Malignant PEComa: a case report with emphasis on clinical and morphological criteria

<p>Abstract</p> <p>Background</p> <p>Malignant perivascular epitheliod cell tumor (PEComa) is a very rare entity composed of distinctive perivascular epitheliod cells with variable immunoreactivity for melanocytic and muscle markers. At present this neoplasm does not have a known normal cellular counterpart and the natural history is often unpredictable. Up to now, few cases of PEComa have been described and treatment modalities are still controversial, particularly in advanced conditions.</p> <p>Case presentation</p> <p>We handled the case of a 42-year-old man with unresectable PEComa of the abdomen. A 7 cm hepatic hypodense lesion between segment V and VIII 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 mediastinum. The patient underwent an explorative laparotomy for uncontrolled intrabdominal hemorrhage without a well-defined preoperative tumor 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, hematomas evacuation and omentectomy. The diagnosis of PEComa was made after surgery on the basis of morphological and immunohystochemical criteria. Radiological and intra operative findings suggest that the mass has an hepatic origin with diffuse involvement of hepatic capsule and suspensory ligaments. The patient received medical support care with blood and plasma transfusions. In our experience, PEComa was clinically malignant, leading to a fatal outcome 25 days after hospital admission of patient.</p> <p>Conclusions</p> <p>Here we report and discuss the peculiar clinical, radiological and morphological presentation of unresectable PEComa. Although in the majority of the reported series, PEComas show a more better prognosis, our case presents with a particular aggressive biological behaviour. The importance of a correct preoperative diagnosis, the need for more effective targeted therapies based on tumor molecular knowledge and evidence-based clinical studies are emphasized together with a revision of the concerning scientific literature.</p

The GAPS Programme with HARPS-N at TNG: XII. Characterization of the planetary system around HD 108874

In order to understand the observed physical and orbital diversity of extrasolar planetary systems, a full investigation of these objects and of their host stars is necessary. Within this field, one of the main purposes of the GAPS observing project with HARPS-N at TNG is to provide a more detailed characterization of already known systems. In this framework we monitored the star, hosting two giant planets, HD 108874, with HARPS-N for three years in order to refine the orbits, to improve the dynamical study and to search for additional low-mass planets in close orbits. We subtracted the radial velocity (RV) signal due to the known outer planets, finding a clear modulation of 40.2 d period. We analysed the correlation between RV residuals and the activity indicators and modelled the magnetic activity with a dedicated code. Our analysis suggests that the 40.2 d periodicity is a signature of the rotation period of the star. A refined orbital solution is provided, revealing that the system is close to a mean motion resonance of about 9:2, in a stable configuration over 1 Gyr. Stable orbits for low-mass planets are limited to regions very close to the star or far from it. Our data exclude super-Earths with Msini 73 5M 95 within 0.4 AU and objects with Msini 73 2M 95 with orbital periods of a few days. Finally we put constraints on the habitable zone of the system, assuming the presence of an exomoon orbiting the inner giant planet. Based on observations made with the Italian Telescopio Nazionale Galileo (TNG) operated on the island of La Palma by the Fundaci\uf3n Galileo Galilei of the INAF at the Spanish Observatorio del Roque de los Muchachos of the IAC in the frame of the programme Global Architecture of Planetary Systems (GAPS).Table A.1 is also available at the CDS via anonymous ftp to http://cdsarc.u-strasbg.fr (http://130.79.128.5) or via http://cdsarc.u-strasbg.fr/viz-bin/qcat?J/A+A/599/A9

VizieR Online Data Catalog: Qatar-1 differential light curve (Covino+, 2013)

R-band light curves of Qatar-1b transits obtained with the Asiago 1.82m and Calar Alto 1.23m telescopes.(1 data file)