Disease Information for Histiocytoma, malignant fibrous

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Clinical Manifestations
Signs & Symptoms
Lymphadenopathy
Splenomegaly
Typical Clinical Presentation
Presentation/Fractures Pathologic Minimal Trauma
Disease Progression
Course/Chronic disorder
Course/Chronic only
Diagnostic Test Results
Pathology
BX/Bone biopsy/abnormal
X-RAY
Xray/Epiphysis Lesions/Site
Associated Diseases & Rule outs
Associated Disease & Complications
Cold autoimmune hemolytic anemia
Histiocytoma, malignant fibrous
Disease Mechanism & Classification
Class
CLASS/Soft Tissue Involvement
CLASS/Bone disorder (ex)
CLASS/Skeletal (category)
Pathophysiology
Pathophysiology/Histiocyte/macrophage disorder (ex)
Process
PROCESS/Neoplastic (category)
PROCESS/Sarcoma/site Lymph node mets/predilection
PROCESS/Sarcoma/site Retroperitoneum/mesentery predilection
Synonyms
Synonym
Fibrous histiocytoma malignant, Fibrous histiocytoma malignant (morphologic abnormality), Fibrous histiocytomas malignant, Fibroxanthoma malignant, Fibroxanthosarcoma, Fibroxanthosarcoma (morphologic abnormality), HISTIOCYTOMA MALIGNANT FIBROUS, Histiocytomas Malignant Fibrous, Malignant Fibrous Histiocytoma, Malignant Fibrous Histiocytoma of Soft Tissue, Malignant Fibrous Histiocytoma of the Soft Tissue, Malignant fibrous histiocytoma stage unspecified, Malignant Fibrous Histiocytomas, Malignant fibroxanthoma, MFH, Synonym/Fibroxanthosarcoma, Synonym/Malignant histiocytoma/fibrous/bone
Definition

Primary malignant bone tumors are rare; Four types make up the majority; Malignant fibrous histiocytoma (MFH) - a pleomorphic sarcoma of storiform pattern without differentiation; Osteosarcoma - similar to malignant fibrous histiocytoma with differentiation to osteoid production; Osteosarcoma - cellular cartilaginous lesion with abundant binucleate cells, myxoid areas, and pushing borders; Ewing sarcoma - small, blue-round cell neoplasm;

Genetics: Ewing sarcoma has 11/22 chromosomal translocation and EW5-FLI-1 fusion protein; Osteosarcomas shows loss of retinoblastoma and p53 suppressor genes and amplification of the genes C-myc, mdm-2, SAS, and cyclin-dependent kinase;Prevalence in USA: Rare: 5000 bone and soft tissue sarcomas per year, a practicing orthopedic surgeon may see one primary malignant tumor of bone in every five years of practice; Ewing sarcoma is less common in blacks; Age:MFH - teens and elderly; Osteogenic sarcoma - teens and early twenties; Chondrosarcoma - very young and very old; Ewing sarcoma - children, teens, and early twenties ; Male = Female; Pain with weight bearing, at rest and at night; Swelling; Bone Tenderness ; Fracture with minor trauma; Minor injury may bring attention to lesion; MFH often follows irradiation or arises in old bone infarct;

Osteosarcoma has association with loss of suppressor retinoblastoma and p53 genes; Chondrosarcoma may arise in pre-existing enchondroma or exostosis; Multiple enchondromatosis (Ollier"s disease) - chondrosarcoma; Multiple hereditary exostosis - chondrosarcoma; Previous irradiation, risk factor for MFH; Previous history of bilateral retinoblastoma - osteosarcoma; Pagets disease osteosarcoma; DDX: Solitary metastatic lesion or myeloma especially in the patient over age 40; Lymphoma at any age; Benign bone tumors and benign bone tumors that look aggressive (aneurysmal bone cyst, giant cell tumor, eosinophilic granuloma); Infection (osteomyelitis); Metabolic bone disease (osteopenia, Pagets,

hyperparathyroidism; Synovial diseases (pigmented villonodular synovitis; [Griffith clincal advisor 2005]

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External Links Related to Histiocytoma, malignant fibrous
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NEJM (The New England Journal of Medicine)
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