Parosteal osteosarcoma dedifferentiating into telangiectatic osteosarcoma: importance of lytic changes and fluid cavities at imaging

Purpose  This study was performed to assess the imaging findings in cases of parosteal osteosarcoma dedifferentiated into telangiectatic osteosarcoma. Parosteal osteosarcoma is a low-grade well-differentiated malignant tumor. Dedifferentiation into a more aggressive lesion is frequent and usually visible on imaging as a central lytic area in a sclerotic mass. Only one case of differentiation into a telangiectatic osteosarcoma has been reported. As it has practical consequences, with a need for aggressive chemotherapy, we looked for this rather typical imaging pattern. Materials and methods  Review of 199 cases of surface osteosarcomas (including 86 parosteal, of which 23 were dedifferentiated) revealed lesions suggesting a possible telangiectatic osteosarcoma on imaging examinations in five cases (cavities with fluid). Histology confirmed three cases (the two other only had hematoma inside a dedifferentiated tumor). There were three males, aged 24, 28, and 32. They had radiographs and CT, and two an MR examination. Results  Lesions involved the distal femur, proximal tibia, and proximal humerus. The parosteal osteosarcoma was a sclerotic, regular mass, attached to the cortex. A purely lytic mass, partially composed of fluid cavities was easily detected on CT and MR. It involved the medullary cavity twice, and remained outside the bone once. Histology confirmed the two components in each case. Two patients died of pulmonary metastases and one is alive. Conclusion  Knowledge of this highly suggestive pattern should help guide the initial biopsy to diagnose the two components of the tumor, and guide aggressive treatment.     

毛细血管扩张型骨肉瘤影像与病理对照分析

目的　探讨毛细血管扩张型骨肉瘤影像与病理表现特征。方法　分析 12例毛细血管扩张型骨肉瘤临床、影像及病理资料。结果　临床特征为肿瘤好发于长骨干骺端 ,下肢多见 ,少有血清碱性磷酸酶增高。X线、CT特征为溶骨性与膨胀性骨破坏 ;肿瘤破坏区和软组织肿块内多发小囊变和液 -液平面 ;皮质变薄 ,多发筛孔样破坏 ;骨膜反应及放射状骨针较常见。病理组织学特征为典型骨肉瘤肿瘤细胞和多发血窦及“彩带样”结构。结论　毛细血管扩张型骨肉瘤具有一定的临床、影像及组织学特征     

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.     

Telangiectatic osteosarcoma of the skull base.

We present a patient with a distinctive lesion of the skull base, which, at imaging, showed expansile destruction of the sphenoid bone, hemorrhage with fluid-fluid levels, and contrast enhancement of a solid portion. These features ordinarily suggest either giant cell tumor or aneurysmal bone cyst; however, pathologic examination confirmed instead a rare variant of osteosarcoma of the telangiectatic type. Although imaging findings can indicate any of these possibilities, biopsy is an essential step in arriving at the diagnosis.     

Magnetic resonance for the study of osteosarcoma

The authors report their experience with MR imaging in the study of osteosarcoma. Two main elements were evaluated: signal characteristics and loco-regional staging. Seventy-one patients were studied: 65 of them had central long-bone osteosarcoma, and 6 had telangiectatic long-bone osteosarcoma. T1- and T2-weighted spin-echo sequences were employed and all cases were scanned on 3 planes (sagittal, coronal, and axial). In 28 patients MR imaging was performed both before and after preoperative chemotherapy. The obtained data were compared to surgical and pathological findings. With the exception of the typical signal patterns of quite-osteoblastic osteosarcoma (which presents with low signal on both T1- and T2-weighted sequences), no particular signal features were observed which could help distinguish the different types of osteosarcoma. MR imaging is the method of choice in loco-regional staging for, in our series, it allowed a rational and adequate surgical planning. For this purpose, at least a longitudinal T1- and an axial T2-weighted images are required.     

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.     

Telangiectatic osteosarcoma: radiologic-pathologic comparison

PURPOSE: To describe the imaging characteristics of a large series of telangiectatic osteosarcomas with pathologic findings for comparison. MATERIALS AND METHODS: The authors retrospectively reviewed 40 pathologically confirmed telangiectatic osteosarcomas. Patient demographics and images from radiography (n = 36), bone scintigraphy (n = 17), angiography (n = 4), computed tomography (CT) (n = 25), and magnetic resonance (MR) imaging (n = 27) were evaluated by three authors in consensus for lesion location, size, and intrinsic characteristics. There were 27 men (68%) and 13 women (32%) in the study, with an age range of 4-83 years (mean age, 24 years). RESULTS: Lesions frequently affected the femur, tibia, and humerus. Radiographs showed geographic bone lysis, a wide zone of transition, and matrix mineralization. CT demonstrated low attenuation, MR demonstrated high signal intensity on T2-weighted images, and both demonstrated hemorrhage, which simulated the appearance of aneurysmal bone cyst. Viable sarcomatous tissue surrounding hemorrhagic and/or necrotic regions was best seen at contrast material-enhanced CT and MR imaging, with thick peripheral, septal, and nodular enhancement in all cases. Subtle matrix mineralization in this viable tissue was best seen at CT. An associated soft-tissue mass was also seen in 19 of 25 cases (76%) at CT and in 24 of 27 cases (89%) at MR imaging. CONCLUSION: CT and MR imaging findings of telangiectatic osteosarcoma often include thick nodular tissue (and matrix mineralization at CT) in a largely hemorrhagic and/or necrotic osseous lesion with an associated soft-tissue mass, which allows distinction from aneurysmal bone cyst.     

Low-grade parosteal osteosaracoma of the ulna with dedifferentiation into high-grade osteosarcoma

 This report describes a low-grade parosteal osteosarcoma of the ulna with dedifferentiation into high-grade osteosarcoma. The clinical, pathological, and imaging findings are described and discussed.     

血管扩张型骨肉瘤的临床、影像和病理分析

目的 进一步分析血管扩张型骨肉瘤的临床、影像及病理学的诊断特征和诊断方法,以加深对其认识和提高诊断水平.方法 回顾性分析10例经病理证实的血管扩张型骨肉瘤资料并结合文献深入分析其临床、影像和病理学表现.10例均行X线平片和MR检查,其中2例同时行CT检查.结果 10例血管扩张型骨肉瘤发生于股骨下端5例、股骨颈1例、肱骨上端和胫骨上段各2例.10例X线平片和2例CT 图像上肿瘤表现为溶骨性骨质破坏,伴骨体轻度膨胀4例,骨破坏区大部分边缘不清且无硬化边,均有Codman三角和大小不一的软组织肿块,肿块内无明显肿瘤性成骨;10例MRI上肿瘤大部或完全由多发囊腔构成,均出现骨膜反应,其中7例囊腔内见少量散在的较小液-液平面.10例病理学上肿瘤均有类似动脉瘤样骨囊肿的血腔结构,但囊壁或囊间隔中见恶性肿瘤细胞以及数量不一的肿瘤性成骨组织,其中6例仅见少量灶状的肿瘤性骨样组织.结论 影像学上类似动脉瘤样骨囊肿的表现和一般恶性骨肿瘤生长方式的特点,以及病理上的血腔结构和囊壁或囊间隔内的恶性肿瘤细胞是血管扩张型骨肉瘤的影像和病理学诊断特征.密切结合临床、影像和病理分析有助于临床及时正确诊断.     

血管扩张型骨肉瘤的临床、影像和病理分析

目的 进一步分析血管扩张型骨肉瘤的临床、影像及病理学的诊断特征和诊断方法,以加深对其认识和提高诊断水平.方法 回顾性分析10例经病理证实的血管扩张型骨肉瘤资料并结合文献深入分析其临床、影像和病理学表现.10例均行X线平片和MR检查,其中2例同时行CT检查.结果 10例血管扩张型骨肉瘤发生于股骨下端5例、股骨颈1例、肱骨上端和胫骨上段各2例.10例X线平片和2例CT 图像上肿瘤表现为溶骨性骨质破坏,伴骨体轻度膨胀4例,骨破坏区大部分边缘不清且无硬化边,均有Codman三角和大小不一的软组织肿块,肿块内无明显肿瘤性成骨;10例MRI上肿瘤大部或完全由多发囊腔构成,均出现骨膜反应,其中7例囊腔内见少量散在的较小液-液平面.10例病理学上肿瘤均有类似动脉瘤样骨囊肿的血腔结构,但囊壁或囊间隔中见恶性肿瘤细胞以及数量不一的肿瘤性成骨组织,其中6例仅见少量灶状的肿瘤性骨样组织.结论 影像学上类似动脉瘤样骨囊肿的表现和一般恶性骨肿瘤生长方式的特点,以及病理上的血腔结构和囊壁或囊间隔内的恶性肿瘤细胞是血管扩张型骨肉瘤的影像和病理学诊断特征.密切结合临床、影像和病理分析有助于临床及时正确诊断.     

Fluid-fluid levels in giant cell tumors of bone: report of two cases

Fluid-fluid levels have been described in association with aneurysmal bone cysts, telangiectatic osteosarcoma, and a chondroblastoma. We report two cases of giant cell tumors of bone with fluid-fluid levels identified by computed tomography and, in one case, by magnetic resonance imaging. This finding has not previously been associated with giant cell tumors. The radiographic features of the fluid-fluid levels cannot be distinguished from those reported in other osseous neoplasms.     

血管扩张型骨肉瘤的临床、影像和病理分析

目的 进一步分析血管扩张型骨肉瘤的临床、影像及病理学的诊断特征和诊断方法,以加深对其认识和提高诊断水平.方法 回顾性分析10例经病理证实的血管扩张型骨肉瘤资料并结合文献深入分析其临床、影像和病理学表现.10例均行X线平片和MR检查,其中2例同时行CT检查.结果 10例血管扩张型骨肉瘤发生于股骨下端5例、股骨颈1例、肱骨上端和胫骨上段各2例.10例X线平片和2例CT 图像上肿瘤表现为溶骨性骨质破坏,伴骨体轻度膨胀4例,骨破坏区大部分边缘不清且无硬化边,均有Codman三角和大小不一的软组织肿块,肿块内无明显肿瘤性成骨;10例MRI上肿瘤大部或完全由多发囊腔构成,均出现骨膜反应,其中7例囊腔内见少量散在的较小液-液平面.10例病理学上肿瘤均有类似动脉瘤样骨囊肿的血腔结构,但囊壁或囊间隔中见恶性肿瘤细胞以及数量不一的肿瘤性成骨组织,其中6例仅见少量灶状的肿瘤性骨样组织.结论 影像学上类似动脉瘤样骨囊肿的表现和一般恶性骨肿瘤生长方式的特点,以及病理上的血腔结构和囊壁或囊间隔内的恶性肿瘤细胞是血管扩张型骨肉瘤的影像和病理学诊断特征.密切结合临床、影像和病理分析有助于临床及时正确诊断.     

Extraskeletal osteosarcoma of the jaw

Extraskeletal osteosarcoma is a rare malignancy, which accounts for 1–2% of all soft tissue sarcomas; occurrence of these tumors in the head and neck is extremely rare. We present a case of extraskeletal osteosarcoma arising in the subcutaneous tissue of the jaw angle, the central portion of which displayed calcification. Imaging findings were interesting in terms of a high grade malignancy in that the tumor was small and well circumscribed; its appearance mimicked a calcified lymph node. The differential diagnosis of this lesion as well as the imaging and pathological features of extraskeletal osteosarcoma are discussed.     

Telangiectatic dedifferentiation of a parosteal osteosarcoma

A unique case of parosteal osteosarcoma (POS) of the proximal femur, with areas of telangiectatic dedifferentiation, in a 28-year-old woman is reported. The patient had a 7-week history of pain and swelling in her right thigh. A biopsy diagnosis of POS was established. The patient was treated with two cycles of intra-arterial chemotherapy, followed by limb salvage surgery. Histological examination of the resected specimen showed POS with areas of dedifferentiation composed of high-grade telangiectatic osteosarcoma with associated secondary aneurysmal bone cyst change. Received: 17 April 1998 Revision requested: 11 May 1998 Revision received: 9 May 2000 Accepted: 11 May 2000     

CT and MRI features of recurrent tumors and second primary neoplasms in pediatric patients with retinoblastoma

OBJECTIVE: The aim of our study was to describe the CT and MRI findings of recurrent tumors and second primary (malignant and benign) neoplasms in patients with retinoblastoma and to evaluate imaging features to assist in distinguishing them. MATERIALS AND METHODS: Records of 445 pathologically confirmed retinoblastomas were retrospectively reviewed. Thirty-four patients with recurrent retinoblastomas and 15 patients with second primary neoplasms who underwent CT and MRI were evaluated by two radiologists with agreement by consensus. RESULTS: Invasive patterns of recurrent tumors included type A, intraocular tumor (n = 13); type B, intraorbital tumor with spread into the optic nerve shown as enlargement and marked enhancement of the optic nerve on contrast-enhanced CT or MRI (n = 6); and type C, tumor extending to the lateral aspect of the orbit and invading the brain via the sphenoidal bone (n = 2). Thirty-eight percent of patients with recurrent tumors had distant metastases (n = 7) or leptomeningeal metastases (n = 6). Leptomeningeal metastases were found only in recurrent tumors. Second primary neoplasms included osteosarcoma (n = 5), rhabdomyosarcoma (n = 5), meningioma (n = 4), and other tumors (n = 3). A significant difference was seen between the patients' ages at the time of diagnosis of recurrent tumors and second primary neoplasms (p < 0.0001). Extraorbital tumors were found more frequently among second primary neoplasms than among recurrent tumors (p < 0.001). CONCLUSION: Both recurrent tumors and second primary neoplasms in patients with retinoblastoma often show characteristic imaging features. The tumor distribution on CT and MRI may help in differentiating recurrent tumors and second primary neoplasms.     

Post-Paget telangiectatic osteosarcoma of the skull

Sarcomatous transformation is the most dreaded complication of Paget’s disease. We report on a case of post-Paget telangiectatic osteosarcoma of the skull, a variant of osteogenic osteosarcoma, in a 79-year-old woman. We discuss the radiological pattern in relationship to the differential diagnosis. Received: 27 July 1998 Revision requested: 24 August 1998, 23 December 1998 Revision received: 25 November 1998, 12 April 1999 Accepted: 13 April 1999     

Large telangiectatic focal nodular hyperplasia presenting with normal radionuclide studies: case report

A 9 cm-lesion of telangiectatic focal nodular hyperplasia was incidentally identified in a 31-yr-old female. Despite a typical appearance by X-ray computed tomography and ultrasonography, scintigraphy with technetium-99m-(99mTc) colloid, 99mTc-diethyliminodiacetic acid, and 99mTc-labeled red cells failed to demonstrate any abnormalities. These findings are felt to reflect the relative lack of architectural disruption that histologically characterizes this particular lesion. The present report described the imaging characteristics of the telangiectatic form of focal nodular hyperplasia.     

Fluid-fluid levels in bone and soft tissue tumors demonstrated by MR imaging]

Fluid-fluid levels in bone tumors have been described in aneurysmal bone cysts and other cystic tumors of bones and soft tissue tumors. We experienced three bone tumors (simple bone cyst, bone metastasis, and osteosarcoma) and three soft tissue tumors (fibrosarcoma, two cases of cavernous hemangioma) that showed fluid-fluid levels on MR, and investigated their cause. Causes included blood in the cystic spaces, hemorrhage in the tumor, the telangiectatic component of the osteosarcoma, and the cavernous component of the hemangioma. No specific diagnosis could be made based on the finding of fluid-fluid levels. We conclude that fluid-fluid levels on MR are rather nonspecific findings in bone and soft tissue tumors and that the diagnosis should be made on the basis of other radiological and clinical findings.     

Isolated lower extremity metastases, 9 years after initial diagnosis of retinoblastoma

We report the development of isolated lower extremity metastases, in a child, 9 years after her initial diagnosis and treatment of bilateral infantile retinoblastomas. The radiographic, scintigraphic, computed tomographic, and magnetic resonance imaging findings are discussed. The dominant metastatic focus was blastic, involving the medial cuneiform. Additional occult lesions were found in the base of the second metatarsal, middle cuneiform, navicular and tibial diaphysis. An open biopsy confirmed the diagnosis. The occurrence of late distant metastases is rare in the USA.This case was presented at the 31st Annual Closed Members Meeting of the International Skeletal Society held in Malta, October 2004.     

Soft tissue osteosarcoma with telangiectatic features: MR imaging findings in two cases

Extraskeletal osteosarcomas are rare tumors, and the telangiectatic variety is the least common histological variety in this group. This report describes the clinical and MR imaging findings in two cases arising in the pretibial soft tissues. Both tumors demonstrated marked inhomogeneity with T2-weighted spin echo and STIR sequences. One of the tumors revealed numerous fluid levels within the lesion. A review of the MRI features of these tumors is provided. Osteosarcoma with telangiectatic features should be considered in the differential diagnosis of a soft tissue mass with fluid-fluid levels in patients 40 years of age or older.