, until macroscopically normal bone is reached. The residual cavity is then filled with autogenous cancellous bone grafts. Other fractures, e.g. those resulting from solitary bone cysts, may be treated as if normal bone were concerned. On the other hand, the treatment of a pathological fracture resulting from a primary malignant bone tumor is subordinate to the management of the primary disease, which may be life-threatening. A pathological fracture means a poorer prognosis because of contamination of the surrounding tissues. The risk of local recurrence is high, so that in most cases wide or radical resection or amputation is required.     

Osteosarcoma of bone and its important recognizable varieties.

Osteosarcoma of bone is a recognizable entity if the histopathologist designates tumors as such when their malignatn cells produce osteoid substance even if only in small foci. Such definition distinguishes this lesion from other sarcomas that arise in bone, especially chondrosarcoma and fibrosarcoma. There is a general tendency to consider that osteosarcomas represent a stereotyped form of disease for which new modalities of treatment can be applied and assessed. The question of whether a given osseous lesion is actually malignant and not a benign neoplasm or even a reactive non-neoplastic condition simulating a malignant tumor may be difficult for the histopathologist. Pathologists without considerable experience in the diagnosis of bone tumors find this question especially vexing. The establishment of a valid diagnosis of osteosarcoma introduces the additional problem that the 11 varieties considered in this paper may pose significant recognizable variations in the clinical capability of the disease. It is apparent that the physician must recognize the known clinicopathologic and prognostic factors of these subtypes in his assessment of the overall problem.     

Diaphyseal aneurysmal bone cysts (ABCs) of long bones in extremities: Analysis of surgical management and comparison with metaphyseal lesions

IntroductionAneurysmal bone cysts (ABCs) are benign lesions of long bone metaphysis affecting mostly medullary region in younger age below 20 years of life. These may be originated rarely either in the cortex or in the superficial regions of diaphysis. The study highlighted the differences of diaphyseal lesions from the usual metaphyseal ones in view of their clinical, radiological and biological behavior and also discussed their management in brief.Material and methodsWe reviewed a total of 84 aneurysmal bone cysts over the past fourteen years (2004–2017) and evaluated their surgical outcomes retrospectively. Total ten lesions were diaphyseal cortical in location (group I), while 74 lesions were conventional metaphyseal type (group II).ResultsWe observed that cortical ABCs were occurred commonly in diaphysis of femur, humerus, tibia and radius while presentation was at an older age than metaphyseal type. Radiographically these were eccentric lesion and more prone for pathological fractures than conventional type. These are differentiated radiographically from other benign lesions but also mimicking malignant conditions including low grade surface osteosarcoma and telangiectatic osteosarcoma while resemble similar to these on histopathological examination (HPE).ConclusionBiological behavior of cortical lesions does not differ significantly than conventional type but these are more prone for pathological fractures so these eccentric cortical ABC lesions should be treated with adequate internal fixation along with curettage and bone grafting. Although incidence of cortical or surface variety of ABCs is rare but surgeons might evaluate its severity in view of aggressive benign or malignant lesions of diaphysis.     

Arteriographic findings in bone tumors: experience with 170 cases

From September 1978 to June 1987, arteriography was performed respectively in 62 cases of osteosarcoma, 21 cases of giant cell tumor and 87 cases of other benign or malignant of bone tumors. In 7 cases contrast medium was injected through indwelling catheter in all other cases it was injected by direct puncture which we think should be of first choice. Arteriographic findings in osteosarcoma were neovascularity and hypervascularity (98.5%), tumor staining (56.4%), early venous drainage (48.3%), arterial displacement (67.7%), and arterial caliber changes (50.1%). Some of the features were more prominent in certain types of osteosarcoma, such as tumor staining was mostly seen in osteolytic osteosarcoma. The arteriographic findings of other malignant bone tumors were similar to those of osteosarcoma. Malignant angioarchitecture usually was not found in benign tumors, but displacement of the artery may be occasionally seen. Extraskeletal part of osteosarcoma and giant cell tumor cannot be shown clearly in plain X-ray film, but it could be clearly seen in angiography. Usually, the extraskeletal part in osteosarcoma is larger than that in giant cell tumor.     

Aneurysmal bone cyst

Aneurysmal bone cysts are rare skeletal tumors that most commonly occur in the first two decades of life. They primarily develop about the knee but may arise in any portion of the axial or appendicular skeleton. Pathogenesis of these tumors remains controversial and may be vascular, traumatic, or genetic. Radiographic features include a dilated, radiolucent lesion typically located within the metaphyseal portion of the bone, with fluid-fluid levels visible on MRI. Histologic features include blood-filled lakes interposed between fibrous stromata. Differential diagnosis includes conditions such as telangiectatic osteosarcoma and giant cell tumor. The mainstay of treatment is curettage and bone graft, with or without adjuvant treatment. Other management options include cryotherapy, sclerotherapy, radionuclide ablation, and en bloc resection. The recurrence rate is low after appropriate treatment; however, more than one procedure may be required to completely eradicate the lesion.     

脊柱单发椎体溶骨性病变的CT灌注成像研究

目的 探讨CT灌注成像技术对脊柱单发椎体溶骨性病变良、恶性鉴别诊断的价值.方法 前瞻性研究30例脊柱单发椎体溶骨性破坏患者的多螺旋CT灌注表现,经前臂肘静脉用CT自动高压注射器团注Omnipaque(300 mgI/ml)60～70 ml,总扫描时间100 s.用Perfusion软件计算获得病变组织的血流量(blood flow,BF)和血容量(blood volume,BV)的伪彩图,测量病变中心区、边缘区BF和BV,计算边缘-中心血流量差异率(rim-to-center differential blood flow ratio,Rrim-cenBF)和边缘一中心血容量差异率(rim-to-center differential blood volume ratio,Rrim-cenBV),绘制时间-密度曲线.结果 表现为高灌注者20例(16例转移瘤,3例骨髓瘤和1例脊索瘤);表现为程度不一的中、高灌注者5例(3例骨巨细胞瘤和2例动脉瘤样骨囊肿);表现为低灌注者5例(1例神经鞘瘤和4例脊柱结核).恶性骨肿瘤边缘区的BF(t=2.881)、BV (t=5.284)明显高于中心区(P<0.01),良性骨肿瘤、结核差异无统计学意义.恶性骨肿瘤Rrim-cenBF值、Rrim-cenBV值明显高于良性骨肿瘤、结核.恶性骨肿瘤的时间一密度曲线呈快速上升趋势,达到峰值后保持相对稳定;良性骨肿瘤保持平缓持续上升的趋势;结核上升幅度较小且持续、平稳,仅比正常肌肉组织略高.结论 CT灌注成像技术反映了良、恶性骨肿瘤以及结核的不同血供特点,在鉴别脊柱单发椎体溶骨性病变的良、恶性鉴别方面具有一定的临床价值.     

Soft-tissue tumors and tumor-like lesions of the foot. An analysis of eighty-three cases

The cases of eighty-three patients who had a soft-tissue tumor or tumor-like lesion in the foot or ankle were retrospectively analyzed to determine the relative frequency of the lesions and which factors, if any, could be used to identify them preoperatively. Seventy-two (87 per cent) of the lesions were benign, with ganglion cysts and plantar fibromatoses being the most common, and eleven (13 per cent) were malignant tumors, five (45 per cent) of which were synovial sarcomas. The age of the patient and the location of the lesion were the two most important factors that characterized the malignant tumors. For eight patients (12 per cent), radiographs were helpful in identifying the nature of the lesion. The sex of the patient, a history of trauma, the duration of the symptoms, the size of the lesion, and the presence of pain or of neurological symptoms were not useful in discriminating a benign lesion from a malignant tumor.     

Osteosarcoma of bone

The definition of osteosarcoma requires that a malignant tumor of bone produce osteoid or bony matrix. With this as a basic definition, osteosarcoma of bone can be divided into several clinicopathologic entities based on clinical, roentgenographic, and pathologic features. The tumors can be broadly divided into those arising within the bone and those arising on the surface of bone. Most intraosseous osteosarcomas are high-grade malignant tumors that occur in children and adolescents. A small number occur in older patients, and they may be related to a preexisting condition, such as Paget's disease or radiation. The site of the lesion has prognostic importance. Osteosarcoma of the jaws is associated with an especially good prognosis, whereas the same kind of tumor involving the skull has a very poor prognosis. Most osteosarcomas of the surface of bone are well or moderately differentiated and are associated with an excellent prognosis. Received for publication on March 17, 1998; accepted on April 28, 1998     

DNA-PCNA dual-staining analysis of bone and soft tissue tumors with flow cytometry

To detect the biological behavior of bone and soft tissue tumors or tumorous lesions is often difficult, but it is essential when planning therapeutic strategy. The DNA-Proliferating-cell nuclear antigen (PCNA) dual-staining analysis method, using flow cytometry, was employed to discover whether it could be of any use in this problem. Specimens were obtained from 9 benign bone lesions, 9 benign soft tissue lesions, 6 malignant bone tumors (3 low-grade, 3 high-grade), and 15 malignant soft tissue tumors (8 low-grade, 7 high-grade). From each of these specimens, DNA histograms, PCNA scores, and cell cycle fractions were acquired and analyzed. There was no significant relationship between DNA ploidy or the PCNA score and the malignancy of the tumors. However, a high PCNA score was observed in the recurrent lesions of four benign tumors. High-grade malignant tumors showed significantly higher PCNA scores than low-grade malignancies (P=0.01). All except one benign lesions showed G0, G1, and M fractions only. In malignant tumors, the G2 cell fraction was significantly higher in high-grade than in low-grade malignancies (P=0.01). One patient with a malignant tumor with marked G1, S, and G2 fractions showed remarkable response to preoperative chemotherapy and radiation therapy. These findings indicate that this method has the ability to disclose the biological nature of cells and provide important information on bone and soft tissue tumors.     

Curettage of benign bone tumors and tumor like lesions: A retrospective analysis

Background:

Curettage is one of the most common treatment options for benign lytic bone tumors and tumor like lesions. The resultant defect is usually filled. We report our outcome curettage of benign bone tumors and tumor like lesions without filling the cavity.

Materials and Methods:

We retrospectively studied 42 patients (28 males and 14 females) with benign bone tumors who had undergone curettage without grafting or filling of the defect by any other bone graft substitute. The age of the patients ranged from 14 to 66 years. The most common histological diagnosis was that of giant cell tumor followed by simple bone cyst, aneurysamal bone cyst, enchondroma, fibrous dysplasia, chondromyxoid fibroma, and chondroblastoma and giant cell reparative granuloma. Of the 15 giant cell tumors, 4 were radiographic grade 1 lesions, 8 were grade 2 and 3 grade 3. The mean maximum diameter of the cysts was 5.1 (range 1.1-9 cm) cm and the mean volume of the lesions was 34.89 cm³ (range 0.94-194.52 cm³). The plain radiographs of the part before and after curettage were reviewed to establish the size of the initial defect and the rate of reconstitution, filling and remodeling of the bone defect. Patients were reviewed every 3 monthly for a minimum period of 2 years.

Results:

Most of the bone defects completely reconstituted to a normal appearance while the rest filled partially. Two patients had preoperative and three had postoperative fractures. All the fractures healed uneventfully. Local recurrence occurred in three patients with giant cell tumor who were then reoperated. All other patients had unrestricted activities of daily living after surgery. The rate of bone reconstitution, risk of subsequent fracture or the incidence of complications was related to the size of the cyst/tumor at diagnosis. The benign cystic bone lesions with volume greater than approximately 70 cm³ were found to have higher incidence of complications.

Conclusion:

This study demonstrates the natural healing ability of bone without filling with bone grafts or bone graft substitutes. In selected sizes and locations of the benign lytic tumors and tumor like lesions extended curettage alone can be sufficient.     

Premalignant tumors and conditions of bone.

Most bone sarcomas arise in apparently normal bone. However, some sarcomas arise in preexisting benign bone tumors or in nonneoplastic conditions. Some of the lesions, such as multiple exostoses and Ollier's disease, have a propensity to develop chondrosarcoma. Rarely does a benign giant cell tumor become malignant without prior irradiation, malignant transformation of other benign tumors, such as osteoblastoma and chondroblastoma, is a medical curiosity. Among nonneoplastic conditions, radiation changes, long-standing chronic osteomyelitis, and Paget's disease have definite premalignant connotations. However, the risk of developing cancer in these conditions is still low.     

Severe life-threatening hypersensitivity reaction to polidocanol in a case of recurrent aneurysmal bone cyst

Aneurysmal bone cysts (ABC) are expansile lytic lesions constituting around 1% of all benign bone tumors with an annual incidence of 1.4/100000. A variety of treatments are available ranging from curettage with or without bone grafting (autologous or allogeneic), curettage with use of adjuvants [Polymethylmethacrylate (PMMA) bone cement, high speed burr, phenol, liquid nitrogen], wide en-block excision with or without reconstruction, selective arterial embolization of the feeding vessels, radiation therapy, high precision megavoltage radiotherapy and percutaneous radio-nuclide ablation, sclerotherapy (ethibloc, aetoxisclerol, alcohol gel, polidocanol). The optimal treatment is debatable due to various indications and contraindications of different modalities of treatment. Recent data suggest that percutaneous sclerotherapy with polidocanol is safe and effective alternative to surgery for treatment of ABCs as it has minimal side effects. We are reporting the first case of life-threatening adverse reaction to intra-lesional polidocanol in a three-year-old boy with a proximal femoral aneurysmal bone cyst. The importance of reporting this case is to make people aware regarding the adverse reaction of polidocanol and to highlight the precautions one should follow while using polidocanol for aneurysmal bone cysts.     

特制人工髋关节假体在髋部骨肿瘤的应用分析

目的探讨特制人工假体在髋部肿瘤保肢术中的应用价值。方法回顾性分析我院自1999年3月至2005年5月髋部原发肿瘤患者行特制人工假体置换术后疗效、并发症及处理资料。本组肿瘤病例中,髋臼11例,股骨近端15例;肿瘤类型:软骨肉瘤3例,骨肉瘤6例,滑膜肉瘤1例,骨巨细胞瘤12例,良性纤维组织瘤2例,动脉瘤样骨囊肿2例。假体类型:人工全髋关节假体17例,人工双极股骨头7例,马鞍式关节假体2例。结果随访时间为18个月～6年,平均4年3个月;10例恶性肿瘤局部复发率40%,保肢率60%,16例中间性及良性肿瘤复发率12.5%,保肢率87.5%,参照Enneking(MSTS)评定标准平均得分19分,优良率76.9%。结论特制人工髋关节假体具有良好的术后肢体功能,是髋部骨肿瘤较为满意的保肢治疗方法之一。     

Surgical therapy of benign and low-grade malignant intramedullary chondroid lesions of the distal femur: intralesional resection and bone cement filling with or without osteosynthesis

Surgical treatment of benign and low-grade malignant intramedullary chondroid lesions at the distal femur is not well analyzed compared to higher-grade chondrosarcomas. Localization at the distal femur offers high biomechanical risks requiring sophisticated treatment strategy, but scientific guidelines are missing. We therefore wanted to analyze a series of equally treated patients with intralesional resection and bone cement filling with and without additional osteosynthesis. Twenty-two consecutive patients could be included with intralesional excision and filling with polymethylmethacrylate bone cement alone (n?=?10) or with compound bone cement osteosynthesis using a locking compression plate (n?=?12). Clinical and radiological outcome was retrospectively evaluated including tumor recurrences, complications, satisfaction, pain, and function. Mean follow-up was 55 months (range 7–159 months). Complication rate was generally high with lesion-associated fractures both in the osteosynthesis group (n?=?2) and in the non-osteosynthesis group (n?=?2). All fractures occurred in lesions that reached the diaphysis. No fractures were found in meta-epiphyseal lesions. No tumor recurrence was found until final follow-up. Clinical outcome was good to excellent for both groups, but patients with additional osteosynthesis had significantly longer surgery time, more blood loss, longer postoperative stay in the hospital, more complications, more pain, less satisfaction, and worse functional outcome. Intralesional resection strategy was oncologically safe without local recurrences but revealed high risk of biomechanical complications if the lesion reached the diaphysis with an equal fracture rate no matter whether osteosynthesis was used or not. Additional osteosynthesis significantly worsened final clinical outcome and had more overall complications. This study may help guide surgeons to avoid overtreatment with additional osteosynthesis after curettage and bone cement filling of intramedullary lesions of the distal femur. Meta-epiphyseal lesions will need additional osteosynthesis rarely, contrary to diaphyseal lesions with considerable cortical thinning.     

Myelolipoma mimicking osteosarcoma in the distal femur

Introduction and importancePeriosteal reactions indicate malignant bone tumors, including osteosarcoma; establishing an accurate diagnosis is key to determining the most appropriate treatment strategy. We describe a rare case of myelolipoma in the distal femur metaphysis with massive extraskeletal lesions and periosteal reactions.Case presentationA 25-year-old woman was referred to our hospital to treat a gradually expanding mass around her knee that grew to the size of a baby's head. She had a history of hydrocephalus caused by congenital cytomegalovirus infection and was bedridden for life. Radiography showed a prominent osteoblastic rim and osteolytic lesion with a moth-eaten appearance. Osteosarcoma was suspected due to excessive extraskeletal invasion and periosteal reactions. T1- and T2-weighted magnetic resonance images showed a high-signal-intensity homologous lesion. Biopsy specimens contained adipose and hematopoietic tissues. A myelolipoma was diagnosed. Due to her fragility, surgical intervention was suspended. Two years after diagnosis, the tumor size did not change.Clinical discussionMyelolipomas are benign tumors that typically arise from the adrenal gland and rarely develop in the extremities. This type of tumor typically does not cause any tumor-related symptoms or endocrine disturbances and has been reported as a type of incidentaloma. To effectively manage myelolipoma patients, differential diagnosis of tumors mimicking malignant bone tumors is important.ConclusionWe successfully managed a destructive ectopic myelolipoma in the distal femoral metaphysis, with massive extraskeletal lesions and periosteal reactions. Clinicians should appropriately differentiate myelolipoma from tumors mimicking malignant bone tumors.     

Treatment of bone tumor using external fixator

External fixation has been widely used for the treatment of limb length discrepancy, deformity correction, and bone defect using distraction osteogenesis. We have been using this technique for the treatment of obstacles due to benign bone tumor and reconstruction after malignant bone tumor resection. In this paper, we introduce the usefulness of external fixation for the treatment of various benign bone tumors and limb salvage surgery for reconstruction after tumor resection using distraction osteogenesis, according to the tumor site. The advantages of external fixation for the treatment of benign bone tumor include a possibility of complex deformity correction without treating the tumor itself and reconstruction of the aggressive benign bone tumor as well as solid fixation with wires, even for weak bone due to benign bone tumors. The advantages of treating malignant bone tumor include regeneration of living bone of sufficient strength and durability, biological affinity, resistance against infection, and lifelong restored function. The disadvantages include delayed union at the docking site and pin-or wire-tract infection, patient's psychological stress, and the procedure is time consuming. We conclude that external fixation is very efficient for the treatment of benign and malignant bone tumors.     

T-lymphocyte subsets in peripheral blood of patients with bone tumors

Peripheral blood T-cell subsets of 54 patients with malignant or benign bone and soft tissue tumors and 20 age-and sex-matched normal controls were studied by using monoclonal antibodies. T-cell subsets were also studied before and after operation in ten patients with osteosarcoma. The number of T cells (OKT 11) and OKT 4 cells significantly decreased in malignant bone and soft tissue tumors. The number of OKT 8 cells increased. Statistically significant decrease in the T4/T8 ratio and the number of macrophage were also found in malignant bone and soft tissue tumors. In giant cell tumor of bone, the number of T cells and OKT 4 cells were also statistically decreased. But, the number of OKT 8 cells and macrophage remained unchanged. The ratio of T 4/T 8 was nearly normal. There was no difference in T cell subsets between benign bone tumors and normal controls. In malignant bone tumors, the number of T cells and T 4/T 8 ratio in some degree may be as a predictor for prognosis.     

Overexpression of hexokinase-2 in giant cell tumor of bone is associated with false positive in bone tumor on FDG-PET/CT

Introduction

The aim of the current study was to evaluate the usefulness of maximum standardized uptake value (SUV_max) in 2-deoxy-2-F¹⁸-fluoro-d-glucose positron emission tomography combined with computed tomography (18F-FDG-PET/CT) for preoperative differential diagnosis between benign and malignant bone tumors.

Materials and methods

Seventy-nine patients with bone tumors were examined by FDG-PET prior to histopathological diagnosis. The SUV_max was calculated and compared between benign and malignant lesions, and among different histopathological subgroups, to identify false-positive histological subtypes.

Results

There was a statistically significant difference in the SUV_max of benign (3.7?±?3.3; n?=?17) and malignant (5.3?±?3.3; n?=?62) bone tumors. However, receiver operating characteristic curve analysis revealed the poor accuracy of this distinction. The cut-off value was determined to be 2.6, while the value of sensitivity and specificity was calculated to be 74.2 and 64.7?%, respectively. Giant cell tumor of bone (9.0?±?2.0; n?=?5) displayed a higher SUV_max than osteosarcoma (4.2?±?2.3; n?=?18). Immunohistochemical analysis demonstrated that markers of these cancers, hexokinase-2 (HK-2) and glucose transporter type 1 (GLUT-1), supported our findings.

Conclusion

The poor accuracy of SUV_max in 18F-FDG-PET/CT in distinguishing malignant from benign bone tumors was confirmed; some benign bone tumors showed high FDG uptake. Giant cell tumor of bone was a major false-positive histopathological subtype of bone tumors, showing high FDG accumulation. HK-2 contributed significantly to FDG uptake, whereas GLUT-1 appeared to play no role in FDG uptake in giant cell tumor of bone.