Conus ependymoma with holocord syringohydromyelia and syringobulbia.

We report a 24-year-old woman with an intramedullary conus ependymoma associated with holocord syringohydromyelia and syringobulbia. The tumor was removed and surgery for decompression of the syringohydromyelia was not considered at the first operation. In the follow-up examinations, MRI showed significant and steady improvement of syringohydromyelia. Symptoms associated with syringohydromyelia also disappeared. The cause of syringohydromyelia accompanying intradural spinal cord tumors appears to be either direct blockade of the central canal or secondary interruption of the central canal flow by compression of the perimedullary cerebrospinal fluid flow. As removal of the mass often corrects both these likely causes of the syringohydromyelia, no additional treatment for the drainage of the hydromyelia cavity syrinx is usually necessary.

A novel surveillance protocol for stage I nonseminomatous germ cell testicular tumours.

OBJECTIVES: To report the results of a novel surveillance policy for stage I nonseminomatous germ cell tumours (NSGCTs). PATIENTS AND METHODS: Between 1978 and 2000, 132 patients (median age 28 years, range 16-52) who were regularly followed were included in a new surveillance policy. All pathology specimens were studied retrospectively by the same pathologist for embryonal carcinoma, yolk sac tumour and lymphovascular invasion components. A loose surveillance protocol was designed in which computed tomography (CT) was used only for the first year. RESULTS: The median (range) follow-up was 38 (6-265) months; the relapse rate was 24% and all occurred before 23 months, with 87% diagnosed within the first year. Platinum-based chemotherapy was given to patients with relapse, and surgery used after chemotherapy in seven. Among all the risk factors, an embryonal carcinoma component was the only significant predictor of relapse. The overall survival rate was 99%. CONCLUSION: The presence of embryonal carcinoma in the primary pathology is the only risk factor determining the relapse rate of the present surveillance policy for stage I NSGCTs. The overall survival was no different from those reported for retroperitoneal lymph node dissection and primary chemotherapy. Decreasing the frequency of CT in the first year and totally eliminating it after 1 year reduces the cost of surveillance. The possible compliance problems of patients are also minimized, without changing the overall survival. This surveillance protocol for patients with stage I NSGCT has reduced costs and provided a better quality of life for the patients, without jeopardizing the final outcome.

Prognostic significance of flow cytometric DNA analysis in patients with malignant pleural mesothelioma.

Malignant pleural mesothelioma (MPM) due to environmental exposure to asbestos and erionite is a relatively common cancer in Turkey. In this study, we investigated the value of flow cytometric (FCM) DNA analysis and other prognostic factors such as age and etiologic factor in the patients with MPM, treated with surgery+/-combination chemotherapy+/-radiotherapy. A total of 40 patients with a median age of 50 (range 30-68) were included in the study. Twenty-nine patients had asbestos exposure in etiology, while 11 had fibrous zeolite (erionite). Paraffin-embedded tumor specimens were studied by FCM for DNA analysis. Twelve patients (30%) had aneuploid tumors and 28 (70%) had diploid ones. Mean S-phase fraction (SPF; %) was 9.1+/-1.1 and proliferation index (PI, SPF+G2/M phase; %) was 11.3+/-0.9. While the median overall survival (OS) was 10+/-2 months (6-14; 95% CI), 1-year survival rate was 45.2%. Only PI was found to be statistically significant for OS in univariate analysis (P=0.013). PI was also found to be an independent prognostic factor for all patients (P=0.035). Aneuploidy was significantly higher in erionite group compared with asbestos group. Male predominance and poor survival were also prominent in erionite group, though not statistically significant. In conclusion, PI is an independent prognostic factor for patients with MPM and the biologic features of the disease may show differences with respect to different etiologies.

The analysis of gastrointestinal amyloidosis in 78 patients with chronic renal failure.

Systemic amyloidosis is not a single disease, but the product of a variety of diseases. Amyloid proteins are insoluble fibrils that are deposited extracellularly in many organ tissues. They stain with Congo red and appear apple green under polarized light. Definitive diagnosis and classification ofamyloidosis requires histologic examination of tissue samples. Gastrointestinal tract involvement is common, and all parts of the system can be affected Immunohistochemical studies have shown that amyloid deposited in the gastrointestinal system is most often of the AA, A kappa, or A lambda types. Another type of amyloidprotein, beta-2 microglobulin (beta2M), predominantly affects the musculoskeletal system, and is usually seen in patients who have been on long-term hemodialysis. Mixed systemic amyloidosis (beta2M and AA) is seen only rarely in these patients. In this study, we attempted to answer why this is so, and examined whether or not mixed amyloidosis is related to amyloidogenesis. We studied gastrointestinal tissues from 78 chronic renal failure patients who had systemic amyloidosis with gastrointestinal involvement. A total of 115 endoscopic samples and 1 jejunal resection specimen were analysed immunohistochemically. Immunohistochemical testing using a panel of antisera directed against two major amyloid fibril proteins (AA-Monoclonal, Dako-, and beta2M-Polyclonal, Dako-) showed that all samples contained AA amyloid, but not beta2M type protein. These findings can be explained by the patients' relatively short average duration of hemodialysis and the predominance of endoscopic biopsy samples in our study.

Simian virus 40 is not a cofactor in the pathogenesis of environmentally induced malignant pleural mesothelioma in Turkey.

Malignant pleural mesothelioma (MPM) continues to be a public health problem in Turkey, where exposure to environmental asbestos and fibrous zeolite (erionite) is the main cause of the disease. However, less than 5% of exposed individuals develop the disease, and numerous cases of MPM are documented each year in which the patient has no known exposure to either of these minerals. Thus, additional unknown factors act independently or as co-carcinogens in the development of MPM. Simian Virus 40 (SV40) may act as a co-carcinogen with asbestos in the pathogenesis of occupationally induced MPM. To determine if SV40 plays a role in the development of MPM in Turkey, we used PCR analysis to investigate if SV40 DNA sequences were present in 29 mesothelioma specimens from patients previously exposed to asbestos or erionite. PCR analysis revealed that all 29 tissue specimens from our patients did not contain SV40 DNA. 15 specimens from patients suffering from tuberculosis pleuresy were also SV40 negative. One mesothelioma and one osteosarcoma from Italy tested positive for SV40. Our results indicate that inorganic fibers, asbestos, and erionite remain the only known causal factors of mesothelioma in Turkey. The absence of SV40 in Turkish specimens and its presence in Italian specimens may be related to the fact that SV40-contaminated vaccines were not administered in Turkey.

Congenital broncho-oesophageal fistula associated with bronchiectasis in adults. Report of two cases and review of the literature.

Congenital broncho-oesophageal fistula is a rare entity in adult patients. This anomaly may cause various symptoms such as respiratory infections, coughing bouts when eating or drinking and even haemoptysis. Even rarer than its occurrence with the above-mentioned symptoms is its presentation with bronchiectasis. A congenital broncho-oesophageal fistula presenting with bronchiectasis in a 28-year-old male and 36-year-old female are described. In reported cases, symptoms of chronic recurrent pulmonary suppuration were initially attributed to alternative aetiologies. In both cases, with such an unusual presentation, the observation of the fistulous tract was coincidental. Surgical division of the fistula associated with lobectomy resulted in complete resolution of symptoms.

Hamartoma of the larynx: a case of unusual bleeding after microlaryngoscopy.

We report on a rare complication of surgery in a 36-year-old man who had undergone removal of a hamartoma of the larynx. The patient had developed a submucosal lesion in the left ventricular fold, which was removed by surgical microlaryngoscopy. Following the excision, the patient began to experience an excessive amount of bleeding at the surgical site. Before hemostasis was achieved, the patient had lost almost 1,000 ml of blood. Although the occurrence of hemorrhage during surgical laryngoscopy is rare, surgeons should be aware of and prepared for this possibility.

The importance of increasing the number of gastric biopsies in the diagnosis of Helicobacter pylori.

BACKGROUND/AIMS: The role of Helicobacter pylori in various gastroduodenal diseases is universally accepted. In this study, we aimed to determine the proper number and sites of the gastric biopsies in order to achieve the highest diagnostic yield through the use of a urease test and histopathology. We also compared the histological findings encountered in patients who had Helicobacter pylori (H. pylori) colonization. METHODOLOGY: Fifty patients referred for upper gastrointestinal endoscopy for dyspeptic complaints were included in the study. Our mapping protocol included 2 biopsies from antrum and 2 biopsies from corpus. We obtained 2 biopsies from each biopsy site for urease test and histopathological assessment. Golden standard positivity for the presence of H. pylori colonization was defined as concomitantly positive urease test and histologically detected bacteria found at the same biopsy site. RESULTS: Forty-three patients had H. pylori colonization. Colonization rates of H. pylori, sensitivities of urease testing, and histopathology in 4 biopsy sites were not statistically different. Sensitivity of urease testing was 81.4% for 1 biopsy and 100% for 4 cumulative biopsies. Sensitivities of histological assessment were 93% and 100% for 1 and 4 biopsies, respectively. CONCLUSIONS: Results of this study suggest that 2 biopsies for urease testing and 1 biopsy for histopathology obtained from the antrum or corpus of the stomach were sufficient to obtain the highest statistically significant diagnostic sensitivity.

Congenital orbital teratoma: a clinicopathologic case report.

A 5-day-old infant boy was noted to have severe left proptosis at birth. The left eye protruded superotemporally through the palpebral fissure and had exposure keratopathy. There was frank left afferent pupillary defect. Computerized tomography (CT) showed a left orbital soft tissue mass with foci of calcification. Magnetic resonance imaging (MRI) studies revealed a left orbital mass with solid and cystic portions without intracranial extension. As the eye was considered to be nonsalvagable, a lid-sparing type, modified exenteration was performed. Histopathologic examination demonstrated various mature tissues of all three embryonic germinal cell lines. This case represents one of the rare examples of true congenital orbital teratoma, which is an uncommon cause of hideous proptosis in the neonate. MRI may prove useful in differentiating this tumor from more common conditions.

Malignant melanoma in xeroderma pigmentosum patients: report of five cases.

Xeroderma pigmentosum is a rare genetic disease transmitted via a recessive gene with an altered reaction of the epidermis to light. Fifty per cent of patients develop a skin tumour by 8 years of age. The majority of patients may have multiple tumours, but metastasis is rare. In the last 25 years we have treated 24 xeroderma pigmentosum patients in our clinic. Only five patients had developed cutaneous malignant melanoma during their follow-up. Three of the patients were from the same family, melanoma occurring in three of five affected individuals. All xeroderma pigmentosum patients with malignant melanoma had received classical treatment modalities. Except one case of fulminant pattern, all four patients had long disease-free survival. Although early detection and treatment of these cutaneous malignancies will reduce morbidity and mortality, genetic counselling remains the most important protective measure for xeroderma pigmentosum.

Primary extramedullary plasmacytoma of the liver.

Extramedullary plasmacytoma of the liver is a rare tumour, only two cases of which have been reported so far. A third case arising in a 22 year old woman, who presented with abdominal pain and enlargement of the liver, is described. Ultrasound and a computed tomography scan showed a solitary hepatic mass, 12 cm diameter, involving both lobes of the liver. Serum immunoelectrophoresis revealed an IgG kappa monoclonal gammopathy. Histologically, the tumour was composed of mature plasma cells with mild atypia. The plasma cells infiltrated the liver parenchyma and showed kappa light chain restriction. The monoclonal nature of the tumour was also demonstrated by PCR amplification of the immunoglobulin heavy chain genes. There was no evidence of bone involvement and repeated bone marrow aspirates and biopsy specimens were normal. The patient was treated with eight courses of chemotherapy. One year after diagnosis, the patient is well, the size of the tumour has decreased and the paraproteinaemia has disappeared.

The correlation between prostate-specific antigen and age. Analysis of prostate-specific antigen values from 4,846 Turkish men with symptomatic benign prostatic hyperplasia.

OBJECTIVE: To analyze serum prostate-specific antigen (PSA) levels of men proven to have benign prostatic hyperplasia (BPH) and to document any correlation between PSA, age and resected or enucleated prostate tissue. PATIENTS AND METHODS: Serum PSA values, age and weight of specimens of 4,846 men who underwent prostatectomy in the Ankara region between January 1, 1991, and December 24, 1995, were reviewed retrospectively. RESULTS: Serum PSA values correlated directly with age (Pearson's r = 0.45, p < 0.00001). The mean PSA values of men in each decade of age differed significantly (p < 0.0001) from all other decades. However, the correlation between the weight of the prostatectomy specimen and age or PSA did not reach statistical significance. CONCLUSION: The effect of age on PSA is evident in men with symptoms of BPH. The existence of symptomatic BPH should also be considered together with age when interpreting PSA levels.

Isolated chylopericardium secondary to intrathymic lymphangiomatous malformation.

A case of chylopericardium associated with lymphangiomatous malformation of the thymus is presented. The specific diagnosis of chylopericardium was made by pericardiocentesis and analysis of the fluid. Despite the pericardiocentesis and pericardial tube drainage, pericardial effusion recurred and the patient underwent pericardiectomy. At operation the thymus, which contained a tumor and showed diffuse hyperplasia, was excised and pericardiectomy was performed. The 2-year-old patient recovered uneventfully without reaccumulation of the chyle. The pathologic diagnosis of the mass was intrathymic ectatic lymphangiomatous malformation.