Expression of histone H3 cell cycle-related gene, vimentin and MYC genes in pediatric brain tumors. A preliminary analysis showing the different malignant cell growth potential.

Eleven pediatric brain tumors were studied for the histone H3, Vimentin and MYC gene expression. H3, an S phase cell cycle-related gene (ccr), was found prevalently expressed in tumors with a high mitotic index (MI). Vimentin gene, which contributes to maintaining the cell structure but is also demonstrated to be an early responder gene to growth stimulation was found variously expressed. The different expression of Vimentin gene in the examined samples suggests the active proliferation of the tumor cells. Analysis of MYC gene expression was found increased only in a mesenchymal chondrosarcoma while in other samples MYC mRNA was undetectable. Medulloblastoma, chondrosarcoma, and choroid plexus carcinoma have high S phase H3 gene expression associated with a high MI. Differently an astrocytoma shows a low MI associated with high H3 gene expression. This first preliminary report of H3, Vimentin and MYC gene expression in brain tumors demonstrates that malignant cells are characterized by a different gene expression and different growth potentials.

Expression of multiple drug resistance gene, MDR1, and N-myc oncogene in an Italian population of human neuroblastoma patients.

Thirty-four patients of an Italian population affected by neuroblastoma (NB) were evaluated at diagnosis for multidrug resistance gene (MDR1) and N-myc oncogene amplification. No patients showed MDR1 amplification, while extra copies of the N-myc gene were found in 9 out of 34 patients (26%). N-myc amplification was correlated (p = 0.008) with a shorter progression-free survival. RNA was purified from fresh tumor biopsies and analysed in 29 NB samples. MDR1 gene expression was found to be increased in 5 out of 29 tumor samples at onset (17%) and in 1 out of 3 at relapse, but none of them expressed both MDR1 and N-myc genes simultaneously. No correlation was found between MDR1 or N-myc genes expression and tumor progression. MDR1 mRNA transcription may occur spontaneously after onset, suggesting that certain NB tumors could be resistant to antineoplastic drugs at onset. All 5 patients showing MDR1 mRNA transcription achieved complete or partial clinical remission after polychemotherapy. This was presumably due to inclusion in the therapeutic protocol of a high dose of Cisplatin, a drug not susceptible to the effects of the MDR1 gene product. Our findings show that cells which actively transcribe for the MDR1 gene are present in several untreated NB patients. No gene amplification was detected and probably the MDR1 gene expression is regulated at the transcriptional level.

[Mesentericoportal Rex-shunt as a treatment for extrahepatic portal vein thrombosis]. / Mesentericoportaler Rex-Shunt als Therapiekonzept bei Pfortaderthrombose des Kindes.

The most common cause of portal hypertension in children with healthy livers is the prehepatic block. A 7-year-old girl had presented with portal vein thrombosis after umbilical vein catheterization in the newborn period. She suffered from collateral circulation with recurrent bleeding episodes due to esophageal varices (stage III-IV) and developed hypersplenism. Ultrasound demonstrated an open branch of the left portal vein. Direct splenoportography showed an open and communicating superior mesenteric vein. Liver biopsy was normal. An autologous left jugular vein graft was used to create a bypass from the superior mesenteric vein to the umbilical portion of the left intrahepatic portal vein (mesentericoportal Rex-shunt). Postoperatively, normal intrahepatic portal vein flow was demonstrated by ultrasound. After 2 years of follow-up, the patient is asymptomatic with no signs of portal hypertension. In contrast to classic portosystemic shunt operations, this bypass restores physiological portal vein flow, thus avoiding the possible consequences of longterm portosystemic shunting and low-grade encephalopathy.

[Sporadic unilateral adrenal medullary hyperplasia--a rare cause of hypertension]. / Die sporadische unilaterale adrenomedulläre Hyperplasie--eine seltene Ursache des Bluthochdrucks.

Sporadic adrenomedullary hyperplasia (AMH) is characterized by a clinical history of hypertension, increased plasma and/or urinary catecholamine levels and histomorphometric evidence of increased adrenal medullary mass in the absence of MEN 2 syndrome. The case of a 42-year-old female patient is reported who presented with typical clinical and laboratory findings of episodic hypertension and elevated plasma and urinary catecholamines. Sonography and computed tomography revealed no abnormality, but 131I-metaiodobenzylguanidine (131I-MIBG) scintigraphy showed increased uptake in the right adrenal. Transabdominal unilateral adrenalectomy was performed. The right adrenal gland was macroscopically inconspicuous. Upon histomorphometry, however, an increased adrenal medullary cell mass was shown, thus confirming AMH. Two years following surgery the patient is asymptomatic and normotensive.

[Pancreatico-bronchial fistula with communication to a pseudoaneurysm of the arteria lienalis as a rare complication in chronic pancreatitis]. / Pankreatikobronchiale Fistel mit Anschluss an ein Pseudoaneurysma der Arteria lienalis als seltene Komplikationen einer chronischen Pankreatitis.

Thoracic manifestations of internal pancreatic fistulas caused by chronic pancreatitis are rare conditions. The three main types of these manifestations are mediastinal pseudocysts, pancreatico pleural fistulas and pancreaticobronchial fistulas. We report on one patient with the clinical presentation of all three thoracic internal pancreatic fistulas with a communication to a pseudoaneurysm of the splenic artery caused by chronic alcohol-related pancreatitis. Conservative therapy over four weeks was not successful. Resection of the pseudoaneurysm, debridement of the mediastinal pseudocyst and duodenum preserving resection of the pancreas treated all complications and prevents recurrence in this patient with chronic pancreatitis.

[Gastric substitute following gastrectomy]. / Magenersatz nach Gastrektomie.

Gastric substitute following gastrectomy. Following gastrectomy a variety of different reconstructive alternatives with different advantages and disadvantages have been proposed. Direct termino-lateral esophagojejunostomy with preservation of the duodenal passage or roux-en-Y jejuno-jejunostomy may be considered as current standard route of reconstruction. With current knowledge formation of a jejunal reservoir with a pouch length of 15 cm seems to be advantageous over simple roux-en-Y reconstruction. Preservation of the duodenal passage offers objective biochemical advantages, while its clinical implications have yet to be confirmed. True long term results of randomised studies are still pending. Thus, the technically easier pouch formation using the roux-en-Y reconstruction may currently be favored. Jejunoplicatio as an antirefluxive component of reconstruction is optional. Its value may well lie in the securement of a critical esophagojejunal anastomosis.

N-myc oncogene amplification in a pediatric case of glioblastoma multiforme.

A sample of a primary brain tumor of glial cell origin was surgically removed from a 7-year-old girl. The histopathological analysis showed a heterogeneous tumor containing highly cellular areas composed of small, poorly differentiated cells with frequent mitoses suggestive of a glioblastoma multiforme. There were also areas presenting as features of lower-grade astrocytoma. Strong immunohistochemical staining for glial fibrillar acidic protein was demonstrated, while vimentin, neurofilament, and S-100 protein were all positive in just the astrocytic part of the tumor. The DNA extracted from a fresh tumor sample at diagnosis was processed by Southern blot analysis and hybridized with a 2.0 kb N-myc oncogene probe recognizing the first intron and the second exon of the human gene. A 20-fold amplification of the oncogene was found. The possible role of such a molecular alteration is discussed in light of the clinical presentation and histopathological features.