Osteogenic sarcoma with pulmonary metastasis visualized by bone imaging

Extraosseous soft tissue metastases from osteosarcoma occasionally accumulate Tc-99m MDP. The authors present a case of osteogenic sarcoma of the femur with a pulmonary metastasis detected by bone imaging with radiographic correlation including CT and magnetic resonance imaging. The implication of the detection of osseous and extraosseous metastasis in these patients by bone imaging is discussed. The changing role of bone imaging in the initial workup and follow-up of patients with osteogenic sarcoma is reviewed in light of the change in course and prognosis of the disease as a result of recent advances in adjuvant chemotherapy.     

继发性骨肉瘤的临床特点及影像学表现

目的:分析继发性骨肉瘤的临床特点及影像学表现.方法:回顾性分析经手术病理证实的25例继发性骨肉瘤患者的病例资料.25例均行X线及CT检查(5例行增强CT),16例行MRI增强检查.结果:本组患者的中位年龄45岁(14～76岁),中位恶变时间为8年(0.5～18.0年).25例中骨纤维结构不良、骨母细胞瘤等良性肿瘤或肿瘤样病变恶变12例,表现为溶骨性破坏、软组织肿块形成及病灶迅速增大等与原发骨肉瘤相仿的影像学改变;骨巨细胞瘤植骨后恶变继发性骨肉瘤8例、金属植入相关的继发性骨肉瘤2例,均表现为移植骨吸收、周围出现明显软组织肿块及肿瘤骨形成;放疗后继发性骨肉瘤2例,以成骨性骨质破坏为主;Paget病继发性骨肉瘤1例,表现为在患骨增粗、不规则变形基础上,出现溶骨性破坏及软组织肿块.病理结果:镜下有不同程度的细胞异形性、肿瘤骨形成.所有病例术后随访时间为1.5～12.0年(平均3.4年),8例出现肺部转移.结论:病理组织学上继发性骨肉瘤与原发性骨肉瘤无明显差异,但结合其临床资料及影像学表现可明确诊断.     

Parosteal osteogenic sarcoma arising in cranial bones: clinical and radiologic features in eight patients

Parosteal osteogenic sarcoma is a distinct surface bone tumor with a better prognosis than conventional osteogenic sarcoma. We studied eight histologically proved cases of cranial parosteal osteogenic sarcoma. The tumors were identical in histologic appearance to parosteal osteosarcoma arising in long bones. Clinically, the tumor presented as a hard, painless, nodular scalp mass. The prevalence in women outnumbered that in men by 3:1, with most cases occurring between the second and third decades of life. Plain radiographs showed a rounded, sessile bone growth of variable size arising from the outer table of the skull. The tumor was heavily ossified centrally with variable margins and, at times, with radiating bony spicules at the periphery. No satellite bone nodules were noted in adjacent soft tissues. In three cases a fine radiolucent cleft was demonstrated between the tumor and the underlying outer table on the tangential radiographs or CT. After en bloc resection of the tumor, follow-ups for 20 years in one patient and 1 year in two patients showed no recurrence. Parosteal osteosarcoma of the skull is a rare low-grade tumor that usually arises from the outer table of the skull and has distinctive radiologic features that should distinguish it from other exophytic cranial bone neoplasms.     

Imaging findings of extrapulmonary metastases of osteosarcoma

PURPOSE: To review imaging findings of extrapulmonary metastasis from osteosarcoma and to evaluate them for any consistent pattern and correlation between imaging findings. MATERIALS AND METHODS: This study was retrospectively conducted in 13 patients with extrapulmonary metastasis of pathologically confirmed osteosarcoma. We evaluated the radioisotope (RI) scans (n=16), ultrasonography (USG) (n=4), computed tomography (CT) scans (n=10), MRIs (n=6), clinical records, and pathological reports for assessment of imaging findings and correlation between radiologic findings and RI uptake of the lesions. Points evaluated were the following: uptake on RI scans, presence of mineralization on CT, and MRI, size, enhancement pattern, attenuation on CT, signal intensity (SI) on MRI, and echogenicity on USG. RESULTS: Extrapulmonary metastatic sites were diverse, including another bone other than the primary site (n=6), lymph node (n=4), pleura (n=2), liver (n=2), pancreas (n=1), kidney (n=1), peritoneum (n=1), muscle (n=1), and subcutaneous fat layer (n=1). One patient had tumor growth within the pulmonary artery and jejunum. Among 21 metastatic sites in 13 patients, bone scan was performed in 16 cases and RI uptake was detected in 10 lesions. Calcification was detected in eight lesions on radiologic imaging, including plain radiography, USG, CT, and MRI. Two lesions showed RI uptake without definite calcification or ossification on MRI and plain radiography, respectively. We analyzed the enhancement pattern and mass size in 18 metastatic sites and these factors had poor correlation with uptake on RI scans. CONCLUSION: The sites and imaging findings of extrapulmonary metastasis of osteosarcoma were variable. All the lesions with mineralization were detectable on RI scans prior to radiologic imaging. RI scan has a limited role in the evaluation of metastatic lesions without mineralization.     

Bone Tumors Occurring in the Soft Tissues: A Review of the Clinical,Imaging, and Histopathologic Findings

Although rare in everyday practice, malignancies that classically arise from bone or cartilage have been reported to arise de novo in various soft tissues in the body, resulting in a diagnostic challenge for the clinician, radiologist, and pathologist.Differential diagnoses of bone tumors often depend on anatomic location of the lesion. For example, the classic location of osteosarcoma is in the metaphysis of long bones about the knee. Histologically osteosarcoma is characterized by tumor cells that directly produce osteoid, bone, or cartilaginous matrix. In extraskeletal osteosarcoma, the clinical and radiologic picture is very different from a conventional osteosarcoma. They occur in older patients, present as a soft tissue mass often coincidentally following trauma and have a worse prognosis. The imaging characteristics are often nonspecific with mineralized elements in a well-defined soft tissue mass. The mineralized elements may or may not be visible. Magnetic Resonance sequences demonstrate a well circumscribed soft tissue mass with hemorrhagic and enhancing solid components. The pathologic features of extraskeletal osteosarcoma on a microscopic scale are identical to that of skeletal lesions. Likewise, conventional chondrosarcomas present in older patients with a growing, painful soft tissue prominence most commonly involving the long tubular bones. In extraskeletal chondrosarcoma however, the presentation is in somewhat younger patients with a painful soft tissue prominence typically in the head (meninges), neck, or upper leg. The pathologic features are most often that of a myxoid chondrosarcoma which is characterized by strands of small cells over a myxoid matrix. Imaging features include chondroid matrix, heterogenous contrast enhancement, and amorphous internal calcification on Computed Tomography. On Magnetic Resonance sequences the matrix has a low signal on all sequences, and variable inhomogeneity depending on grade of the lesion. Other extraskeletal bone tumors include Ewing's sarcoma and osteoid osteoma amongst other lesions.Although these malignancies may be rare clinical entities, they often exhibit characteristic clinical, imaging, and histopathological findings although differing in treatment and prognosis. Knowledge of these and other common mimicking lesions will help guide the clinician and radiologist to make an accurate diagnosis.     

Imaging findings in pseudocystic osteosarcoma

OBJECTIVE: Our purpose was to describe four female patients with osteosarcoma whose clinical and imaging findings primarily suggested either simple or aneurysmal bone cyst. All lesions were osteolytic, intracompartmental, and expanded bone without periosteal reaction. None of the patients presented during the peak age incidence for osteosarcoma. From imaging to histologic diagnosis, the discovery of osteosarcoma ranged from 1 week to 3 years. CONCLUSION: Atypical osteosarcoma may rarely mimic simple or aneurysmal bone cyst radiologically and may show a nonmalignant rate of growth. It may be more frequently encountered in females and may not present during the peak age incidence for osteosarcoma. Microscopically, the tumors were not cystic, necrotic, or telangiectatic but were conventional osteosarcoma and osteoclast-rich osteosarcoma.     

长骨骨干骨肉瘤X线、CT及MRI表现

目的 分析长骨骨干骨肉瘤X线、CT和MRI表现,探讨有关的临床特点和鉴别诊断.方法 28例长骨骨干骨肉瘤患者,均经手术与病理证实,其中病变位于股骨干18例、腓骨干4例、肱骨干4例、胫骨干2例.所有患者均行X线、CT和MR检查,对其影像学表现与手术病理结果进行对照,并由双盲法分析确认.结果 28例中,X线和CT显示广泛骨质破坏16例,骨膜反应22例.X线显示软组织肿块18例,肿瘤骨和瘤样钙化12例.CT平扫显示软组织肿块22例,增强扫描显示软组织肿块24例,肿瘤骨和瘤样钙化16例.MRI显示骨质破坏和骨膜反应10例,软组织肿块26例,其周围可见软组织水肿及骨髓水肿.骨膜反应在SE T1WI上呈等低信号,T2WI呈等信号.软组织肿块在T1WI为等信号,T2WI及STIR呈等高信号.软组织水肿及骨髓水肿在T2WI及STIR呈高信号.MRI增强检查显示病灶均呈不均匀强化,骨髓水肿和软组织肿块均见强化.结论 X线、CT和MRI从不同方面反映长骨骨干骨肉瘤的影像病理特点,其发病率低,骨破坏范围大,无病理性骨折.成骨型骨干骨肉瘤较易诊断,溶骨型应与Ewing瘤、恶性巨细胞瘤等鉴别.

Abstract：

Objective To explore the findings of diaphysial osteosarcoma in long bone on X-ray,CT and MRI, and discuss their clinical features and manifestations for differential diagnosis. Methods Twenty-eight cases with diaphysial osteosarcoma in long bone proved by surgery and pathology were reviewed retrospectively. Eighteen tumors were located in the femur, 4 in fibula, 4 in humerus and 2 in tibia. All of the patients were examined by X-ray, CT and MRI. The imaging manifestations on X-ray, CT and MRI were analyzed, and the relationship of the imaging features with the pathological types was also observed. The imaging signs were correlated with the pathologic findings with a double blind method. Results Of the 28 cases, there were 16 cases with large bone destruction, 22 cases with periosteal reaction on X-ray and CT. On X-ray, 18 cases showed soft tissue mass and 12 cases with neoplastic bone and tumor calcification.While on CT, 22 cases showed soft tissue mass on plain scan and 2 more cases displayed soft tissue mass after the injection of contrast mediun. Sixteen cases showed neoplastic bone and tumor calcification on CT.On MRI, there were 10 cases with bone destruction and periosteal reaction with iso- and hypo-intense on T1WI and iso- signals on T2WI. Twenty-six cases showed soft tissue edema and bone marrow on MRI. The soft mass were iso-signals on T1 WI and iso-hyperintense signals on T2 WI or STIR. The soft tissue edema was found hyperintense signals on T2WI or STIR. The lesions had heterogeneous enhancement especially in bone marrow with edema and adjcent soft tissue. Conclusion The X-ray, CT and MRI can reflect the pathological changes of diaphysial osteosarcoma in long bone from different aspects. Lower incidence, large bone destruction and no pathological fracture were the features of diaphysial osteosarcoma. The osteogenic type is diagnosed easily, but the osteolytic lesion should be differentiated from Ewing sarcoma, malignant giant cell tumor of bone and so on.     

Mineralization in musculoskeletal leiomyosarcoma: radiologic-pathologic correlation

OBJECTIVE: Mineralization in leiomyosarcoma, a malignant tumor of smooth muscle, has not been widely recognized. In this article, we report our experience with four cases of primary leiomyosarcoma of soft tissue or bone in which mineralization was visible on either radiography or CT. In none of the cases was the diagnosis of leiomyosarcoma considered before biopsy. In one case of a soft-tissue leiomyosarcoma, the presence of mineralization was a factor that led to the misinterpretation of the needle biopsy specimen as soft-tissue osteosarcoma. CONCLUSION: Histologically, mineralization in leiomyosarcoma appears to be caused by either nonneoplastic ossification or dystrophic mineralization in the tumor. This feature can cause leiomyosarcomas to be confused with other neoplasms.     

Intracortical osteoblastic osteosarcoma with oncogenic rickets

Intracortical osteosarcoma is the rarest variant of osteosarcoma, occurring within, and usually confined to, the cortical bone. Oncogenic osteomalacia, or rickets, is an unusual clinicopathologic entity in which vitamin D-resistant osteomalacia, or rickets, occurs in association with some tumors of soft tissue or bone. We present a case of oncogenic rickets associated with intracortical osteosarcoma of the tibia in a 9-year-old boy, whose roentgenographic abnormalities of rickets disappeared and pertinent laboratory data except for serum alkaline phosphatase became normal after surgical resection of the tumor. Histologically, the tumor was an osteosarcoma with a prominent osteoblastic pattern. An unusual microscopic feature was the presence of matrix mineralization showing rounded calcified structures (calcified spherules). Benign osteoblastic tumors, such as osteoid osteoma and osteoblastoma, must be considered in the differential diagnosis because of the relatively low cellular atypia and mitotic activity of this tumor. The infiltrating pattern with destruction or engulfment of normal bone is a major clue to the correct diagnosis of intracortical osteosarcoma. The co-existing radiographic changes of rickets were due to the intracortical osteosarcoma.     

Bone scintigraphy in two cases of primary vertebral osteosarcoma in adults

The primary vertebral osteosarcoma in adults is a rare tumor which represents less than 2% of all osteosarcomas. We present the cases of two men (40 and 33 years old) who began with pain and neurological compression symptoms. The imaging methods used to study the tumors were X-rays, CT, MRI and bone scintigraphy with 99mTc-HMDP. After the pathological diagnosis, the tumors were removed surgically and the treatment was completed with chemotherapy and radiotherapy. In this report the authors review the published cases of vertebral osteosarcoma, its epidemiology, clinical presentation and characteristics in the different imaging techniques.     

Recurrent spontaneous pneumothoraxes as a complication of osteosarcoma metastases: a case report

Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. Osteosarcomas are highly aggressive tumors that historically have had a dismal prognosis. However, the survival rate has improved significantly with the addition of adjuvant and neoadjuvant chemotherapy. Here, we present a case report of a 13-year-old male with a history of a left humeral osteosarcoma whose course was complicated by recurrent sarcoma-related pneumothoraces. Despite recurrent pneumothoraces being a relatively uncommon complication of osteosarcoma, they present a great challenge to providing treatment that optimizes outcomes and quality of life for patients.     

Primary osteosarcoma of a metatarsal bone

Although osteosarcoma is the most common primary bone malignancy of childhood and adolescence that is not related to marrow cells, involvement of the short tubular bones is uncommon. In contrast to more conventional sites, where the tumor is usually high grade and found in adolescents, osteosarcoma of the small bones is more likely to be low grade, and is often seen in older individuals. We present a case of low-grade primary osteosarcoma of a metatarsal bone in a 25-year-old woman. Received: 22 December 1998 Revision requested: 3 February 1999 Revision received: 24 April 2000 Accepted: 26 April 2000     

Clear cell chondrosarcoma of the pelvis in a skeletally immature patient

We report on a case of clear cell chondrosarcoma (CCCS) of the left iliac bone in a 12-year-old skeletally immature boy. Radiographic examination revealed an aggressive osteolytic lesion with areas of mineralization. Fluid-fluid levels were seen on T2-weighted MR images. Laboratory data showed slight elevation of serum alkaline phosphatase. The biopsy specimen showed histological features of CCCS with some resemblance to osteosarcoma, such as prominent irregular osteoid formation among clear tumor cells. Surgical treatment was accomplished without pre- or post-operative chemotherapy. Because of the patient’s age, elevated serum alkaline phosphatase, and histopathology with prominent osteoid production, this case could be confused with osteosarcoma. Although CCCS is an extremely rare bone tumor in children, it is important to be aware that it may arise in a skeletally immature patient. CCCS, unlike osteosarcoma, is not treated with neo-adjuvant chemotherapy. Received: 13 November 1998 Revision requested: 28 December 1998 Revision received: 11 January 1999 Accepted: 14 January 1999     

The clinical and diagnostic imaging findings of osteosarcoma of the jaw

OBJECTIVE: To clarify the valuable clinical features and diagnostic imaging findings regarding the diagnosis of osteosarcoma of the jaw (OSJ). MATERIALS AND METHODS: The initial symptoms and diagnostic imaging findings of 10 patients with OSJ were analysed. The points analysed on the diagnostic images were as follows: any widening of the periodontal ligament space of the teeth on the periphery of the OSJ; the presence of radial spicules and Codman's triangle; any signs of bone destruction; and the patterns of osteogenesis. RESULTS: All patients had pain and/or swelling of the affected site, and all OSJs, except for one edentulous case, showed a widening of the periodontal ligament space of the teeth on the periphery of the OSJ. Radial spicules or Codman's triangle were observed in only three cases (30%). Four out of five mandibular OSJs were osteolytic or osteolytic dominant with bone destruction, while, in contrast, four out of five maxillary OSJs were osteogenic or osteogenic dominant, and three out of the four maxillary OSJs did not show bone destruction. The osteogenic OSJ without bone destruction was similar to some benign cemento-osseous lesions of the jaw and thus was difficult to diagnose as OSJ based on the diagnostic imaging findings alone. CONCLUSION: Even though some OSJ showed features similar to the benign tumours of the jaw bone based on the diagnostic imaging findings, the pain and swelling of the affected site, and the widening of the periodontal ligament space of the teeth on the periphery of OSJ were considered to be common findings, which may help in making an accurate diagnosis of OSJ in this limited series.     

High-activity samarium-153-EDTMP therapy in unresectable osteosarcoma

Despite highly efficacious chemotherapy, patients with osteosarcomas still have a poor prognosis if adequate surgical control cannot be obtained. We applied high-activity Sm-153-EDTMP therapy within a multimodal therapy concept to improve local control of an unresectable osteosarcoma with poor response to initial polychemotherapy. A 21-year-old woman with an extended, unresectable pelvic osteosarcoma and multiple pulmonary metastases was treated with high-activity of Sm-153- EDTMP (150 MBq/kg BW, total 8.1 GBq). Afterwards external radiotherapy of the primary tumor site was performed and polychemotherapy was continued, followed by autologous peripheral blood stem cell reinfusion. Within 48 h after Sm-153-EDTMP application the patient had complete pain relief. After three weeks the response was documented by 3-phase Tc-99m-MDP bone scintigraphy (primary tumor and metastases: decreased tracer uptake), whole-body F-18-FDG-PET (primary tumor and metastases: diminution of glucose metabolism) and thoracic CT (metastases: reduction of size). The present case warrants further evaluation of feasibility and efficacy of this multimodal therapy combination of high-activity Sm-153-EDTMP therapy, external radiation, polychemotherapy and stem cell support for unresectable osteosarcomas.     

Diagnosis of osteosarcoma in a patient previously treated for Ewing sarcoma

Primary malignant bone tumors, whether Ewing sarcoma or osteosarcoma, are a rare type of tumor. The sequential occurrence of two bone sarcomas, Ewing sarcoma and high-grade osteosarcoma, in the same patient at two different locations is an exceptionally rare phenomenon. We present the case of a 13-year-old girl who presented with a high-grade osteoblastic osteosarcoma of the distal femur, 7 years after treatment for Ewing sarcoma of the left pelvis. She did not receive radiation therapy. Following the recent developing multidisciplinary therapy, long-term follow-up for monitoring latent treatment-related adverse effects may be necessary for survivors of primary malignant bone tumors.     

Prognostic significance of (18)F-FDG and (99m)Tc-methylene diphosphonate uptake in primary osteosarcoma.

The purpose of this retrospective analysis was to evaluate the prognostic significance of both initial glucose metabolism as measured by (18)F-FDG PET and osteoblastic activity as measured by (99m)Tc-methylene diphosphonate (MDP) bone scintigraphy in osteosarcoma. METHODS: In 29 patients (18 male, 11 female; age range, 5-41 y) with primary osteosarcoma, (18)F-FDG uptake and (99m)Tc-MDP uptake were measured semiquantitatively (average and maximum tumor-to-nontumor ratios [T/NT(av) and T/NT(max), respectively]) using PET and bone scintigraphy at the time of diagnosis. After chemotherapy, the patients underwent surgery for their primary tumor, and the response was determined histologically. Cumulative overall survival and event-free survival were determined by clinical and imaging follow-up of 7-72 mo (median, 28 mo). RESULTS: Clinical and imaging follow-up revealed that the disease relapsed or failed to achieve complete remission in 9 patients and that 6 patients died of the disease. Both overall and event-free survival were significantly better in patients with a low (18)F-FDG T/NT(max) (less than the median) than in patients with a high (18)F-FDG T/NT(max) (at least the median). The negative relationship of (18)F-FDG T/NT(av), (99m)Tc-MDP T/NT(max), and (99m)Tc-MDP T/NT(av) with overall and event-free survival did not reach a level of significance. (18)F-FDG uptake values correlated moderately and positively with (99m)Tc-MDP uptake values, but a level of significance was reached only between (18)F-FDG T/NT(max) and (99m)Tc-MDP T/NT(av). CONCLUSION: The initial glucose metabolism of primary osteosarcoma as measured by (18)F-FDG PET using T/NT(max) provides prognostic information. High (18)F-FDG uptake correlates with poor outcome. Thus, (18)F-FDG uptake may be complementary to other well-known factors in judging the prognosis in osteosarcoma.     

Rare primary cranial vault and base of the skull tumors in children. Report of 30 cases with a short literature review

Twenty-six cases of rare primary cranial vault tumors are reported, together with 4 cases of primary tumors of the base of the skull and 3 cases of monostotic cranial neuroblastoma. Whereas some rare primary cranial vault tumors may present with characteristic radiographic patterns (e.g. hemangioma, aneurysmal bone cyst, osteoma, progonoma), most of them can be recognised only after histology. The most frequent tumor in the region of previous irradiation is osteosarcoma. The only "common" primary bone tumor of the base of the skull is chordoma. The radiological differential diagnosis of primary tumors of the skull vault and base is discussed.     

Primary vertebral osteosarcoma: imaging findings

PURPOSE: To evaluate patient age and sex and location and imaging appearances of primary vertebral osteosarcoma (PVOS) compared with histologic subtypes. MATERIALS AND METHODS: Retrospective review (1915-2001) of imaging findings in patients with histologically proved primary osteosarcoma of vertebral column was performed. Two radiologists in consensus reviewed findings for location, origin site, matrix pattern, and spinal canal invasion and compared them with histologic subtypes. Radiation-induced, Paget, metastatic, and multifocal osteosarcoma were excluded. RESULTS: Of 4,887 osteosarcoma cases, 198 (4%) were PVOS arising from vertebral column. There were 103 female and 95 male patients (age range, 8-80 years; median age, 34.5 years). Involvement included cervical (27 patients), thoracic (66 patients), lumbar (64 patients), and sacral (41 patients) spine. Adequate imaging findings were available in 69 patients, and involvement of two levels was seen in 12 (17%). In nonsacral spine, most tumors (44 cases) arose from posterior elements, with partial involvement of vertebral body. Lesions confined to vertebral body were less frequent (12 cases). Sacral tumors involved body and sacral ala. The most common histologic subtypes were osteoblastic (47 patients), chondroblastic (12 patients), telangiectatic (four patients), fibroblastic (four patients), small cell (one patient), and epithelioid (one patient). The majority (55 cases) demonstrated osteoid matrix mineralization; 17 showed marked mineralization. Five cases with marked mineralization were confined to vertebral body, with "ivory vertebra" appearance. Purely lytic pattern was seen in 14 (20%) cases. Lytic pattern was seen in four (100%) telangiectatic, three (75%) fibroblastic, three (25%) chondroblastic, three (6%) conventional osteoblastic, and one (100%) small-cell subtypes. Invasion of spinal canal was common (84% of cases). Appearance simulating osteoblastoma without soft-tissue mass was present (seven cases). Pathologic compression fractures were identified (seven patients). CONCLUSION: This study provides age and sex distribution and location and imaging features in a large series of PVOS.     

骨膜骨肉瘤的影像学诊断与病理学分析

目的:探讨骨膜骨肉瘤的影像学及组织病理学特点。方法:收集6例骨膜骨肉瘤患者的临床及影像资料;6例均摄X线平片,5例行CT扫描,4例行MRI扫描;对其影像学及组织病理学表现进行分析和总结。结果:骨膜骨肉瘤好发于胫骨,影像学表现为发生在长骨的骨干或相当于干骺部位置的骨表面肿块,病灶内见局灶性骨化或环状钙化(本组3例),边缘可见骨膜反应(本组2例),肿瘤下骨皮质不受侵,但不侵犯骨髓腔。骨膜骨肉瘤组织学上由大量分叶软骨构成,肿瘤细胞呈明显异型性。结论:骨膜骨肉瘤是位于长骨骨皮质表面的恶性骨肿瘤,影像表现与病理表现相结合可作出正确诊断。