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1.
Guo-Jing Zhang Ruo-Tong Li Yang Zhou Fei Huang Zhi-Cheng Zhao Wei-Dong Li Wei-Hua Fu 《International journal of clinical and experimental pathology》2015,8(9):11691-11697
Solitary fibrous tumor (SFT) which is an extremely rare clinical entity has been reported infrequently. Most commonly it is distinguished into pleural and extrapleural forms, with same morphological resemblance. There has been many literatures reported regarding extrapleural form of SFT but few cases of SFT originating from small bowel mesentery have been reported till now. We here report one case of SFT of small bowel mesentery with some eventful postoperative bowel obstruction and literature review. 相似文献
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Kuroda K Ishizawa S Kudo T Uotani H Hosokawa A Tanaka T Kitano H Hori T Tsukada K Sugiyama T Fukuoka J 《Pathology international》2008,58(4):239-243
Malignant mesothelioma is an uncommon lethal neoplasm in the serous membrane in which peritoneal mesothelioma is a rarer form. Herein is reported a case of malignant mesothelioma presenting as a localized mass inside the mesentery causing focal luminal obstruction of the small intestine. The diagnosis of malignant mesothelioma was obtained on repeat double balloon endoscopic biopsy. Partial resection of the small intestine along with the mesentery was performed, followed by a course of chemotherapy. No relapse of the disease has been found in the 8 months' follow up radiologically. To the best of the authors' knowledge this is the first reported case of localized malignant mesothelioma arising inside the mesentery. Mesothelioma should be considered as the differential diagnosis when small bowel obstruction occurs with unknown primary neoplasm. 相似文献
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Angiosarcoma is a rare soft-tissue neoplasm occurring most often in the skin and the subcutaneous tissues and very rarely in the gastrointestinal tract. We report a case of a 25-year-old woman who presented with a small intestinal angiosarcoma associated with angiosarcomatosis. The diagnosis was established on surgical intestinal resection, that showed a high-grade angiosarcoma with epithelioid component and foci of agressive form of hemangioendothelioma. Immunohistochemical study revealed tumour cell positivity with endothelial markers CD31 and factor VIII whereas CD34 and epithelial markers were negative. The tumour displayed KIT (CD117) immunoreactivity without KIT or PDGFRA mutation on molecular analysis. Clinical and pathological features as well as differential diagnosis of this rare entity in gastrointestinal tract are discussed. 相似文献
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Mesenteric fat necrosis causing bowel obstruction is a rare occurrence with only one case reported in humans. It is due to accidental or surgical trauma to the adipose tissue with extracellular liberation of fat or enzymatic lysis of fat due to the release of lipases resulting in fibrosis. Preoperative imaging may often be misleading and fail to identify fat necrosis as the cause of bowel obstruction. As surgical intervention is the only suitable treatment option in cases of failed conservative treatment, the diagnosis is made postoperatively. There is no published advice on the management of mesenteric fat necrosis. We recommend safe operating techniques to minimize the risk of developing fat necrosis and its potential harmful consequences. 相似文献
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We report a case of a child with features of Down syndrome (DS) but with an atypical karyotype. Initial chromosome analysis was 46,XX,dup(21q).ish 21(wcp21+). The father's chromosomes were normal. However, the mother was found to have mosaicism for a pericentric inversion of chromosome 21 (19/30 cells). The revised chromosome result of the child was 46,XX,rec(21)dup(21q)inv(21)(p12q21.1)mat. A literature review of similar cases (hereafter referred to as rec dup(21q)) was conducted to aid counselling about recurrence risks and the prognosis for this child. All previous reports of rec dup(21q) were secondary to a maternal pericentric inversion. Male carriers did not seem to be at risk of having offspring with the rec dup(21q), although the number of male carriers was limited. In those with rec dup(21q), the risk of congenital heart disease was similar to that of trisomy 21. In reported cases, the facial appearance was suggestive of Down syndrome but perhaps less striking. Although the data are limited, there is an indication the developmental disabilities and short stature are milder in those with rec dup(21q) compared to trisomy 21. These observations promote the concept that the region of chromosome 21 proximal to the duplication contains genetic information contributing to the expression of some features of Down syndrome. 相似文献
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目的探讨唐氏综合征和畸胎瘤发生可能存在的相关性。方法通过对一例唐氏综合征合并畸胎瘤报告及相关文献复习,概述唐氏综合征可能引起畸胎瘤的原因。结果唐氏综合征患者生殖细胞成熟延迟,癌基因高表达,DNA抗损伤及修复能力下降,促性腺激素水平增高等因素是引起原始生殖细胞突变形成畸胎瘤的原因。结论鉴于唐氏综合征和畸胎瘤以及生殖细胞肿瘤的相关性,临床医生应重视相关检查。 相似文献
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Schnater JM Schouten-van Meeteren AY Heins YM Aronson DC 《Cancer Genetics and Cytogenetics》2005,156(1):77-79
After an uneventful pregnancy, a boy was born by vacuum extraction at 40.6 weeks' gestation. Physical examination revealed several malformations due to a partial trisomy 9p [karyotype: 46,XY, dup(9)(p13p24)]. Three months after birth, the boy presented with a hepatoblastoma without distant metastases which was treated with chemotherapy combined with surgery. At the last follow-up, 15 years after the resection of the hepatoblastoma, he was still in complete remission. To our knowledge this is the first case report of a patient with a constitutional partial trisomy 9p associated with hepatoblastoma. 相似文献
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We report a fortuitous occurrence of a small bowel leiomyosarcoma infected with Schistosoma mekongi in an elderly patient from Kampuchea. S mekongi is endemic in countries along the lower Mekong River basin (Laos, Thailand, and Kampuchea), where its intermediate snail hosts (Lithoglyphopsis aperta) are found. The diagnosis was made by examining the numerous ova that were embedded in the tumor and along the small bowel wall. The ova with their small lateral spines are easily confused with those of Schistosoma japonicum, but are consistently smaller and round. The patient presented with lower gastrointestinal bleeding secondary to tumor invasion of the small bowel rather than from heavy parasitic infection. Because of the spindle-cell morphology of the tumor, an exuberant fibroblastic host reaction to the ova was considered during frozen section examination. Unlike the more common species of schistosoma (eg, Schistosoma mansoni and Schistosoma haematobium) that are associated with the development of malignant neoplasms, we believe our case represents a chance finding and that the possible role of S mekongi infection in the neoplastic process is unlikely. 相似文献
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Morgagni hernia is a rare diaphragmatic hernia accounting for only 2% of the congenital diaphragmatic defects. A case of Morgagni hernia was diagnosed radiologically in a 12-months-old male with Down syndrome, with recurrent respiratory distress and chest deformity. The 2-dimensional echocardiography was normal. The diagnosis of Morgagni hernia was confirmed by barium studies. The patient underwent a corrective surgery at 18 months of age following which his symptoms subsided. Literature review revealed only 18 cases of Morgagni hernia with Down syndrome reported till date, with age of presentation varying from neonatal age group to 12 years of age. The mode of presentations varied from asymptomatic detection to severe respiratory distress. The possible mechanism of association and its clinical implication has been discussed. The case emphasises a need for diaphragmatic hernia to be looked for as a possible cause of respiratory distress in Down syndrome. 相似文献
12.
Koukoulis G Ke Y Henley JD Cummings OW 《Archives of pathology & laboratory medicine》2001,125(10):1331-1334
CONTEXT: The pathology of small bowel obstruction in Crohn disease has not been studied extensively. Stricture formation has been attributed mainly to fibrosis, although muscularization of the submucosa has been discussed previously. OBJECTIVE: To identify additional pathologic changes in Crohn disease that could be involved in the formation of strictures. DESIGN: We reviewed 50 ileal resections from patients with Crohn disease. The histopathologic slides were reviewed initially without knowledge of the macroscopic or clinical findings. We identified an unusual muscular proliferation that we refer to as obliterative muscularization of the submucosa, defined as a thick and continuous muscle layer from the mucosal base to the muscularis propria that is at least 1 cm in length. Subsequently, histopathologic findings were correlated with macroscopic and clinical findings. RESULTS: Obliterative muscularization of the submucosa was present in 14 specimens, and in 11 of these 14 it was topographically restricted to strictures. Submucosal fibrosis was observed in sections from adjacent regions. Obliterative muscularization of the submucosa, including thick-walled vessels and hyperplastic nerves but not prominent scarring, was more common in specimens with strictures; the difference was statistically significant (P <.001). CONCLUSIONS: Obliterative muscularization of the submucosa may be pathogenetically involved in the formation of strictures either directly by causing a sustained spasm, or indirectly by minimizing the vasoprotective role of the submucosa, impairing repair and enhancing scarring. 相似文献
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This paper reports on the successful treatment by in-vitro fertilization (IVF) of a couple in whom the male partner had Kartagener's syndrome. His spermatozoa were severely asthenozoospermic with deficient dynein arms and disordered microtubular configuration. On computer-assisted sperm analysis (CASA) motile spermatozoa displayed straight non-progressive motility with minimal amplitude of lateral head displacement and none were hyperactivated. This is the first case report in which spermatozoa with axonemal disruption in a man with immotile cilia syndrome (ICS) have been shown to be able to penetrate the zona pellucida and fertilize oocytes. IVF may be a suitable treatment for certain variants of ICS. 相似文献
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We experienced a rare case of thoracic outlet syndrome caused by hypertrophic nonunion of the first rib. A diagnosis was made mainly upon provocative tests and imaging studies. Pain and tingling could be reproduced and the radial pulse obliterated by the hyperabduction test. Abundant callus formation on the posterior aspect of the first rib with fracture line was visible on plain radiograph. Two-dimensional computed tomography showed right thoracic outlet narrowing mainly caused by the mass-effect of the callus. Dynamic arteriographic studies revealed an external compression of the right subclavian artery and duplex ultrasonography demonstrated a reduction in right subclavian artery blood flow when the shoulder is in 90 degrees of abduction. Surgery was performed after the conservative management for three months which failed to relieve the patient of his complaints. Resection of the first rib via transaxillary approach was undergone uneventfully in combination with the myotomy of the scalenus anticus muscle. At postoperative one year follow up, the patient was free of symptoms, and had a full range of motion of the right shoulder with no evidence of arterial insufficiency. 相似文献
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Der Sheng Sun Ok Ran Shin Young Mi Ku Young-Seok Kim Kyung-Jin Seo 《International journal of clinical and experimental pathology》2014,7(9):6345-6349
Squamous cell carcinoma arising from the small intestine is rare and difficult to identify as a primary or metastatic feature. We report a case of small intestinal squamous cell carcinoma manifesting as subacute peritonitis due to perforation. An 80-year-old man was admitted to our hospital with intermittent postprandial abdominal pain. He was diagnosed with acute peritonitis due to gastrointestinal perforation. During explorative laparotomy, a perforation site was detected in the jejunum and segmental resection to correct the perforation was performed including the perforation site located at the 70 cm inside the jejunum from the Treitz ligament. The pathology results revealed squamous cell carcinoma in the resected segment of the jejunum with two perforation sites. 相似文献
18.
Jhala DN Eltoum I Carroll AJ Lopez-Ben R Lopez-Terrada D Rao PH Pettenati MJ Siegal GP 《Human pathology》2003,34(12):1354-1357
Osteosarcomatous transformation in fibrous dysplasia is unusual. The incidence is increased in patients with concomitant Mazabraud's syndrome and McCune-Albright syndrome. We report the cytological, histological, and cytogenetic findings of this rare entity arising from a mass in the right elbow of a 44-year-old African-American woman. The fine-needle aspiration (FNA) findings were diagnostic of malignancy, with markedly atypical spindle and polygonal cells admixed with osteoid. The diagnosis of osteosarcoma by FNA was subsequently further confirmed by histological evaluation of an above-elbow amputation specimen. Fluorescence in situ hybridization and comparative genomic hybridization demonstrated trisomies of chromosomes 5 and 7 in the fibrous dysplasia and osteosarcoma. In addition, multiple chromosomal abnormalities were also noted in the osteosarcoma. We are unaware of any previous reports of the cytogenetic findings in the tissue of this rare condition, and argue for the value of FNA in the evaluation of such patients under selected conditions. 相似文献
19.
Um JW Kim KH Kang MS Choe JH Bae JW Hong YS Suh SO Kim YC Whang CW Kim SM 《Journal of Korean medical science》1999,14(6):679-681
Macroamylasemia is a condition of persistent, elevated serum amylase activity with no apparent clinical symptoms of a pancreatic disorder. In Korea, however, no such case has been reported to date. We report a case of a 17-year-old female diagnosed with macroamylasemia and acute appendicitis. One day earlier, she developed epigastric and right lower quadrant abdominal pain. She was characterized by high level of serum amylase, but normal lipase. Amylase isoenzyme analysis demonstrated increased fraction of salivary type and follow-up amylase level was persistently increased. Immunofixation disclosed the macroamylase binding with an immunoglobulin, consisting of IgA and kappa chain. The patient was treated by appendectomy, and the abdominal pain subsided. 相似文献