A man with a big tumour on his back

A 47-year-old man was seen with a big tumour on his back. The tumour had started growing a year earlier, but the man never consulted a doctor. Histopathology showed melanoma, and there were no signs of metastastatic disease. The tumour was surgically removed.     

阴道间质瘤的临床表现、病理与治疗

目的:探讨胃肠道外阴道间质瘤(extra-gastrointestinalstromalstromaltumour,EGIST)临床表现、病理、治疗。方法:对1例于6月3日~7月13日住院治疗的患者(术后病理分析为中度恶性阴道间质瘤)用免疫组化方法观察4种抗体CD117、CD34、S-100、SMA并进行分析。结果:CD117(+)、CD34(+)、S-100(-)、SMA(-)。结论:胃肠道外间质瘤诊断主要依靠病理及免疫组化,诊断明确有利于该病的治疗及预后。     

A case of multiple jejunal diverticula with strongyloidiasis and malabsorption syndrome

We reported multiple jejunal diverticula with strongyloidiasis and malabsorption syndrome. To our knowledge, 140 cases including the present one have been reported in Japan. We reviewed these cases in this report.     

Diagnostic image (235). A woman with a tumour on the thorax

An 81-year-old woman presented with a tumour on the right hemithorax due to transthoracic progression of an intrathoracic reactivated tuberculosis infection.     

Familial jejunal atresia with 'apple-peel' variant.

We report two siblings who had jejunal atresia which we believe to be familial. The parents of these siblings were first cousins. The first child had jejunal atresia with mesenteric agenesis and 'apple-peel' configuration; the second child had jejunal atresia with a V-shaped mesenteric defect. Other reported cases of familial atresia of the small intestine are reviewed.     

Diagnostic image (376). A man with a tumour in the anal seam

A 50-year-old man presented with a sacral tumour due to a sacrococcygeal chordoma.     

Basal metabolic rate in children with a solid tumour

OBJECTIVE: To study the level of and changes in basal metabolic rate (BMR) in children with a solid tumour at diagnosis and during treatment in order to provide a more accurate estimate of energy requirements for nutritional support. DESIGN: An observational study. SETTING: Tertiary care at the Centre for Paediatric Oncology, University Hospital Nijmegen. SUBJECTS: Thirteen patients were recruited from a population of patients visiting the University Hospital Nijmegen for treatment. All patients asked to participate took part in and completed the study. INTERVENTION: BMR was measured by indirect calorimetry, under stringent, standardised conditions, for 20 min and on three different occasions in all patients. Continuous breath gas analysis using a mouthpiece was performed. Weight, height and skinfold measurements were performed before each measurement. MAIN OUTCOME MEASURES: BMR was expressed as percentage of the estimated reference value, according to the Schofield formulas based on age, weight and sex, and in kJ (kcal) per kg of fat-free mass. RESULTS: At diagnosis, the BMR was higher than the estimated reference BMR in all patients and 44% of the patients were considered hypermetabolic. Mean BMR (as percentage of reference) was significantly increased (11.6% (s.d. 6.7%); P=0.001), but decreased during treatment in 12 of the 13 patients (mean decrease 12.7% (s.d. 3.9%); P<0.0001). Furthermore, a significant negative correlation (P=-0.67; P=0.01) was found between the change in BMR and tumour response. CONCLUSIONS: These data suggest that the BMR of children with a solid tumour is increased at diagnosis and possibly during the first phase of oncologic treatment. This may be important when determining energy requirements for nutritional support.     

Gastric adenocarcinoma with lymphoid stroma. About nine cases

AIM: the purpose of this work is to study the pathological features and the outcome of lymphoid stroma gastric carcinoma and its relation with Epstein-Bar Virus (EBV) and the imortance of the EBV serology in detection of early reccurence. METHODS: between january 1990 and december 2004, 155 patients underwent gastric resection for gastric carcinoma. Nine of them had lymphoid stroma gastric carcinoma. Over all survival as well as disease free survival were determined. Detection of reccurence was based on clinical exam and on findings yielded by endoscopy, radiological exams and EBV serology. A comprison of survival rate of patients beteween current gastric carcinoma and those with lymphoid stroma gatsric carcinoma was done using the Log Rank test. RESULTS: the patients (7 men and 2 women) had a mean age of 61 years (41 to 82). The tumor was in the antrum in 4 cases, in the body of the stomach in 3 cases and in the cardia in 2 cases. The mean tumor size was 8 cm with deep invasion of the stomach wall in 5 cases and ganglionnary invasion in 6 cases out of 9. EBV was detected in all patients by in situ hybridation. Post operative morbidity and mortality were nil. Global survival and disease free survival were, respectively, 53.3% and 65.6% in lymphoid stroma gatsric carcinoma, and 43.4% and 63.5% for current gatsric carcinoma. Three patients died of a reccurent disease after 6, 30 and 33 months. The remaing 6 patients didn't have signs of reccurence. CONCLUSION: lymphoid stroma gastric carcinoma is a bulky, local disease. In our patients, it was always associated with EBV. Over all survival was better than survival of current gastric carcinoma, although there was no significance. EBV serology can be an effective screening procedure of reccurence.     

Partial jejunal volvulus due to a mesenteric cystic lymphangioma

Mesenteric cysts are unusual benign tumours that include lymphangioma. Their clinical presentation is variable and acute symptoms can be produced due to complications. This tumour appears especially in childhood, and its prognosis after surgical removal is excellent. We present the case of a 15 year old female patient with symptoms of postprandial abdominal pain and palpation of smooth mass in hypogastrium. Radiological studies showed a big polilobular mass of cystic substance that included a portion of jejune with incomplete volvulus. The treatment was the removal of the cyst and a jejunal portion and the pathological diagnosis was mesenteric cyst lymphangioma. The patient is asymptomatic more than three years after the intervention.     

A retrospective review of the course of patients with pancreatitis discharged on jejunal feedings.

INTRODUCTION: Although parenteral nutrition (PN) has been the standard nutrition therapy in patients with pancreatitis requiring nutrition support, it is associated with a higher rate of catheter-related sepsis and gut atrophy. Research suggests enteral nutrition (EN) is possible in patients with pancreatitis without exacerbating symptoms when infused jejunally. The purpose of this study was to review the course of patients with resolving pancreatitis discharged to home on EN. METHODS: The medical records of 33 patients with a percutaneous endoscopic gastrostomy tube with jejunal extension (PEG-J) or nasojejunal tube (NJ) who received home EN were reviewed. The data collected included duration of EN, formula used, goal and maximum tolerated flow rates, anthropometric measures, and gastrointestinal complications. RESULTS: Ninety-seven percent of patients received a standard polymeric formula providing an average of 1845 +/- 421 kcal/d. Forty-two percent of patients took pancreatic enzyme supplements. Complications occurring in patients included nausea and vomiting (42%), feeding rate intolerance (18%), diarrhea (12%), and PEG site infection (27%). Seventy-seven percent of patients achieved nutritional goals. CONCLUSION: Standard polymeric EN seems to be safe and efficacious in the home setting for patients with resolving pancreatitis.     

Imatinib, a specific tyrosine-kinase inhibitor, for the treatment of patients with a gastrointestinal stroma cell tumor

The c-kit oncogene, encoding for a transmembrane growth-factor receptor with tyrosine-kinase activity (KIT), plays an important role in the tumourigenesis of gastrointestinal stroma-cell tumours (GISTs). GISTs are a heterogeneous group of mesenchymal tumours, mainly localised in the abdomen. Immunohistochemical staining using antibodies directed towards the c-kit protein on the cell surface, is a useful tool in the often difficult differential diagnosis of GISTs from other mesenchymal tumours. The correct diagnosis of GISTs is crucial for the new treatment option with imatinib, the tyrosine-kinase inhibitor specifically targeted against KIT. The clinical development of imatinib for patients with GISTs has been extraordinarily rapid. As a result of the short period of time between the drug's experimental introduction and actual registration, many unanswered questions about its use for the treatment of GISTs remain. These can only be answered in well-designed clinical trials. It is therefore recommended that patients with these rare tumours are referred to a centre with experience in the diagnosis and treatment of these soft-tissue sarcomas.     

血管内皮素-1和血管内皮细胞生长因子在胃肠道间质瘤中的表达及相关性研究

目的 探讨血管内皮素-1(endothelin-1,ET-1)和血管内皮细胞生长因子(VEGF)在胃肠道间质瘤(GISTs)中的表达及其在预后判断中的价值.方法 应用免疫组织化学S-P法对86例GISTs检测,观察ET-1和VEGF在GISTs中的表达情况.结果 ET-1和VEGF在GISTs中的阳性表达率分别为66.28％ (57/86)和72.09％(62/86),两者的表达呈正相关性(r=0.347,P＜0.01),GISTs中ET-1、VEGF的表达与患者性别、年龄和原发部位均无明显相关性(P＞0.05);在极低危和低危、中危、高危三组中,ET-1、VEGF的阳性表达率分别依次为40.00％(6/15)、65.22％(30/46)、84.00％(21/25)和33.33％ (5/15)、76.09％ (35/46)、88.00％ (22/25),阳性表达率均随恶性程度增加而升高(x2 =8.171,14.710,P＜0.05);有浸润转移组、无浸润转移组ET-1、VEGF的阳性表达率分别为76.47％ (39/51)、51.43％ (18/35)和88.24％ (45/51)、48.57％ (17/35),差异有统计学意义(x2=10.002,16.230,P＜0.05);在肿瘤直径＜2 cm、2～5 cm和＞5 cm三组,ET-1、VEGF的阳性表达率分别依次为33.33％ (5/15)、61.11％ (11/18)、77.36％ (41/53)和40.00％ (6/15)、66.67％(12/18)、83.02％ (44/53),随直径增大依次升高.直径＜2 cm和＞5 cm组间比较差异均有统计学意义(x2=10.354,11.116,P＜0.05);在细胞核分裂数＜5/50HP和≥5/50HP两组,ET-1、VEGF的阳性表达率分别为39.39％(13/33)、83.02％ (44/53)和45.45％(15/33)、88.68％ (47/53),后者均明显高于前者,差异有统计学意义(x2=17.317,18.886,P＜0.05).ET-1和VEGF阳性患者5年生存率均显著低于相应阴性组患者(x2 =6.124,6.287,P＜0.05).结论 ET-1和VEGF在GISTs中的表达有联合倾向,可能提示肿瘤的恶性程度及其预后,并可作为判断预后的有用标志物.同时可能给GISTs的抗血管分子治疗提供理论基础.