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1.
Maria Sandbacka Mervi Halttunen Varpu Jokimaa Kristiina Aittomäki Hannele Laivuori 《Orphanet journal of rare diseases》2011,6(1):1-5
Background
Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment.Methods
From January 2000 all patients under 18 years of age diagnosed with "rare paediatric tumours" were centrally registered by the Italian centres participating in the TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]). The clinical data of children with a thymic epithelial tumour registered as at December 2009 were analyzed for the purposes of the present study.Results
Our series comprised 4 patients with thymoma and 5 with carcinoma (4 males, 5 females; median age 12.4 years). The tumour masses were mainly large, exceeding 5 cm in largest diameter. Based on the Masaoka staging system, 3 patients were stage I, 1 was stage III, 1 was stage IVa and 4 were stage IVb. All 3 patients with stage I thymoma underwent complete tumour resection at diagnosis and were alive 22, 35 and 93 months after surgery. One patient with a thymoma metastasizing to the kidneys died rapidly due to respiratory failure. Thymic carcinomas were much more aggressive, infiltrating nearby organs (in 4 cases) and regional nodes (in 5), and spreading to the bone (in 3) and liver (in 1). All patients received multidrug chemotherapy (platinum derivatives + etoposide or other drugs) with evidence of tumour reduction in 3 cases. Two patients underwent partial tumour resection (after chemo-radiotherapy in one case) and 4 patients were given radiotherapy (45-54 Gy). All patients died of their disease.Conclusions
Children with thymomas completely resected at diagnosis have an excellent prognosis while thymic carcinomas behave aggressively and carry a poor prognosis despite multimodal treatment. 相似文献2.
Amarjit Anand Eva Maria Tsapakis Ali A Narvani Ali Alhakim Steve R Cannon Eleftherios Tsiridis 《World journal of surgical oncology》2007,5(1):1-3
Background
As a result of improvements in diagnostic accuracy, the primary source of the tumour is identified in more than 99% of cases presenting with a malignancy. Whilst the axial skeleton is a common site of metastases, the sternum is rarely affected, especially by isolated metastases.Case presentation
We report a case of a 68 year old male who was referred to the surgical outpatient clinic with a six month history of sternal pain. The patient was known to have essential thrombocythaemia, which had recently transformed into acute myeloid leukaemia but a sternal biospy showed mucinous adenocarcinoma. He had not localising symptoms and full evaluation failed to localise the primary tumour.Conclusion
Solitary sternal metastases are rare and when found an underlying neoplasm is usually identified allowing targeted treatment. If however, there is no symptomatic tumour, the metastasis should simply be treated symptomatically. 相似文献3.
Background
Pericardial tumours are unusual and may be difficult to characterise with imaging. They manifest as large, non-contractile, solid masses within the pericardium. Presenting symptoms include heart failure, arrythmias, sudden death, cyanosis and chest pain.Case presentation
We describe a case of massive pericardial fibroma in a 52 year old woman, who presented with palpitations only.Conclusion
We illustrate the different imaging modalities available to image this tumour prior to surgical resection, and indicate the strengths and weaknesses of each. 相似文献4.
L Zachau C Zeckey J Schlue J Sander C Meyer-Heithuis M Winkler J Klempnauer H Schrem 《World journal of surgical oncology》2012,10(1):1-8
Background
The guidelines established by the National Comprehensive Cancer Network do not describe mucinous histology as a clinical factor that should influence the therapeutic algorithm. However, previous studies show conflicting results regarding the prognosis of colorectal mucinous adenocarcinoma. In this study, we described the clinicopathological features of mucinous adenocarcinoma in Japan, to identify optimal therapeutic strategies.Methods
144 patients with mucinous and 2673 with non-mucinous adenocarcinomas who underwent primary resection in two major centers in Yokohama, Japan were retrospectively evaluated for clinicopathological features and treatment factors. A multivariate analysis for overall survival followed by the comparison of overall survival using Cox proportional hazard model were performed.Results
Patients with mucinous adenocarcinoma had larger primary lesions, higher preoperative CEA levels, a deeper depth of invasion, higher rates of nodal and distant metastasis, and more metastatic sites. A multivariate analysis for overall survival revealed a mucinous histology to be an independent prognostic factor. In the subgroup analysis stratified by stage, Patients diagnosed as StageIII and IV disease had a worse survival in mucinous adenocarcinoma than non-mucinous, while survival did not differ significantly in patients diagnosed as Stage0-II disease. In StageIII, local recurrence in rectal cases and peritoneal dissemination were more frequently observed in patients with a mucinous histology.Conclusions
Our study indentified that mucinous adenocarcinoma was associated with a worse survival compared with non-mucinous in patients with StageIII and IV disease. In rectal StageIII disease with mucinous histology, additional therapy to control local recurrence followed by surgical resection may be a strategical alternative. Further molecular investigations considering genetic features of mucinous histology will lead to drug development and better management of peritoneal metastasis 相似文献5.
Tieppo C Betterle C Basso D Mescoli C Rugge M Martini C Zorzetto V Maddalo G Cazzagon N Nitti D Farinati F 《Cancer immunology, immunotherapy : CII》2011,60(7):1057-1060
Context
Gastric type I carcinoid is a rare neoplasm, deriving from enterochromaffin-like cells (ECL), mainly affecting women with autoimmune gastritis. The approach to treatment, either endoscopic, medical or surgical, is not well defined, particularly in multifocal tumours or carcinoids with rapid growth/frequent recurrence.Objective
To determine whether an anti-G17 vaccination might interfere on the natural history of gastric type I carcinoid.Setting
Padua teaching Hospital, outpatient clinic.Design and patients
Three patients with type I gastric carcinoid in autoimmune gastritis were administered, after informed consent and ethic committee approval, with a vaccine against gastrin 17 (G17), a synthetic peptide that stimulates specific and high-affinity anti-G17 antibodies, and followed up endoscopically and clinically for a mean of 36?months.Main outcome measures
Gastric histology and specifically carcinoid growth/recurrence and trend in time in gastrin, G17, pepsinogens, chromogranin A and clinical parameters.Results
Following vaccination, carcinoid regression was observed in 2/3 patients and, in one of the patients, even the disappearance of ECL hyperplasia, with a reduced ECL cells stimulation, confirmed by a significant reduction in chromogranin A levels. Regression was observed in the two patients that showed a more clear local response to the vaccine. Increased autoantibody titre was observed, but no appearance of new autoimmune diseases.Conclusions
Anti-G17 vaccination induced regression of type I gastric carcinoid and could be considered for the treatment of this tumour, when endoscopic removal is not indicated. 相似文献6.
Background
Leiomyomas are benign tumours that originate from smooth muscles. They are often seen in the uterus, but also in the renal pelvis, bladder, spermatic cord, epididymis, prostate, scrotum or the glans penis. Leiomyomas of the tunica albuginea are extremely rare.Case presentation
A 59-year-old white male has noted an asymptomatic tumour on the right side of his scrotal sac for several years. This tumour has increased slowly and caused local scrotal pain. An inguinal incision was performed, in which the hypoplastic testis, the epididymis and the tumour could be easily mobilized. Macroscopically the tumour showed a solid round nonencapsulated whorling cut surface. Histologically the diagnosis of a leiomyoma was made.Conclusion
We report here a very interesting and rare case of a leiomyoma of the tunica albuginea. Leiomyomas can be a possible differential diagnosis in this area.Virtual Slides
http://www.diagnosticpathology.diagnomx.eu/vs/2585095378537599 相似文献7.
Moayad M Aljarabah Neil R Borley James MD Wheeler 《International Seminars in Surgical Oncology : ISSO》2007,4(1):20
Background
appendiceal tumours are rare, they may be encountered unexpectedly in any acute or elective abdominal operation, many of these tumours are not appreciated intraoperatively and are diagnosed only during formal histopathological analysis of an appendicectomy specimen. Herein we present a case of appendiceal adenocarcinoma presenting as left-sided large bowel obstruction, we also review the literature of unusual presentations of appendiceal tumours.Case Presentation
we report a case of left sided large bowel obstruction found to be secondary to an appendiceal adenocarcinoma. The patient presented with abdominal pain, distension and constipation, CT scan showed large bowel obstruction thought to be due to a sigmoid tumour, on laparotomy the appendix was also noted to be abnormal. A low Hartman's was performed with en-bloc total hysterectomy and bilateral salpigo-oophorectomy. A separate ileocaecal resection with end ileostomy was also performed, pathology specimens showed that the primary neoplasm was the appendix with metastasis to the distal sigmoid.Conclusion
appendiceal tumours are rare, they usually present as acute appendicitis, other presentations are far less common.8.
Luigi Boni Angelo Benevento Gianlorenzo Dionigi Francesca Rovera Mario Diurni Renzo Dionigi 《World journal of surgical oncology》2005,3(1):1-7
Background
Parathyroid carcinoma is a rare malignancy, with an incidence of 0.5 to 4% of all cases of primary hyperparathyroidism. Surgery is the only curative treatment.Case presentation
We report the case of a 66-year-old man referred for a large suspicious substernal goitre associated with severe hypercalcemia due to hyperparathyroidism. After normalization of serum calcium levels, patient underwent surgery. The voluminous cervicomediastinal firm mass could not be removed through the cervical incision; therefore a cervicothoracic approach was employed. Histopathology revealed a giant parathyroid cancer of 450 grams. A review of the literature was also undertaken to summarize the current treatment approaches for this rare malignancy.Conclusion
Parathyroid cancer is usually not recognized either preoperatively or intra-operatively. En bloc resection of the tumour with the adjacent tissue is the treatment of choice and it is very important to avoid the rupture of the capsule during operation. Neither tumour size, nor the lymph node status appears to play a role in prognosis. The management of parathyroid carcinoma is a challenge even for experienced surgeons. 相似文献9.
Yamamoto Daigo Yamada Masanori Okugawa Homa Tanaka Kanji 《World journal of surgical oncology》2004,2(1):1-4
Background
Malignant pleural mesothelioma is a rare malignancy. The outcome remains poor despite complete surgical resection.Patients and methods
Eleven patients with histologicaly proven epithelial type malignant pleural mesothelioma undergoing extrapleural pneumonectomy with systemic chemotherapy and/or radiotherapy before and after surgical resection were retrospectively reviewed.Results
Ten out of 11 patients underwent complete surgical resection, of these 7 patients had stage I disease. Of these 7 patients, 5 are alive without any recurrence, a 2-year survival rate of 80% was observed in this group. There was no operative mortality or morbidity.Conclusion
Extrapleural pneumonectomy with perioperative adjuvant treatment is safe and effective procedure for epithelial type malignant pleural mesothelioma. 相似文献10.
Andersson E Villabona L Bergfeldt K Carlson JW Ferrone S Kiessling R Seliger B Masucci GV 《Cancer immunology, immunotherapy : CII》2012,61(8):1243-1253
Background
In recent years, evidence is accumulating that cancer cells develop strategies to escape immune recognition. HLA class I HC down-regulation is one of the most investigated. In addition, different HLA haplotypes are known to correlate to both risk of acquiring diseases and also prognosis in survival of disease or cancer. We have previously shown that patients with serous adenocarcinoma of the ovary in advanced surgical stage disease have a particularly poor prognosis if they carry the HLA-A02* genotype. We aimed to study the relationship between HLA-A02* genotype in these patients and the subsequent HLA class I HC protein product defects in the tumour tissue.Materials and methods
One hundred and sixty-two paraffin-embedded tumour lesions obtained from Swedish women with epithelial ovarian cancer were stained with HLA class I heavy chain (HC) and β2-microglobulin (β2-m)-specific monoclonal antibodies (mAb). Healthy ovary and tonsil tissue served as a control. The HLA genotype of these patients was determined by PCR/sequence-specific primer method. The probability of survival was calculated using the Kaplan–Meier method, and the hazard ratio (HR) was estimated using proportional hazard regression.Results
Immunohistochemical staining of ovarian cancer lesions with mAb showed a significantly higher frequency of HLA class I HC and β2-m down-regulation in patients with worse prognosis (WP) than in those with better prognosis. In univariate analysis, both HLA class I HC down-regulation in ovarian cancer lesions and WP were associated with poor survival. In multivariate Cox-analysis, the WP group (all with an HLA-A02* genotype) had a significant higher HR to HLA class I HC down-regulation.Conclusions
HLA-A02* is a valuable prognostic biomarker in epithelial ovarian cancer. HLA class I HC loss and/or down-regulation was significantly more frequent in tumour tissues from HLA-A02* positive patients with serous adenocarcinoma surgical stage III–IV. In multivariate analysis, we show that the prognostic impact is reasonably correlated to the HLA genetic rather than to the expression of its protein products. 相似文献11.
Esam Aboutaleb Manish Kothari Osama Damrah Roben Canelo 《International Seminars in Surgical Oncology : ISSO》2009,6(1):17
Background
Gastrointestinal stromal tumours are rare (GIST). However, the incidence of GIST among neurofibromatosis type 1 (NF-1) patients is approximately 3.9-25%. GIST can present clinically in different ways such as abdominal pain, gastrointestinal bleeding and obstruction.Case report
We present 51 year female patient admitted with Background of neurofibromatosis type 1 admitted with melena. OGD has been done and showed duodenitis with large volume fresh blood in distal duodenum but no obvious bleeding point. Exploratory laparotomy revealed smooth nodular masses on the serosal surface of jejunum. Small bowel resection and side-to-side anastomosis were performed. Histopathoogical examination revealed small bowel gasrointestinal stromal tumour with low risk malignant potential.Conclusion
The incidence of GIST among neurofibromatosis type 1 (NF-1) patients is not uncommon and we should pay attention to gastrointestinal manifestation in such patients.12.
13.
Justin S Gundara Raul Alvarado-Bachmann Nicholas Williams Sivakumar Gananadha Anthony Gill Thomas J Hugh Jaswinder S Samra 《World journal of surgical oncology》2011,9(1):1-8
Background
Compromised physiological reserve, comorbidities, and the natural history of pancreatic cancer may deny pancreatic resection from elderly patients. We evaluated outcomes of elderly patients amenable to pancreatic surgery.Methods
The medical records of all patients who underwent pancreatic resection at our institution (1995-2007) were retrospectively reviewed. Patient, tumor, and outcomes characteristics in elderly patients aged ≥ 70 years were compared to a younger cohort (<70y).Results
Of 460 patients who had surgery for pancreatic neoplasm, 166 (36%) aged ≥ 70y. Compared to patients < 70y (n = 294), elderly patients had more associated comorbidities; 72% vs. 43% (p = 0.01) and a higher rate of malignant pathologies; 73% vs. 59% (p = 0.002). Operative time and blood products consumption were comparable; however, elderly patients had more post-operative complications (41% vs. 29%; p = 0.01), longer hospital stay (26.2 vs. 19.7 days; p < 0.0001), and a higher incidence of peri-operative mortality (5.4% vs. 1.4%; p = 0.01). Multivariable analysis identified age ≥ 70y as an independent predictor of shorter disease-specific survival (DSS) among patients who had surgery for pancreatic adenocarcinoma (n = 224). Median DSS for patients aged ≥ 70y vs. < 70y were 15 months (SE: 1.6) vs. 20 months (SE: 3.4), respectively (p = 0.05). One, two, and 5-Y DSS rates for the cohort of elderly patients were 58%, 36% and 23%, respectively.Conclusions
Properly selected elderly patients can undergo pancreatic resection with acceptable post-operative morbidity and mortality rates. Long term survival is achievable even in the presence of adenocarcinoma and therefore surgery should be seriously considered in these patients. 相似文献14.
15.
Mariana R B De Mello Dulcineia M Albuquerque Fernanda Gon?alves Pereira-Cunha Krizzia B Albanez Katia B B Pagnano Fernando F Costa Konradin Metze Irene Lorand-Metze 《Diagnostic pathology》2012,7(1):1-8
Background
Ezrin is a cytoskeletal protein that is involved in tumor growth and invasion. It has been suggested that Ezrin expression plays an important role in tumor metastasis. This study is aimed to investigate the clinicopathological significance of Ezrin overexpression in gastric adenocarcinomas.Methods
Ezrin protein expression was examined by immunohistochemistry in 26 normal gastric mucosa, 32 dysplasia, and 277 gastric adenocarcinomas. The relationship between Ezrin expression and the clinicopathological features of gastric cancers was analyzed. In addition, a gastric cancer cell line, MKN-1, was also used for immunofluorescence staining to evaluate the distribution of Ezrin protein.Results
Ezrin protein located in the cytoplasm and/or membrane in the migrating gastric cancer cells, and it mainly concentrated at the protrusion site; however, only cytoplasmic distribution was observed in the non-migrating cancer cells by immunofluorescence staining. The positive rate of Ezrin protein expression was significantly higher in gastric adenocarcinoma and dysplasia compared with that in the normal gastric mucosa. Moreover, expression frequency of Ezrin protein increased significantly in lymph node metastasis and late clinical stages. Consistently, strong expression of Ezrin was significantly correlated with poor prognosis of gastric cancer.Conclusion
The detection of Ezrin expression can be used as the marker for early diagnosis and prognosis of gastric adenocarcinoma.Virtual Slides
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2303598677653946 相似文献16.
Background
To support decisions about surgical treatment of elderly patients with cancer, population-based estimates of postoperative mortality (POM) rates are required.Methods
Electronic records from the Rotterdam Cancer Registry were retrieved for octogenarians and nonagenarians who underwent resection in the period 1987–2000. POM was defined as death within 30 days of resection and both elective and emergency operations were included.Results
In a series of 5.390 operated patients aged 80 years and older, POM rates were 0.5% for breast cancer, 1.7% for endometrial cancer and 4.2% for renal cancer. For patients with colorectal cancer, POM increased from 8% for the age group 80–84 to 13% for those 85–89 to 20% in nonagenarians. For stomach cancer, the respective figures were 11%, 20% and 44%.Conclusion
These results show that resections can be performed at acceptable risk in selected elderly patients with cancer. 相似文献17.
Background
Malignant gastrointestinal stromal tumors (GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal tract. Myogenic gastrointestinal stromal tumor, a distinctive morphologic variant is characterized by an unusually prominent myxoid stromal background.Case presentation
We report a case of myxoid variant of GIST in a 42 years old woman presenting as an epigastric mass associated to an ovarian cyst and elevated CA-125. Histologically, the lesions was composed of a proliferation of spindle cells in an abundant myxoid stroma, without evidence of atypia or anaplasia. Immunohistochemical stains showed strong positive staining with muscle actin, positive staining with CD34 and weak positive staining with CD117, while showed negative for S-100.Conclusion
At surgery every effort should be made to identify the origin of the tumor. A complete surgical removal of the tumor should be obtained, as this is the only established treatment that offers long term survival. 相似文献18.
Shiang-Fu Huang Wen-Yu Chuang Sou-De Cheng Li-Jen Hsin Li-Yu Lee Huang-Kai Kao 《World journal of surgical oncology》2010,8(1):1-5
Background
The most common type of carcinoma associated with ulcerative colitis (UC) is adenocarcinoma. We present a case of primary rectal small cell carcinoma in a patient with a history of UC.Methods
A 34-year-old male diagnosed with UC for 10 years was not consistent with the usual annual follow-up and presented with mucoid-bloody diarrhea. Colonoscopy revealed a rectal mass 2 cm distant from the anal verge. The patient underwent a total proctocolectomy with preservation of the anal sphincters, construction of an ileal reservoir, anastomosis of the reservoir to the anus (J configuration) and protective loop ileostomy.Results
Histological examination showed undifferentiated small cell carcinoma.Conclusions
This is the first case of small cell carcinoma in a background of UC reported to be treated surgically and the patient and has no reccurence 18 months postoperatively. 相似文献19.
Markos Ioannou Panayiotis J Papagelopoulos Ioannis Papanastassiou Ioanna Iakovidou Stamatios Kottakis Nikolaos Demertzis 《World journal of surgical oncology》2008,6(1):1-5
Background
Morbid obesity strongly predicts morbidity and mortality in surgical patients. However, obesity's impact on outcome after major liver resection is unknown.Case presentation
We describe the management of a large hepatocellular carcinoma in a morbidly obese patient (body mass index >50 kg/m2). Additionally, we propose a strategy for reducing postoperative complications and improving outcome after major liver resection.Conclusion
To our knowledge, this is the first report of major liver resection in a morbidly obese patient with hepatocellular carcinoma. The approach we used could make this operation nearly as safe in obese patients as it is in their normal-weight counterparts. 相似文献20.
Leda Guzmán María Soledad Depix Ana María Salinas Rosa Roldán Francisco Aguayo Alejandra Silva Raul Vinet 《Diagnostic pathology》2012,7(1):1-9