Cystic angiomatosis: case report and review of the literature

 The objective of this article was to offer a better characterization of the typical clinical presentation, radiologic findings, histology, treatment approaches, and differential diagnosis of cystic angiomatosis, a rare condition of which previous reports have been confusing because of unclear diagnostic criteria, different classifications, and variations in terminology. A case report using the improved imaging techniques of computed tomography scanning is presented in addition to an analysis and review of the previous literature, which relied heavily on plain film radiography, biopsy, and necropsy for diagnosis. A case report of a 26-year-old man initially symptomatic at age 12 is presented. Although a rare condition, cystic angiomatosis must be considered in pediatric and young adult patients presenting with diffuse, multifocal, cystic skeletal lesions, with or without visceral involvement.     

Osteoblastic response as a healing reaction to chemotherapy mimicking progressive disease in patients with small cell lung cancer

The osteoblastic response (OR) phenomenon as a healing reaction during effective chemotherapy—defined by the appearance of new osteoblastic bone lesions while disease response in other tumor sites was well documented—has previously been described for breast and prostate cancer. The purpose of this study was to investigate this phenomenon that could erroneously be interpreted as progressive disease in patients with small cell lung cancer (SCLC) and to establish guidelines for interpretation of follow-up computed tomography (CT) examinations in this situation. Twenty-four patients with newly diagnosed SCLC and bone metastases were retrospectively included in this study. The characteristics of bone lesions in CT examinations were correlated with bone scintigraphy and magnetic resonance imaging, if available. In target lesions the CT density quantified in Hounsfield units (HU) was evaluated at baseline and during follow-up. New osteoblastic lesions occurred during follow-up in 17 of 24 patients. OR was proven in 4 patients and considered most likely in 11 patients; mean density increase in target lesions was 153 HU. The study indicates that osteoblastic response as a healing reaction seems to occur in the majority of patients with SCLC and bone metastases and should not be misinterpreted as progressive disease.     

Osteolytic lesions and bacillary angiomatosis in HIV infection: radiologic differentiation from AIDS-related Kaposi sarcoma

Bacillary angiomatosis is a newly recognized multisystem bacterial infectious disease seen in the setting of human immunodeficiency virus (HIV) infection. The disease is marked by cutaneous vascular lesions that contain a bacterium similar to the cat scratch disease bacillus. Antibiotic therapy leads to the resolution of both cutaneous and systemic manifestations. Of 17 HIV-infected patients with cutaneous lesions of bacillary angiomatosis, six (35%) had symptomatic osteolytic bone lesions that improved following antibiotic therapy. The authors describe the appearance of the bone lesions on radiographs, computed tomographic (CT) scans, magnetic resonance (MR) images, and radionuclide studies. Osteolytic lesions are a relatively common feature of bacillary angiomatosis in patients with HIV infection. The presence of bone lesions aids in differentiation of bacillary angiomatosis from acquired immunodeficiency syndrome-related Kaposi sarcoma, which has similar cutaneous abnormalities but no associated bone lesions.     

Cystic angiomatosis with splenic involvement: unusual MRI findings

Cystic angiomatosis is a rare disorder with a poor prognosis. We describe a case of a 33-year-old woman who presented with long-standing bone pain, hemolytic anemia, and an enlarged spleen. Radiologically, multiple osseous lesions with a mixed pattern of lytic and sclerotic areas were seen within the shoulders, spine, and pelvis. On CT and MRI of the abdomen, the spleen was markedly enlarged, with internal hyperdense foci on non-contrast CT scan, corresponding to low signal intensity areas on all MR pulse sequences. After administration of contrast, a mottled enhancement pattern throughout the entire spleen was seen both on CT and MRI. Cystic angiomatosis was proven by histological analysis of a biopsy specimen of an involved vertebra and histopathological examination of the spleen after subsequent splenectomy. This is the first report of a patient with disseminated cystic angiomatosis with splenic involvement in which the MRI features differ from the previous reports. Instead of the usual pattern consisting of multiple well-defined cystic lesions, a diffuse involvement replacing the entire spleen, with heterogeneous signal intensities on T2-weighted images and heterogeneous enhancement pattern, was seen in our patient.     

The doughnut sign in patients with multiple myeloma

Bone lesions with a ring-shaped appearance (the doughnut sign) have been encountered during routine reporting of bone scintigrams performed on patients with multiple myeloma. Such a ring-shaped appearance has been revealed in four (8%) of the last 50 bone scintigrams performed on patients with myeloma at the Royal Marsden Hospital. In contrast, a review of the last 300 bone scintigrams performed on patients with bone metastases failed to reveal any such ring-shaped appearance. Examination of the case notes and radiographs of the multiple myeloma patients did not reveal any correlation between the presence of the ring-shaped appearances and the histology, biochemistry, treatment, or course of the disease nor were any specific radiographic features noted. Three of the four patients have had local radiotherapy to the sites of the lesions. We conclude that the ring-shaped appearance on bone scintigrams is usually related to myelomatous bone lesions, particularly following local radiotherapy to these lesions. The reason for this appearance in three of the four scintigrams may be the stimulation of vascularity and osteoblastic activity around the periphery of the myelomatous lesions by the response of the lesions to radiotherapy.     

Giant cell reparative granuloma of the sphenoid bone

We present 2 patients with giant cell reparative granuloma (GCRG) of the sphenoid bone. The first patient is an 8-year-old boy with involvement of the greater wing, and the second is a 53- year-old man with a lateral pterygoid plate mass. Both patients presented with rapid expansion of lytic bone lesions, which had solid and cystic components and lacked matrix calcification. Biopsies were indeterminate for definitive diagnoses. The radiologic appearance, location, and incidence of the lesions, and the patient's age and medical history are helpful aids in narrowing the differential diagnosis of sphenoid bone lesions. However, the imaging and, occasionally, even the histologic findings may not suggest the specific diagnosis of GCRG, which must be added into the differential diagnosis of rapidly enlarging cystic bone lesions of the sphenoid bone.     

Temporal progression of skeletal cystic angiomatosis

Cystic angiomatosis is a rare, benign, multifocal disorder of bone and viscera, in which angiomatous deposits of both vascular and lymphatic elements result in bone lysis and organ dysfunction. We report on a case of late-onset cystic angiomatosis in a Caucasian woman who first presented at age 35 years with a lytic expansile lesion of the proximal humerus, initially diagnosed as low-grade hemangio-endothelioma. This was treated with injection of cement and prophylactic pinning. However, the lesion continued to grow, and, 5 years later, she was discovered to have disseminated bony involvement, initially thought to represent metastatic disease. However, further investigation revealed a diagnosis of cystic angiomatosis, and the patient was treated with bisphosphonates. Follow-up over a 15-year period since her initial presentation at age 35 years has shown osteosclerotic conversion of many of the lesions, with development of numerous pathologic stress fractures that have failed to heal, despite operative intervention.     

Myositis ossificans: MR appearance with radiologic-pathologic correlation

We reviewed retrospectively the MR images of eight histologically proved cases of myositis ossificans and correlated the MR appearance with the histologic findings, as well as with other radiologic studies. Patients with available MR images were chosen from a group of 326 cases in our radiologic archives of histologically proved and radiologically correlated myositis ossificans. In addition to MR images, all patients had plain radiographs, six had CT scans, and two had arteriograms. On T2-weighted spin-echo MR, the lesions were relatively well defined and inhomogeneous and had intermediate to high signal intensity. The latter corresponded to a central proliferating core of fibroblasts and myofibroblasts with a myxoid stroma resembling nodular fasciitis, rimmed by osteoblasts with bone production. Edema surrounded lesions less than a few months old. T1-weighted images of early lesions were normal or showed evidence of a mass by displacement of fat planes. Hemorrhage and fluid-fluid levels were seen in one lesion of intermediate duration. Mature lesions tended to be well defined with inhomogeneous signal intensity, similar to that of fat, representing areas of fat situated between bone trabeculae within the lesion. We present the MR appearance of myositis ossificans and correlate it with other radiologic studies and the histologic findings. The varying appearance of myositis ossificans relates to the histologic changes that occur as the disorder progresses. Knowledge of the MR appearance of myositis ossificans is important in that the lesion has many of the MR imaging characteristics frequently associated with malignancy.     

Tumour-like lesions in the femur with cementum-like material

Five hundred and seventy nine juvenile bone cysts were analysed to evaluate the radiological and histological structures. Most of the juvenile bone cysts showed a typical appearance radiographically; histologically they had a cyst wall consisting of fibrous tissue without an epithelial lining. In many of these lesions newly formed bone trabeculae, granulation tissue, calcium deposits, and in some cases focal deposits of a homogeneous material resembling cementum of tooth were observed. In 28 lesions a well circumscribed, round focus was observed within the proximal femur or femoral neck radiologically, showing dense calcification with a small central radiolucency. In some cases, the intraosseous focus was almost solid without a cystic appearance. Histologically the unusual bone lesions consisted almost exclusively of calcified cementum devoid of cells. Lesions of this type were found exclusively in the proximal femur of patients between 47 and 56 years of age. Because of the radiological and histological appearance we would like to call this solid lesion cementoma of long bones. Operative therapy is not necessary.     

Skeletal changes in congenital fibrinogen abnormalities

We report an anatomico-radiologic study of humerus, femur, and tibia from a case of total congenital afibrinogenemia. Juxtatrabecular hemorrhages occur mainly in metaphyses and seem to be related to normal lines of stress. They may lead to the formation of intraosseous cysts and to a remodelling of bone trabeculae.The radiologic lesions in a second case, diagnosed as congenital dysfibrinogenemia, are similar to those found in Case 1 (femoral trabeculae remodelling) but also resemble some alterations described in hemophilia (pseudotumor of the right iliac bone). Anatomic study of the lesions in Case 2 was not possible.The significance of these observations could be better defined by a more extended skeletal study (radiologic and when feasible anatomic) of patients with congenital clotting defects and especially with inherited disorders of the fibrinogen molecule. It would also be worthwhile investigating manifest or latent hemostatic disorders (particularly at the fibrinogen level) in patients with solitary or aneurysmal bone cysts, and even with bone infarct or unexplained trabecular remodelling.     

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.     

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.     

CT findings in skeletal cystic echinococcosis

PURPOSE: To evaluate the CT findings of skeletal cystic echinococcosis. MATERIAL AND METHODS: CT findings of 7 patients with pathologically confirmed skeletal cystic echinococcosis were evaluated. RESULTS: There were 4 men and 3 women, aged 36-75 years. Hydatid cysts were located in the spine (n=2), a rib (n=3), the pelvis and a vertebra (n=1), the pelvis and the left femur (n=1). The size of the lesions varied from 1 cm to 15 cm. CT showed well defined, single or multiple cystic lesions with no contrast enhancement, no calcification, no daughter cysts, and no germinal membrane detachment. The cystic lesion had a honeycomb appearance in 2 cases; there was pathologic fracture in 2 cases, bone expansion in 5 cases, cortical thinning in 6 cases, cortical destruction in 6 cases, bone sclerosis in 1 case, and soft tissue extension in 6 cases. CONCLUSION: Preoperative differential diagnosis of skeletal cystic lesions should include cystic echinococcosis, especially in endemic areas, since this diagnosis may easily be missed unless kept in mind.     

Multiple intracerebral cavernous angiomas

Eight patients (seven women and one man) with multiple intracerebral cavernous angiomas (cavernomas), also known as angiomatosis cerebri, were examined with high-field magnetic resonance imaging (MRI). Although previous articles have referred to such cases, a series similar to the one reported here has apparently not been described in the radiology literature. The patients presented with seizures, progressive neurologic deficit or cerebral hemorrhage. In all eight cases the multiplicity of the lesions was an incidental finding in the magnetic resonance images. The MRI appearance of the cavernomas, although characteristic, is similar to that of other angiographically occult intracranial vascular malformations, in particular thrombosed arteriovenous malformations and mixed vascular malformations, as well as that of hemorrhagic metastases. Additional criteria, such as the absence of edema, the presence of calcifications and the temporal evolution of the cavernomas on serial scans, should allow cavernomas to be differentiated from hemorrhagic metastases. The exquisite sensitivity in detecting angiomatosis cerebri and the ability to show the evolution of internal hemorrhage in individual lesions make MRI the method of choice for diagnosing and following this condition.     

Infantile polycystic kidney disease: an imaging dilemma

Infantile and adult type polycystic kidney diseases are 2 disparate genetic disorders and generally are easily distinguishable on the basis of clinical, pathologic, and radiologic findings. We present 3 children with infantile polycystic kidney disease, ages 9 months to 6 years, in whom the excretory urogram and/or renal ultrasound or gross anatomical appearance of the kidneys resembled adult polycystic kidney disease. The findings from these 3 patients emphasize the importance of renal and liver biopsies in the diagnosis of cystic kidney disease in young children.     

Infantile polycystic kidney disease: An imaging dilemma

Infantile and adult type polycystic kidney diseases are 2 disparate genetic disorders and generally are easily distinguishable on the basis of clinical, pathologic, and radiologic findings. We present 3 children with infantile polycystic kidney disease, ages 9 months to 6 years, in whom the excretory urogram and/or renal ultrasound or gross anatomical appearance of the kidneys resembled adult polycystic kidney disease. The findings from these 3 patients emphasize the importance of renal and liver biopsies in the diagnosis of cystic kidney disease in young children.     

Cerebellar hemangioblastoma: computed tomographic, angiographic and clinical correlation in seven cases

Seven patients with histologically confirmed cerebellar hemangioblastomas are reviewed in order to establish computed tomographic (CT), angiographic and clinical characteristics and correlations. Noncontrast CT scans demonstrated most cystic lesions but did not always depict nodules on the cyst wall and small solid tumors. Contrast-enhanced CT scans demonstrated the solid and cystic types of tumors but in some cases angiography better delineated them and the presence of nodules. The CT appearance reflected the pathologic characteristics, which included vascular channels, cyst formation and nodules. Solid tumors enhanced homogeneously and could not be definitively distinguished from other cerebellar neoplasms by CT alone. One atypical undifferentiated tumor appeared as a solid mass with a necrotic center. The CT appearance of associated retinal angiomatosis is also described. Hydrocephalus, gait or appendicular ataxia was the initial clinical presentation in these patients.     

Fibrous dysplasia of bone: case report with autopsy study 80 years after the original clinical recognition of the bone lesions

 We present a report of the histological features of the bone lesions in a case of polyostotic fibrous dysplasia studied at autopsy in a patient aged 88 years. While much of the fibrous tissue of the lesions contained the trabeculae of non-lamellar bone that are a feature of fibrous dysplasia in young patients, other parts of the lesions showed evidence of osteoclastic remodelling of bone trabeculae, and even of the replacement of some of these structures by mature lamellar bone.     

Radiologic morphology of low-grade primary central nervous system lymphoma in immunocompetent patients

BACKGROUND AND PURPOSE: Primary central nervous system lymphomas (PCNSLs) are usually high-grade and are rarely low-grade non-Hodgkin lymphomas (NHLs). On MR imaging, PCNSLs typically present as contrast-enhancing lesions in contact with the subarachnoid space without evidence of necrosis. We evaluated the radiologic morphology and clinical characteristics of low-grade PCNSLs, hypothesizing that they may differ from high-grade PCNSLs. METHODS: Records were reviewed from 332 patients screened for inclusion in 3 multicenter prospective trials. MR imaging scans were obtained from all patients and were centrally reviewed by 2 consultant neuroradiologists. RESULTS: Ten patients (3%) with low-grade PCNSLs (7 men and 3 women; median age, 59 years; age range, 19-61 years) were identified. Four patients had one lesion, 2 patients 2 lesions, and 4 patients had multiple lesions. The following radiologic features infrequently seen in high-grade PCNSLs were found in a substantial proportion of patients: location in deep structures or spine (n = 6); lack of periventricular location (n = 5); hyperintensity on T2-weighted images (n = 10); moderate or absent contrast enhancement (n = 6); and heterogeneous contrast enhancement (n = 5). In 8 patients, >2 of these features were present in at least one lesion, and, thus, the radiologic appearance was assessed atypical of high-grade PCNSLs. The atypical radiologic appearance in combination with atypical or mild symptoms resulted in a false or delayed diagnosis. CONCLUSION: Low-grade PCNSLs may have a variable and atypical radiologic morphology compared with high-grade PCNSLs with the risk of false or delayed diagnosis.