Acute leriche syndrome due to paradoxical embolism.

Paradoxical embolism, describing the passage of venous or right-sided cardiac thrombus into the arterial or systemic circulation through patent foramen ovale, is an uncommon cause of acute arterial occlusion. Here, we report acute Leriche syndrome in a young woman attributable to paradoxical embolism. Ischaemia, patent foramen ovale, and venous thrombosis were the triad of evidence for paradoxical embolism.     

Trigeminal amyloidoma: case report and review of the literature.

The authors present a case of amyloid infiltration involving the trigeminal nerve that mimicked a malignant cavernous sinus tumor with perineural tumor infiltration. A 64-year-old man presented with trigeminal nerve numbness. Imaging revealed a plaque-like enhancing lesion along the right lateral cavernous sinus extending anteriorly into Meckel's cave and involving the proximal V2 and V3 branches of the trigeminal nerve. The patient underwent an extradural frontotemporal craniotomy with middle fossa exposure of the cavernous sinus to diagnose and treat the presumed malignant cavernous sinus tumor. A reddish mass involving the lateral dural wall of the cavernous sinus was resected. The gasserian ganglion, V2, and V3, the latter of which was biopsied, were enlarged. Permanent histopathological studies showed microscopic eosinophilic, amorphous material, which stained positive for Congo red, and an absence of neoplastic cells. The final diagnosis was amyloidoma. Thus, amyloidomas can involve the trigeminal nerve or ganglia and should be considered in the differential diagnosis of a cavernous sinus lesion mimicking a tumor. Patients may have symptomatic improvement of trigeminal neuropathy with resection of the amyloidoma outside the nerve capsule that is compressing the nerve, while resection of the lesion from within the capsule may result in permanent trigeminal nerve dysfunction.     

Regulation des programmierten Zelltod in aggressiven Fibroblasten

Apoptosis is a central physiological mechanism for maintaining cellular stability in tissue. Synovial fibroblasts, which play a central role in the pathogenesis of rheumatoid arthritis (RA), show a resistance to apoptosis. Several molecular mechanisms are involved in such resistance. Thus, soluble Fas can bind Fas ligands (Fas-L) and hinder Fas-L induced apoptosis in fibroblasts. SUMO-1 (a small ubiquitin-like modifier) attaches to proteins post-translationally. This appears to be significantly involved in apoptosis resistance in RA fibroblasts. SUMO-1 levels are substantially increased in synovial fibroblasts from RA patients. A change in the post-translational SUMOlation pattern could represent a new target for changing the stable activation of synovial fibroblasts in RA.     

The high prognostic value of the histologic grade at the deep invasive front of tongue squamous cell carcinoma

BACKGROUND: Although many histopathologic factors in squamous cell carcinoma of the tongue predict the prognosis, the major predictive factors have not been identified clearly. This study analyzed the prognostic value of the histologic grade at the deep invasive front of tongue squamous cell carcinoma. METHODS: The clinicopathologic features of 124 consecutive patients seen between January 1985 and December 1999 with previously untreated squamous cell carcinoma of the tongue were reviewed. Their mean age was 58.5 years (range: 23-90) and the male-female ratio was 1.8: 1 (79 men and 45 women). There were 41, 40, 30, and 13 cases at stage I to stage IV, respectively. The clinicopathologic factors, especially the histologic grade at the deep invasive front (invasive front grade, IFG), were analyzed to determine factors predicting prognosis. RESULTS: The 5-year disease-free survival rate of the patients treated with curative aim only was 66.7%. Clinicopathologic factors significantly associated with the prognosis were T classification, tumor size, stage classification, tumor depth, macroscopic appearance, cervical lymph node metastasis (nodal metastasis), microvascular invasion, and IFG. In a multivariate analysis, patients with tumor depth >/=4 mm, IFG >/=8 points, and nodal metastasis had a reduced disease-free survival and IFG >/=11 points had a predictive value for nodal metastasis (odds ratio: 7.34; P = 0.0019). CONCLUSION: This study found that a high IFG malignancy score had a high prognostic value for squamous cell carcinoma of the tongue.     

Bedeutung molekular-zytogenetischer Befunde bei Speicheldrüsentumoren am Beispiel des Mukoepidermoidkarzinoms

Chromosomentranslokationen in Tumoren führen nicht selten zur Ausbildung von Fusionsgenen, die für Proteine mit onkogenen Eigenschaften codieren können. Mukoepidermoidkarzinome sind charakterisiert durch eine Translokation t(11;19), die in 60% der untersuchten Tumoren vorkommt. Diese chromosomale Umlagerung führt zu einem Fusionsgen, das aus dem Exon 1 des MECT 1-Gens und den Exons 2–5 des MAML 2-Gens zusammengesetzt ist. In der Folge bildet sich ein Fusionstranskript, wodurch unabhängig von Notch-Liganden eine Aktivierung der Transkription von HES 1, einem Notch-Zielgen, erfolgt. Durch die veränderte Funktion von MAML 2 kommt es zur Unterbrechung des NOTCH-Signaltransduktionsweges, was einen neuen Mechanismus in der Tumorgenese vermuten lässt. Der Nachweis der Translokation mittels FISH und/oder RT-PCR könnte von erheblichem diagnostischem und möglicherweise auch prognostischem Interesse sein. Zuvor müssen allerdings die molekularen Ereignisse, die einem scheinbar identischen chromosomalen Rearrangement in Warthin-Tumoren zugrunde liegen, geklärt werden. Warthin-Tumoren sind gutartige Speicheldrüsentumoren mit einer vom Mukoepidermoidkarzinom abweichenden Histomorphologie und Histogenese.     

Ultrasonographic study on kidneys in patients with acute renal failure]

Ultrasonotomograms of 22 kidneys were obtained in 11 patients with renal-acute renal failure (renal-ARF). The underlying diseases of renal-ARF were acute tubular necrosis in 8 patients and acute on-set chronic glomerulonephritis in 3 patients. They were treated by hemodialysis in 10 patients and intermittent peritoneal dialysis in 1 patient. Ultrasonic measurement of the size of kidneys revealed that the thickness (anterior-posterior diameter) and the ratio of thickness to length (T/L) were greater in patients with ARF than in those with chronic renal failure and normal renal function. The patients with a low value of T/L (under 0.60) had a significantly greater urine volume than those with high a value of T/L (0.60 or more). The sonographic features of renal-ARF kidneys were marked increase in parenchymal echogenicity and appearance of hypoechoic swollen renal pyramids with sharpness of the corticomedullary border. In the course of ARF, these sonographic changes gradually disappeared when the patients had recovered from ARF. However, the prognosis was poor in patients with severer sonographic findings. We believe that repeated ultrasonic examination of the kidneys in patients with renal-ARF is useful for not only differential diagnosis of post-renal urinary obstruction but evaluating the course of ARF.     

Auriculo-ventricular block and distal myopathy with rimmed vacuoles and desmin storage.

A young patient had an auriculo-ventricular block and a distal myopathy with muscle biopsy findings suggestive of inclusion body myositis. What was most unusual was the presence of numerous sarcoplasmic bodies identified as desmin by electron microscopy and immunocytochemistry. The nosological situation of this condition is discussed.