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Orthopaedics & Traumatology, Surgery &... Feb 2015Simple and aneurysmal bone cysts are benign lytic bone lesions, usually encountered in children and adolescents. Simple bone cyst is a cystic, fluid-filled lesion, which... (Review)
Review
Simple and aneurysmal bone cysts are benign lytic bone lesions, usually encountered in children and adolescents. Simple bone cyst is a cystic, fluid-filled lesion, which may be unicameral (UBC) or partially separated. UBC can involve all bones, but usually the long bone metaphysis and otherwise primarily the proximal humerus and proximal femur. The classic aneurysmal bone cyst (ABC) is an expansive and hemorrhagic tumor, usually showing characteristic translocation. About 30% of ABCs are secondary, without translocation; they occur in reaction to another, usually benign, bone lesion. ABCs are metaphyseal, excentric, bulging, fluid-filled and multicameral, and may develop in all bones of the skeleton. On MRI, the fluid level is evocative. It is mandatory to distinguish ABC from UBC, as prognosis and treatment are different. UBCs resolve spontaneously between adolescence and adulthood; the main concern is the risk of pathologic fracture. Treatment in non-threatening forms consists in intracystic injection of methylprednisolone. When there is a risk of fracture, especially of the femoral neck, surgery with curettage, filling with bone substitute or graft and osteosynthesis may be required. ABCs are potentially more aggressive, with a risk of bone destruction. Diagnosis must systematically be confirmed by biopsy, identifying soft-tissue parts, as telangiectatic sarcoma can mimic ABC. Intra-lesional sclerotherapy with alcohol is an effective treatment. In spinal ABC and in aggressive lesions with a risk of fracture, surgical treatment should be preferred, possibly after preoperative embolization. The risk of malignant transformation is very low, except in case of radiation therapy.
Topics: Adolescent; Biopsy; Bone Cysts; Bone Cysts, Aneurysmal; Child; Female; Fractures, Spontaneous; Humans; Magnetic Resonance Imaging; Male; Prognosis; Risk Factors; Treatment Outcome; Young Adult
PubMed: 25579825
DOI: 10.1016/j.otsr.2014.06.031 -
American Journal of Hematology May 2020Multiple myeloma accounts for approximately 10% of hematologic malignancies. (Review)
Review
DISEASE OVERVIEW
Multiple myeloma accounts for approximately 10% of hematologic malignancies.
DIAGNOSIS
The diagnosis requires ≥10% clonal bone marrow plasma cells or a biopsy proven plasmacytoma plus evidence of one or more multiple myeloma defining events (MDE) namely CRAB (hypercalcemia, renal failure, anemia, or lytic bone lesions) features felt related to the plasma cell disorder, bone marrow clonal plasmacytosis ≥60%, serum involved/uninvolved free light chain (FLC) ratio ≥100 (provided involved FLC is ≥100 mg/L), or >1 focal lesion on magnetic resonance imaging (MRI).
RISK STRATIFICATION
The presence of del(17p), t(4;14), t(14;16), t(14;20), gain 1q, or p53 mutation is considered high-risk multiple myeloma. Presence of any two high risk factors is considered double-hit myeloma; three or more high risk factors is triple-hit myeloma.
RISK-ADAPTED INITIAL THERAPY
In transplant eligible patients, induction therapy consists of bortezomib, lenalidomide, dexamethasone (VRd) given for approximately 3-4 cycles followed by autologous stem cell transplantation (ASCT). In high-risk patients, daratumumab, bortezomib, lenalidomide, dexamethasone (Dara-VRd) is an alternative to VRd. Selected standard risk patients can get additional cycles of induction, and delay transplant until first relapse. Patients not candidates for transplant are typically treated with VRd for approximately 8-12 cycles followed by lenalidomide; alternatively these patients can be treated with daratumumab, lenalidomide, dexamethasone (DRd).
MAINTENANCE THERAPY
After ASCT, standard risk patients need lenalidomide maintenance, while bortezomib-based maintenance is needed for patients with high-risk myeloma.
MANAGEMENT OF REFRACTORY DISEASE
Most patients require a triplet regimen at relapse, with the choice of regimen varying with each successive relapse.
Topics: History, 21st Century; Humans; Multiple Myeloma; Prognosis; Risk Factors
PubMed: 32212178
DOI: 10.1002/ajh.25791 -
Insights Into Imaging Mar 2021Osteoid osteoma is a painful, benign and common bone tumor that is prevalent in young adults. The typical clinical presentation consists of pain that becomes worse at... (Review)
Review
Osteoid osteoma is a painful, benign and common bone tumor that is prevalent in young adults. The typical clinical presentation consists of pain that becomes worse at night and is relieved by nonsteroidal anti-inflammatory drugs. The most common imaging finding is a lytic lesion, known as a nidus, with variable intralesional mineralization, accompanied by bone sclerosis, cortical thickening and surrounding bone marrow edema, as well as marked enhancement with intravenous contrast injection. When the lesion is located in typical locations (intracortical bone and the diaphyses of long bones), both characteristic clinical and radiological features are diagnostic. However, osteoid osteoma is a multifaceted pathology that can have unusual presentations, such as intraarticular osteoid osteoma, epiphyseal location, lesions at the extremities and multicentric nidi, and frequently present atypical clinical and radiological manifestations. In addition, many conditions may mimic osteoid osteoma and vice versa, leading to misdiagnosis. Therefore, it is essential to understand these musculoskeletal diseases and their imaging findings to increase diagnostic accuracy, enable early treatment and prevent poor prognosis.
PubMed: 33683492
DOI: 10.1186/s13244-021-00978-8 -
Seminars in Interventional Radiology Oct 2018Percutaneous, image-guided musculoskeletal biopsy, due to its minimal invasive nature, when compared with open surgical biopsy, is a safe and effective technique which... (Review)
Review
Percutaneous, image-guided musculoskeletal biopsy, due to its minimal invasive nature, when compared with open surgical biopsy, is a safe and effective technique which is widely used in many institutions as the primary method to acquire tissue and bone samples. Indications include histopathologic and molecular assessment of a musculoskeletal lesion, exclusion of malignancy in a bone/vertebral fracture, examination of bone marrow, and infection investigation. Preprocedural workup should include both imaging (for lesion assessment and staging) and laboratory (including coagulation tests and platelet count) studies. In selected cases, antibiotic prophylaxis should be administered before the biopsy. Core needle biopsy of musculoskeletal lesions has a diagnostic accuracy that ranges from 66 to 98% with higher diagnostic yield for lytic, large-size, malignant lesions and when multiple and long specimens are obtained. Reported complication rates range between 0 and 10% and usually do not exceed 5%, with a suggested threshold of 2%. The purpose of this review article is to illustrate the technical aspects, the indications, and the methodology of percutaneous image-guided bone biopsy that will assist the interventional radiologist to perform these minimal invasive techniques.
PubMed: 30402003
DOI: 10.1055/s-0038-1669467 -
Insights Into Imaging Oct 2018Calvarial lesions are often asymptomatic and are usually discovered incidentally during computed tomography or magnetic resonance imaging of the brain. Calvarial lesions... (Review)
Review
Calvarial lesions are often asymptomatic and are usually discovered incidentally during computed tomography or magnetic resonance imaging of the brain. Calvarial lesions can be benign or malignant. Although the majority of skull lesions are benign, it is important to be familiar with their imaging characteristics and to recognise those with malignant features where more aggressive management is needed. Clinical information such as the age of the patient, as well as the patient's history is fundamental in making the correct diagnosis. In this article, we will review the imaging features of both common and uncommon calvarial lesions, as well as mimics of these lesions found in clinical practice. TEACHING POINTS: • Skull lesions are usually discovered incidentally; they can be benign or malignant. • Metastases are the most frequent cause of skull lesions. • Metastatic lesions are most commonly due to breast cancer in adults and neuroblastoma in children. • Multiple myeloma presents as the classic "punched out" lytic lesions on radiographs. • Eosinophilic granuloma is an osteolytic lesion with bevelled edges.
PubMed: 30232767
DOI: 10.1007/s13244-018-0643-0 -
American Journal of Hematology Jan 2016There have been major advances in the diagnosis, staging, risk-stratification, and management of multiple myeloma (MM). In addition to established CRAB (hypercalcemia,... (Review)
Review
There have been major advances in the diagnosis, staging, risk-stratification, and management of multiple myeloma (MM). In addition to established CRAB (hypercalcemia, renal failure, anemia, and lytic bone lesions) features, new diagnostic criteria include three new biomarkers to diagnose the disease: bone marrow clonal plasmacytosis ≥60%, serum involved/uninvolved free light chain ratio ≥100, and >1 focal lesion on magnetic resonance imaging. MM can be classified into several subtypes based on baseline cytogenetics, and prognosis varies according to underlying cytogenetic abnormalities. A Revised International Staging System has been developed which combines markers of tumor burden (albumin, beta-2 microglobulin) with markers of aggressive disease biology (high-risk cytogenetics and elevated serum lactate dehydrogenase). Although the approach to therapy remains largely the same, the treatment options at every stage of the disease have changed. Carfilzomib, pomalidomide, panobinostat, daratumumab, elotuzumab, and ixazomib have been approved for the treatment of the disease. These drugs combined with older agents such as cyclophosphamide, dexamethasone, thalidomide, bortezomib, and lenalidomide dramatically increase the repertoire of regimens available for the treatment of MM. This review provides a concise overview of recent advances in MM, including updates to diagnostic criteria, staging, risk-stratification, and management.
Topics: Antineoplastic Agents; Consolidation Chemotherapy; Disease-Free Survival; Hematopoietic Stem Cell Transplantation; Humans; Maintenance Chemotherapy; Multiple Myeloma; Neoplasm Staging; Recurrence
PubMed: 26565896
DOI: 10.1002/ajh.24236 -
Microbial Cell (Graz, Austria) Oct 2022Holins are generally believed to generate large membrane lesions that permit the passage of endolysins across the cytoplasmic membrane of prokaryotes, ultimately... (Review)
Review
Holins are generally believed to generate large membrane lesions that permit the passage of endolysins across the cytoplasmic membrane of prokaryotes, ultimately resulting in cell wall degradation and cell lysis. However, there are more and more examples known for non-lytic holin-dependent secretion of proteins by bacteria, indicating that holins somehow can transport proteins without causing large membrane lesions. Phage-derived holins can be used for a non-lytic endolysin translocation to permeabilize the cell wall for the passage of secreted proteins. In addition, clostridia, which do not possess the Tat pathway for transport of folded proteins, most likely employ non-lytic holin-mediated transport also for secretion of toxins and bacteriocins that are incompatible with the general Sec pathway. The mechanism for non-lytic holin-mediated transport is unknown, but the recent finding that the small holin TpeE mediates a non-lytic toxin secretion in opened new perspectives. TpeE contains only one short transmembrane helix that is followed by an amphipathic helix, which is reminiscent of TatA, the membrane-permeabilizing component of the Tat translocon for folded proteins. Here we review the known cases of non-lytic holin-mediated transport and then focus on the structural and functional comparison of TatA and TpeE, resulting in a mechanistic model for holin-mediated transport. This model is strongly supported by a so far not recognized naturally occurring holin-endolysin fusion protein.
PubMed: 36262927
DOI: 10.15698/mic2022.10.785