Association between phakomatosis and neoplasia in children pathology. IV Pediatric Clinic experience

The risk to developing a neoplasm is increased when associated to a patient phakomatosis (Recklinghausen neurofibromatosis, Bourneville's tuberous sclerosis). We analysed 6 cases with phakomatosis and tumours, admitted in the Department of Oncopediatry, between 1993-1998; five of these children had neurofibromatosis and one Bourneville's disease. The associated tumours were hematologic malignancies (juvenile myeloid chronic leukemia) and solid tumors (rhabdomyosarcoma, hepatic carcinoma, CNS tumour, NHL optic glioma). The diagnosis was confirmed by microscopic examination of the bioptic material in all cases. Tumoral staging was performed by clinics, biology and imagistic investigations. All cases had extensive and aggressive tumours at the moment of diagnosis, We noticed a poor response and an early relapse after chemotherapy. A special follow-up and a different management has to be established for the patients with phakomatosis, in order to have a good oncological prophylaxis.     

Lateral intra-extraspinal cervical schwannomas: Partial versus total resection

The study involved 27 lateral cervical schwannomas surgically treated in the last 40 years utilizing either partial or total resection. The study aims to compare the results of total and partial resection of spinal lateral schwannoma and to assess the values of partial resection of large lateral cervical schwannomas. We performed a retrospective analysis of 27 patients operated for lateral cervical schwannomas in the period 1971–2010, out of which 11 had partial resection. The average follow-up of the patients was 136.4 months, comparing pre- and postoperative clinical picture, time to relapse, dynamics of yearly growth of schwannomas, and the need for re-resection. The average period of preoperative complaint in lateral cervical schwannomas was 19.6 months. Unilateral cervicobrachialgia as a manifestation of radiculopathy was observed in 85% of LCSs, and, was, as a rule the first symptom of the disease. Surgical treatment of 27 patients with LCSs resulted in recovery or improvement of complaints in 80% of cases. The treatment effect did not depend on the degree of resection. Reoperation was done, on average, 16.3 years after partial resection. The residues of lateral cervical schwannomas stopped growing 15 years after partial resection and underwent cystic degeneration. Partial resection of lateral cervical schwannomas is an excellent alternative to total resection, not only for the elderly or in cases of increased surgical risk, but also as a method of choice since it is more readily available to a larger number of surgeons and associated with low percentages of surgically relevant relapses and high percentages of improvement.     

Expression of glypican 3 in ovarian and extragonadal germ cell tumors

Germ cell tumors (GCTs), rare malignancies that occur in a wide range of locations and display variable histologic patterns, may pose diagnostic challenges. Glypican 3 (GPC3), a membrane-bound heparan sulfate proteoglycan, has been shown to be a novel diagnostic marker in testicular GCT. However, GPC3 expression in ovarian and extragonadal GCT has not been reported. We evaluated GPC3 immunoreactivity in GCTs from 63 patients (57 children and 6 adults), including 14 ovarian and 20 extragonadal primary GCTs and 8 metastases along with 21 primary testicular GCTs for comparison. All 33 yolk sac tumors (YSTs) and both choriocarcinomas were immunoreactive for GPC3. In contrast, a minority of immature (4/10) and mature (4/35) teratomas were positive. No positivity was seen in 6 embryonal carcinomas or 5 germinomas. GPC3 is differentially expressed in ovarian and extragonadal GCTs, with expression predominantly observed in YSTs and choriocarcinoma.     

Surgical treatment of gastrointestinal autonomic nerve tumors (GANT) in children-2 case reports

Gastrointestinal autonomic nerve tumors form an uncommon subcategory of stromal tumors of the intestinal tract although their histologic appearance is similar to other gastrointestinal stromal tumors. Our aim was to evaluate our experience in the diagnosis and therapy of these kinds of tumors. Two patients were admitted to the Pediatric Surgery Clinic in Niš with abdominal pain and a palpable mass in the abdomen. After excision, the tumor tissue was sampled, sent for histopathological diagnosis, and examined by light microscopy, immunohistochemistry, and electron microscopy. Postoperatively, both patients recovered without complications. The patient with the tumor bulk in the mesentery of the small bowel had no evidence of tumor progression 6 years after surgery. In the second case, a giant tumor was present along the greater curvature of gaster. Even with a tumor of this size, there were no signs of progression 10 years after surgery. Radical surgical resection of gastrointestinal autonomic nerve tumors seems to be the curative approach to date, and long-term survival is possible even with large tumors.     

High interleukin 12 and low interleukin 10 production after in vitro stimulation detected in sepsis survivors

Objective To evaluate viability of isolated peripheral blood mononuclear cells (PBMC) and production of cytokines in vitro after stimulation as prognostic factors for survival in sepsis patients.Design Prospective study of the biological response of PBMC in the onset of severe sepsis.Setting Research laboratory of molecular biology and immunology and university hospital ICU, Faculty of Medicine, Trakia University.Patients Twenty-three patients meeting the criteria for severe sepsis, and 14 control subjects.Interventions Isolated PBMC were stimulated in vitro with: C3-binding glycoprotein (C3bgp; 30 µg), lipopolysaccharide (30 µg), phytohemagglutinin (20 µg), pokeweed mitogen (30 µg), and dexamethasone (500 µg).Measurements and results We measured the levels of interleukins (IL) 6, 10, and 12 in culture supernatants. Stimulation with C3bgp and phytohemagglutinin led to significantly lower PBMC secretion of IL-6 in nonsurvivors than in survivors and healthy donors. Stimulation with C3bgp, lipopolysaccharide, and pokeweed mitogen considerably reduced IL-12 production in nonsurvivors. Stimulation with lipopolysaccharide and pokeweed mitogen caused immune cells in nonsurvivors to produce higher levels of IL-10 than in survivors. Survival of PBMC reduced viability for nonsurvivors PBMC, both spontaneously and as induced by lipopolysaccharide or pokeweed mitogen.Conclusions The viability of PBMC at the onset of sepsis and enhanced production of IL-12 and diminished production of IL-10 after stimulation with all stimuli used may be a favorable prognostic factor in sepsis.     

Novel Approach for Synthesis of Graphene-like Phases by Pulsed Laser Ablation in a Flow-Mode Suspension

The present study investigates the possibility of obtaining graphene-like phases (defected graphene, graphene oxide, and reduced graphene oxide) as fine suspensions by applying a novel pulsed laser ablation (PLA) approach in flow mode. Two types of suspensions of microcrystalline graphite in aqueous suspensions and two types of microcrystalline graphite in suspensions of 6% hydrogen peroxide solution were irradiated in a quartz tube through which they flow. The third (λ = 355 nm) and fourth harmonics (λ = 266 nm) of an Nd:YAG laser system (15 ns pulse duration and 10 Hz pulse repetition rate) were used. The morphology of the obtained particles was studied by transmission electron microscopy (TEM). Their phase composition and structure were explored by X-ray photoelectron spectroscopy, X-ray diffractometry, and Raman spectroscopy.     

Rat liver plasma membrane damage in hexachlorobenzene intoxication and its potentiation by ethanol

Chronic feeding of male Wistar rats with food containing hexachlorobenzene (HCB) at 17.5 mmol/kg induced elevation of serum amino-transferases and bilirubin content, increase of microsomal cytochrome P-450 concentration, and decrease of 5'-nucleotidase, K+,Na+- and Mg2+-adenosine triphosphatase activities in liver plasma membrane preparations. These changes were potentiated by ethanol consumption suggesting a possible role of liver plasma membrane damage in the pathogenesis of HCB intoxication.     

Intraraphedorsal kainic acid induces paroxysmal electroencephalographic changes in some brain structures of cats

In acute experiments on cats the effect of kainic acid microinjected into the raphe dorsal nucleus on the EEG activity recorded from various brain structures was investigated. Kainic acid in doses of 0.2 to 32.0 nmol was injected by glass micropipets and an air-pressure system. The analysis of the EEG and the EEG power analysis in the usual frequency bands (delta, theta, alpha, and beta) revealed an activating to hyperexcitatory effect of kainic acid. This hyperexcitatory effect was manifested by the appearance of paroxysmal EEG patterns of different types which developed in some cases into seizure episodes or an epileptic state. Abnormal EEG changes occurred first in the thalamus and mesencephalic reticular formation. A dose-response relationship existed: the duration of the EEG with pathological patterns increased whereas the latency of their appearance decreased with increase of the dose of kainic acid. Five to seven hours after the injection there were no severe histopathologic changes in the region of the raphe dorsal nucleus. The hyperexcitatory effect of kainic acid on brain excitability might be a result of the excitatory action of kainic acid and of triggering of circuits of neuronal hyperactivity in structures connected with the raphe dorsal nucleus.     

A comparative analysis of the postoperative complications of thyroid cancer surgery related to surgical approach

A retrospective study of the postoperative complications in 129 patients treated for thyroid cancer in the clinics of surgery of the Higher Medical Institute for a period of 9 years (1988-1996) was performed. The majority of the patients (90) were treated by total thyroidectomy. Subtotal thyroidectomy was performed in 21 and thyroid lobectomy in 15 patients. Postoperative complications occurred in 56 of the operated patients. Of these 5 had permanent and 51 transient complications. Most of the complications (80.35 +/- 3.56%) ensued from total thyroidectomy. The variety of the complications included permanent hypoparathyroidism in 3 patients, transient hypocalcemia in 37 patients, injury of the recurrent laryngeal nerves in 11 patients, esophageal damage in 1 patient, hematoma within the operation field in 1 patient and suppuration of the operative wound in 1 patient. Four of the patients with injury of the recurrent laryngeal nerve suffered from obstruction of the airways which required temporary tracheostoma to be performed. No lethal postoperative outcome was observed. The data of the study are discussed in relation with the literature data.