Une ostéomalacie liée à un diabète phosphoré – rôle de la scintigraphie osseuse couplée à la TEMP-TDM

We report the case of a 59-year-old woman, investigated for disabling pain of the left thigh, unrelated to any traumatic event. Interrogation had found diffuse pain of myalgia-type and arthralgia-type for approximately a year without local inflammatory signs and insufficiency fractures of both calcaneus two years before. The Technetium 99m-labeled hydroxymethylene diphosphonate (^99mTc-HDP) whole-body bone scintigraphy evidenced multiple hot spots on the higher third of left femur, rib cage, sternum, scapulae, pelvis, right hip and both calcaneus. Moreover, a more diffuse and heterogeneous prominent uptake appeared on rib cage, spine and pelvis. These images suggested a diffuse metastatic disease of the skeleton. The single photon emission computerized tomography guided by computerized tomography (SPECT/CT), centered on lumbar spine, pelvis and the upper end of femurs showed that the multiple hot spots were infact bone fractures. These findings pointed diagnosis to a metabolic disease. The clinical context was in favour of an osteomalacia. Further explorations showed an osteomalacia related to phosphate diabetes. A thorough work-up did not reveal any known aetiology. To date, idiopathic phosphate diabetes seems the most likely diagnosis. Nuclear medicine input in osteomalacia is discussed.     

Severe Hypophosphatemic Osteomalacia Secondary to Fanconi Syndrome Due to Adefovir: A Case Report

ObjectiveGeneralized proximal, type 2, renal tubular acidosis, also known as Fanconi syndrome, is a generalized dysfunction of the proximal renal tubule characterized by impaired reabsorption and increased urinary loss of phosphate and other solutes, such as uric acid, glucose, amino acids, and bicarbonate. Chronic hypophosphatemia is the second most common cause of osteomalacia after vitamin D deficiency in adult patients and can have a heterogeneous presentation, ranging from mild symptoms such as muscle weakness and skeletal pain to more severe presentation, such as disabling myopathy, severe bone and joint pain, difficulty walking, and even bone fractures.MethodsThis report describes a case of severe hypophosphatemic osteomalacia with multiple fragility fractures induced by adefovir, which was worsened and confounded by a previous treatment with zoledronic acid and required prolonged intravenous potassium phosphate administration.ResultsWe highlight the limited diagnostic value of dual X-ray absorptiometry and bone scintigraphy in this challenging diagnosis. Bone metabolism should always be assessed in patients treated with adefovir for early detection of osteomalacia due to Fanconi syndrome.ConclusionAlthough rare, this condition may be life-threatening and mimic other bone metabolic disorders that are treated with drugs that may further impair phosphate balance. (Endocr Pract. 2014;20:e246-e249)     

Histiocytose langerhansienne osseuse multifocale : intérêt de la scintigraphie osseuse planaire

Langerhans cell histiocytosis is a disease of the reticuloendothelial system characterized by a clonal proliferation of dendritic cells of Langerhans. This is a rare disease that primarily affects children and young adults. It can take many forms, from a single eosinophilic granuloma to widespread lesions involving multisystem life-threatening. Bone involvement is the most common, it can be uni- or multifocal. We report a case of multifocal bone Langerhans cell histiocytosis, revealed in a child aged 3 years and touched his left temporal bone and right femur. The bone scintigraphy has found a third location in right scapular. The outcome was favorable after chemotherapy. The bone lesions are characterized by lytic lesions and are responsible for pain, swelling and fractures. All the imaging techniques (radiographs, CT, and MRI) contribute to the formation of the diagnosis. Bone scintigraphy is a very sensitive examination that allows the initial staging and follow-up on treatment of skeletal manifestations.     

Oncogenic osteomalacia associated with mesenchymal tumor in the middle cranial fossa: a case report

ABSTRACT: INTRODUCTION: Tumor-induced osteomalacia is a paraneoplastic syndrome of hypophosphatemia. Osteomalacia causes multiple bone fractures and severe pain. CASE PRESENTATION: We report the case of a 57-year-old Japanese man with tumor-induced osteomalacia associated with a middle cranial fossa bone tumor. The tumor was successfully resected by using a middle fossa epidural approach. His phosphate level recovered to a normal range immediately after the surgery. CONCLUSIONS: It is rare that tumor-induced osteomalacia originates from the middle skull base. This report suggests that, if patients have a clinical and biochemical picture suggestive of tumor-induced osteomalacia, it is crucial to perform a meticulous examination to detect the tumor or the lesion responsible for the tumor. The serum level of fibroblast growth factor 23 is the most reliable marker for evaluating the treatment outcome of tumor-induced osteomalacia.     

Histiocytose langerhansienne : place actuelle de l’imagerie

Langerhans cell histiocytosis (LCH) is a rare disease of the reticuloendothelial system which consists in various clinical manifestations from a single lytic bone lesion to multisystemic lesions with organ dysfunction. One single bone lesion, the most frequent presentation, has a good prognosis. We relate here the case of a 4-year-old child who had a pain of his right hip with lameness and fever. The bone scintigraphy (BS) highlighted an increased uptake of the right iliac wing. Biopsy of the lesion led to the diagnosis of LCH. The progression was spontaneously favourable with relief of pain after the biopsy. This case report allows us to highlight the most recent guidelines regarding the classification of the disease and the indication of imaging. Staging is currently based on chest radiograph, abdominal ultrasound and skeletal radiograph survey. The role of BS, MRI and FDG PET/CT for staging remains to be clarified.     

CT与MRI扫描三维重建在四肢骨关节隐匿性骨折诊断中的应用

摘要 目的：探讨电子计算机断层扫描(Computed Tomography,CT)与磁共振成像(Magnetic resonance imaging,MRI)扫描三维重建在四肢骨关节隐匿性骨折诊断中的应用。方法：2016年9月到2019年10月选择在本院诊治的下拟诊为四肢骨关节隐匿性骨折118例,所有患者都给予CT与MRI扫描三维重建诊断,记录影像学特征与判断诊断价值。结果：在118例患者中,最终确诊为四肢骨关节隐匿性骨折98例,无骨折20例,其中腕关节骨折34例,踝关节骨折22例,膝关节骨折15例,肘关节骨折15例,肩关节骨折8例,髋关节骨折4例。在98例确诊的四肢骨关节隐匿性骨折中,MRI三维重建显示双边征、骨质破坏、充气征、软组织影等比例显著都高于CT (P<0.05)。CT与MRI三维重建诊断四肢骨关节隐匿性骨折的敏感性为89.8 %和99.0 %,特异性为95.0 %和100.0 %,误诊率分别为9.3 %和0.8 %,MRI三维重建诊断的敏感性高于CT ,漏诊率低于CT。结论：CT与MRI扫描三维重建在四肢骨关节隐匿性骨折诊断中的应用都有很好的价值,特别是MRI三维重建能清晰显示骨折特征,具有更高的诊断敏感性,能减少漏诊率,可作为四肢骨关节隐匿性骨折的首选检查方法。     

A rare association of hyperparathyroidism and Turner's syndrome--a case report

We present the clinical, laboratory, radiological and pathological findings in the case and review the literature. Our patient, a 37-year-old woman of short stature, was referred because of musculoskeletal pain. After primary evaluation, she underwent treatment with calcium and vitamin D supplement with the diagnosis of osteomalacia in Turner's syndrome. The rise of serum calcium during medical therapy, which was an unusual finding, attracted the clinician's attention to another underlying disorder. Further evaluation revealed primary hyperparathyroidism due to an adenoma of the parathyroid gland. Even though this is a rare diagnosis, its presence should be considered in any patient with Turner's syndrome presenting with severe osteoporosis and a rise in serum calcium during treatment.     

Fine needle aspiration diagnosis of osteitis fibrosa cystica (brown tumor of bone): a case report

BACKGROUND: Fine needle aspiration (FNA) cytologic diagnosis of bone lesions is controversial; opponents cite its low sensitivity and proponents emphasize its cost-effectiveness, specificity and rapid turnaround time. The focus of FNA cytology is most often to exclude malignancy, which may contribute to incomplete information sharing on the part of the health care team and the published low sensitivity of diagnostic cytology of osseous lesions. It is therefore incumbent upon cytopathologists to formulate a complete differential diagnosis of osseous lesions. CASE: A 72-year-old man, admitted for severe abdominal pain, underwent diagnostic computed tomography (CT) examination that incidentally revealed multiple abdominal and pelvic lytic bone lesions. CT-guided FNA of a lesion revealed bland histiocytic and spindled cells, prominent hemosiderin pigment and scattered multinucleated cells, findings consistent with osteitis fibrosa cystica. Subsequent consultation with the medical team revealed the patient's underlying secondary hyperparathyroidism. CONCLUSION: This case emphasizes the importance of correlating clinical history and radiologic findings to the accurate cytologic diagnosis of bone lesions. The benign cytomorphologic features of brown tumor should not be overlooked or be considered nondiagnostic in the hunt for suspected malignancy. FNA allowed confident exclusion of a malignant process and prevented unnecessary surgery and its inherent risks.     

Clinical usefulness of bisphosphonates in oncology: treatment of bone metastases, antitumoral activity and effect on bone resorption markers

The present article overviews the role of bisphosphonates for the treatment and prevention of bone metastases and their antiangiogenic effects and antitumoral activity. The skeleton is a frequent and clinically relevant site of metastasis in cancer patients. The major events related to bone metastases include bone pain, bone loss, hypercalcemia, spinal cord compression, and fractures. On the basis of their radiographic features, bone metastases are classified as osteoblastic, osteoclastic, or mixed. The primary goals of treatment of bone metastases are reduction of the risk of pathological fractures and other skeletal-related events, and pain control. Bisphosphonates are used to prevent pathological fractures by inhibition of osteoclasts. Recent studies suggest that bisphosphonates have some direct antitumoral activity, mainly mediated through the blockade of angiogenic pathways. Further clinical studies are needed to determine the optimal treatment duration, timing and schedule of bisphosphonates, assess their role as adjuvant therapy for the prevention of bone metastases, and establish their antiangiogenic activity in association with standard cytotoxic and hormonal drugs for treatment of patients with advanced disease.     

A study of complaints and their relation to vertebral destruction in patients with osteoporosis

Patients with spinal osteoporosis suffer from vertebral deformation, loss of height and back pain, as well as from functional limitations and alterations of mood. So far little is known about the extent of these clinical symptoms at all and whether they are related in a predictable manner to the fractures or damages of bone structure. In the present study we investigated the relation between vertebral deformation and clinical symptoms in 70 patients with osteoporosis. Clinical data like pain, functional limitations and parameters of mood were examined by a standardized questionnaire. The numbers of vertebral fractures were determined, and the vertebral destruction was quantified using the Spine Deformity Index (SDI). The symptoms and functional limitations were graded and correlated to the SDI and the number of fractures. Our results underline a relation between the extent of vertebral deformation and the reduction in quality of life by pain, functional limitations and alterations of mood. This relationship was absent or less evident, if the number of fractures was taken into account. Besides the difficulties concerning the grading and quantification of clinical symptoms and outcome of disease, our study revealed that there is a causal relation between the extent of vertebral destruction measured by the SDI and the extent of these clinical parameters.     

Progress in osteoporosis and fracture prevention: focus on postmenopausal women

In the past decade, we have witnessed a revolution in osteoporosis diagnosis and therapeutics. This includes enhanced understanding of basic bone biology, recognizing the severe consequences of fractures in terms of morbidity and short-term re-fracture and mortality risk and case finding based on clinical risks, bone mineral density, new imaging approaches, and contributors to secondary osteoporosis. Medical interventions that reduce fracture risk include sufficient calcium and vitamin D together with a wide spectrum of drug therapies (with antiresorptive, anabolic, or mixed effects). Emerging therapeutic options that target molecules of bone metabolism indicate that the next decade should offer even greater promise for further improving our diagnostic and treatment approaches.     

Imaging Non-Specific Wrist Pain: Interobserver Agreement and Diagnostic Accuracy of SPECT/CT,MRI, CT,Bone Scan and Plain Radiographs

Purpose

Chronic hand and wrist pain is a common clinical issue for orthopaedic surgeons and rheumatologists. The purpose of this study was 1. To analyze the interobserver agreement of SPECT/CT, MRI, CT, bone scan and plain radiographs in patients with non-specific pain of the hand and wrist, and 2. to assess the diagnostic accuracy of these imaging methods in this selected patient population.

Materials and Methods

Thirty-two consecutive patients with non-specific pain of the hand or wrist were evaluated retrospectively. All patients had been imaged by plain radiographs, planar early-phase imaging (bone scan), late-phase imaging (SPECT/CT including bone scan and CT), and MRI. Two experienced and two inexperienced readers analyzed the images with a standardized read-out protocol. Reading criteria were lesion detection and localisation, type and etiology of the underlying pathology. Diagnostic accuracy and interobserver agreement were determined for all readers and imaging modalities.

Results

The most accurate modality for experienced readers was SPECT/CT (accuracy 77%), followed by MRI (56%). The best performing, though little accurate modality for inexperienced readers was also SPECT/CT (44%), followed by MRI and bone scan (38% each). The interobserver agreement of experienced readers was generally high in SPECT/CT concerning lesion detection (kappa 0.93, MRI 0.72), localisation (kappa 0.91, MRI 0.75) and etiology (kappa 0.85, MRI 0.74), while MRI yielded better results on typification of lesions (kappa 0.75, SPECT/CT 0.69). There was poor agreement between experienced and inexperienced readers in SPECT/CT and MRI.

Conclusions

SPECT/CT proved to be the most helpful imaging modality in patients with non-specific wrist pain. The method was found reliable, providing high interobserver agreement, being outperformed by MRI only concerning the typification of lesions. We believe it is beneficial to integrate SPECT/CT into the diagnostic imaging algorithm of chronic wrist pain.     

TEP/TDM au FDG et bilan initial des lymphomes : du diagnostic au pronostic

Lymphoma represents a very heterogeneous pathologic disease group and its initial assessment, treatment strategy and prognosis is closely related within each histological subtype. The diagnosis of lymphoma disease is often due to the development of a tumour mass at a lymph node area, or within an organ with or without systemic symptoms. However, all these signs remain non specific. Biopsy is the only reliable diagnostic tool, which can be carried out surgically or guided by the morphological and functional imaging. Once the histological diagnosis is determined by the WHO classification, the staging procedure aim is to provide the prognosis to assist the therapeutic decision. Morphologic imaging primarily based on CT remains the reference in exploring most cases. But in the last decade, the introduction of functional imaging using FDG-PET/CT has dramatically changed the therapeutic management of lymphoma. The additional clinical value provided by initial FDG-PET/CT could lead to an alteration of the therapeutic strategy, as well as to the optimization of radiation therapy or to the establishment of prognostic score and lead to improved lymphoma patient survival in the future.     

Impact de l’imagerie hybride TEMP/TDM dans le diagnostic et la prise en charge de l’ostéome ostéoïde

IntroductionThe osteoid osteoma is a major bone benign tumors in children. It is more common in boys. The femoral and tibial locations account for 50% of cases. Clinically, it is revealed by nocturnal pain relieved by aspirin. The pain may precede by several months the radiographic abnormality. The purpose of our work is to elucidate the contribution of SPECT/CT in addition to the planar bone scintigraphy in the diagnosis and treatment of osteoid osteoma about two cases.Case reportClinical case No. 1: a 10-year-old child who presented a limp nocturnal pain in the left hip evolving for 4 months. The radiograph of the pelvis showed bone condensation subtrochanteric left femur. SPECT/CT showed an image for an osteoid osteoma. Surgical resection of the home revealed in the histological study an osteoid osteoma of the left femoral neck. The evolution was marked by an immediate pain relief and full recovery of the left lower limb mobility. Clinical case No. 2: an 11-year-old child who had a limp nocturnal pain at the upper end of the left femur evolving for 2 months. The pain was paroxysmal, relieved by salicylates. The X-ray of the pelvis showed a metaphyseal image with peripheral condensation and thickening of the cortex. SPECT/CT showed an image for an osteoid osteoma. The intervention was a tumor excision resection. Histopathological examination revealed a small nidus consistent with an osteoid osteoma. The evolution was marked by an immediate pain relief and normalization of the mobility of the left lower limb.DiscussionThe SPECT/CT can increase the sensitivity and specificity of planar bone scintigraphy. It confirms the location of osteoid osteoma and defines its anatomical relationships in order to optimize surgical management.ConclusionThe SPECT/CT contributes significantly to the diagnosis of osteoid osteoma when radiological images are atypical or unusual clinical expression.     

Pathological femoral fractures due to osteomalacia associated with adefovir dipivoxil treatment for hepatitis B: a case report

ABSTRACT: We present a case of a 62-year-old man who underwent total hip arthroplasty for treatment of pathologic femoral neck fracture associated with adefovir dipivoxil-induced osteomalacia. He had a 13-month history of bone pain involving his shoulders, hips, and knee. He received adefovir dipivoxil for treatment of lamivudine-resistant hepatitis B virus infection for 5 years before the occurrence of femoral neck fracture. Orthopedic surgeons should be aware of osteomalacia and pathological hip fracture caused by drug-induced renal dysfunction, which results in Fanconi's syndrome.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1600344696739249.     

Preoperative Tumor Localization by Means of Venous Sampling for Fibroblast Growth Factor-23 in a Patient with Tumor-Induced Osteomalacia

ObjectiveTo report on a novel strategy for tumor localization in a 62-year-old man with hypophosphatemic tumor-induced osteomalacia (TIO).MethodsRepeated computed tomographic and magnetic resonance imaging scans failed to localize any tumor in a patient with adult-onset hypophosphatemic osteomalacia. Therefore, venous sampling for fibroblast growth factor-23 (FGF23)—a circulating hormone that has been identified as a causative factor for TIO—in major veins was conducted. Serum FGF23 was measured from collected samples by an intact FGF23 enzyme-linked immunosorbent assay.ResultsVenous sampling suggested a local increase in serum FGF23 in the left femoral vein; this finding prompted performance of octreotide scintigraphy restricted to the left leg. A tumor was located at the lateral condyle of the left femur, which was also confirmed by magnetic resonance imaging. Surgical resection of the tumor normalized the serum phosphorus and 1,25-dihydroxyvitamin D₃ levels within 5 to 10 days, and FGF23 declined to normal levels within 24 hours. Histologic analysis supported the diagnosis of a soft-tissue giant cell tumor.ConclusionOur study case demonstrates the diagnostic complexity and difficulties in localizing a small tumor in a patient with TIO. Venous sampling for FGF23 may be helpful in tumor localization in sporadic cases of hypophosphatemic osteomalacia, especially when noninvasive diagnostic techniques prove insufficient. (Endocr Pract. 2008;14:362-367)     

16层螺旋CT三维重建与DR平片检查对外伤性肋骨骨折的诊断价值比较研究

目的:研究16层螺旋电子计算机断层扫描(CT)三维重建与数字化摄影(DR)平片检查对外伤性肋骨骨折的诊断价值,为临床诊治提供参考。方法:将2017年6月至2018年6月期间于本院接受诊治的82例外伤性肋骨骨折患者作为研究对象,所有患者均接受16层螺旋CT三维重建与DR平片检查,观察并记录患者的骨折发生部位,并比较两种诊断方法对外伤性肋骨骨折诊断的准确率、灵敏度、特异性、阳性预测值、阴性预测值以及漏诊情况。结果:82例外伤性肋骨骨折患者经影像学与临床诊断明确发生骨折179处,多发性骨折发生率为62.20%,单发性骨折发生率为37.80%,骨折肋骨段位中4-10段骨折发生率最高为69.83%,骨折肋骨水平阶段中腋肋骨折发生率最高为59.78%。相较于DR平片,16层螺旋CT三维重建诊断外伤性肋骨骨折的准确率、灵敏度、特异性、阳性预测值、阴性预测值更高,漏诊率更低,差异有统计学意义(P0.05)。结论:相较于DR平片检查,应用16层螺旋CT三维重建检查外伤性肋骨骨折能明显提高临床诊断的准确率、灵敏度和特异性,减少漏诊,可为临床诊治提供更可靠的信息,值得临床推广。     

Oncogenic Osteomalacia Cured by Removal of an Organized Hematoma

ObjectiveTo describe a patient with oncogenic osteomalacia whose symptoms were rapidly resolved after surgical removal of an organized hematoma of the hip.MethodsA case report is presented, including clinical and laboratory findings. The relevant literature is reviewed, and the current understanding of oncogenic osteomalacia is summarized.ResultsIn September 1996, a 44-year-old black woman presented with a 2-year history of bone pain, progressive muscle weakness, depression, osteomalacia, and hypophosphatemia. Her condition did not improve with use of calcitriol and phosphate replacement. During the previous year, her serum phosphorus levels were low, ranging from 1.0 to 2.2 mg/dL, and the levels of serum 1,25-dihydroxyvitamin D [1,25-(OH)₂D] were very low, ranging from < 5 to 19.4 pg/mL (normal, 15 to 60). The serum 25-hydroxyvitamin D levels were low, ranging from 8 to 14 ng/mL (normal, 9 to 52). The higher values were noted after she had received large doses of phosphate, 1,25-(OH)₂D, and vitamin D. During the previous year, her serum alkaline phosphatase levels were high, ranging from 253 to 314 U/L; serum calcium and parathyroid hormone levels were normal. The abnormalities on physical examination were obesity and a 10- by 10-cm firm, poorly demarcated mass superior to the left greater trochanter. A computed tomographic scan of this region showed a water-density fluid collection in the left buttock measuring 7.8 by 7.8 cm, consistent with a chronic hematoma. The mass was resected, and histopathologic examination revealed features of an organized hematoma with areas of myxoid changes and cartilaginous metaplasia. Postoperatively, the patient’s strength improved, and the levels of serum phosphorus and 1,25-(OH)₂D became supranormal.ConclusionThe symptoms and laboratory abnormalities of this patient with oncogenic osteomalacia promptly resolved after resection of an organized hematoma of the left hip. (Endocr Pract. 2005;11:190-193)     

Massive primary hepatic tuberculoma mimicking hepatocellular carcinoma in an immunocompetent host

Tuberculoma of the liver is rare in an immunocompetent individual. We report a 26-year-old man with upper abdominal pain, abnormal liver function, and raised inflammatory markers. Abdominal computed tomography (CT) scan revealed a mixed attenuation lesion measuring 6 x 5 cm occupying most of the left lobe of the liver. Subsequent histology and culture confirmed tuberculous abscess. Following antituberculous therapy, repeat CT scan revealed complete resolution of the initial findings. This case illustrates the diagnostic difficulties of hepatic tuberculosis (TB) and the importance of considering TB in patients with hepatic lesions.     

Intérêt de l’imagerie hybride TEMP-TDM dans l’histiocytose langerhansienne osseuse. À propos d’un cas

Langerhans’ cell histiocytosis is a non-malignant proliferative disease of unknown etiology. It is a rare illness affecting mainly children and young adults with a male predominance. It can affect one or many organs. The bone locations are the most frequent. We present one case of multifocal Langerhans’ cell histiocytosis in a 22-year-old male patient followed for diabetes insipidus with an enlargement of the pituitary stalk at the magnetic resonance imaging (MRI), and a lytic bone lesion revealed in lumbar spine (L2) by ^99mTc-HMDP scintigraphy conducted in planar mode, and then in Single Photon Emission Computed Tomography coupled to Computed Tomography (SPECT/CT) in hybrid mode. To this end, we propose to highlight diagnostic gain of SPECT/CT compared with SPECT and planar scintigraphy to characterize radiotracer uptake abnormalities in bone in Langerhans’ cell histiocytosis.