RESUMEN
An inflammatory myofibroblastic tumor (IMT) is a rare benign hepatic neoplasm that is characterized by a mass of localized proliferations of fibroblasts and infiltrations of mononuclear inflammatory cells. An IMT can occur in a variety of locations, including the lung, orbit, parotid, pleura and stomach. However, they have rarely been encountered in the liver. An IMT of the liver most often presents in young adults. The clinical presentations of a hepatic IMT vary: asymtomatic, fever, abdominal pain, palpable mass, vomiting, obstructive jaundice, anemia and hepatosplenomegaly. Their radiographic appearance and clinical presentation make them extremely difficult to distinguish from a malignant neoplasm, preoperatively. Therefore, surgical management is used when a malignancy can not be excluded preoperatively or when the IMT produces a biliary obstruction. We encountered a 31-year-old male patient who is still alive for 38 months following a resection for symtomatic IMT.
Asunto(s)
Adulto , Humanos , Masculino , Adulto Joven , Dolor Abdominal , Anemia , Fiebre , Fibroblastos , Granuloma de Células Plasmáticas , Ictericia Obstructiva , Neoplasias Hepáticas , Hígado , Pulmón , Miofibroblastos , Órbita , Pleura , Estómago , VómitosRESUMEN
An undifferentiated (embryonal) sarcoma of the liver (USL) is a rare and highly malignant hepatic neoplasm of mesenchymal origin. This tumor almost exclusively affects pediatric patients, with a poor prognosis. The highest incidence is noted in pediatric patients, usually from 5~10 years of age, but only 43 cases of USL have been reported in adults worldwide since it was first as a clinicopathological entity. With a USL in adults, most patients are known to have died within 1 year of diagnosis. The absence of specific symptoms, rapid tumor growth, normality of common tumor markers and the consequential delay in diagnosis often result in significant enlargement of the tumor, with a poor prognosis. Various therapeutic modalities have been attempted in adult patients, but only a few long-term survivors have been reported. Herein, our experience of a 42-year-old female patient who is still alive, 55 months after the first resection for a USL is reported.
Asunto(s)
Adulto , Femenino , Humanos , Diagnóstico , Incidencia , Neoplasias Hepáticas , Hígado , Pronóstico , Sarcoma , Sobrevivientes , Biomarcadores de TumorRESUMEN
No abstract available.