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Intradural plasmacytoma originating from the filum terminale in the setting of multiple myeloma: illustrative case.

BackgroundMultiple myeloma (MM) has the propensity to spread to vertebral bodies; however, extramedullary plasmacytomas involving the central nervous system are extremely rare.ObservationsThe authors report the first intradural extramedullary plasmacytoma in the lumbar region of the spine in a patient with preexisting MM. They present a 50-year-old female with severe back and radicular pain and progressive neurological deficit.LessonsMM is typically treated with chemotherapy and radiation therapy. However, in this unique case, resection proved to be a key part of treatment.

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… In contrast, theirs was a solitary plasmacytoma that arose from the ventral dura and showed adjacent vertebral body hyperostosis. 6 Hyperostotic reaction and/or tumor calcification have been reported as signs of plasmacytoma. 11 There is no consensus on how plasma cells spread to the dura mater, but …

Overexpression of FGFR3, Stat1, Stat5 and p21Cip1 correlates with phenotypic severity and defective chondrocyte differentiation in FGFR3-related chondrodysplasias.

Achondroplasia (ACH) and thanatophoric dysplasia (TD) are human skeletal disorders of increasing severity accounted for by mutations in the fibroblast growth factor receptor 3 (FGFR3). Attempts to elucidate the molecular signaling pathways leading to these phenotypes through mouse model engineering have provided relevant information mostly in the postnatal period. The availability of a large series of human fetuses including 14 ACH and 26 TD enabled the consequences of FGFR3 mutations on endogenous receptor expression during the prenatal period to be assessed by analysis of primary cultured ch

Decision-Making in Clival Mass Lesions: Risk Factors for Malignant Disease and an Illustrative Case Example.

Introduction  Clival tumors are rare and heterogeneous. Although some benign prototypical sellar lesions may present as clival tumors, the likelihood of malignant disease is higher. Here we define a novel algorithm for the workup and management of clival masses through an illustrative case of colorectal adenocarcinoma metastasis to the clivus. Methods  In this case report, the best practice guidelines for managing clival masses are described through a literature review and refined by senior author consensus. We conducted a focused systematic review to characterize the present case in the conte

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… Meningiomas are the most common benign primary central nervous system neoplasm, and frequently demonstrate iso- to hyperdense CT and iso- to hypointense T1- and T2-weighted MRI findings. 10 Characteristic features of meningiomas include the presence of a wide dural base with an enhancing dural tail, hyperostosis …

Retrospective Analysis of Clinicopathological Characteristics of Lacrimal Gland Pleomorphic Adenoma and Mechanism of Tumorigenesis by the Imbalance Between Apoptosis and Proliferation.

BACKGROUND Lacrimal gland pleomorphic adenoma (LGPA) is the most common clinically benign epithelial tumor of the lacrimal gland and is predominantly comprised of epithelial cells and interstitial components. At present, the exact pathogenesis of LGPA remains unclear. Previous research has indicated that the occurrence of LGPA may be related to excessive cell proliferation. MATERIAL AND METHODS This study observed the clinicopathological characteristics of LGPA and investigated the tumorigenesis mechanism of cell over-proliferation caused by the imbalance between apoptosis and proliferation. A

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… 32016935 7 Wang Y Song L Guo J Xian J Value of quantitative multiparametric MRI in differentiating pleomorphic adenomas from malignant epithelial tumors in lacrimal gland Neuroradiology 2020 62 9 1141 47 32430642 10.1007/s00234-020-02455-3 8 Behshad S Sethi P Valenzuela AA The malignancy masquerader: Hyperostosis …

Reducing diagnostic delay in hypophosphatasia: a case series of 14 patients presenting to general rheumatology.

OBJECTIVES: Hypophosphatasia (HPP) is a rare genetic metabolic bone disease that can cause chronic pain and fractures. Its hallmark is a persistently low serum ALP. HPP is now recognised by many osteoporosis specialists, but other specialists, such as rheumatologists and primary care physicians, may be less aware of this condition, causing diagnostic delay and possible harm to these patients. Our objective was to highlight features that can reduce this delay. METHODS: We retrospectively analysed 14 patients that presented with musculoskeletal pain to general rheumatology clinic at St. George'

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… However, one study found that HPP in children can cause a chronic auto-inflammatory process in bones and joints similar to CRMO (chronic recurrent multifocal osteomyelitis) or SAPHO (synovitis, acne, pustulosis, hyperostosis) syndrome, both of which are associated with psoriasis [ 17 ]. …

Rare Causes of Musculoskeletal Pain: Thinking beyond Common Rheumatologic Diseases.

OBJECTIVES: Rare metabolic bone diseases can present with symptoms mimicking more common rheumatological conditions including spondyloarthritis, osteoarthritis, and fibromyalgia. Increasing awareness of these rare diseases within the rheumatology community is vital to ensure that affected patients are diagnosed and appropriately treated. The literature includes several reports of tumour-induced osteomalacia initially diagnosed as rheumatic disease, but other rare diseases such as X-linked hypophosphatemia (XLH) and hypophosphatasia (HPP) also deserve attention. Here, we describe two cases of a

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… In addition to AS, our XLH case's rheumatologist also considered a diagnosis of diffuse idiopathic skeletal hyperostosis (DISH), due to ectopic bone formation in the spine. …

Utility of the fast-field-echo resembling a computed tomography using restricted echo-spacing sequence in evaluating ossification of the posterior longitudinal ligament of the cervical spine.

BACKGROUND: Ossification of the posterior longitudinal ligament (OPLL) is a degenerative disease of the cervical spine. Due to the ectopic calcification of the posterior longitudinal ligament, the spinal cord or nerve roots may be compressed, producing neurological symptoms. Computed tomography (CT) is considered the "gold standard" for the diagnosis of OPLL; however, its ability to visualize soft-tissue lesions is limited. This study aimed to evaluate the utility of the magnetic resonance imaging (MRI) using the fast-field-echo resembling a computed tomography using restricted echo-spacing (F

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… Prevalence of Diffuse Idiopathic Skeletal Hyperostosis Diagnosed by Whole Spine Computed Tomography: A Preliminary Study. Clin Orthop Surg 2018 ; 10 : 41 - 6 . 10.4055/cios.2018.10.1.41 29564046 PMC5851853 2 Boody BS Lendner M Vaccaro AR . …

Е-постеры.

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An Unrecognized Ligament and its Ossification in the Craniocervical Junction: Prevalence, Patient Characteristics, and Anatomic Evidence.

BACKGROUND: In the craniocervical junction, the ligaments between the anterior foramen magnum and the anterior arch of the atlas are not wel … ligamentum flavum, transverse ligament, and apical ligament, as well as diffuse idiopathic skeletal hyperostosis (DISH). The association between these structures was also assessed. This unreported ossification was called the capped dens sign. It was defined and graded from 1 to 3. Grade 3 was defined as the typical capped dens sign. Cervical spine MRI was used …

Utility of REMS-Derived Fragility Score and Trabecular Bone Score in Evaluating Bone Health in Type 2 Diabetes Mellitus.

Background/Objectives: A significantly higher fracture risk characterizes Type 2 diabetes mellitus (T2DM) patients when compared to the non-diabetic population, even though their average bone mineral density (BMD) tends to be normal or high. This elevated risk is primarily driven by defective bone quality. The trabecular bone score (TBS) and radiofrequency echographic multispectrometry (REMS) have recently been proposed to improve the assessment of bone quality in T2DM individuals. This study aimed to evaluate whether TBS and REMS can improve the identification of osteoporosis and fracture ris

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… The discrepancy between REMS and DXA measurements may be explained, at least in part, by the ability of REMS to eliminate artifacts commonly observed in patients with T2DM, such as osteophytes, degenerative joint changes, vascular calcifications, and diffuse idiopathic skeletal hyperostosis, which typically …

Asia-Pacific consensus statement on medication-related osteonecrosis of the jaw in patients with osteoporosis.

A unified consensus statement on medication-related osteonecrosis of the jaw (MRONJ) has not yet been established among the Asian member countries or regions of the Asian Federation of Osteoporosis Societies (AFOS). This study aimed to develop a consensus on MRONJ in patients with osteoporosis across these countries and regions. In this study, the term "Asia-Pacific" refers specifically to the Asian member countries and regions of AFOS. A structured survey consisting of nine MRONJ-related questions was distributed across 10 countries and regions to assess the level of agreement and summarize r

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… Other important differential diagnoses include suppurative osteomyelitis of odontogenic origin; diffuse sclerosing osteomyelitis associated with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome [ 34 ]; and radiation-induced osteonecrosis. … Diagnostic points and possible origin of osteomyelitis in synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome: a radiographic study of 77 mandibular osteomyelitis cases Rheumatology 42 2003 1398 1403 12867589 10.1093/rheumatology/keg396 35 Traboulsi-Garet B. Jorba-García A. …

The Schnitzler syndrome.

The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of

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… Osteocondensation is the most frequent radiological finding and the most common radiological pattern is a sclerotic bone marrow involvement with cortical hyperostosis of distal femora and proximal tibiae, and a lack of suggestive signs of malignancy. …

Consensus Recommendations for the Diagnosis and Management of X-Linked Hypophosphatemia in Belgium.

X-linked hypophosphatemia (XLH) is the most common genetic form of hypophosphatemic rickets and osteomalacia. In this disease, mutations in the PHEX gene lead to elevated levels of the hormone fibroblast growth factor 23 (FGF23), resulting in renal phosphate wasting and impaired skeletal and dental mineralization. Recently, international guidelines for the diagnosis and treatment of this condition have been published. However, more specific recommendations are needed to provide guidance at the national level, considering resource availability and health economic aspects. A national multidiscip

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… ARHR1 may present as a sclerosing bone dysplasia with hyperostosis and very dense vertebral bodies ( 54 ). DMP1 as well as PHEX are highly expressed in osteocytes, although it remains unknown how they alter the phosphate-FGF23 set point ( 55 ). … Hypophosphatemic osteosclerosis, hyperostosis, and enthesopathy associated with novel homozygous mutations of DMP1 encoding dentin matrix protein 1 and SPP1 encoding osteopontin: The first digenic SIBLING protein osteopathy? Bone (2020) 132:115190. 10.1016/j.bone.2019.115190 62. …

Diagnosis and treatment of Paget's disease of bone: position paper from the Italian Society of Osteoporosis, Mineral Metabolism and Skeletal Diseases (SIOMMMS).

INTRODUCTION: Paget's disease of bone is a focal skeletal disorder causing bone deformities and impairing bone quality. Despite the prevalence of asymptomatic cases is increasing, the progression of the disease can lead to invalidating complications that compromise the quality of life. Doubts on clinical and therapeutic management aspects exist, although beneficial effects of antiresorptive drugs, particularly bisphosphonates are known. However, limited information is available from randomized controlled trials on the prevention of disease complications so that somewhat contrasting positions a

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… No bone expansion or cortical breach Hyperostosis frontalis interna Usually affects the outer calvarial table more prominently Erdheim Chester disease Osteosclerotic lesions are generally symmetrical and do not lead to bone deformity Table 2 Main bone disorders to be excluded in the diagnostic process … No bone expansion or cortical breach Hyperostosis frontalis interna | Usually affects the outer calvarial table more prominently Erdheim Chester disease | Osteosclerotic lesions are generally symmetrical and do not lead to bone deformity The largest description of histomorphometry and histological characteristics …

Identified Medications Causing Medication-Related Osteonecrosis of the Jaw: A Literature Review.

Medication-related osteonecrosis of the jaw (MRONJ) is a serious adverse event that occurs in patients treated with certain medications, leading to a major side effect that can result in substantial morbidity. MRONJ is known to be associated with antiresorptive (AR) and antiangiogenic (AA) medicines, but recent evidence suggests that other medications may also be involved. Recognizing a broad range of drug-induced risk factors is important, notably in light of the increasing number of drugs associated with MRONJ. The primary objective of this review is to update and synthesize findings from th

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… conditions, including (a) osteoporosis; (b) Paget’s disease of bone; (c) malignancy-related conditions like MM, bone metastasis, and hypercalcemia; (d) adjuvant therapy for breast cancer (even without bone metastasis); or (e) other conditions such as osteogenesis imperfecta, synovitis, acne, pustulosis, hyperostosis …

Pyoderma gangrenosum: pathogenetic mechanisms and their implications for treatment.

Pyoderma gangrenosum (PG) is a rare inflammatory skin disease belonging to the group of neutrophilic dermatoses. The pathogenesis of PG involves a predisposing genetic background that facilitates a dysregulated innate and adaptive immune response, with an imbalance between pro-inflammatory and anti-inflammatory mediators, leading to neutrophil-driven inflammatory damage. Several immunosuppressants and immunomodulatory drugs are currently available for the treatment of PG, in combination with topical therapies, wound management and pain control strategies. Systemic corticosteroids and cyclospor

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… Hidradenitis Suppurativa-Related autoinflammatory syndromes: an updated review on the Clinics, Genetics, and treatment of pyoderma gangrenosum, acne and suppurative hidradenitis (PASH), pyogenic Arthritis, pyoderma gangrenosum, acne and suppurative hidradenitis (PAPASH), Synovitis, acne, Pustulosis, hyperostosis … Hidradenitis Suppurativa-Related autoinflammatory syndromes: an updated review on the Clinics, Genetics, and treatment of pyoderma gangrenosum, acne and suppurative hidradenitis (PASH), pyogenic Arthritis, pyoderma gangrenosum, acne and suppurative hidradenitis (PAPASH), Synovitis, acne, Pustulosis, hyperostosis …

Proceedings 27th Symposium ESVN‐ECVN

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Neurology

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… However, in a study of canine brain tumors, CT correctly predicted histologic type in 86% of cases. 107 Meningiomas appear isodense to hyperdense, homogenous, and brightly enhanced on CT, and calcification is easily recognized ( Figure 27-5 ). 115 Hyperostosis, frequently seen in the calvarium overlying … Note the hyperdense area of calcification ventrally and the hyperostosis in the overlying calvaria. (Courtesy Dr. Kerry Bailey, Oradell Animal Hospital.) …

Nuances in the treatment of malignant tumors of the clival and petroclival region.

Introduction Malignancies of the clivus and petroclival region are mainly chordomas and chondrosarcomas. Although a spectrum of malignancies may present in this area, a finite group of commonly encountered malignant pathologies will be the focus of this review, as they are recognized to be formidable pathologies due to adjacent critical neurovascular structures and challenging surgical approaches. Objectives The objective is to review the literature regarding medical and surgical management of malignant tumors of the clival and petroclival region with a focus on clinical presentation, diagnost

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… Hyperostosis, increased vascular markings, and calcification are characteristic findings of meningiomas on CT; however, malignant meningiomas can be more destructive of bony structures and more invasive of soft tissue, which can also be evident on MRI ( Fig. 5 ). …

Informed appropriate imaging for low back pain management: A narrative review.

Most patients with acute low back pain (LBP), with or without radiculopathy, have substantial improvements in pain and function in the first 4 weeks, and they do not require routine imaging. Imaging is considered in those patients who have had up to 6 weeks of medical management and physical therapy that resulted in little or no improvement in their LBP. It is also considered for those patients presenting with suspicion for serious underlying conditions, such as cauda equina syndrome, malignancy, fracture and infection. In western country primary care settings, the prevalence has been suggeste

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… These include the Anderson and Romanus lesions in seronegative spondyloarthropathies, the subligamentous oedema in diffuse idiopathic skeletal hyperostosis, haemodialysis spondyloarthropathy, neuropathic spine and infectious spondylodiscitis [70] , [86] . …