Resumen. COLLAZO ALVAREZ, Hiralio; TORRECILLA SILVERIO, Dewar; MORALES FLORAT, Jorge Luis y COLLAZO MARIN, Stephens Yecc. Malignant. CASO CLÍNICO. Malignant oropharyngeal fibrous histiocytoma. Resection and radial reconstruction with fasciocutaneous flap. Histiocitoma fibroso maligno de. LETTERS TO THE EDITOR. Malignant fibrous histiocytoma – a rare hepatic tumor . Histiocitoma fibroso maligno, un tumor hepático raro.
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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Undifferentiated pleomorphic sarcoma UPS is an aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis.
UPS ranks hisriocitoma 4th most common soft tissue sarcoma with a slight male preponderance. The incidence has been evaluated to be close to 0. The tumor arises most commonly during the sixth and seventh decades of life.
The most common sites of involvement include lower extremities mainly thigh followed by upper arms, retroperitoneum, viscera, head and neck in childhood.
Primary osseous UPS most commonly occurs in distal femur, proximal tibia, proximal femur and humerus. Malgno the skin, UPS presents as a relatively painless, rapidly enlarging nodule. Anorexia, malaise, fever and weight loss are present in retroperitoneal and inflammatory forms of UPS. Most UPS recur locally; distant metastases are common the most frequent is lung.
Regional metastases are rare. UPS is thought to be derived from a primitive mesenchymal cell capable of differentiating into histiocytes, fibroblasts, myofibroblasts and osteoclasts.
Histiocitoma fibroso maligno
The etiology of the tumor remains unknown. Prior radiation therapy is a likely risk factor in some cases. Any tumor mass over 5 cm is suspected to be a sarcoma. Magnetic resonance imaging MRI is the imaging method of choice for limbs and shows a high fibros on T2 weighted images. Histology of biopsy specimen prior to any treatment is crucial to reach diagnosis and shows pleomorphic spindle cell population with large atypical cells frequently exhibiting numerous irregular mitotic figures, associated regions of hemorrhage and necrosis, associated lymphohistiocytic infiltrate and invasion of dermis.
Immunohistochemical staining is negative for S, HMB, CD34 and cytokeratin which assists in ruling out other hhistiocitoma tissue tumors.
Most cases previously diagnosed as malignant fibrous histiocytoma have been reclassified into other histological types of sarcoma. When occurring in skin, UPS is difficult to differentiate from atypical fibroxanthoma or dermatofibrosarcoma protuberans see this term. Histological differential diagnoses include leiomyosarcoma, rhabdomyosarcoma, lymphoma, and melanoma see these terms.
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Immediate surgery of a mass without knowledge of its histological nature is strongly discouraged because it is associated with an increased risk of death due to inappropriate resection and increased risk of relapse.
UPS is best treated by wide surgical excision. Sometimes amputation may be necessary to remove dibroso whole lesion. Chemotherapy CHT with ifosfamide, trabectedin, dacarbazine, pazopanib have demonstrated efficacy in UPS and are registered and available in most European Union countries.
The childhood variant appears have better prognosis. Other search option s Alphabetical list. Summary and related texts. Check this box if you wish to receive a copy of your message. Disease definition Undifferentiated pleomorphic sarcoma UPS is an aggressive sarcoma of soft tissues or bone that can arise from any part of the body, clinically presenting as swelling, mass, pain, pathological fracture and occasional systemic features and is characterized by high local recurrence and significant metastasis.
Summary Epidemiology UPS ranks the 4th most common soft tissue sarcoma with a slight male preponderance. Clinical description The tumor arises most commonly during the sixth and seventh decades of life.
Etiology UPS is thought to be derived from a primitive mesenchymal cell capable of differentiating into histiocytes, fibroblasts, myofibroblasts and osteoclasts.
Histiocitoma fibroso maligno, un tumor hepático raro
Diagnostic methods Any tumor mass histipcitoma 5 cm is suspected fubroso be a sarcoma. Differential diagnosis When occurring in skin, UPS is difficult to differentiate from atypical fibroxanthoma or dermatofibrosarcoma protuberans see this term. Detailed information Article for general public English Professionals Review article Deutsch Clinical practice guidelines English Additional information Further information on this disease Ffibroso s 2 Gene s 0 Clinical signs and symptoms Other website s 7.
Health care resources for this disease Expert centres Diagnostic tests 4 Patient organisations 32 Orphan drug s Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.