Jiri Ferda

Gastrointestinal stromal tumor of the pancreas: case report with documentation of KIT gene mutation

Sir, mesenchymal tumors of the pancreas are exceedingly rare, accounting for less than 1% of all pancreatic tumors, and most of them have been reported in small series or as single case reports. This group of pancreatic tumors includes leiomyosarcoma, malignant peripheral nerve sheath tumor, malignant fibrous histiocytoma, liposarcoma, rhabdomyosarcoma,hemangiopericytoma,schwannomaandsolitary fibrous tumor. In the gut, mesenchymal tumors are more common, and the majority of these are formed by gastrointestinal stromal tumors (GISTs). GISTs are defined as KIT-positive mesenchymal spindle cell or epithelioid neoplasms showing differentiation toward the interstitial cell of Cajal. GISTs occur most commonly in the stomach (60–70%), small intestine (20–25%), colorectum (5%) and esophagus (<5%). They were also occasionally reported in extragastrointestinal sites,suchasurinarybladder,gallbladder,omentumandmesentery[1].WereportacaseofaGIST of the pancreatic head in a 70-year-old woman exhibiting all diagnostic histopathological, immunohistochemical and molecular genetic features, which has not yet been properly documented in this location. Although Neto et al. publishedacaseofpancreatic GISTrecently,evaluationofc-kit mutations as well as computed tomography (CT) scan or microphotograph documenting pancreatic location of the tumor is missing in their report [7].

Length of Occlusion Predicts Recanalization and Outcome After Intravenous Thrombolysis in Middle Cerebral Artery Stroke

Background and Purpose— The length of large vessel occlusion is considered a major factor for therapy in patients with ischemic stroke. We used 4D-CT angiography evaluation of middle cerebral artery occlusion in prediction of recanalization and favorable clinical outcome and after intravenous thrombolysis (IV-tPA). Methods— In 80 patients treated with IV-tPA for acute complete middle cerebral artery/M1 occlusion determined using CT angiography and temporal maximum intensity projection, calculated from 4D-CT angiography, the length of middle cerebral artery proximal stump, occlusion in M1 or M1 and M2 segment were measured. Univariate and multivariate analyses were performed to define independent predictors of successful recanalization after 24 hours and favorable outcome after 3 months. Results— The length of occlusion was measureable in all patients using temporal maximum intensity projection. Recanalization thrombolysis in myocardial infarction 2 to 3 was achieved in 37 individuals (46%). The extension to M2 segment as a category (odds ratio, 4.58; 95% confidence interval, 1.39–15.05; P=0.012) and the length of M1 segment occlusion (odds ratio, 0.82; 95% confidence interval, 0.73–0.92; P=0.0007) with an optimal cutoff value of 12 mm (sensitivity 0.67; specificity 0.71) were significant independent predictors of recanalization. Favorable outcome (modified Rankin scale 0–2) was achieved in 25 patients (31%), baseline National Institutes of Health Stroke Scale (odds ratio, 0.82; 95% confidence interval, 0.72–0.93; P=0.003) and the length of occlusion M1 in segment (odds ratio, 0.79; 95% confidence interval, 0.69–0.91; P=0.0008) with an optimal cutoff value of 11 mm (sensitivity 0.74; specificity 0.76) were significant independent predictors of favorable outcome. Conclusions— The length of middle cerebral artery occlusion is an independent predictor of successful IV-tPA treatment.

Dual-Phase Dual-Energy CT in Patients Treated with Erlotinib for Advanced Non-Small Cell Lung Cancer: Possible Benefits of Iodine Quantification in Response Assessment.

AbstractObjectivesTo investigate the relationship of dual-phase dual-energy CT (DE-CT) and tumour size in the evaluation of the response to anti-EGFR therapy in patients with advanced non-small cell lung cancer (NSCLC).MethodsDual-phase DE-CT was performed in 31 patients with NSCLC before the onset of anti-EGFR (erlotinib) therapy and as follow-up (mean 8 weeks). Iodine uptake (IU; mg/mL) was quantified using prototype software in arterial and venous phases; arterial enhancement fraction (AEF) was calculated. The change of IU before and after therapy onset was compared with anatomical evaluation in maximal transverse diameter and volume (responders vs. non-responders).ResultsA significant decrease of IU in venous phase was proved in responders according to all anatomical parameters (p=0.002–0.016). In groups of non-responders, a significant change of IU was not proved with variable trends of development. The most significant change was observed using the anatomical parameter of volume (cut-off 73 %). A significant difference of percentage change in AEF was proved between responding and non-responders (p=0.019–0.043).ConclusionDual-phase DE-CT with iodine uptake quantification is a feasible method with potential benefit in advanced assessment of anti-EGFR therapy response. We demonstrated a decrease in vascularization in the responding primary tumours and non-significant variable development of vascularization in non-responding tumours.Key Points• Dual-phase DE-CT is feasible for vascularization assessment of NSCLC with anti-EGFR therapy. • There was a significant decrease of iodine uptake in responding tumours. • There was a non-significant and variable development in non-responding tumours. • There was significant difference of AEF percentage change between responders and non-responders.

Multidetector computed tomography of chest trauma: indications, technique and interpretation

BackgroundChest traumas are a significant cause of mortality and morbidity, especially in the younger population.MethodsDiagnostic imaging plays a key role in their management. Multidetector computed tomography (MDCT) is the most important imaging method in this field. Its advantages include especially high speed and high geometric resolution in any plane.ResultsThe method allows us to view large parts of the body with minimal motion artifacts and to create accurate multiplanar and three-dimensional (3D) reformations, which make the diagnosis significantly more accurate. Because of its advantages MDCT has become the first-choice method in high-energy traumas.ConclusionThis article summarises the position of MDCT in the diagnostic algorithm of chest injuries, technical aspects of the examination and imaging findings in traumas of the individual chest compartments.Teaching Points• Diagnostic imaging plays a key role in the management of high-energy chest trauma.• MDCT is the most important imaging method in this kind of injury, as detailed information can be acquired in a short acquisition time.• Multiplanar and three-dimensional (3D) reformattings make the diagnosis significantly more accurate.

Cystic and necrotic papillary renal cell carcinoma: prognosis, morphology, immunohistochemical, and molecular-genetic profile of 10 cases.

Conflicting data have been published on the prognostic significance of tumor necrosis in papillary renal cell carcinoma (PRCC). Although the presence of necrosis is generally considered an adverse prognostic feature in PRCC, we report a cohort of 10 morphologically distinct cystic and extensively necrotic PRCC with favorable biological behavior. Ten cases of type 1 PRCC with a uniform morphologic pattern were selected from the 19 500 renal tumors, of which 1311 were PRCCs in our registry. We focused on precise morphologic diagnosis supported by immunohistochemical and molecular-genetic analysis. Patients included 8 men and 2 women with an age range of 32-85 years (mean, 62.6 years). Tumor size ranged from 6 to 14 cm (mean, 9.4 cm). Follow-up data were available in 7 patients, ranging from 0.5 to 14 years (mean, 4 years). All tumors were spherical, cystic, and circumscribed by a thick fibrous capsule, filled with hemorrhagic/necrotic contents. Limited viable neoplastic tissue was present only as a thin rim in the inner surface of the cyst wall, consistent with type 1 PRCC. All cases were positive for AMACR, OSCAR, CAM 5.2, HIF-2, and vimentin. Chromosome 7 and 17 polysomy was found in 5 of 9 analyzable cases, 2 cases demonstrated chromosome 7 and 17 disomy, and 1 case showed only chromosome 17 polysomy. Loss of chromosome Y was found in 5 cases, including 1 case with disomic chromosomes 7 and 17. No VHL gene abnormalities were found. Papillary renal cell carcinoma type 1 can present as a large hemorrhagic/necrotic unicystic lesion with a thick fibroleiomyomatous capsule. Most cases showed a chromosomal numerical aberration pattern characteristic of PRCC. All tumors followed a nonaggressive clinical course. Large liquefactive necrosis should not necessarily be considered an adverse prognostic feature, particularly in a subset of type 1 PRCC with unilocular cysts filled with necrotic/hemorrhagic material.

Elastofibromatous Changes in Tissues From Spinal Biopsies. A Degenerative Process Afflicting a Small but Important Subset Of Patients Operated for Spinal Canal Compression: Report of 18 Cases

Elastofibroma is a tumorlike lesion occurring usually in the subscapular region of elderly females. In this study, 18 cases of elastofibromatous tissue retrieved from the spinal canal were analyzed to elucidate its frequency and possible clinical associations. The patients included 8 men and 10 women with a mean age of 63.4 years (range, 40-84 years). The elastic nature of the material was confirmed by Weigert’s elastic stain and immunohistochemically. Elastofibroma was diagnosed in 6 patients, and elastofibroma-like tissue was found in 11 samples. The finding of elastofibromatous tissue was associated with clinical diagnosis of juxtaarticular cysts (3 cases), thickening of ligamentum flavum (4 cases), extradural expansion (2 cases), metastatic involvement of the dorsal spine (3 cases), and a vertebral fracture (1 case). Two cases were located above the transpedicular fixation. Elastofibromatous tissue may be found in approximately 2.6% of all spinal biopsies and it may take part in compression of spinal cord and nerve roots.

Bilateral post-traumatic carotid dissection as a result of a strangulation injury.

Carotid trauma is always very serious. Post-traumatic carotid dissection is rather rare and can be diagnosed late. Authors present a case report of post-traumatic bilateral carotid dissection and its spontaneous recovery. Symptoms, diagnosis, and treatment are discussed.

Idiopathic portal hypertension: a case report

SummaryBACKGROUND: Idiopathic portal hypertension (IPH) is a disease of unknown etiology which manifests itself in hypercirculation and hypertension in the portal venous system and splenomegaly with hypersplenism. METHODS: We describe a case of IPH in a young sportsman with the characteristic clinical, sonographic, and computed-tomography picture. Open splenectomy was performed. RESULTS: Preoperative examination revealed an interesting elevation of endothelin-1 (ET-1) and vasoadhesive molecule-1 (VCAM-1) in the serum. Postoperative course was without complications. One year after operation the patient is fine without any symptomatology of IPH. CONCLUSIONS: ET-1 and VCAM-1 will probably have a significant influence on the development of IPH. ET-1 causes hypercirculation and hypertension in the portal venous system, and VCAM-1 causes adhesion of lymphocytes to the endothelium of the portal tracts and their migration into the wall, mainly in a presinusoidal way, followed by fibrotization of the venous wall. Splenectomy was performed in this case, after which the clinical symptomatology of IPH disappeared.ZusammenfassungGRUNDLAGEN: Die idiopathische portale Hypertension (IPH) ist eine Krankheit unbekannter Genese, welche zu einer Hypervaskularisation und portalen Hypertension mit Splenamagalie und Hypersplenismus führt. METHODIK: Kasuistik von IPH mit typischen klinischen, sonographischen und CT-radiologischen Zeichen. Eine offene Splenektomie wurde durchgeführt. ERGEBNISSE: Präoperativ fanden sich erhöhtes Endothelin-1 (ET-1) und vasoadhäsives Molekül-1 (VCAM-1) im Serum. Der postoperative Verlauf war komplikationsfrei, ein Jahr nach der Operation zeigt der Patient keine Hinweise für IPH. SCHLUSSFOLGERUNGEN: ET-1 und VCAM-1 dürften eine Rolle in der Pathogenese der IPH spielen. ET-1 mediiert die Hypervaskularisation und Hypertension im portal-venösen System, VCAM-1 mediiert die Adhäsion und Transmigration von Lymphozyten in das Endothel. Durch Splenektomie wurde die IPH erfolgreich behandelt.

Unusual Case of Pheochromocytoma With a Surprising Response to the Intravenous Administration of Norepinephrine

A 34-year-old woman with a 1-year history of hypertension was admitted to the internal department of the catchment hospital on March 31, 2000, with dyspnea, chest discomfort, and vomiting. On April 11, cephalea with paroxysmal hypertension appeared. An ultrasonographic examination showed a suspected right adrenal pheochromocytoma. On April 18, the patient was transferred to our hospital, and a right adrenal tumor was diagnosed by CT scan. After her admission to the coronary unit, periodic fluctuations of blood pressure (and heart rate) were found. The blood pressure ranged between 65/40 and 270/150 mm Hg, with a 10-minute periodicity. During hypertension, pallor was present, accompanied by cephalea and sinus bradycardia, which was changing …

Urgent Extracranial-Intracranial Bypass in the Treatment of Acute Hemodynamic Ischemic Stroke: Case Report

Extracranial-intracranial (EC-IC) bypass surgery is performed to allow flow augmentation in selected cases of occlusive cerebrovascular disease. The majority of EC-IC bypasses are described as an elective procedure in the prevention of hemodynamic ischemic stroke. There is only limited and controversial experience of superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis performed under urgent circumstances as a treatment of acute cerebral ischemia. We describe a unique case of a 75-year-old patient presenting with acute progressive hemodynamic ischemia after carotid endarterectomy (CEA), which developed contralaterally to the performed CEA in the region of chronic internal carotid artery (ICA) occlusion. Urgent performance of a standard STA-MCA bypass rapidly improved the cerebral hemodynamic and had an excellent therapeutic effect. The patient had recovered completely within 4 weeks of surgery. The role of urgent EC-IC bypass for stroke treatment is discussed.