8830/3 Fibroxanthoma, malignant



Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells} and isn synonymous with undifferentiated high grade pleomorphic sarcoma.



Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells} and isn synonymous with undifferentiated high grade pleomorphic sarcoma.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic and spindle cells arranged in a storiform pattern.

According to the 2003 WHO Classification
3
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells and is synonymous with undifferentiated high grade pleomorphic sarcoma.



Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification
4
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells and is synonymous with undifferentiated high grade pleomorphic sarcoma.

Primary malignant histiocytoma of bone predominantly arises in the metaphyseal regions of the long bones of the lower extremities, particularly the femur (30-45%), followed by tibia and humerus. The tumour is osteolytic and usually destroys the cortex; soft tissue extension is common. The margins are irregular and ill defined. In addition to the characteristic mixed population of spindle, histiocytoid and pleomorphic cells, varying amounts of multinucleated osteoclast-type giant cells, foamy cells and chronic inflammatory cells can be found within the lesion. The characteristic features of MFH are very similar to those of fibrosarcoma, and distinction between the two tumours can be arbitrary.
MFH of bone is a relatively rare tumour which affects adults and shows a predilection for males. MFH is a highly malignant neoplasm; most tumours are high grade tumours. Metastasis is frequent, particularly to the lungs(45-50%). The recommended treatment is wide surgical excision; preoperative chemotherapy is indicated in patients with histologically high grade lesions. Favourable prognostic factors are younger age at manifestation (<40 years), adequate surgical margins and histological low grade
5
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



.



Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68
ICD10: C65-C68
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification
6
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells} and isn synonymous with undifferentiated high grade pleomorphic sarcoma.



Kidney
ICD-O-3 topography code: C64
ICD10: C64
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification
7
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells} and isn synonymous with undifferentiated high grade pleomorphic sarcoma.

Renal malignant fibrous histiocytomas are very rare tumours thought to arise from the renal capsule. They are large and fleshy masses with haemorrhage and necrosis. MFHs usually have pararenal and retroperitoneal extension and may extend into the renal and caval veins
8
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



.



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification
9
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells} and isn synonymous with undifferentiated high grade pleomorphic sarcoma.



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

Malignant fibrous histiocytoma (MFH) is a pleomorphic sarcoma characterized by the presence of fibrohistiocytic cells and spindle cells arranged in a storiform pattern. According to the 2003 WHO Classification
10
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



, this term is now used as a diagnosis of exclusion for sarcomas composed largely of myofibroblasts or undifferentiated mesenchymal cells} and isn synonymous with undifferentiated high grade pleomorphic sarcoma.