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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 -
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 -
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 -
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 -
Polish Journal of Radiology 2017The sternum is an uncommon site for neoplastic involvement and metastases are far commoner than primary neoplasms. Of the primary tumours, malignant lesions are more... (Review)
Review
The sternum is an uncommon site for neoplastic involvement and metastases are far commoner than primary neoplasms. Of the primary tumours, malignant lesions are more frequent than the benign lesions. Early diagnosis and treatment is prudent in such neoplasms not only to halt disease progression but also to prevent circulatory compromise resulting from the mass effect on the mediastinum. Sound knowledge of neoplasms affecting the sternum and their imaging appearance is essential to arrive at an early diagnosis and also to obviate biopsy in cases with classical imaging findings. Neoplastic involvement of the sternum is extremely unusual and should be considered malignant unless proven otherwise. Imaging may help in arriving at the diagnosis of these lesions, together with other factors such as patient's age, type of lesion (lytic/sclerotic or mixed), matrix mineralization, multiplicity and involvement of other sites.
PubMed: 29662571
DOI: 10.12659/PJR.901226 -
Indian Journal of Orthopaedics Oct 2009Multiple myeloma is characterized by clonal proliferation of plasma cells usually of the B cell type. The skeletal manifestations are usually osteolytic lesions whose...
Multiple myeloma is characterized by clonal proliferation of plasma cells usually of the B cell type. The skeletal manifestations are usually osteolytic lesions whose differential diagnosis includes primary and secondary bone tumor. This tumor is characterized by the presence of abnormal paraprotein 8 in blood and urine. However, one to five per cent of the cases do not have any protein. Hence they are termed nonsecretory. It often poses a diagnostic dilemma when it is presented to orthopedic surgeons with no clear features of the disease. Our case report exemplifies such a diagnostic dilemma. A high index of suspicion must be borne in mind when excluding multiple myeloma as a cause of pain, pathological fracture or lytic lesion.
PubMed: 19838394
DOI: 10.4103/0019-5413.55979