RESUMO
We report the case of a 19 month old boy referred to our institution because of a pelvic tumor initially identified as an embryonal rhabdomyosarcoma and treated with surgery and chemotherapy. Eight years after the initial surgery, a local tumor recurrence with bone metastasis was found. Histological examination and immunohistochemistry showed a double differentiation with both muscular and neuronal cells. This double differentiation was retrospectively found in the initial tumor, then allowing the diagnosis of malignant ectomesenchymoma also called gangliorhabdomyosarcoma. This rare tumor, occurring mainly during childhood, is composed of neuroblasts and / or ganglion cells and of malignant mesenchymal cells (usually rhabdomyosarcomatous cells).
Assuntos
Mesenquimoma/patologia , Neoplasias Pélvicas/patologia , Rabdomiossarcoma/patologia , Neoplasias Ósseas/secundário , Diferenciação Celular , Diagnóstico Diferencial , Evolução Fatal , Neoplasias Femorais/secundário , Gânglios/patologia , Humanos , Imuno-Histoquímica , Lactente , Masculino , Mesenquimoma/tratamento farmacológico , Mesenquimoma/cirurgia , Recidiva Local de Neoplasia , Neurônios/patologia , Neoplasias Pélvicas/tratamento farmacológico , Neoplasias Pélvicas/cirurgia , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/cirurgia , Rabdomiossarcoma EmbrionárioRESUMO
Inflammatory pseudotumors (IPT) of the liver are rare benign tumors of unknown origin. Most previously reported cases were initially misdiagnosed and treated surgically; thus, their course under medical treatment remains unclear. We report an additional case of hepatic IPT with a favorable outcome under medical treatment without surgical resection. New imaging and pathologic features of this disease are presented that may facilitate preoperative recognition and appropriate treatment.
