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Hepatic Ewing's Sarcoma - Case Report
Apresentação do caso
Female patient, 39 years old, complaining of abdominal pain, presenting on magnetic resonance imaging of the abdomen an expansive hepatic formation infiltrating the inferior vena cava. ewing sarcoma/PNET in the liver, investigation of other sites of injury was performed, but no bone lesions or lesions in other organs were found, thus being defined as primary extraosseous Ewing's sarcoma of the liver.
Primitive neuroectodermal tumor (PNET) is a malignant neoplasm that commonly involves the central and skeletal nervous system and is composed of small, round and uniform cells. Because of the undifferentiated appearance of the tumor cells, it appears that the original cell may be an undifferentiated mesenchymal cell. These sarcomas predominantly affect bones and deep soft tissue and rarely affect visceral organs. PNET can be found in the chest (44%), abdomen and pelvis (44%), extremities (20%) and 6% in the head and neck areas. Primary visceral involvement has been described with increasing frequency in the pancreas, vagina, stomach, small intestine, ovaries, esophagus, and kidneys. Patients with localized disease have estimated 5-year overall survival rates of about 70% due to considerable progress in local and systemic therapy over the past 4 decades. Treatment with surgery alone for extraosseous PNET is considered insufficient, and multimodal treatment with chemotherapy and radiotherapy is often performed. Despite the multimodal treatment, most patients have rapid spread of the disease.
PNETs are small round cell tumors that belong to the Ewing's sarcoma family of tumors. These tumors are divided into two main categories: central (derived from the neural tube) and peripheral (outside the central nervous system). Although it is an uncommon liver tumor associated with a poor prognosis, PNET should be considered in the differential diagnosis of liver masses.
Primitive neuroectodermal tumor; Liver; chemotherapy; Ewing's sarcoma.
Fígado / Pâncreas / Vias Biliares
RHUANA SILVA MEDRADO, BERNARDO MOREIRA OLIVEIRA