Résumé
Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The tumor appeared to arise from the prostatic region and enlarged to compress the bladder leading to bilateral hydronephrosis. Immunohistochemical studies for the resected tumor confirmed the presence of mixed ganglioneuroma and rhabdomyosarcoma establishing the diagnosis of malignant ectomesenchymoma. However, in the initial biopsy specimen of tumor, poorly differentiated round to oval cells positive for both desmin and vimentin staining were uniformly noted and the incorrect diagnosis of rhabdomyosarcoma was made. Intensive multi-agents chemotherapy, surgery and radiotherapy had failed in preventing the development of local recurrences. Subsequently, invasion of pubic bone and lung metastases ensued. This report enlightens the need for immunohistochemistry to seek possible neuroectodermal components in a tumor specimen suggestive of rhabdomyosarcoma.
Sujets)
Humains , Mâle , Douleur abdominale , Biopsie , Desmine , Diagnostic , Traitement médicamenteux , Pseudokystes mucoïdes juxta-articulaires , Ganglioneurome , Hydronéphrose , Immunohistochimie , Poumon , Métastase tumorale , Plaque neurale , Pubis , Radiothérapie , Récidive , Rhabdomyosarcome , Vessie urinaire , VimentineRésumé
We report a 10-year-old male with steroid-responsive nephrotic syndrome which was complicated by cerebral venous sinus thrombosis including superior sagittal sinus and left popliteal artery thrombosis following 5 months later. At each thrombotic episode, he suffered from relapse of massive proteinuria with hypovolemic crisis, and from hemoconcentration with dehydration due to vomiting or diarrhea. Brain MR angiography confirmed thromboses of superior sagittal sinus, straight sinus, vein of Galen and occipital sinus. Diagnosis of left popliteal artery thrombosis was made according to the clinical observations including pulseless left dorsalis pedis artery, pain and coldness of left lower leg, and reperfusion injury of calf muscle after heparinization. Each thrombotic episode was successfully managed with heparinization, oral warfarin therapy and fresh frozen plasma for the antithrombin III replacement.