Acute Promyelocytic Leukemia: an Experience on 95 Greek Patients Treated in the All-Trans-Retinoic Acid Era

Acute promyelocytic leukemia (APL) is highly curable with the combination of all-transretinoic acid (ATRA) and anthracycline based chemotherapy, but the percentage of early deaths remains high. In the present study, we report the clinical, immunophenotypic, cytogenetic and molecular characteristics and outcome of APL patients diagnosed and treated in various Hospitals of Greece and Cyprus

Distillation modeling and control

Bibliography: p. 233-237

Extrahepatic right portal vein ligation allows parenchyma-sparing en bloc resection of segments 7,8 and 4a for liver tumors engaging the right and middle hepatic veins

Right trisectionectomy for posterior liver tumors engaging the right and middle hepatic veins may lead to post-hepatectomy liver failure if the anticipated liver remnant is small. In such patients we developed a parenchyma-sparing one-step approach, that includes extrahepatic right portal vein ligation accompanied by en bloc resection only of segments 7, 8 and 4a and resection of the right and middle hepatic veins. The technique was applied in 3 patients with normal liver function, where according to the preoperative computed tomography the volume of segments 1, 2 and 3 ranged between 17% and 20% of the total liver volume. In all patients liver biochemistry improved rapidly postoperatively and a doubling of volume of segments 1,2 and 3 was achieved by the third postoperative week, as extrahepatic right portal vein ligation ameliorated reperfusion injury of the remaining segments 5 and 6 and induced hypertrophy of segments 1, 2, 3 and 4b. There was no mortality or long-term complications. Patients are alive and free of disease 74, 50 and 17 months after the operation, respectively. We propose that the term “extended upper right sectionectomy” may be considered for the en bloc resection of segments 7,8 and 4a, in future revisions of the Brisbane 2000 terminology of hepatic anatomy and resections

LARGE ADRENAL ONCOCYTOMA WITH UNCERTAIN MALIGNANT POTENTIAL: CASE REPORT AND REVIEW OF LITERATURE

Objective: To report a case of oncocytoma; a relatively rare adrenal tumor, which most commonly is detected as an adrenal incidentaloma. Methods: We present a case report, including laboratory, imaging, and pathologic findings, of a 47-year-old obese woman who had hypertension and an incidentally found large, left adrenal mass. Results: On the basis of the hormonal evaluation, this mass was a nonsecreting adrenal tumor, which histologically proved to be an oncocytoma with borderline malignant characteristics. A collective analysis of the few cases of adrenal oncocytoma published in the medical literature showed that our case corresponded to the previously published cases in preponderant location (left side) as well as the general size (11.4 cm in the largest dimension) and weight (372 g). Conclusion: Adrenal oncocytoma should be included in the differential diagnosis of adrenal incidentalomas, especially if large tumors are detected. In addition, a long-term follow-up is suggested because there are no certain clues about the true potential of this tumor. (Endocr Pract. 2010;16:641-645

A novel germline mutation of the VHL gene in a Greek family with Von Hippel–Lindau disease

Von Hippel–Lindau disease (VHL) is an autosomal dominant disorder, caused by mutations of the VHL gene showing a strong genotype–phenotype correlation. The present report concerns a 16-year-old girl with VHL (retinal, spinal cord and cerebellar haemangioblastomas and pancreatic cysts), her father (retinal and spinal cord haemangioblastomas) and the phenotypically healthy mother and younger brother and sister. DNA extraction, PCR and direct sequencing of the VHL entire coding and intronic flanking sequences, were performed according to standard procedures. In the index patient and her father a novel heterozygous germline was identified; nonsense mutation (p.145X) in exon 2 of VHL, leading to a truncated VHL protein lacking the last 66 amino acids. This is the first report of a novel VHL mutation in patients with VHL associated with haemangioblastomas and pancreatic cysts but not renal cell carcinoma