Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas

Chordomas and chondrosarcomas are malignant tumors that are reported to have similar clinical presentations and radiological features but different behaviors and outcomes. The aim of this retrospective study was to determine whether specific radiological features of skull-base chordomas or chondrosarcomas are correlated with histopathology, and thus allow preoperative diagnosis. The study involved 32 classic chordomas, 6 chondroid chordomas and 4 chondrosarcomas (42 tumors total). For each case, tumor size and extent, the detailed anatomy involved, and magnetic resonance imaging and computed tomography findings were analyzed. Tumor extent was assessed using a novel method that assessed presence/absence in 18 defined skull-base zones. The chondrosarcomas presented significantly earlier in life than the chordomas (means, 20.5 years versus 36 years, respectively). At time of diagnosis, the median tumor volume was 23 cm(3) (range, 1.2-78.8 cm(3)) and the mean tumor extent was 6.7+/-2.9 zones. There were no differences between chordomas and chondrosarcomas, or between the two chordoma subgroups, with respect to lesion volume or extent. Comparison of other imaging findings revealed no features that were diagnostic for either chordoma or chondrosarcoma. The data support previous claims that chondrosarcomas present earlier in life than chordomas, but this finding is not diagnostic. There is wide variation in the extent of skull-base chordomas and chondrosarcomas, and in the specific anatomical structures these tumors involve. None of the MRI or CT features of these tumors appear to be useful for differentiating chordomas from chondrosarcomas preoperatively. For surgical planning, specific, area-oriented definition of tumor extent might provide more useful information than tumor-type classification schemes.     

Extraskeletal mesenchymal chondrosarcoma: case report

This is a case report of an extraskeletal mesenchymal chondrosarcoma (ESMC) that originally occurred in the retroperitoneum of a 24-year-old female and later metastasized to the left proximal humerus. Mesenchymal chondrosarcomas are very rare in comparison to conventional chondrosarcomas and even more so when arising from an extraskeletal location. In this report, we discuss the major characteristics of ESMC and offer a review of the current knowledge regarding this rare disease entity.     

Primary retroperitoneal extraskeletal mesenchymal chondrosarcoma: a computed tomography diagnosis

Mesenchymal chondrosarcomas, although very rare compared with conventional chondrosarcomas, are one of the few primary malignant tumours of bone that sometimes also arise in the soft tissues. Here we present a rare case of retroperitoneal extraskeletal mesenchymal chondrosarcoma (ESMC) in a 50-year-old female that showed characteristic extensive calcification. In this report, we discuss CT features of ESMC with pathological correlation.     

Carbon Ion Radiotherapy for Chordomas and Low-Grade Chondrosarcomas of the Skull Base

PURPOSE: To prospectively evaluate outcome and toxicity after carbon ion radiotherapy (RT) in chordomas and low-grade chondrosarcomas. PATIENTS AND METHODS: Between September 1998 and December 2001, 74 patients were treated for chordomas and chondrosarcomas with carbon ion RT at the "Gesellschaft für Schwerionenforschung" (GSI). Seven patients reirradiated with reduced carbon ion doses after conventional RT were excluded from the analysis, leaving 67 evaluable patients (44 chordomas and 23 chondrosarcomas) who received a full course of carbon ion therapy. Tumor-conform application of carbon ion beams was realized by intensity-controlled raster scanning with active energy variation. Three-dimensional treatment planning included intensity modulation and biological plan optimization. A median dose of 60 GyE was applied to the target volume within 20 consecutive days at a dose of 3.0 GyE per fraction. RESULTS: Median follow-up was 15 months (range 3-46 months). At 3 years, actuarial local control was 100% for chondrosarcomas and 87% for chordomas, respectively. Partial tumor remission was observed in 14/44 (31%) chordoma patients and in 4/23 (17%) chondrosarcoma patients. At 3 years, actuarial overall survival was 100% for chondrosarcomas and 89% for chordomas, respectively. No severe side effects > CTC degrees III have been observed. CONCLUSIONS: These data demonstrate the clinical efficiency and safety of scanning beam delivery of carbon ion beams in patients with skull base chordomas and chondrosarcomas. The observation of tumor regressions at a dose level of 60 GyE may indicate that the biological effectiveness of carbon ions in chordomas and chondrosarcomas is higher than initially estimated.     

A rare cause of laryngeal distress: chondrosarcoma of the larynx

Laryngeal chondrosarcomas are much less aggressive lesions than chondrosarcomas that arise elsewhere in the body. Two cases of chondrosarcoma of the larynx encountered during a seven year period are presented, with a review of the literature. Computed tomographic (CT) and magnetic resonance imaging (MRI) findings are described. The MRI findings of laryngeal chondrosarcoma are compared to those of cartilaginous tumors elsewhere in the body. The differential diagnosis, prognosis, and treatment of laryngeal chondrosarcomas are discussed.     

Angiographic features of chondromas and chondrosarcomas

Angiography was performed in 19 cases of pathologically confirmed chondromatous tumors. These tumors, due to their specific intercellular nutrition via diffusion, are relatively less vascular than osteoid and fibrous tissue tumors. All benign chondromatous tumors revealed normal angiographic features. In cases of chondrosarcomas, there was a variety of angioarchitecture, ranging from normal to increased vascularity. Heterogeneous vascularity was seen in the majority of hypervascular chondrosarcomas and was related to the cell composition of the tumor. There is a close relationship between the number of vessels in a tumor and its degree of clinical and histopathologic malignancy. The most vascular part of a tumor is the least differentiated and should be used for biopsies. Angiography can be used for the grading of chondrosarcomas, and probably for assessing the clinical prognosis.     

Dedifferentiated chondrosarcoma of bone with leiomyosarcomatous mesenchymal component: a case report

Dedifferentiated chondrosarcomas are well-described aggressive tumors arising from low-grade chondrosarcomas and containing high-grade mesenchymal sarcomatous components. These tumors are important to consider in the differential diagnosis of well-defined lytic lesions in the pelvis associated with large soft-tissue components. We present the plain film, computed tomographic, and angiographic findings of the first such reported tumor containing a leiomyosarcomatous component.     

Two rare cases of craniofacial chondrosarcoma

The craniofacial region is a rare site for chondrosarcomas.These tumors may have osseous or extraosseous origin.Extraosseous chondrosarcomas have the same histological features as osseous chondrosarcomas.Chondrosarcomas usually present in the fifth to seventh decades of life,although several cases with younger age at presentation have been reported.They usually present as a painless mass that gradually progresses to various complaints,such visual impairment,nasal obstruction,and dental abnormalities.In this article,we present two cases of chondrosarcoma occurring at rather unusual locations.It is important to keep this rare malignancy in the list of differential diagnoses for a mass in the head and neck region,as these tumors may not always show the features typical of this malignancy.     

Neutron boost irradiation of soft tissue and chondrosarcomas at the West German Tumour Centre in Essen

In this retrospective study, the results of postoperative neutron boost therapy for 40 soft tissue- and 18 chondrosarcomas are presented. In 65.5% and 34.5% of all cases, microscopic to macroscopic tumour residual was left postoperatively. Locally advanced and moderate to highly malignant tumours were found at surgery in 81% and 69%, respectively. Except for seven patients with a whole series of neutrons to 16 Gy, the other cases were treated with initially 40 to 50 Gy photons and a subsequent neutron boost of 4 to 6 Gy. The median follow-up was 29.3 and 36.2 months for the soft tissue- and chondrosarcomas. The actuarial three-year local control and overall survival rates were 69.3% and 91% for soft tissue- vs. 55.6% and 62.9% for chondrosarcomas. With regard to the extension of surgery (microscopic or macroscopic residual), local control rates of 80% and 60% for soft tissue- vs. 62.5% and 40% for chondrosarcomas were observed. The overall local failure rate was 32.8% with a median disease-free interval of ten respectively eleven months for soft tissue and chondrosarcomas. 17 out of 19 local recurrences were due to inadequate irradiation portals for the neutron boost or to insufficient total doses in the initially treated photon target volume.     

FDG-PET in differential diagnosis and grading of chondrosarcomas.

PURPOSE: The purpose of this work was to investigate the usefulness of PET with [18F]2-deoxy-2-fluoro-D-glucose (FDG) in the differential diagnosis and grading of chondrosarcomas. METHOD: Four enchondromas, one osteochondroma, and six chondrosarcomas were prospectively studied by FDG-PET. The significance of the standardized uptake value (SUV) was evaluated in comparison with clinical, radiological, and histopathological findings of the tumors. RESULTS: The SUV ranged from 0.7 to 1.3 (mean 0.96+/-0.22) in benign cartilage tumors and from 1.3 to 3.3 (2.23+/-0.80) in chondrosarcomas. This SUV difference between benign and malignant tumors was statistically significant (p = 0.011) in contrast to considerable overlap of clinical and radiological findings. Among chondrosarcomas, the SUV of one Grade I tumor was 1.3; those of four Grade II tumors were 1.3, 1.9, 2.5, and 3.1; and that of one Grade III tumor was 3.3. CONCLUSION: Our preliminary study showed that FDG-PET could be an objective and quantitative adjunct in the differential diagnosis and grading of chondrosarcomas.     

Intramedullary chondroid tumors of bone: correlation of abnormal peritumoral marrow and soft-tissue MRI signal with tumor type

 Objective. To determine whether enchondromas and chondrosarcomas can be differentiated on the basis of peritumoral MR signal abnormality. Design. STIR and T2-weighted MRI images were retrospectively assessed for the presence and extent of abnormal peritumoral marrow and soft-tissue signal. The cause of the peritumoral signal abnormality was determined by histologic correlation with resection specimens. The presence or absence of bone destruction was noted. Patients. Twenty-three patients were studied: ten with enchondromas (three men, seven women; ages 33–73 years) and 13 with chondrosarcomas (seven men, six women; ages 25–88 years). Results. Abnormal peritumoral marrow signal was present on STIR images around none of 10 enchondromas and all of 13 chondrosarcomas (P<0.0001). The marrow signal abnormality corresponded histologically to fine marrow fibrosis in all cases. Adjacent abnormal soft-tissue signal was present on STIR images around none of ten enchondromas and eight (62%) of 13 chondrosarcomas (P=0.0026). Abnormal soft-tissue signal was more common around high-grade than low-grade chondrosarcomas (100% vs 38%, P=0.028), and was more extensive (mean extent 28 mm vs 8 mm; P>0.04). In the subset of tumors without bone destruction, peritumoral marrow signal abnormality was present around none of ten enchondromas and all of five chondrosarcomas (P=0.0003); abnormal soft-tissue signal was present around none of ten enchondromas and two of five chondrosarcomas (P>0.05). Conclusion. Abnormal marrow or soft-tissue signal around a chondroid tumor is suggestive of chondrosarcoma, even in the absence of bone destruction. STIR images are necessary for adequate detection of peritumoral signal abnormalities.     

Evaluation of diffusion-weighted imaging for the differential diagnosis of poorly contrast-enhanced and T2-prolonged bone masses: Initial experience

PURPOSE: To determine whether quantitative diffusion-weighted imaging (DWI) is useful for characterizing poorly contrast-enhanced and T2-prolonged bone masses. MATERIALS AND METHODS: We studied 20 bone masses that showed high signal intensity on T2-weighted images and poor enhancement on contrast-enhanced T1-weighted images. These included eight solitary bone cysts, five fibrous dysplasias, and seven chondrosarcomas. To analyze diffusion changes we calculated the apparent diffusion coefficient (ADC) for each lesion. RESULTS: The ADC values of the two types of benign lesions and chondrosarcomas were not significantly different. However, the mean ADC value of solitary bone cysts (mean +/-SD, 2.57 +/- 0.13 x 10(-3) mm(2)/second) was significantly higher than that of fibrous dysplasias and chondrosarcomas (2.0 +/- 0.21 x 10(-3) mm(2)/second and 2.29 +/- 0.14 x 10(-3) mm(2)/second, respectively, P < 0.05). None of the lesions with ADC values lower than 2.0 x 10(-3) mm(2)/second were chondrosarcomas. CONCLUSION: Although there was some overlapping in the ADC values of chondrosarcomas, solitary bone cyst, and fibrous dysplasia, quantitative DWI may aid in the differential diagnosis of poorly contrast-enhanced and T2-prolonged bone masses.     

Magnetic resonance imaging of cartilaginous tumors: a retrospective study of 79 patients

Objective: Hyaline cartilaginous tumors are characterized by extremely high signal intensity on T2-weighted images. Recently, some distinctive MR features of cartilaginous bone tumors were reported in small series. Low signal intensity septa surrounding high signal intensity cartilage lobules were seen on T2-weighted images in low-grade chondrosarcomas. On spin-echo T1-weighted images after Gd contrast injection, marked ‘septal’ or ‘ring-and-arc’ enhancement was observed in low-grade chondrosarcomas and enchondromas. The purpose of this study was to determine sensitivity and specificity of these MR findings in diagnosis of cartilaginous tumors, and to assess the value of MR in diagnostic workup of these lesions. Materials and methods: Retrospective evaluation of MR findings in 79 cartilaginous tumors and in 79 non-cartilaginous tumors. All lesions were biopsy proven. Each MR examination was independently reviewed by two experienced radiologists without knowledge of clinical data, radiographic and/or CT findings, or histological diagnosis. All lesions were evaluated for morphology (lobular or non-lobular), presence of a high signal intensity mass on T2-weighted images, presence of low signal intensity septa separating high signal intensity lobules on T2-weighted images, and evidence of septal (‘ring-and-arc’) enhancement.Results: None of the reviewed parameters is useful in diagnosing osteochondromas. Since osteochondromas have a characteristic appearance on plain radiography, the value of MR imaging in the workup of these lesions remains limited. MR findings in enchondromas have a low specificity and a low sensitivity. Low-grade chondrosarcomas, often hard to diagnose on plain radiography and difficult to differentiate from enchondromas, are characterized by the MR tandem of ‘low signal intensity septa on T2-weighted images’ together with ‘septal or ring-and-arc enhancement’ (sensitivity 92.3%, specificity 76.5%). High-grade chondrosarcomas are easily recognized on plain radiography. Conclusions: In differentiating cartilaginous from non-cartilaginous tumors, MR features are highly specific but lack sensitivity. Grading potentials of MR parameters are promising due to the high accuracy in diagnosing low-grade chondrosarcomas.     

MRI differentiation of low-grade from high-grade appendicular chondrosarcoma

Objectives

To identify magnetic resonance imaging (MRI) features which differentiate low-grade chondral lesions (atypical cartilaginous tumours/grade 1 chondrosarcoma) from high-grade chondrosarcomas (grade 2, grade 3 and dedifferentiated chondrosarcoma) of the major long bones.

Methods

We identified all patients treated for central atypical cartilaginous tumours and central chondrosarcoma of major long bones (humerus, femur, tibia) over a 13-year period. The MRI studies were assessed for the following features: bone marrow oedema, soft tissue oedema, bone expansion, cortical thickening, cortical destruction, active periostitis, soft tissue mass and tumour length. The MRI-features were compared with the histopathological tumour grading using univariate, multivariate logistic regression and receiver operating characteristic curve (ROC) analyses.

Results

One hundred and seventy-nine tumours were included in this retrospective study. There were 28 atypical cartilaginous tumours, 79 grade 1 chondrosarcomas, 36 grade 2 chondrosarcomas, 13 grade 3 chondrosarcomas and 23 dedifferentiated chondrosarcomas. Multivariate analysis demonstrated that bone expansion (P?=?0.001), active periostitis (P?=?0.001), soft tissue mass (P?<?0.001) and tumour length (P?<?0.001) were statistically significant differentiating factors between low-grade and high-grade chondral lesions with an area under the ROC curve of 0.956.

Conclusions

On MRI, bone expansion, active periostitis, soft tissue mass and tumour length can reliably differentiate high-grade chondrosarcomas from low-grade chondral lesions of the major long bones.

Key Points

? Accurate differentiation of low-grade from high-grade chondrosarcomas is essential before surgery ? MRI can reliably differentiate high-grade from low-grade chondrosarcomas of long bone ? Differentiating features are bone expansion, periostitis, soft tissue mass and tumour length ? Presence of these four MRI features demonstrated a diagnostic accuracy (AUC) of 95.6?% ? The findings may result in more accurate diagnosis before definitive surgery     

Craniofacial chondrosarcomas: imaging findings in 15 untreated cases

Radiographic findings of 15 untreated chondrosarcomas of the cranial and facial bones were reviewed. These tumors have a propensity to occur in the wall of a maxillary sinus, at the junction of sphenoid and ethmoid sinuses and vomer, and at the undersurface of the sphenoid bone. Because of its slow-growing nature, chondrosarcomas tend to be large, multilobulated, and sharply demarcated when detected. Frequent bone changes are a combination of erosion and destruction, with sharp transitional zones and absent periosteal reaction. Tumor matrix calcifications, not necessarily chondroid, are almost always present. Both CT and MR may be necessary for thorough evaluation of tumor extent.     

Chondrosarcomas of the skull base: MR imaging features.

The magnetic resonance (MR) images from 17 patients with chondrosarcomas of the skull base were retrospectively reviewed to characterize the size, location, signal intensity, and extension of these tumors. Eleven patients with chondrosarcomas received intravenously administered gadopentetate dimeglumine. In 16 patients, computed tomographic (CT) scans were obtained to evaluate intratumorous mineralization and bone erosion. On short repetition time (TR)/echo time (TE) MR images, chondrosarcomas generally had low to intermediate signal intensity; on long TR/TE MR images, they generally had very high signal intensity. Signal heterogeneity on long TR/TE MR images was seen in 10 of 17 tumors (59%) and was caused by matrix mineralization, fibrocartilaginous elements, or both. Matrix mineralization was demonstrated with CT in seven of the 16 chondrosarcomas. Chondrosarcomas showed marked enhancement after administration of gadopentetate dimeglumine in either a heterogeneous (n = 8) or homogeneous (n = 3) pattern. The information about the size and extent of these neoplasms was important in the choice of surgical approaches for gross total resection of tumor.     

Extraskeletal mesenchymal chondrosarcoma of the rectus sheath

 Mesenchymal chondrosarcomas (MSCs) are a rare form of chondrosarcoma which usually arise in bone. Extraskeletal chondro-sarcomas constitute a minority (14–25%) of MSCs. We describe the imaging features of an extraskeletal mesenchymal chondrosarcoma that arose from the rectus abdominus muscle.     

颅内软骨肉瘤的CT及MRI表现

目的　探讨颅内软骨肉瘤的CT、MRI特征。方法　对经手术病理证实的 3例颅内软骨肉瘤的临床资料进行了回顾性分析。结果　 3例CT平扫显示不规则分叶状肿块 ,瘤周水肿不明显 ,2例伴有瘤内钙化及局部颅骨侵蚀。MRI显示肿瘤在T1WI为低、等信号 ,T2 WI为高信号 ;MRI增强时T2 WI中央低信号部分不强化 ,周边强化明显。结论　颅内软骨肉瘤好发于颅底软骨联合处 ,常伴瘤内钙化和局部颅骨侵蚀 ,但须依靠病理及免疫组化染色确诊与鉴别诊断     

Characterization of cartilaginous tumors with201Tl scintigraphy

Histological diagnosis and grading of cartilaginous tumors are closely correlated with patient prognosis; consequently, they are essential elements. We attempted to clarify the characteristics of 201Tl uptake in various histological types of cartilaginous tumors and to assess its clinical value. METHODS: Twenty-two cases with histologically proven cartilaginous tumors (3 enchondromas, 15 conventional chondrosarcomas (grade I = 9, II = 5, III = 1), 3 mesenchymal chondrosarcomas, and 1 de-differentiated chondrosarcoma) were examined retrospectively. Planar 201Tl images were recorded 15 min following intravenous injection of 201Tl (111 MBq). 201Tl uptake in the tumor was evaluated visually employing a five-grade scoring system: 0 = no appreciable uptake, 1 = faint uptake above the background level, 2 = moderate uptake, 3 = intense uptake but lower than heart uptake and 4 = uptake higher than heart uptake. RESULTS: 201Tl uptake scores were 0 in 3 of 3 enchondromas, 9 of 9 grade I, and 4 of 5 grade II conventional chondrosarcomas. 201Tl uptake scores were 1 among 1 of 5 grades II and a grade III conventional chondrosarcoma. Mesenchymal chondrosarcoma and de-differentiated chondrosarcoma displayed 201Tl uptake scores of 2 or 3. CONCLUSIONS: Absence of elevated 201Tl uptake in cartilaginous tumors was indicative of enchondroma or low-grade conventional chondrosarcoma. However, in instances in which 201Tl uptake is obvious, high-grade chondrosarcoma or variant types should be considered.     

Unusual metastatic chondrosarcoma detected with bone scintigraphy

A case is presented of a scapular chondrosarcoma which showed intense uptake of Tc99m MDP in the primary tumour and in pleural and osseous metastases. Additionally, there was intense visualization of the malignant pleural effusion. The bone scintigraphy correctly diagnosed the extent of the involvement. This finding is unusual because chondrosarcomas in such a disseminated state are relatively rare.