[Lipoid proteinosis (Urbach-Wiethe's Disease)]. / Lipoid proteinozis (Urbach-Wiethe Hastaligi).

A 25-year-old female patient was admitted to our outpatient clinic with postpartum hoarseness. Punch biopsy specimens obtained from the larynx and sublingual region revealed multi-folded squamous epithelium with a hyperkeratosis pattern and amorphous hyaline material aggregation. This aggregation was also remarkable around the vessels. Histochemical examination showed periodic acid-schiff-positive staining of the basal membrane with negative staining with Congo red. Physical examination revealed papule-like itchy lesions in the whole body and hoarseness. Ophthalmological examination showed bilateral blepharitis. Tooth loss and yellowish papule formation in the oral mucosa were remarkable. The patient was diagnosed with lipoid proteinosis characterized by typical vocal cord involvement and conventional histopathological and clinical findings.

Prognostic Significance of Two Dimensional AgNOR Evaluation in Local Advanced Rectal Cancer Treated with Chemoradiotherapy.

The prognostic significance of AgNOR proteins in stage II-III rectal cancers treated with chemoradiotherapy was evaluated. Silver staining was applied to the 3µm sections of parafin blocked tissues from 30 rectal cancer patients who received 5-FU based chemoradiotherapy from May 2003 to June 2006. The microscopic displays of the cells were transferred into the computer via a video camera. AgNOR area (nucleolus organizer region area) and nucleus area values were determined as a nucleolus organizer regions area/total nucleus area (NORa/ TNa). The mean NORa/TNa value was found to be 9.02±3.68. The overall survival and disease free survival in the high NORa/TNa (>9.02) patients were 52.2 months and 39.4 months respectively, as compared to 100.7 months and 98.4 months in the low NORa/TNa (<9.02) cases. (p<0.001 and p<0.001 respectively). In addition, the prognosis in the high NORa/TNa patients was worse than low NORa/TNa patients (p<0.05). In terms of overall survival and disease-free survival, a statistically significant negative correlation was found with the value of NORa/TNa in the correlations tests. Cox regression analyses demostrated that overall survival and disease-free survival were associated with lymph node status (negative or positive) and the NORa/TNa value. We suggest that two-dimensional AgNOR evaluation may be a safe and usable parameter for prognosis and an indicator of cell proliferation instead of AgNOR dots.

Prognostic value of NF-κB, CD9, and VEGF in gastrointestinal stromal tumors.

AIM OF THE STUDY: Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the gastrointestinal system. We aimed to determine whether nuclear transcription factor κB (NF-κB), CD9 and vascular endothelial growth factor (VEGF) have prognostic value in patients with GIST. MATERIAL AND METHODS: Thirty-five patients with GIST, who were diagnosed in the Pathology Department of Erciyes University, were included in the study. Cases were classified based on the 2002 NIH consensus. CD9, VEGF, and NF-κB immunohistochemistry were applied to GIST cases positive for CD117 and CD34, which are used to evaluate GISTs immunohistochemically. RESULTS: Although there are no statistically significant differences between NF-κB (p = 0.329), CD9 (p = 0.269), and VEGF (p = 0.372) and risk groups, 79.22% of cases that stained positive for NF-κB, 81% of cases that stained positive for CD9, and 80% of cases that stained positive of VEGF were in the high risk group. CONCLUSIONS: It was found that NF-κB, CD9, and VEGF, which are important in predicting behaviors of other malign tumors, were expressed at high rates in high risk group GISTs. This can be used to determine prognosis with tumor diameter, mitosis rate under 50 BBS, Ki-67 proliferation index and other parameters.

A huge solitary fibrous tumor localized in the pancreas: a young women.

CONTEXT: Solitary fibrous tumor is an uncommon spindle cell tumor which were first described in 1931 at pleura; it should be seen rarely in extra-pleural localization. CASE REPORT: We report the ninth case of pancreatic solitary fibrous tumor in a 24-year-old woman who presented with mild epigastric pain radiating to the back and chronic constipation. Imaging studies confirmed a solitary mass in the epigastric region that begins from posterior of stomach, fills little curvature and extends to pelvis, invades vascular structures by encircling them and extends to retroperitoneal regions that was considered as it may have mesenchymal origin. The patient underwent an enucleation of the mass which was diagnosed as solitary fibrous tumor, supported by immunohistochemical studies showing positivity for CD34, vimentin and SMA. CONCLUSION: There is limited data regarding biological behavior of solitary fibrous tumors with extra-pleural localization, because they are rare tumors. They are generally asymptomatic and slow growing tumors and it is difficult to distinguish them from other mesenchymal tumors. These issues as well as the prior nine cases are discussed.

Is Every Patient Followed up as a Papillary Thyroid Cancer Patient Really That?

UNLABELLED: We report the case of a 64-year-old man followed up for two years as suffering from differentiated thyroid cancer (DTC). In the patient's follow up, despite thyroglobulin level and I-131 whole body scan results being normal, metastases were identified at the 4th thoracic vertebra corpus by MR. Histopathological findings were carcinoma metastases. F-18 FDG PET/CT showed increased metabolic activity in the right renal mass, bilaterally in the surrenal gland, multiple lymph nodes in the thoracic and abdominal para-aortic region and in multiple vertebral and pelvic bones. An excisional biopsy of the right renal mass was reported as renal cell carcinoma. Immunohistochemical staining performed retrospectively to the first thyroid preparation showed renal cell carcinoma metastases. Consequently, any patient who presents with a thyroid nodule can also be considered as possibly suffering from metastatic disease. F-18 FDG PET/CT can provide valuable information in finding the primary focus and metastases. CONFLICT OF INTEREST: None declared.

Cyclooxygenase-2 expression in gastrointestinal stromal tumours.

BACKGROUND: While it is well known that cyclooxygenase-2 (COX-2) expression is increased in colorectal adenoma and carcinoma, there is only limited information on its status in stromal tumours. METHODS: Immunohistochemical COX-2 staining was performed on a total of 42 confirmed gastrointestinal stromal tumours (GISTs) in the Pathology Department of Gaziantep University and the findings were compared with various other parameters. RESULTS: We found a statistically significant correlation between the tumor mitosis and COX-2 expression in GISTs. However, there was no relationship between COX-2 expression and death rate, presence of metastasis, tumour size, risk staging, usage of tyrosine kinase inhibitors, and complete resection rate. CONCLUSIONS: In the light of these findings, the usage of COX-2 inhibitors with or without tyrosine kinase inhibitors in GIST patients may be helpful in the adjuvant setting to prevent or delay recurrence. Moreover, we need more studies to define the status of COX-2 inhibitors in GISTs.

Prognostic significance of CD9 expression in locally advanced gastric cancer treated with surgery and adjuvant chemoradiotherapy.

The tetraspanin transmembrane protein CD9 plays an important role in inhibiting cell motility in numerous neoplastic cell lines, including lung, gastric, pancreatic, and bladder carcinomas. The prognostic importance of CD9 in the survival of gastric carcinoma patients has not been examined to date, and in the present study, we attempted to define its prognostic value. The study included 49 (35 men and 14 women) patients with locally advanced (stages II-IV) gastric cancer. The median age was 55 years (range, 22-73 years). Surgery was the initial treatment for all patients, followed by adjuvant chemoradiotherapy. Tissue sections were evaluated immunohistochemically with a monoclonal anti-CD9 antibody. Of the 49 patients with gastric adenocarcinoma, 11 (22.4%) were CD9-positive, and 38 (77.6%) were CD9-negative. A significant prognostic value in disease-free survival and overall survival was observed in T classification and CD9 positivity. In conclusion, CD9 expression in gastric cancer appears to be associated with poor prognosis.

Which urine marker test provides more diagnostic value in conjunction with standard cytology- ImmunoCyt/uCyt+ or Cytokeratin 20 expression.

BACKGROUND: Because of the poor sensitivity of urinary cytological findings for the diagnosis of especially low grade urinary bladder carcinoma, new molecular diagnostic methods have been proposed. We decided to verify the ImmunoCyt/uCyt+ (UCyt+) test and cytology combination and cytokeratin 20 (CK20) and cytology combination in urine as possible diagnostic and monitoring tool for bladder cancer. METHODS: Evaluation of CK20 expression and UCyt+ was performed in urine of 90 patients of which 54 with bladder cancer with primary/recurrent diagnosis (low grade urothelial carcinoma (LGUC) = 23/8 patients, high grade urothelial carcinoma (HGUC) = 18/5 patients), and 36 patients as control; except of neoplastic bladder disease patients. For the evaluation of the three tests, CK20 and UCyt+ tests were combined with urine cytology and compared with each other. RESULTS: The overall sensitivity detected for each tumor marker was as follows: for urine cytology was 75.9% and UCyt+ was 83.3%, for CK20 70.4%, while the specificity was 66.7% for urine cytology and 86.1% for UCyt+ and 83.3% for CK20. The sensitivity of cytology and UCyt+ combination was higher (88.9%) than the sensitivity cytology and CK20 combination (77.8%). The simultaneous use of the three markers, sensitivity was reaching 92.5%. CONCLUSION: The UCyt+ test and CK20 expression are valid tools for the performance of adjunctive analyses with conventional cytologic examination.

Radiological findings of isolated hepatic tuberculosis in a child with tubulointerstitial nephritis-uveitis syndrome.

Isolated hepatic tuberculosis is a rare manifestation of tuberculosis in children although its incidence has been increasing, especially in immunosuppressed patients. Imaging studies frequently present a diagnostic challenge, especially for the nodular form. Thus a high index of suspicion is required for diagnosis. On US, hepatic tuberculosis may rarely present as a macronodular form (also called pseudotumour or tuberculoma). We report a 15-year-old girl with multiple hepatic macronodular masses due to isolated hepatic tuberculosis.

Different seizure types and skin lesions in oculocerebrorenal syndrome of Lowe.

Oculocerebrorenal syndrome of Lowe is an X-linked recessive disorder localized to Xq24-26.1. The phenotypic features of this disorder are Fanconi-type renal failure, mental retardation, and various eye abnormalities. Seizures may accompany the disease, and the skin-related findings are poorly defined. This case of a 9-year-old patient, diagnosed as having and followed for oculocerebrorenal syndrome of Lowe, has been presented for his seizures, which were initially myoclonic but subsequently atonic, and for his skin findings, understood to be trichoepithelioma, cystic in nature, and stemming from mature hair follicles. In monitoring the disease, the manifestation of the seizures as atonic seizures accompanied by focally initiated secondary generalized epileptic discharges is a finding previously undefined in oculocerebrorenal syndrome of Lowe. Moreover, the presence of dermal findings of a cystic nature is reported in few cases of this syndrome. In this rare syndrome, it is necessary to be aware of the presence of atonic seizures, which have an association with the progression of the disease that has not been previously reported in the literature, and of the cystic dermal lesions as part of the syndrome.

Effect of interleukin-10 on tissue damage caused by organophosphate poisoning.

Organophosphate poisoning is a common cause of severe morbidity and mortality among patients admitted to emergency departments. Tissue damages as a consequence of organophosphate poisoning are frequently reported, but preventing this potentially severe complication has not been the subject of much research. We tested whether interleukin-10, a cytoprotective agent, could prevent or diminish pathological signs of tissue damages caused by organophosphate poisoning. Thirty rats were divided into three equal groups (n = 10). Group 1 (sham) did not receive any agent during the experiment. Group 2 (control) received 0.8 g/kg of fenthion intraperitoneally, followed by 6 ml/kg of intraperitoneal normal saline 30 min and 3 hr later. Group 3 (treatment) received 0.8 g/kg of fenthion intraperitoneally, followed by 2 microg/kg of interleukin-10 intraperitoneally 30 min and 3 hr later. All rats were killed under anaesthesia after 6 hr and tissue samples were obtained from liver, kidneys and lungs. Even organophosphate poisonings do not cause significant clinical problems; several degrees of damages could be observed in liver, kidneys and lungs. These damages could be reduced by interleukin-10 treatment.

Inferior vena cava obstruction due to cecum adenocarcinoma.

In this paper, we report the radiologic findings of cecal adenocarcinoma with caval invasion in a 60-year-old man. Caval invasion was confirmed by ultrasonographically guided fine-needle aspiration biopsy and by surgery. To our knowledge, this is the first case report demonstrating malignant caval thrombus due to colon cancer. In addition, we suggest that fine-needle aspiration biopsy may be helpful for prompt diagnosis and treatment in such a case.

Hepatic von Meyenburg complex simulating biliary cystadenocarcinoma.

Multiple bile duct hamartomas [von Meyenburg complex (VMC)] are benign liver malformations that include biliary cystic lesions, with congenital hepatic fibrosis being the cause of ductal plate malformations. This rare entity is usually isolated and detected incidentally in 0.6-5.6% of reported autopsy series. Its clinical significance is that it may be misdiagnosed as malignant liver disease. Imaging findings of VMC are nonspecific. We present a rare case of VMC in a patient who presented with epigastric and upper right abdominal pain, and jaundice of 2 months' duration. Ultrasonography, computed tomography, and magnetic resonance imaging showed a periportal multicystic mass simulating biliary cystadenoma/cystadenocarcinoma. The patient underwent laparotomy. A diagnosis of VMC was made after histopathologic examination of the wedge biopsy of the liver.

MR cholangiopancreatography findings of heterotopic pancreatic tissue in the distal common bile duct.

Heterotopic pancreas is defined as the presence of pancreatic tissue at sites other than the pancreas. Involved sites may be the stomach, duodenum, proximal jejunum, ileum, congenital duodenal web, Meckel's diverticulum, ampulla of Vater, and the main pancreatic duct. We report the magnetic resonance cholangiopancreatography findings of a patient who had biliary obstruction due to heterotopic pancreas tissue at the distal common bile duct.

Acute lymphoblastic leukemia associated with brucellosis in two patients with fever and pancytopenia.

Brucellosis is a disease involving the lymphoproliferative system, which may lead to changes in the hematological parameters; however, pancytopenia is a rare finding. However, malignant diseases in association with brucellosis are rarely the cause of pancytopenia. Herein, two cases with fever and pancytopenia, diagnosed as simultaneous acute lymphoblastic leukemia and brucellosis are presented. Anti-leukemic therapy and brucellosis treatment were administered simultaneously, and normal blood parameters obtained. The first patient is in complete remission; the other recovered from the brucellosis, but later died due to a leukemic relapse.

Single dose of anti-transforming growth factor-beta1 monoclonal antibody enhances liver regeneration after partial hepatectomy in biliary-obstructed rats.

BACKGROUND: Transforming growth factor (TGF) beta is a potent inhibitor of hepatocyte DNA synthesis and liver regeneration. TGF-beta(1) expression progressively increases in obstructive jaundice. We investigated the effect of TGF-beta(1) blockage on liver regeneration in rats induced with obstructive jaundice. MATERIALS AND METHODS: Male Wistar-albino rats were divided into three groups: sham, control, and study groups. In the study and control groups, the common bile duct was ligated and divided, and 7 days later a partial hepatectomy was performed. In the study group, anti-TGF-beta(1) monoclonal antibody (10-microg single dose) was administered immediately after the 70% hepatectomy. In the control group, those rats in which obstructive jaundice was induced received normal saline after the 70% hepatectomy, and nonjaundiced rats received anti-TGF-beta(1) monoclonal antibody after the 70% hepatectomy. Rats were sacrificed after 48 or 72 h. Relative liver weight, AST, ALT, total and conjugated bilirubin, and TGF-beta(1) levels were measured. The mitotic index and proliferating cell nuclear antigen (PCNA) labeling index were evaluated as histopathologic parameters. RESULTS: At 72 h, the TGF-beta(1) level in the study group was similar to that in the sham group, whereas TGF-beta(1) in the study group was significantly lower than that of the jaundiced control group at 48 or 72 h (P < 0.001). The relative liver weight, mitotic index, and PCNA labeling index were significantly higher in the study group than in the jaundiced control group at 48 and 72 h (P < 0.001). The AST, ALT, and TGF-beta(1) levels were significantly higher in the jaundiced control group compared to the study group after 48 and 72 h, whereas these values were significantly lower in the nonjaundiced control group (P < 0.001). CONCLUSIONS: In obstructive jaundiced rats, TGF-beta(1) blockage with anti-TGF-beta(1) monoclonal antibody after liver resection improved liver regeneration both morphologically and functionally.

Experimental posterior perforating ocular injury in rabbit eyes: effects of radiotherapy with or without intravitreal cyclosporin on vitreous proliferation.

Double perforating ocular injuries were made in 30 rabbits and the effects of radiotherapy (RT) alone or in combination with cyclosporin (CS) on vitreous proliferation investigated. Thirteen rabbits in group 1 received RT alone (600 cGy), and 13 rabbits in group 2 received RT combined with 100 microg of intravitreal CS. No treatment was given to 4 rabbits in the control group (group 3). All animals were ophthalmologically examined on the 1st, 3rd, 7th, 14th and 28th days following the injury and the clinical findings scored; they were sacrificed on the 28th day, and histopathological scoring was made. The median histopathological score of the RT group (4.0; range: 0-8) was significantly higher than that of the CS + RT group (1.0; range: 0-5; p = 0.018). We conclude that intravitreal CS may be used as an adjunctive agent to RT to inhibit intraocular proliferation following penetrating ocular injury in rabbits.

Importance of anticoagulant proteins in chronic liver diseases.

BACKGROUND/AIMS: This study was conducted to elucidate the importance of anticoagulant proteins in chronic liver disease and their possible role as markers in determining the severity of the liver disease. METHODS: This study was conducted on 35 patients with cirrhosis, 15 patients with chronic active hepatitis and 10 healthy controls. Coagulation inhibitor proteins such as protein C, protein S and antithrombin, as well as D-dimer level and thrombin time, which reflect fibrin degradation products, were measured. Cirrhotic patients were categorized as Child A, B and C and chronic active hepatitis patients as mild or moderate activity according to the modified Knodell histopathologic classification. The parameters were compared between patient groups and healthy controls. RESULTS: In comparison with controls, the cirrhotics had significantly decreased protein C, protein S, antithrombin levels and increased D-dimer levels. The Child B and Child C patients differed significantly with respect to protein C and antithrombin levels only. In the chronic active hepatitis patients, protein S, protein C and fibrinogen were within normal limits, whereas antithrombin was low. CONCLUSIONS: In chronic active hepatitis, the antithrombin level may be used as an early marker of hepatocellular damage. In cirrhotics, protein C and antithrombin may be useful for assessment of hepatocellular damage, whereas D-dimer may be important for the transition to decompensation.