Adrenocortical carcinoma extending into the inferior vena cava in a patient with right kidney agenesis: Surgical approach and review of literature

INTRODUCTIONAdrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis and the association with tumor thrombus into the inferior vena cava (IVC) is not common. The best treatment is represented by radical surgery.PRESENTATION OF CASEWe describe a case of a large ACC of the left adrenal gland extending into the IVC through the left renal vein in a young patient with agenesis of the right kidney and signs of acute renal failure. A midline laparotomy was performed, subsequently extended by a left thoracophrenotomy through the 7th intercostal space in order to control the proximal surface of the mass and the thoracic aorta. The tumor was completely excised preserving the kidney, and thrombectomy was performed by a cavotomy with a temporary caval clamping, without cardiopulmonary by-pass (CPB).DISCUSSIONWe discuss surgical approaches reported in literature in case of ACC with intracaval extension. The tumor must be completely resected and the thrombectomy can be performed by different approaches: cavotomy with direct suture, partial resection of caval wall without reconstruction, resection of vena cava with graft reconstruction. These procedures could require a CPB, with an increased mortality. In our case we preserved the kidney and a thrombectomy without CPB was performed.CONCLUSIONIntracaval extension of ACC does not represent a contraindication to surgery. The best treatment of intracaval thrombus should be the cavotomy with direct suture. The CPB is not always required. In presence of renal agenesis, the preservation of the kidney is mandatory.     

Lysine Acetylation in Sexual Stage Malaria Parasites Is a Target for Antimalarial Small Molecules

Therapies to prevent transmission of malaria parasites to the mosquito vector are a vital part of the global malaria elimination agenda. Primaquine is currently the only drug with such activity; however, its use is limited by side effects. The development of transmission-blocking strategies requires an understanding of sexual stage malaria parasite (gametocyte) biology and the identification of new drug leads. Lysine acetylation is an important posttranslational modification involved in regulating eukaryotic gene expression and other essential processes. Interfering with this process with histone deacetylase (HDAC) inhibitors is a validated strategy for cancer and other diseases, including asexual stage malaria parasites. Here we confirm the expression of at least one HDAC protein in Plasmodium falciparum gametocytes and show that histone and nonhistone protein acetylation occurs in this life cycle stage. The activity of the canonical HDAC inhibitors trichostatin A (TSA) and suberoylanilide hydroxamic acid (SAHA; Vorinostat) and a panel of novel HDAC inhibitors on early/late-stage gametocytes and on gamete formation was examined. Several compounds displayed early/late-stage gametocytocidal activity, with TSA being the most potent (50% inhibitory concentration, 70 to 90 nM). In contrast, no inhibitory activity was observed in P. falciparum gametocyte exflagellation experiments. Gametocytocidal HDAC inhibitors caused hyperacetylation of gametocyte histones, consistent with a mode of action targeting HDAC activity. Our data identify HDAC inhibitors as being among a limited number of compounds that target both asexual and sexual stage malaria parasites, making them a potential new starting point for gametocytocidal drug leads and valuable tools for dissecting gametocyte biology.     

Dilated intracranial translucency and blake's pouch cyst: First‐trimester ultrasound markers of occipital cephalocele diagnosed using novel three‐dimensional reslicing technique

Abnormal intracranial translucency (IT) (fourth ventricle) and a Blake's pouch cyst with normal brain stem cavity may be valuable first‐trimester call signs of defects in the skull base. Here, we report a case of presumptive two‐dimensional sonographic diagnosis of occipital cephalocele that was posed at the time of 11–13 weeks aneuploidy scan. The two‐dimensional sonographic finding elicited a detailed fetal neuroscan that was performed using either multiplanar mode or a novel three‐dimensional reslicing and lightening technique. The use of three‐dimensional sonographic software and offline “navigation” within the volume of interest enabled operators to capture a diagnostic snapshot of the condition, enhancing quality imaging and early detection of the encephalic lesion. © 2013 Wiley Periodicals, Inc. J Clin Ultrasound 42 :157–161, 2014     

Quality of life in long-term survivors following treatment for Hodgkin's disease during childhood and adolescence in the German multicentre studies between 1978 and 2002

Purpose

The purpose of this study was to cross-sectionally assess quality of life (QoL) in survivors of childhood Hodgkin's disease (HD) in a cohort treated for HD in the successive German–Austrian therapy studies HD-78, HD-82, HD-85, HD-87, HD-90, HD-95, respectively, in accordance with the HD-Interval-Treatment recommendation between 1978 and 2002.

Patients and methods

Data from QoL questionnaires were provided by 1,202 (66 %) of 1,819 invited survivors. These included the EORTC QLQ-C30 and socio-demographic variables. Data of a homogenous sub-sample (n?=?725) defined by age (21–41 years) and event- free-survival (no progress, relapse or secondary malignancies) were compared to an age-adjusted German reference sample (n?=?659).

Results

While the global and physical QoL scores were comparable to those of the general population, survivors' mean scores were more than 10 points lower on the EORTC QLQ-C30 scales “Emotional” and “Social Functioning”. On the symptom scales, higher mean scores, exceeding 10 points, were obtained for the scales “Fatigue” and “Sleep”. In general, there was a gender effect showing lower functioning and higher symptom levels in women, most prominently in the group of young women (21–25 years). The results within the group of HD survivors could not be associated with the time since treatment, the age of HD survivors at diagnosis or the extent of therapy burden.

Conclusion

Clinicians engaged in follow-up care should be sensitive to aspects of fatigue and related (emotional) symptoms in HD childhood cancer survivors and encourage their patients to seek further support if needed.     

Genetic susceptibility to pancreatic cancer and its functional characterisation: The PANcreatic Disease ReseArch (PANDoRA) consortium

Pancreatic cancer is the fourth leading cause of cancer deaths in the European Union and in the USA, but little is known about its genetic susceptibility. The PANcreatic Disease ReseArch (PANDoRA) consortium was established to unite the efforts of different research groups; its aim is to create a large bio-database to uncover new genetic factors for pancreatic cancer risk, response to treatment, and patient survival. So far 2220 cases of pancreatic adenocarcinoma, a smaller number of cases of endocrine pancreatic tumours (n = 86), chronic pancreatitis (n = 272) and 3847 healthy controls have been collected. As a collective effort of the consortium, SNPs associated with pancreatic adenocarcinoma risk from a genome-wide association study performed in Caucasians were replicated. The possibility that the same genetic polymorphisms may influence patient survival as well was also addressed. This collective effort is particularly important for pancreatic cancer because it is a relatively rare disease for which little is known about aetiopathogenesis and risk factors. The recruitment of additional collaborators and partner institutions is continuously on-going.