XXXX : a case report with emphasis on clinical and morphological criteria.

@entity1 @entity1824 @entity548 @entity5 @entity146 @entity797 @entity1410 @entity6830
BACKGROUND: Malignant perivascular epitheliod cell @entity5 ( @entity1824 ) is a very rare entity composed of distinctive perivascular epitheliod cells with variable immunoreactivity for melanocytic and muscle markers. At present this @entity5 does not have a known normal cellular counterpart and the natural history is often unpredictable. Up to now, few cases of @entity1824 have been described and treatment modalities are still controversial, particularly in advanced conditions. CASE PRESENTATION: We handled the case of a 42-year-old @entity1 with unresectable @entity1824 of the abdomen. A 7 cm hepatic hypodense lesion between segment V and @entity797 of the liver and diffuse intraperitoneal nodules of 0,3-3,5 cm along the right subcapsular hepatic region, were documented by a CT scan. Radiological images showed abnormal lymph nodes of the right internal mammary chain and anterior @entity6830 . The @entity1 underwent an explorative laparotomy for uncontrolled @entity548 without a well-defined preoperative @entity5 diagnosis. At surgery, multiple lobulated nodules containing hemorrhagic fluid on the liver surface, peritoneum and omentum were confirmed. The procedure had a palliative intent and consisted of hemostasis, @entity1410 evacuation and omentectomy. The diagnosis of @entity1824 was made after surgery on the basis of morphological and immunohistochemical criteria. Radiological and intra operative findings suggest that the mass has an hepatic origin with diffuse involvement of hepatic capsule and suspensory ligaments. The @entity1 received medical support care with blood and plasma transfusions. In our experience, @entity1824 was clinically malignant, leading to a fatal outcome 25 days after hospital admission of @entity1 . CONCLUSIONS: Here we report and discuss the peculiar clinical, radiological and morphological presentation of unresectable @entity1824 . Although in the majority of the reported series, @entity1824 show a more better prognosis, our case presents with a particular @entity146 biological behaviour. The importance of a correct preoperative diagnosis, the need for more effective targeted therapies based on @entity5 molecular knowledge and evidence-based clinical studies are emphasized together with a revision of the concerning scientific literature.
