Chondromyxoid fibroma of the acromium with soft tissue extension

Chondromyxoid fibroma is an unusual, benign tumor of cartilaginous origin and represents less than 1% of all primary bone tumors. It usually involves the long bones around the knee joint or the flat bones of the pelvis or ribs. Soft tissue extension is also thought to be rare in these lesions. They are usually eccentrically located in the metaphyses of the long bones and centrally in the flat bones. The radiographic appearances are characteristically those of a single, lytic lesion with lobulated margins, septations, cortical expansion and a sclerotic rim. Histologically, they display a lobulated pattern with spindle-shaped cells lying within a myxoid matrix with areas of hyaline cartilage. The differential diagnosis includes giant cell tumor, chondroblastoma or enchondroma as well as chondrosarcoma. The rarity of these lesions may render the diagnosis difficult to make, especially when the lesion involves an unusual site such as the acromium. Received: 21 June 1999 Revision requested: 27 September 1999 Revision received: 27 October 1999 Accepted: 28 October 1999     

髂骨原发囊样骨肿瘤及瘤样病变的影像学诊断

目的:分析髂骨囊样骨肿瘤及肿瘤样病变的影像学表现。方法:回顾性分析经穿刺或/和手术病理证实的46例髂骨囊样骨肿瘤及肿瘤样病变影像学表现。46例全部行X线片检查,38例行CT检查,20例行MRI检查(增强16例)。结果:46例中骨肿瘤29例,其中良性肿瘤12例(骨巨细胞瘤4例,内生软骨瘤、软骨母细胞瘤各2例,血管瘤、骨母细胞瘤、骨样骨瘤、软骨黏液样纤维瘤各1例),恶性肿瘤17例(恶性巨细胞瘤、骨恶性纤维组织细胞瘤各1例,软骨肉瘤、淋巴瘤各4例,骨肉瘤7例),肿瘤样病变17例(单纯性骨囊肿1例,邻关节骨囊肿、动脉瘤样骨囊肿、嗜酸性肉芽肿各2例,骨纤维异常增生症10例)。发病部位为髂翼39例,髋臼7例。病变主要表现为髂骨囊样骨质破坏,呈膨胀性改变30例,硬化环形成24例,病变内见钙化灶14例,软组织肿块20例。结论:常见的髂骨囊样骨肿瘤和肿瘤样病变一般都具有某些特征性的CT表现,必要时综合X线、MRI表现,可提高定性诊断符合率。     

Osteosarcoma in a patient with McCune-Albright syndrome and Mazabraud’s syndrome

Sarcomas infrequently develop in osseous sites of fibrous dysplasia. We report a patient with Mazabraud’s syndrome (polyostotic fibrous dysplasia and soft tissue myxomas) complicated by the development of osteogenic sarcoma in a bone affected by fibrous dysplasia. This is the third case of osteosarcoma within the small population of reported patients with Mazabraud’s syndrome. There may be an increased incidence of malignant transformation in these individuals’ dysplastic bones above that associated with patients suffering from fibrous dysplasia alone. Received: 31 December 1998 Revision requested: 31 January 1999 Revision received: 13 May 1999 Accepted: 14 May 1999     

De novo malignant transformation of giant cell tumor of bone

Two cases of malignant lesions are reported, both of which arose secondary to an originally benign giant cell tumor (GCT) of bone. The first case was a typical benign GCT, which occurred in the left proximal tibia of a 31-year-old woman. The tumor was treated by curettage and bone grafting. However, it recurred twice during 15 years of follow-up. The second recurrence showed that the lesion histologically had turned into malignant fibrous histiocytoma. The second case also started as an initially benign GCT that arose in the left distal femur of a 41-year-old man. The patient underwent curettage and bone grafting. The lesion recurred 13 years postoperatively. The histological appearance of the recurrent tumor showed it to be an osteogenic sarcoma. In both patients, radiation had never been given. Malignant transformation has rarely been reported in patients with GCT of bone who have not received radiation treatment. Received: 12 June 2000 Revision requested: 17 August 2000 Revision received: 13 October 2000 Accepted: 18 October 2000     

Myxoma of the humerus: an exceptional site of origin

Myxoma of bone, outside of the jaws, is exceptional. We present such a tumor in the humerus and discuss the criteria for the diagnosis of myxoma. Received: 10 August 1999 Revision requested: 10 September 1999 Revision received: 14 September 1999 Accepted: 14 September 1999     

Case report 695

A case of a cementifying fibroma in the proximal end of the tibia in a 31-year-old woman is presented. The lesion was expanding but well-defined with amorphous central calcification. The unique, calcified, cementum-like particles in a fibrous stroma have been reported in only four previous cases in long bones. The histological and radiographic differences between a cementifying fibroma, bone cysts with cementum, and a true cementoma are discussed. The radiological differential diagnosis of fibrous dysplasia, giant cell tumor, and low grade central osteosarcoma was considered.     

骨纤维异常增殖症自发恶变15例分析

报告１５例骨纤维异常增殖症（简称骨纤）自发恶变，其中恶变为骨肉瘤８例，纤维肉瘤５例和软骨肉瘤２例。１５例均长期患有骨纤，均未接受过放射治疗。本组恶变者中，多骨型骨纤１１例，单骨型４例。恶变患者的主要临床症状是病变区疼痛和肿胀，而后则出现硬性肿块。恶变的Ｘ线表现为：在原骨纤病变区域内，出现虫噬样或囊状溶骨性破坏，骨纤病变边缘变模糊或消失，皮质破坏并逐渐出现软组织肿块。恶变为骨肉瘤者，肿瘤区内常有不同形态的痛骨；软骨肉瘤则可见环状或斑片状钙化；纤维肉瘤则表现为单纯溶骨性破坏，软组织肿块呈中等密度。     

跟骨和距骨良性囊样病变影像学表现

目的 评价跟骨或距骨良性囊样病变的影像学表现. 资料与方法 回顾性分析27例经穿刺活检或手术病理证实的跟骨或距骨良性囊样病变的影像学表现.全部病例均行X线平片检查,20例行CT检查,10例行MRI检查. 结果 骨囊肿8例,软骨母细胞瘤8例(合并动脉瘤样骨囊肿4例),邻关节囊肿5例,脂肪瘤3例,骨巨细胞瘤2例,骨纤维结构不良1例.平片及CT显示大小不等囊性低密度病灶;MRI信号表现各具特征. 结论 跟骨或距骨良性囊样病变的影像学表现具有一定特征.     

Hyperfunctioning parathyroid carcinoma

Primary hyperparathyroidism is rarely caused by carcinoma. We report a patient who manifested many of the clinical and radiographic features of the disease. When encountering symptomatic hypercalcemia with or without a palpable neck mass, carcinoma should be considered in the differential diagnosis. Patient survival depends on an aggressive surgical approach to the primary lesion and recurrent disease. Received: 27 May 1999 Revision requested: 28 June 1999 Revision received: 22 September 1999 Accepted: 27 September 1999     

Papillary cystic neoplasm of the pancreas in a teenage boy

We present a case of a 13-year-old boy with a left-sided abdominal mass which proved to be a papillary cystic neoplasm of the pancreas. This low-grade malignant lesion of young patients is very rare, and exceedingly rare in males. The prognosis following resection of this tumor is good. We present the ultrasound and computed tomographic picture of this lesion, as well as the gross and microscopic pathology. Received: 10 July 1998; Revision received: 13 November 1998; Accepted: 11 January 1999     

Extraosseous calcified plasmacytoma causing thoracic myelopathy

We report on a rare, calcified, plasma cell tumor of the spine causing progressive myelopathy. Other unusual features were the lack of an osseous lesion at the site of the mass, considerable calcified amyloid within the mass but no identifiable amyloid elsewhere, and normal serum immunoelectrophoresis. Received: 12 February 1999 Revision requested: 18 March 1999 Revision received: 19 April 1999 Accepted: 20 April 1999     

肋骨肿瘤和肿瘤样病变的X线和CT表现

目的：讨论肋骨肿瘤和肿瘤样病变的X线和CT表现,总结各自的特点。方法：取经手术和病理证实,或临床动态观察证实的肋骨病变病例100例;所有病例均行X、CT检查。结果：64例为良性病变,其中24例为骨岛,18例为骨纤维异常增殖症,14例为软骨类良性肿瘤,8例为骨感染性病变,（4例为结核,4例为非特异性感染）。恶性病变36例,其中转移瘤20例,骨髓瘤8例,骨巨细胞瘤8例。结论：肋骨肿瘤和肋骨肿瘤样病变一般具有某些有助于诊断的X线和CT表现,据此可以较好的区分常见肋骨肿瘤和肋骨肿瘤样病变。     

Monostotic fibrous dysplasia of the calcaneus: two case reports

The authors report two unusual cases of aggressive monostotic fibrous dysplasia involving the calcaneus. This lesion occurs on the 2th decade of life. The main symptom is talalgia. On plain films, differential diagnosis includes aggressive cystic or pseudocystic lesions of the calcaneus. On MR imaging, the tumor matrix is more suggestive of the diagnosis. Treatment of this aggressive form of fibrous dysplasia consists of a surgical curettage completed by cryotherapy and graft because of a high level of recurrence.     

Progressive destructive vertebral sarcoid leading to surgical fusion

Skeletal sarcoidosis is rare. This report describes a 31-year-old African American man who presented with a destructive osteolytic lesion of C2 and another lesion in a rib. The lesion at C2 was treated with corpectomy and bone graft. Four months later the lesion recurred and a new lesion was discovered in the cervical vertebral column. The patient declined surgery for instability for another 3 months, choosing to remain in a halo. Seven months following the initial operation, a technetium bone scan showed spread of the disease to the calvarium and thoracic and lumbar vertebrae. The patient had no symptoms referable to these sites. The patient agreed to have his neck fused at this point. For the next 10 months, the patient was on steroids and a further new lesion appeared at L5 without localizing signs or symptoms. The patient declined further evaluation over the next 12 months and is now considered lost to follow-up. Received: 29 July 1999 Revision requested: 26 August 1999 Revision received: 1 September 1999 Accepted: 2 September 1999     

Hemangioma of bone arising in the ulna: imaging findings with emphasis on MR

An 18-year-old woman presented with left elbow joint pain. Radiographs and computed tomographic scan showed a well-defined osteolytic lesion of the left ulna associated with a honeycomb appearance on the radiographs. Magnetic resonance images showed intermediate signal intensity on T1-weighted images and mixed intermediate and high signal intensities on T2-weighted images. Only the periphery of the lesion enhanced with intravenously injected gadolinium-diethylenetriamine pentaacetic acid. The lesion was curetted to avoid pathologic fracture, and a histologic diagnosis of cavernous hemangioma of bone was made. Hemangioma involving the ulna is rare, but should be included in the differential diagnosis of a radiographic osteolytic lesion with a honeycomb appearance. Received: 17 September 1999 Revision requested: 31 October 1999 Revision received: 10 December 1999 Accepted: 13 December 1999     

Vertebral intra-osseous chordoma or giant notochordal rest?

Chordomas of the lumbar vertebral bodies are rare. We report an unusual case of an entirely intra-osseous chordoma of the fifth lumbar vertebra treated by vertebrectomy. Conventional radiographs and scintigraphy were normal. The lesion was well visualised by MR imaging, but showed only slight sclerosis on CT. We give our reasons for making a diagnosis of chordoma rather than giant notochordal rest and discuss the problems of management resulting from this diagnostic dilemma. Received: 25 February 1999 Revision requested: 2 March 1999 Revision received: 15 March 1999 Accepted: 18 March 1999     

眶骨良性肿瘤与瘤样病变的CT诊断

目的分析眶骨良性肿瘤与瘤样病变的CT表现,提高对此类病变的认识。方法回顾32例经手术、病理和临床证实的眶骨良性肿瘤与瘤样病变,包括良性肿瘤17例、瘤样病变15例。结果骨瘤9例,呈均一致密影、海绵骨样密度或磨砂玻璃样改变;骨化性纤维瘤5例,表现为边界清晰的膨胀性骨性肿块,内有骨化、囊变等;2例表皮样囊肿呈囊性密度;1例软骨瘤以钙化、骨化为主;骨纤维异常增殖症10例,受累骨不规则膨大,磨砂玻璃样密度伴囊变,边界不清;郎格罕氏细胞组织细胞增生症3例,不规则骨质破坏,边缘清晰;1例动脉瘤样骨囊肿表现为膨胀性肿块,有薄骨壳;1例石骨症眶骨增厚,呈均一致密影。结论眶骨良性肿瘤与瘤样病变的CT表现各具特征性,CT扫描能清晰显示病变范围、密度、边界以及与邻近结构的关系。     

Primary hemangiopericytoma of the tibia: MR and angiographic correlation

The Magnetic Resonance Imaging (MRI) appearances of primary osseous hemangiopericytoma (HPC) have been rarely described. We report on a 46-year-old Chinese man with primary osseous HPC of the right tibia. The characteristic vascular distribution of this tumor, presenting with a ”spoke-wheel” appearance on MR images and with angiographic correlation, is described. Although not pathognomonic, this MR appearance may be an important finding in suggesting the diagnosis of osseous HPC. Received: 3 March 1999 Revision requested: 26 April 1999 Revision received: 13 September 1999 Accepted: 15 September 1999     

Atypical adult non-calcified pilomatricoma

We present a rare case of a non-calcified pilomatricoma in a 67-year-old man. This tumor was extremely large in size, and its location, in the lower extremity, was very unusual. The clinical, radiographic, and histopathological features are described in detail. The role of magnetic resonance imaging (MRI) in the diagnosis of this entity is discussed. Definite internal reticulations and septations were observed. A possible explanation for this observation is that the high signal intensity reticulations may represent edematous stroma surrounding basaloid cells. Received: 8 July 1999 Revision requested: 28 July 1999 Revision received: 10 September 1999 Accepted: 13 September 1999     

Malignant chondroblastoma presenting as a recurrent pelvic tumor with DNA aneuploidy and p53 mutation as supportive evidence of malignancy

We report a rare case of malignant chondroblastoma, which presented in a 47-year-old man as a recurrent tumor, 18 years following wide excision of a typical pelvic chondroblastoma. Radiologic studies of the recurrent tumor showed a large, lytic, destructive lesion of the right pelvic bones and femur, with a pathologic fracture of the latter, a large pelvic soft tissue mass, and multiple pulmonary metastases. Biopsy tissue showed typical features of chondroblastoma, but also increased nuclear atypia, hyperchromasia, and pleomorphism, compared to the original tumor, and, most significantly, abnormal mitotic figures. Immunohistochemical studies of the recurrent tumor revealed p53 mutation and extensive proliferative activity, and flow cytometric studies showed DNA aneuploidy, none of which was present in the original tumor. The patient received chemotherapy and radiation, but died of disease eight months after presentation. We also review chondroblastoma in general, to assign this unusual lesion to a tumor subtype. Received: 29 March 1999 Revision requested: 27 April 1999 Revision received: 23 June 1999 Accepted: 29 June 1999