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.

Fibrinogen promotes adhesion of monocytic to human mesothelioma cells.

Adhesion between monocytic and mesothelioma or pleural mesothelial cells influences stromal remodeling in pleural neoplasia. We found that cultured monocytic cells (U937) adhere to either human pleural mesothelioma (MS-1) or mesothelial (MeT5A) cells in vitro. 125I-fibrinogen bound specifically and saturably to either cell line, and specific fibrinogen binding increased upon stimulation of these cells with proinflammatory agents such as phorbol myristate (PMA), lipopolysaccharide (LPS) or tumor necrosis factor (TNF-alpha). We purified the fibrinogen receptor protein from a membrane fraction of MS-1 cells and identified it by immunoprecipitation as intercellular adhesion molecule (ICAM-1). Anti-ICAM-1 antibody or antisense oligonucleotides inhibited fibrinogen-mediated cell adhesion and binding of 125I-fibrinogen to mesothelioma or mesothelial cells. Cultured monocytic cells adhere to either mesothelioma or mesothelial cells, and the interaction is promoted by fibrinogen binding ICAM-1 at the cell surface. ICAM-1 is expressed by mesothelioma cells and CD 11b by macrophages in the fibrinous mesothelioma tumor stroma. The data suggest a common mechanism by which monocytic cells could adhere to either malignant mesothelioma cells or the mesothelial surface in pleural neoplasia.

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.

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.

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.

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.

Solitary phalanx plasmacytoma relapse with disseminated extramedullary plasmacytomas and myeloma after short duration of remission.

Solitary bone plasmacytomas account for 5-7% of multiple myeloma cases and are assumed to have a fairly good prognosis, with a long duration of relapse-free survival after primary local treatment. Isolated phalanx plasmacytoma is a very rare entity, because involvement of extremities is seen in less than 1% of all solitary bone plasmacytomas, where they are usually localized centripedally, often in the axial skeleton. A 68 year old patient with a lytic lesion involving 5th phalanx was diagnosed as having a biopsy-proven solitary plasmacytoma, with a negative work-up for coexisting plasma cell dyscrasia. Three and a half months after completion of radiotherapy of the involved phalanx, the patient was readmitted with hypercalcemia, renal insufficiency and subsequently diagnosed as having atypical plasma cell infiltration of marrow, and plasmacytomas involving the right vocal cord and the premaxillary region, as well as pathological ulna fracture. Plasmacytoma of the phalanx, with extreme short duration of remission and an aggressive type of clinical relapse, is in sharp contrast with the natural stable course of a solitary plasmacytoma where the use of systemic treatment is subject to intense debate.

Myasthenia gravis and primary squamous cell carcinoma of the thymus.

Occurrence of primary squamous cell carcinoma of the thymus gland in a 65-year-old man with myasthenia gravis is reported. Histologic and immunohistochemical studies confirmed the diagnosis of a differentiated squamous cell carcinoma. Extensive clinical investigations ruled out another primary site for the tumor. The patient made a full recovery postoperatively. Only three cases of primary squamous cell carcinoma of the thymus gland in association with myasthenia gravis have been reported in the literature.

Blood cyst originating from tricuspid septal leaflet.

Successful removal of a blood cyst of the tricuspid valve in a 34 years old man is reported. The rarity of this cyst in an adult and diagnostic clues are discussed.

Coarctation of the abdominal aorta with left renal artery and splenic artery aneurysms.

Both coarctation of the abdominal aorta, and splenic artery aneurysm reside in the hilus, a rare but extremely important pathological condition. Herein a 36-year-old woman hospitalized for uncontrolled hypertension is presented. Aortography demonstrated segmental abdominal aortic coarctation with left renal artery and splenic artery aneurysms. Medical control of hypertension was not possible and thoracoabdominal bypass with a prosthetic graft was undertaken. Left renal artery aneurysm was resected and aorto-renal saphenous vein bypass was performed. But splenic artery aneurysm was left in situ because it was asymptomatic and less than 2 cm in diameter. Postoperatively the patient was normotensive. Although the pathogenesis of such lesions remain unknown, it may be to congenital or related medial degeneration as demonstrated histologically.

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.

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.

Multicentric angiofollicular lymph-node hyperplasia associated with myasthenia gravis.

Castleman's disease is often referred to as giant lymph-node hyperplasia. Although this localized disorder most commonly involves the mediastinum, recently many patients have been described with multicentric lymph-node hyperplasia associated with various clinical abnormalities. Here a new case of multicentric angiofollicular lymph-node hyperplasia associated with myasthenia gravis and gammopathy is presented. At first, the patient responded to surgery well, but after some months her condition deteriorated: plasmapheresis was then performed, one year after the operation, with great clinical improvement.