Rare cases reports of gastrointestinal stromal tumour (GIST).

The GISTs are rare tumours but even rarer is the localization in some districts. We reported two GISTs of the duodenum, two of the omentum and peritoneum, one of the rectum and one of a Meckel's diverticulum. These exceptional locations are confirmed by the relative difficult diagnosis, obtained in some cases only by the surgical treatment despite the CT and MR. The endoscopy is useful in hemorrhagic and duodenum forms, only for the diagnosis and for the control of blood loss. Surgical treatment in all cases was decisive without the need to make use of adjuvant therapy, with positive long-term results, which excluded the disappearance of relapses or secondary lesions.

Respiratory failure and pharyngeal hematoma as presenting signs of Moschcowitz's syndrome: a case report and literature review.

Moschcowitz's syndrome or thrombotic thrombocytopenic purpura is a quite rare pathology in childhood, being, as a matter of fact, more frequent among adult people. Often it is hard to distinguish from other pathologies in children both for its rare incidence and for the presence of clinical forms that are very heterogeneous and difficult to be classified. We report on a 13 year-old girl suffering from Moschcowitz's syndrome, in whom respiratory failure and pharyngeal hematoma were the first sign of the disease follone by jaundice, hematoma of the arm and limbs. The girl was treated with plasmapheresis with an improvement of her general condition. Since then we have followed up the girl for two years without any reappearance of the symptomatology. To our knowledge this is the first report of this peculiar presentation in children.

A rare location of benign osteoblastoma: case study and a review of the literature.

Osteoblastoma is a solitary, benign bone tumor that is rarely localized in the frontal sinus. It consists of hypocellular mineralized tissue that may form large masses or irregular trabeculae. A 31 year old man came to our attention with a 7 month history of diplopia, photophobia, frontal headhaches and progressive exophthalmos with proptosis of the left eye. The patient was submitted to computed tomography (CT) which allowed to appraise the extension of the lesion. The mass expanded inside the left frontal sinus and the upper ethmoidal cells invading the left orbital roof. Considering the extension of the tumor, the site and the connections with contiguous structures, a combination of endoscopic endonasal technique with intraorbital approach was performed. At histological examination typical features of benign osteoblastoma were observed. The sites of predilection for the tumor are the long bones, vertebral column, and small bones of hands and feet. Its occurrence in the skull and jaw bones is relatively rare and represents only 15% of all osteoblastomas. To our knowledge, only 5 cases of osteoblastoma of the frontal sinus have been previously reported in the English-language literature. This report describes a case of benign osteoblastoma in a rare site, namely, the frontal sinus with particular attention about the differential diagnosis and the treatment.    

Tracheostomy in childhood: new causes for an old strategy.

INTRODUCTION: A review of the available literature has shown that the indications, epidemiology, and complications for tracheostomies are changing, and that no definite guidelines have been established. In the 1970s, the most common indication for tracheostomies in children was acute inflammatory airway obstruction. Modern neonatal intensive care units (ICU) have turned long-term intubation into an alternative to a tracheostomy. Currently, long-term intubation has become the most important indication for tracheostomies in children. PATIENTS AND METHODS: We present our series involving tracheostomies performed in paediatric patients between 2004 and 2008 at our hospital. Sixteen patients underwent tracheostomies for respiratory failure and upper airway obstruction. RESULTS: The total complications rate was 37.5%. In children < 1 year of age, the complications rate was 25%, while in children > 1 year of age, the complications rate was 12.5%. CONCLUSIONS: Long-term intubation and its sequelae have now become one of the most important indications for tracheostomies in the paediatric age group.

Acoustic analysis of voice in Parkinson's disease: a systematic review of voice disability and meta-analysis of studies. / Análisis acústico de la voz en la enfermedad de Parkinson: revisión sistemática de la discapacidad vocal y metaanálisis de estudios.

AIM: To systematically review all the literature, focusing on instrumental quantitative assessment of voice in patients with Parkinson's disease (PD). Furthermore, a meta-analysis was performed to identify the main characteristics of voice disturbances in PD. PATIENTS AND METHODS: Literature searches with the keywords «Parkinson¼ and «voice¼ were conducted in PubMed, EMBASE, Cochrane Library and Web of Science. Main inclusion criteria were: clinically confirmed PD and instrumented measurement of voice parameters with acoustic analysis of voice. RESULTS: Fourteen publications met the inclusion criteria and were included in the meta-analysis. The data within the meta-analysis revealed that several voice parameters including jitter, shimmer and fundamental frequency variation presented significant variations between patients with EP and healthy controls. Significant variations of fundamental frequency, maximum phonation time, harmonic to noise ratio, standard deviation of fundamental frequency were observed, but with a high heterogeneity between the studies. On the other hand, significant variations of noise to harmonic ratio, s/z ratio, variation of amplitude were not observed. CONCLUSION: Acoustic analysis of voice, using an electronic system, allows the identification of changes in voice parameters for predicting the worsening of disease and for targeting specific intervention. Among the voice parameters, jitter and shimmer significantly increased in patients with PD.

TITLE: Análisis acústico de la voz en la enfermedad de Parkinson: revisión sistemática de la discapacidad vocal y metaanálisis de estudios.Objetivo. Revisar de manera exhaustiva la bibliografía referente a la evaluación instrumental cuantitativa de la voz en pacientes con enfermedad de Parkinson (EP) y realizar un metaanálisis para definir las principales características de los trastornos de la voz en la EP. Pacientes y métodos. Búsquedas bibliográficas con las palabras clave «Parkinson¼ y «voice¼ en PubMed, EMBASE, Cochrane Library y Web of Science. Los principales criterios de aceptación fueron: EP con confirmación clínica y medición instrumentada de los parámetros de la voz mediante análisis acústico. Resultados. Catorce publicaciones cumplieron los criterios de aceptación y se incluyeron en el metaanálisis. De los datos incorporados al metaanálisis, se dedujo que varios parámetros vocales, como el jitter, el shimmer y la variación de la frecuencia fundamental, presentan variaciones significativas en los pacientes con EP frente a los controles sanos. Se hallaron variaciones significativas de la frecuencia fundamental y de su desviación estándar, del tiempo máximo de fonación y de la razón armónicos-ruido, si bien con una alta heterogeneidad entre los estudios. En cambio, no se observaron variaciones sustanciales de la razón ruido-armónicos, en el índice s/z ni en la variación de la amplitud. Conclusión. El análisis acústico de la voz por medio de un sistema electrónico permite detectar los cambios de los parámetros vocales de cara a predecir el empeoramiento de la enfermedad y elegir una intervención específica. Entre dichos parámetros, el jitter y el shimmer aumentaron significativamente en los pacientes con EP.

A pilot pharmacokinetic-pharmacodynamic study of benzodiazepine antagonism by flumazenil and aminophylline.

STUDY OBJECTIVES: To develop a pharmacokinetic-pharmacodynamic model using quantitative electroencephalographic (EEG) analysis to compare two separate benzodiazepine antagonists and generate data concerning response variability. DESIGN: A pilot study using a randomized, blinded, crossover design. SETTING: The Neurology Laboratory at the Akron City Hospital campus of SUMMA Health System. PATIENTS: Four healthy volunteers completed the protocol. INTERVENTIONS: Subjects received midazolam 0.1-0.2 mg/kg by intravenous bolus on 3 study days, separated by a minimum washout period of 1 week. Subjects participated in an initial open-label response phase followed by a randomized, crossover trial of each benzodiazepine antagonist. MEASUREMENTS AND MAIN RESULTS: Venous blood samples were obtained to characterize the pharmacokinetics of all study compounds. The EEG parameter of total number of waves/second (recorded from FP1-F3 and FP2-F4 electrodes) in the frequency of 12-30 Hz was used to quantify effect. Flumazenil appeared to prolong the elimination half-life of midazolam significantly (p < 0.05). Theophylline (aminophylline) also appeared to prolong the half-life of flumazenil (p < 0.05). Despite considerable variability, flumazenil resulted in reversal of sedation at concentrations achieved by routine dosing. Resedation was apparent for all subjects following flumazenil reversal. Only partial reversal of sedation by theophylline was achieved by an aminophylline dose of 1-2 mg/kg. CONCLUSIONS: Flumazenil was consistently effective in reversing sedation by midazolam at routinely recommended dosing. Further investigation of aminophylline as a reversal agent should use an estimated dose of 6-8 mg/kg aminophylline. To achieve adequate reversal, some patients may require aminophylline dosages that exceed safe clinical administration.

Experience of endoscopic ultrasound in staging adenocarcinoma of the cardia.

AIM: The introduction of endoscopic ultrasonography (EUS) has generally resulted in a more accurate assessment of gastrointestinal regional tumour stage. Knowing the stage and the extent of oesophageal involvement is extremely important as a guide to the choice of surgical approach in cardia cancer. The aim of this study was to evaluate the ability of EUS to accurately predict depth of tumour invasion (T), node involvement (N) and the tumour's invasion length along the oesophagus. MATERIAL AND METHODS: Thirty-five patients with adenocarcinoma of the cardia were studied by EUS and the EUS pre-operative findings were compared with the pathology findings. RESULTS: The overall accuracy in T staging was 55.2% (16/29 cases). The sensitivity in evaluating T1, T2 and T3 classes was 80%, 38.5% and 70%, respectively. The sensitivity was excellent in evaluating N0 class (100%) (5 cases), but it fell to 66.7% in N1 cases. EUS correctly determined the extent of oesophagus invasion in 75.9% (22/29) of cases; moreover, it had a very high accuracy in distinguishing between tumours with an oesophageal invasion greater or lower than 2 cm (93.1%) (27/29 cases). CONCLUSION: EUS proved to be useful in pre-operative staging of cardia adenocarcinoma and, in particular, in the pre-operative identification of the extent of oesophageal invasion.

Pathological fractures in the proximal part of the femur treated by Zickel-nail fixation.

Twenty-one patients were treated with Zickel nail for neoplastic pathological fracture or impending pathological fracture: in the femoral neck in three, in the intertrochanteric region in three, and in the subtrochanteric region in fifteen. Nineteen patients were restored to bed-chain status by the third postoperative day, and twenty were able to walk. Failure of fixation did not occur and there were no infections.

Roentgenographic search for avascular necrosis of the head of the femur in alcoholics and normal adults.

Roentgenograms of 1,410 hips from 705 alcoholics were compared with those of 200 hips from 100 controls for changes consistent with non-traumatic (idiopathic) necrosis of the femoral head according to the findings diagnostic of necrosis of bone that were outlined by the Decompression Sickness Panel of the British Medical Research Council. Abnormalities were found in 45.3 per cent of the alcoholics and 49 per cent of the controls. None of the findings showed any statistical difference between the controls and the alcoholics. It was concluded that the roentgenographic findings used were not valid in detecting the early or subtle changes of necrosis of the femoral head and that the changes found in both groups are common and probably non-specific.

Benign osteoblastoma: range of manifestations.

A study of twenty-five new cases of osteoblastoma and a review of 172 previously reported cases demonstrated the wide spectrum of manifestations of the lesion. The ratio of males to females was 2:1, with 80 per cent of the patients being under thirty years of age. The duration of complaints prior to diagnosis averaged seventeen months. The majority of the lesions were found in the spine or major long bones of the lower extremity. When complete excision of the tumor was done a cure always was the result, but incomplete curettage also affected a cure in some cases. Three cases presented in detail emphasize the wide variation in biological behavior which this lesion may exhibit. Histological characteristics of aneurysmal bone cyst may frequently appear in the tumor tissue. Radiotherapy does not alter the course of the disease and appears to be contraindicated.

The malignant potential of enchondromatosis.

In a tri-institutional, retrospective study with long-term follow-up, forty-four patients who had multiple enchondromas were identified. Thirty-seven patients did not have hemangiomas (Ollier disease) and seven did (Maffucci syndrome). Of the thirty-seven patients who had Ollier disease, a low-grade chondrosarcoma developed in four; an astrocytoma, in one; and a granulosa-cell ovarian tumor, in one. In four of the seven patients who had Maffucci syndrome, there were six low-grade chondrosarcomas, one high-grade osteosarcoma, one pancreatic adenocarcinoma, one biliary adenocarcinoma, and one astrocytoma. None of the patients in either group died of the skeletal sarcoma, but four of five patients who had a non-skeletal malignant lesion died. From life-table analyses of these patients, we estimated that the incidence of secondary chondrosarcoma in patients who have Ollier disease is about 25 per cent at the age of forty years, and that malignant degeneration is almost a certainty in patients who have Maffucci syndrome. We concluded that periodic surveillance of the brain and abdomen for occult malignant lesions is indicated in patients who have enchondromatosis.

Eosinophilic granuloma and its variations.

The orthopedist is most likely to encounter solitary or multiple forms of eosinophilic granuloma of bone. Management includes curettage for diagnosis and treatment with insertion of autogenous bone. Radiation therapy and chemotherapy are reserved for patients with multiple lesions and for the less common forms of histiocytosis X, i.e., Hand-Schüller-Christian disease and Letterer-Siwe disease.

Helicobacter pylori: a comparison of some diagnostic methods.

We evaluated the performances of three methods (urease-test, cytology and culture) for the detection of Helicobacter pylori infection Fifty-five patients, 28 men and 27 women (ages, 19-77 years) were included in the study. Helicobacter pylori have been detected by urease-test in 41 patients (74.5%), by culture in 39 patients (70.9%), by cytology in 32 patients (58.1%). Urease-test and culture are direct and specific methods in diagnosing Helicobacter pylori infection.

[Superextended lymphadenectomy (D4) in the treatment of gastric adenocarcinoma]. / La linfadenectomia superestesa (D4) nel trattamento dell'adenocarcinoma gastrico.

BACKGROUND: The incidence of paraaortic lymph node metastasis (N4) in relation with the site of the tumour, and survival in patients with gastric cancer who underwent gastric resection and superextended lymphadenectomy (D4), have been analyzed. METHODS: The frequency of paraaortic lymph node metastasis was studied in 132 patients who underwent gastrectomy with D4 lymphadenectomy during the period June 1988 - December 2000. Six patients with plastic linitis and 3 with carcinoma of the gastric stump were excluded from the analysis. RESULTS: In personal experience the most frequent postoperative morbidity were respiratory complication (7.6%) and pancreatic fistula (6.8%). Among the 132 patients the total number of dissected nodes was 6362 and the mean number of dissected nodes per case was 48.2. The total number of retrieved lymph nodes from the paraaortic station was 755 with a mean number 5.7 per patients. N4 nodal involvement was found in 25 (19%) of 132 patients: 14 (36%) patients with carcinoma located in the proximal third, 5 (13%) with tumour located in the middle third and 6 (11%) with carcinoma of the distal third of the stomach. The median survival time and the overall cumulative 5-year survival rate for curatively (R0) resected patients were 74 months and 52% respectively. CONCLUSIONS: The presence of metastasis in paraaortic lymph nodes in 19% of our patients, the low morbidity and mortality, the good survival after superextended lymphadenectomy, suggest that this lymphadenectomy should be considered in the curative surgical treatment of advanced gastric cancer, especially if located in the proximal third of the stomach (N4 in 36% of cases).

[Goseki classification in adenocarcinoma of the cardia]. / La classificazione di Goseki nell'adenocarcinoma del cardias.

BACKGROUND: To assess an additional prognostic value of Goseki histological classification to TNM staging system in adenocarcinoma of the cardia. METHODS: Sixty-one patients curatively resected for advanced (T2, T3 and T4) cardia cancer at the I Division of General Surgery, University of Verona were classified in four different grades according to Goseki. Survival curves were estimated with Kaplan-Meier method and compared by the log-rank test. Multivariate analysis was performed by Cox regression model. c2 test was used to compare Goseki to Lauren classification and grading. After discharge from hospital all patients were followed with a mean follow-up of 39.5 months. RESULTS: Lauren classification and grading were significantly related to tubular differentiation (p<0.01). Kaplan-Meier estimates of survival showed a better 5-year outcome for tumors with good tubular differentiation (19%), even though the difference with poor tubular differentiated tumors was not statistically significant (p'0.06). Diffuse type carcinomas and tumors with poor cytological differentiation showed a worse prognosis at univariate analysis (p<0.01). Multivariate analysis showed no additional prognostic significance of any of the histological classification analyzed. Only T (p<0.02; RR 2.2; IC 1.2-4) and N (p<0.01; RR 5; IC 2.4-11) were independent prognostic factors. CONCLUSIONS: In adenocarcinoma of the cardia, Goseki classification did not add any information to Lauren classification and to TNM staging system.

[Abdominal metastasis of cardias adenocarcinoma]. / Studio della diffusione neoplastica addominale nell'adenocarcinoma del cardias.

BACKGROUND: The aim of the present study was to evaluate the incidence of abdominal lymph node involvement of adenocarcinoma of the gastric cardia in relationship with the site and depth of tumor invasion. METHODS: From July 1988 to April 1998, 79 patients with adenocarcinoma of the gastric cardia underwent surgical curative resection and D2 lymphadenectomy at the 1st Department of General Surgery of Verona University. Among these 79 patients, 16 had an adenocarcinoma of the distal esophagus (type I), 26 patients had an adenocarcinoma of the anatomic cardia (type II) and 37 had a subcardial adenocarcinoma (type III). The frequency of lymph node involvement in each of the lymph nodes as classified by the JRSGC were analyzed. RESULTS: In type I carcinoma positive lymph nodes occurred in 20% of pT1, 33% of pT2 and 100% of pT3. Positive nodes along the lower half of the stomach were never found. In type II carcinoma positive lymph nodes occurred in 57% of pT1, 86% of pT2 and 83% of pT3. Metastasis along the greater curvature in 18% of advanced cancers were found. In type III carcinoma positive lymph nodes occurred in 83% of pT2, 94% of pT3 and in 100% of pT4. Nodes along greater curvature were involved in 21% of advanced cases and also infrapyloric lymph nodes involved in 13% of cases. The type II and III advanced tumors had involved paraortic lymph node in 33% of cases. CONCLUSIONS: These results suggest that for tumors of the cardia an extended lymphadenectomy is necessary to ensure the removal of all metastatic nodes.

[Para-aortic lymph node involvement in gastric adenocarcinoma]. / Envahissement des ganglions para-aortiques dans les adénocarcinomes de l'estomac.

AIMS: To determine the significance of superextended lymphadenectomy (D4) in patients with gastric cancer. The incidence of para-aortic lymph node metastases (N4) was analysed as well as its relationship to the site of the tumour. PATIENTS AND METHODS: The frequency of para-aortic lymph node metastases was assessed in 110 patients who underwent gastrectomy with D4 lymphadenectomy during the period from June 1988 to October 1999; five patients with plastic linitis and three with carcinoma of the gastric stump were excluded from the study. RESULTS: The postoperative mortality rate was 2.7% (n = 3) and the postoperative morbidity rate was 29.1% (n = 32). In our experience the most frequent postoperative complications were pancreatic fistulas (7.3%) and respiratory complications (6.4%). Among the 110 patients, the total number of dissected nodes was 5245 and the mean number of dissected nodes per case was 47.7. The total number of retrieved lymph nodes from the para-aortic station level was 639, with a mean number of 5.8 per patient. N4 nodal involvement was found in 20 (18.2%) out of 110 patients: 12 (33%) patients with a carcinoma located in the proximal third, two (6%) with a tumour located in the middle third and six (15%) with a carcinoma of the distal third of the stomach. CONCLUSION: The presence of para-aortic lymph node involvement in 18.2% of the patients suggests that D4 lymphadenectomy should be considered in the curative surgical treatment of advanced gastric cancer, especially if located in the proximal third of the stomach (N4 + in 33% of the patients).

[Prognostic value of histologic classifications of advanced stomach cancer: comparative study of Lauren's and Goseki's classifications]. / Valore prognostico delle classificazioni istologiche del cancro dello stomaco avanzato: studio comparativo fra classificazione di Lauren e di Goseki.

Controversy exists about the prognostic value of the histological classifications of gastric cancer commonly used. Recently Goseki proposed a new classifying system based on intracellular mucus production and the degree of tubular differentiation. The aim of this study was to compare Lauren and Goseki classifications with particular emphasis on their prognostic significance. Eighty-nine patients, who underwent potentially curative resections (RO) and radical lymphadenectomy for advanced gastric cancer from September 1988 to April 1996 were analysed. Cox regression model was used to evaluate the prognostic significance of Goseki classification, Lauren classification, age, sex, type of lymphadenectomy, depth of tumour invasion (T), node metastases (N) and number of metastatic nodes. A statistically significant correlation between the different Goseki grades and histology according to Lauren was found (p < 0.001). By multivariate analysis the only parameters predictive of long term outcome were depth of tumour invasion, nodal status and histology according to the Lauren classification. Also after excluding the Lauren classification from the analysis, the Goseki histological grading system did not affect survival independently. This study on advanced gastric cancer patients identified depth of invasion, lymph node metastases and Lauren classification as significant independent pathological variables influencing survival. The classification proposed by Goseki did not add anything further to the prognostic informations provided by TNM staging and Lauren classification.

[Results of the surgical treatment of primary gastric lymphoma]. / Risultati del trattamento chirurgico del linfoma gastrico primitivo.

The aim of the study was to verify the long term results obtained in primary gastric lymphoma with a strategy consisting in surgery as first-line treatment. Over the period from January 1988 to December 1999, 44 patients with histologically proven primary gastric lymphoma underwent surgical treatment in the First Department of General Surgery of the University of Verona. Tumours were staged according to the Ann Arbor classification and divided, according to the Kiel classification, into high- and low-grade lymphoma. Patients received adjuvant chemotherapy depending on the grade of malignancy and/or completeness of resection. Of the 44 patients, 40 (90.9%) underwent curative resections, i.e. with complete macroscopic and microscopic tumour removal (R0), consisting in total gastrectomy in 34 cases and subtotal gastrectomy in 6. Twenty-five of 40 patients had stage IE and 15 stage IIE tumours. Adjuvant chemotherapy was given to 33 patients (30 high-grade lymphomas and 3 low-grade lymphomas with N2 metastases). The overall cumulative 10-year survival rate in patients who underwent R0 resection was 79% without any significant differences in 10-year survival between patients with high- and low-grade malignancy (both 79%; P = 0.582) or between patients with or without lymph node metastases (91% and 70%, respectively; P = 0.426). In conclusion, the present investigation suggests that surgery yields prolonged complete remission in a high percentage of patients affected by gastric lymphoma irrespective of histopathologic grade of the disease and nodal involvement.