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A case of synchronous multicentric osteosarcoma is reported in an 11-year-old boy. This very rare condition is characterized by simultaneous involvement of multiple skeletal sites, and the prognosis is generally poor.
Radiolabelled somatostatin analogues are of potential value in the imaging of somatostatin receptor-positive tumours. Recently, somatostatin receptors have been demonstrated in the osteoblast precursor cells. In this preliminary study, we evaluated the uptake characteristics of indium-111 octreotide in two benign and two malignant bone tumours. Tracer accumulation was observed in all four cases, and overall lesion to background ratio (mean+/-SD) was 2. 74+/-0.84 and 2.98+/-1.49 at 4 h and 24 h, respectively. There was no clear relationship between 111In-octreotide accumulation and the benign or malignant nature of the tumour. In one patient, tracer uptake was inhibited by unlabelled octreotide administration. These results suggest that 111In-octreotide can be taken up by benign and malignant bone tumours. The inhibition of tumour uptake by treatment with cold octreotide supports the concept that specific uptake mechanisms are responsible for 111In-octreotide deposition by bone tumours.
Alterations of tumour suppressor genes are considered crucial steps in the development of human cancers. Expressions of p53 protein, a product of the tumour suppressor gene altered most commonly in human cancers examined so far, were investigated immunohistochemically in 18 osteosarcomas and 40 other malignant and benign lesions of bone. A monoclonal antibody clone PAb240, which recognizes a common conformational epitope of mutant p53 proteins, stained nuclei of tumour cells in 12 of 18 osteosarcomas (67%). Six tumours (33%) particularly showed positive immunoreactions in more than half of the tumour cells. PAb240 also stained tumour cells in a small number of other malignant bone tumours, such as malignant fibrous histiocytoma, chondrosarcoma, and Ewing's sarcomas. Furthermore, a small number of cells of giant-cell tumours were positively stained. In contrast, PAb240 was completely negative in 21 benign bone tumours and reactive lesions examined. Another monoclonal antibody clone PAb1801, which reacts with both wild- and mutant-type p53 protein, reacted in nuclei of tumour cells of 7 osteosarcomas (39%). Most of those also reacted with PAb240. PAb1801 was expressed much more frequently in other malignant bone tumours and giant-cell tumours. In addition, PAb1801 showed intranuclear positive reactions in tumour cells of a benign chondroblastoma, and reactive cells such as actively proliferating preosteoblasts in a myositis ossificans and osteoclast-like giant cells in a giant-cell tumour. The immunoelectron-microscopic observation that p53 protein was localized in euchromatic areas of nuclei of osteosarcoma cells supported the specificity of immunoreaction for p53 protein, indicating an active role of p53 protein in the regulation of DNA synthesis and transcription. These findings suggest that point mutation of the p53 gene is frequently involved in the development of osteosarcomas. PAb240 may be a useful tool not only in screening point mutations of the p53 gene in osteosarcomas but also in the differential diagnosis between osteosarcomas and reactive bone-forming lesions. Expressions of mutant p53 protein were not correlated with any clinical or pathological factors examined, although the results should be confirmed in studies of a large number of osteosarcomas.
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In order to evaluate the value of the ultrasonography in the diagnosis of tumor of the knee and its clinical implication, 67 patients with clinically suspected bone tumor of the knee were examined by ultrasound. The ultrasonographic characteristics of different bone tumors were studied and compared with the results of pathologic characters after operation. Ultrasonography can readily visualize the bony destruction and the pathologic change of the periosteum and the soft tissue related to bone tumor. Fifty-two cases of malignant bone tumors and 15 cases of giant cell tumors were diagnosed by ultrasonography. Pathologically, there were 54 cases of malignant bone tumor and 13 cases of giant cell tumor. It was concluded that ultrasonographic examination might be a useful method for the diagnoses of bone tumor of the knee and play an important role in guiding needle biopsy and electing operative method and approach.
UNLABELLED: AIM OF THE STUDY, METHOD: The advantages of a prophylactic care of fracture-endangered, osseous metastasis of the mammary cancer stand opposite to the perioperative risk and to conservative alternatives. As a pathologic fracture cannot surely be excluded while performing a conservative proceeding, a retrospective trial was set up to compare the results of treatment after a pathologic fracture (n = 35) with those undergoing a prophylactic attendance (n = 44). RESULTS: The intraoperative, cardio-pulmonary complications were distributed in balance totally amounting to 20.3% (n = 16). Intraoperative complications concerning surgical procedure (n = 3) exclusively occurred within the fracture group. Generally, postoperative complications arose in 20.3% (n = 16) of all cases, in which the patients belonging to the fracture group were increasingly afflicted [28.6% (n = 11/35) vs. 11.4% (n = 5/44); p < 0.02]. While there were no differences between both groups concerning the postoperative, surgery-technical complications, significantly more patients (91.8% [n = 40/44]) of the prophylactic-care group achieved a complete postoperative usability of the operated area than in the fracture-group [74.3% (n = 26/35)] (p < 0.05). The average survival time tended to be longer within the prophylactic-care group [19.3 +/- 15.6 month (prophylactic-care group) vs. 15.0 +/- 16.9 month (fracture group)]. CONCLUSIONS: The prophylactic treatment of fracture endangered, osseous metastasis of the mammary cancer leads to reduction of the general, postoperative complications compared to the patients with a pathologic fracture. Further, those patients have a better chance to recover full mobility after surgery. Considering the long survival time after the incidence of osseous metastasis at the mammary cancer a prophylactic treatment represents the method of first choice compared with the conservative treatment which persistently contains the risk of fracturing.
In this report, we describe the clinical, imaging, and pathologic findings in a case of diaphyseal telangiectatic osteosarcoma arising 23 years after successful treatment of bilateral retinoblastomas. To our knowledge, telangiectatic osteosarcoma occurring as a second tumor in this setting, outside the radiation treatment field, has not been previously reported. The association of the RB-1 gene defect with retinoblastomas and other tumors is discussed. Also, the computed tomography and magnetic resonance imaging findings in telangiectatic osteosarcoma are described and discussed.
OBJECTIVE: The purpose of our study was to report the imaging characteristics of 12 high-grade surface osteosarcomas, diagnosed, staged, treated and followed up in a single institution. DESIGN AND PATIENTS: There were 3 females and 9 males ranging in age from 15 to 34 years (mean 20 years). The radiological documents (plain films in all patients, CT scans in 8 and MR images in 6) were reviewed and compared with the histological findings. RESULTS: Disease sites were the femur (7), tibia (4) and fibula (1). Tumor size varied from 6 to 17 cm. The cortex appeared normal in two patients, thick in seven and/or thin in five. The tumor showed dense ossification in all except in one case where ossification was slight. Minimal marrow involvement was visible in two cases on CT/MRI. Only one case exhibited a satellite nodule in a previous biopsy tract. Two cases radiologically mimicked periosteal osteosarcoma. CONCLUSION: High-grade surface osteosarcomas are often characterized by the presence of an ossified mass contiguous with and originating from the cortical bone with no or minimal marrow involvement.
Aggressive juxta-articular giant cell tumours of the lower limbs occurring in young patients are a challenge to the average orthopaedic surgeon. Although it is the treatment of choice for these tumours, wide resection creates a problem for the reconstruction of large bone gaps. We describe our results after resection arthrodesis of such tumours using the technique of bone transport over a long intramedullary nail in 27 patients. This is the first and largest study of its kind in the management of giant cell tumours in the literature. All our patients fared well with this mode of treatment, and none had recurrence or major complications.
Limb salvage operations using large custom-made prostheses have become the treatment of choice for juxta-articular tumours at the knee. Resection-arthrodesis has been used since the 1920s and offers a satisfactory long term solution for young active patients. The good functional results after this procedure in 9 patients is presented here. This method should still be considered in selected cases.
Periosteal Ewing's sarcoma is very rare. In this report, we describe three cases of periosteal Ewing's sarcoma, illustrating the principal clinical, radiographic, and histologic features. The patients were aged 15, 16, and 20 years old, with an average of 17 years. The tumor occurred in the humerus in two and in the femur in one. Plain radiography showed a laminated periosteal reaction with a Codman's triangle over a subperiosteal soft-tissue mass or saucerization. The unusual site of Ewing's sarcoma was clearly visualized by computed tomography (CT) and magnetic resonance imaging (MRI) and confirmed by histological examination. Chemotherapy and/or irradiation was employed before and after wide excision. The patients have survived an average of 6 years after the primary surgery.
We performed two rotationplasties (type A1 and A2) as salvage procedures in cases of custom-made endoprosthetic replacements of the distal femur and knee joint. The patients suffered from malignant bone and soft-tissue tumors of the knee, respectively. One of them developed chronic osteomyelitis around both stems of the prosthesis, and three local revisions turned out to be ineffective. The other patient, suffering from parossal osteosarcoma of the distal femur, developed a local recurrence following endoprosthetic reconstruction. The functional abilities following the operation of these two patients suggested that rotationplasty was a viable alternative to above-the-knee amputation in failed or severely complicated total arthroplasty of the knee with massive bone loss.
We investigated a dedifferentiated chondrosarcoma of a 61-year-old woman with an osteosarcomatous high-grade component for p53 alteration. The low-grade cartilaginous and the high-grade osteosarcomatous components of the tumor were macrodissected and evaluated separately by immunohistochemistry and molecular biology. We used PCR-SSCP analysis and direct sequencing to screen exons 4-8 for p53 mutations. The p53 intron 1-polymorphism was investigated for loss of heterozygosity. A functionally relevant p53 missense mutation in codon 193 of exon 6 (A-to-T transversion) with loss of wild-type allele was detected only in the dedifferentiated component. Using the monoclonal antibody DO-1, immunohistochemistry failed to show p53 overexpression. This evidence of p53 mutation may be regarded as at least a co-factor that "switched" the preexisting low-grade conventional chondrosarcoma to a highly malignant dedifferentiated tumor.
BACKGROUND: Malignant change in fibrous dysplasia (FD) is very rare. This study was carried out to establish some characteristic clinical information about this disorder. METHODS: Four cases with a malignant change in FD out of 128 cases with FD were surgically treated and followed up for a median period of 61.3 months. The mean age of the patients was 39.8 years. Clinical features, radiological findings, and the outcome were analyzed for each of the four cases. RESULTS AND CONCLUSION: The sites of the lesions were tibia (2 cases), femur (1 case), and rib (1 case). The forms of FD were monostotic in one case and polyostotic in three cases. Radiologically, plain films and computed tomography (CT) showed osteolytic lesions with poorly delineated margins within and/or near areas having a ground-glass appearance. In the osteolytic lesions, simple cystic changes associated with old FD could be excluded by enhanced magnetic resonance imaging (MRI). Histopathologically, two cases were osteosarcoma, one case was malignant fibrous histiocytoma (MFH), and one case was fibrosarcoma. The management of this disease should be decided according to the type of primary high-grade bone sarcoma. One patient, with MFH, was dead of lung metastasis 13 months after surgery. The others are alive without disease.
An observational cohort study was performed on 90 hip arthroplasties performed in 84 patients for metastatic disease of the hip. Significant improvement was noted with both 3-month and 6-month function scores vs preoperative function scores (P < .001). Complications included 1 intraoperative femur fracture, 2 cases of deep venous thrombosis, 1 peroneal nerve palsy, 1 deep infection, and 5 dislocations. Eight (8.8% of 90 procedures, 9.4% of 84 patients) patients died during the initial hospital stay. Although the risk of mortality after hip arthroplasty for metastatic diseases is perhaps higher than previously expected, improvement in postoperative function scores in surviving patients was significant and perioperative morbidity in this complex patient population was acceptably low.
Adrenal gland metastasis from osteogenic sarcoma is extremely rare and is an atypical location for metastasis. Vascular hepatic exclusion techniques and the use of venovenous bypass with cardiac surgical techniques may be required to resect large adrenal masses that have invaded the inferior vena cava to achieve curative resection. The use of newer chemotherapeutic agents and aggressive surgical resection have prolonged the survival of patients with osteosarcoma.
The application of nuclear magnetic resonance (NMR) techniques to the diagnosis of cancer was first explored by Damadian, who proposed that benign and malignant tissues could be differentiated on the basis of characteristic differences in spin-lattice and spin-spin relaxation times (T1 and T2) as determined in vitro with NMR spectrometers. Damadian's thesis was very controversial and never gained widespread acceptance. Not all investigators were able to confirm his findings. Moreover, it was improbable that NMR would ever play an important role in the diagnosis of malignancy as long as biopsy was necessary to obtain material for analysis. However, the potential usefulness of NMR in oncology was enhanced considerably by the work of Lauterbur, who showed that NMR signals could be spatially encoded to produce images of the examined object. NMR imaging has made feasible the measurement of the T1 and T2 of lesions without biopsy. Unfortunately, initial efforts at characterizing tissues by in vivo determination of proton relaxation times have yielded disappointing results. Nonetheless, NMR imaging will be a powerful tool for evaluating patients with malignant disease because of the unique anatomic information it can provide without exposure of the patient to ionizing radiation. In vivo NMR spectroscopy of 31P and other sensitive nuclei may add a new dimension to clinical and experimental oncology.
A method is described whereby an osteoid osteoma may be conveniently removed on an outpatient basis, using a bone biopsy drill and CT control.