Successful treatment of retroperitoneal giant cell-type malignant fibrous histiocytoma in a 5-year-old boy

Hacettepe University, Engüri, Ankara, Turkey
The Turkish journal of pediatrics (Impact Factor: 0.43). 11/2006; 49(3):307-11.
Source: PubMed


Malignant fibrous histiocytoma, usually seen in patients older than 10 years, is an aggressive soft-tissue sarcoma occurring mostly in the extremities and the trunk, but it is extremely rare in children. We report the clinical, radiological and pathologic features of a five-year-old boy who was diagnosed as a retroperitoneally originated malignant fibrous histiocytoma. The patient with unresectable mass was successfully treated with multidisciplinary approach, with chemotherapy, surgery and radiotherapy, by using combined chemotherapy consisting of vincristine, cisplatinum, adriamycin, cyclophosphamide, actinomycin D and dacarbazine.

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    • "It can also be accompanied by an increased erythrocyte sedimentation rate, weight loss, and fever. MFH originates from undifferentiated mesenchymal cells [3] and can be a complication of radiation, chronic postoperative repair, trauma, surgical incisions or burn scars, and previously diagnosed hematopoietic disease, including Hodgkin's lymphoma, multiple melanoma, and malignant histiocytosis [2] [3]. Malignant fibrous histiocytomas are tumors with a mixed structure, containing fibroblasts and histiocyte-like cells, to varying degrees. "
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    ABSTRACT: Malignant fibrous histiocytoma is the second most common soft-tissue sarcoma in adults. After the extremities, the retroperitoneal space is the second most common site of this tumor. A 50-year-old man presented with a right retroperitoneal, thick-walled, cystic multilocular mass measuring 10 × 10 cm that was thought to be a type CE 5 hydatid cyst preoperatively. However, the postoperative histopathology did not agree with the radiological findings and instead showed a malignant fibrous histiocytoma. The computed tomography and ultrasound/Doppler ultrasound findings of this retroperitoneal mass mimicked a type CE 5 hydatid cyst. We present this case because the surgical management of these two lesions differs and misdiagnosis can be problematic.
    Full-text · Article · Sep 2011