原发性心脏肿瘤八例报告

本文报道了8例非粘液瘤性的心脏原发性肿瘤(5例良性肿瘤,3例恶性肿瘤)的治疗经验、临床表现与手术情况,评价了某些临床特殊检查的价值与意义,并总结讨论了本组8例患者的诊断、治疗及预后的经验与体会。     

Giant gastrointestinal stromal tumour of rare sarcomatoid epithelioid subtype:Case study and literature review

Gastrointestinal stromal tumours(GISTs) are the most common mesenchymal tumours of the gastrointestinal tract,but they represent less than 3% of all gastrointestinal tract malignancies.This is a detailed case study of a 52-yearold male patient treated for very uncommon histological subtype of gastric GIST with atypical clinical presentation,asymptomatic progress and late diagnosis.The resected tumour,giant in diameters,was confirmed to represent the most rare histopathologic subtype of GISTs- sarcomatoid epithelioid GIST.We report this case and review the literature with a special focus on pathomorphological evaluation,biological aggressiveness and prognostic factors.To our knowledge this is the first report of giant GIST of very uncommon sarcomatoid epithelioid subtype.It is concluded that clinicians should pay attention to the fact that initial diagnosis may be delayed due to mildly asymptomatic and non-specific clinical presentation.Asymptomatic tumours diagnosed at a late stage,which is often the case,can be large on presentation.Prognosis for patients diagnosed with GIST depend on tumour size,mitotic rate,histopathologic subtype and tumour location.That is why early diagnosis and R0 resection,which is usually feasible and safe even in giant gastric sarcomatoid epithelioid subtype of GISTs,are the key factors for further treatment and good prognosis.     

Nested stromal-epithelial tumour of the liver:An unusual liver entity

Nested stromal-epithelial tumours(NSETs)of the liver have been reported to be extremely unusual primary hepatic neoplasms.To date,few cases have been described in the literature.NSETs have been defined as non-hepatocytic and non-biliary tumours of the liver consisting of nests of epithelial and spindled cells,myofibroblastic stroma and variable intralesional calcification and ossification.Here,we report a case of a young female who underwent liver resection for a large hepatic lesion that proved to be a calcifying NSET on pathological examination.Details about the clinical and histopathological features of the tumour are reported.     

Unusual cystic presentation of an endocrine carcinoma of the jejunum

The cystic presentation of endocrine tumours is rare and raises difficult diagnostic problems. So far, the only cases of cystic digestive endocrine tumours reported in the literature are of pancreatic origin. We report the unusual observation of a jejunal endocrine carcinoma presenting as a cystic abdominal mass. A 59-year-old woman was referred for chest and abdominal pain. Imaging studies revealed multiple cystic nodules in the liver and a large sus-mesocolic cystic lesion of probable intestinal origin. Biopsies of the extra-hepatic mass and liver nodules showed endocrine tumour. Surgical resection of the jejunal mass and of liver segment III were performed. Histological examination confirmed the diagnosis of jejunal endocrine carcinoma metastatic to the liver. Large areas of the primary and secondary tumours presented an unusual vesicular architecture, responsible for the cystic presentation. No adjuvant treatment was attempted. This observation underlines the difficult diagnostic problems raised by the cystic presentation of digestive endocrine tumours.     

Left posterior hemiblock related to an interventricular septum tumour. First case in the literature.

A 22-year-old man who engaged in intense and regular physical exercise complained of atypical chest pain. The only remarkable abnormality found in the routine clinical work-up was a left posterior hemiblock. The echocardiogram and the magnetic resonance imaging (MRI) study showed a tumour in the posterior and superior aspect of the interventricular septum where the posterior fascicle of the left bundle is located. It was interpreted that the left posterior hemiblock was produced by the tumour.     

Pancreatic paraganglioma with draining vessels

A pancreatic paraganglioma is a rare neoplasm that is difficult to distinguish from a pancreatic neuroendocrine tumour. Here we present a case of pancreatic paraganglioma that was surgically resected following preoperative diagnosis of a pancreatic neuroendocrine tumour. Careful evaluation of the endoscopic ultrasonography findings revealed abundant draining vessels, which could have led to a correct preoperative diagnosis of pancreatic paraganglioma.     

Breast cancer as a systemic disease: a view of metastasis

Breast cancer is now the most frequently diagnosed cancer and leading cause of cancer death in women worldwide. Strategies targeting the primary tumour have markedly improved, but systemic treatments to prevent metastasis are less effective; metastatic disease remains the underlying cause of death in the majority of patients with breast cancer who succumb to their disease. The long latency period between initial treatment and eventual recurrence in some patients suggests that a tumour may both alter and respond to the host systemic environment to facilitate and sustain disease progression. Results from studies in animal models suggest that specific subtypes of breast cancer may direct metastasis through recruitment and activation of haematopoietic cells. In this review, we focus on data implicating breast cancer as a systemic disease.     

Management of the Zollinger–Ellison syndrome in patients with multiple endocrine neoplasia type 1

Jensen RT (Bethesda, MD, USA). Management of the Zollinger–Ellison syndrome in patients with multiple endocrine neoplasia type 1 (Minisymposium: MEN & VHL). J Intern Med 1998; 243 : 477–88. Zollinger–Ellison syndrome (ZES) is the most common symptomatic pancreatic endocrine tumour in patients with MEN-1. Besides the treatment of the usual endocrinopathies seen in patients with MEN-1, the treatment of the ZES requires attention be paid to controlling the gastric acid hypersecretion, to dealing with the gastrinomas per se which are malignant in 18–60% of cases, and to the diagnosis and treatment of gastric carcinoid tumours, that are increasingly seen in these patients. In this article the current management of each of the areas is reviewed and what is known or uncertain discussed, based on our studies at the NIH and data from others. Data from 231 patients including 45 with MEN-1 and 186 without MEN-1 is contrasted in this report. Gastric acid hypersecretion has been controlled in all patients medically with MEN-1 and ZES at the NIH for up to 22 years. The current drugs of choice are H⁺-K⁺ ATPase inhibitors and twice a day dosing is recommended. Periods of parenteral drug therapy (surgery, etc.) and pregnancy require important modifications. The appropriate surgical therapy of the gastrinoma is controversial. Eighty per cent of patients have a duodenal gastrinoma and 20–30% have a pancreatic tumour. Recent studies suggest gastrinoma enucleation combined with duodenotomy rarely results in cure. Aggressive surgery (Whipple resection) can result in cure of gastrinoma but effect on survival is unclear. There are important differences in gastrinoma location, extent, and percentage with aggressive disease in patients with or without MEN-1, which are discussed. Confusion has occurred because of lack of information on the natural history of the gastrinoma compared to the other pancreatic endocrine tumours that occur in MEN-1 and survival data from patients with and without MEN-1 is contrasted. The occurrence of gastric carcinoids in patients with and without MEN-1 with ZES is contrasted and the areas of certainty and disagreement reviewed.     

T cell infiltrate and outcome following resection of intermediate-grade primary neuroendocrine tumours and liver metastases

Background:

Tumour-infiltrating lymphocytes (TILs) have been shown to predict survival in numerous malignancies. The importance of TILs in primary pancreatic neuroendocrine tumours (NETs) and NET liver metastases (NETLMs) has not been defined.

Methods:

We identified 87 patients with NETs and 39 with NETLMs who had undergone resection. Immunohistochemistry was performed to determine TIL counts. Recurrence-free survival (RFS) and overall survival (OS) were determined using the log-rank test.

Results:

The median follow-up time was 62 months in NET patients and 48 months in NETLM patients. Vascular invasion and histologic grade were the only independent predictors of outcome for NETs and NETLMs, respectively. Analysis of intermediate-grade NETs indicated that a dense T cell (CD3+) infiltrate was associated with a median RFS of 128 months compared with 61 months for those with low levels of intratumoral T cells (P = 0.05, univariate analysis). Examination of NETLMs revealed that a low level of infiltrating regulatory T cells (Treg, FoxP3+) was a predictor of prolonged survival (P < 0.01, univariate analysis).

Conclusions:

A robust T cell infiltrate is associated with improved RFS following resection of intermediate-grade NETs, whereas the presence of more Treg correlated with shorter OS after treatment of NETLMs. Further study of the immune response to intermediate-grade NETs and NETLMs is warranted.     

Total pancreatectomy in six patients with intraductal papillary mucinous tumour of the pancreas: the treatment of choice

BackgroundIntraductal papillary mucinous tumours (IPMT) were described as a distinct entity in l982.The extent of surgical resection remains controversial.MethodsSix patients with a diffuse dilatation of the main pancreatic duct were treated with total pancreatectomy for cure of IPMT.ResultsHistological examination showed one IPM adenoma, four IPM non-invasive carcinomas and one IPM invasive carcinoma. In all but one case multifocal extensive intraductal changes were found, affecting either most of the pancreas or the whole organ. All patients survived the operation and remain alive 5–56 months later. Post-pancreatectomy diabetes has been moderately well controlled.DiscussionIPMTs represent a subgroup of pancreatic neoplasms with a favourable prognosis, and the resection should aim at removing all dysplastic foci. In cases with diffuse dilatation of the main pancreatic duct, widespread tumour involvement of the duct system can be expected, so total pancreatectomy should be the operation of choice.     

Solitary tumour metastasis: a rare cause of right ventricular outflow tract obstruction and sudden death

The clinical features of a patient presenting with recent breathlessnessindicated right ventricular outflow tract obstruction, whileechocardiography suggested the presence of tumour in the rightventricle. Before further investigations could be instituted,she died suddenly. Autopsy revealed a haemopericardium secondaryto rupture of the right atrium while the right ventricle wasfound to contain a tumour arising from most of its endocardialsurface. Histology showed this to be a poorly differentiatedmucous secreting carcinoma similar in appearance to her coloniccarcinoma excised 8 years previously. There was no other evidenceof metastatic disease.     

Primary right ventricular osteosarcoma

A 41-year-old man suffering from dyspnea on exertion was found to have a mass located on the right ventricle. Resection of the tumour required reconstruction of the right ventricular outflow tract and replacement of the pulmonary valve. Histopathology confirmed the diagnosis of high-grade osteosarcoma.     

Aortic valve fibroelastoma causing cerebral infarction

We report incidental findings of aortic valve fibroelastomain a patient with embolic complications during routine transthoracicechocardiography.     

Pulmonary glomus tumour: a case initially diagnosed as carcinoid tumour

Pulmonary glomus tumours are rare lesions, with few cases reported previously. Herein, we present the clinical and pathological features of a case of pulmonary glomus tumour. A 29-year-old female patient presented to our clinic complaining of cough, dyspnoea and left-sided chest pain. Computed tomography (CT) of the thorax revealed a nodular lesion causing obstruction of the left main bronchus. Fibreoptic bronchoscopy demonstrated a polypoid mass occluding the left main bronchus 10 mm distal to the main carina. Bronchoscopic biopsy was interpreted histologically as carcinoid tumour. Bronchotomy plus mass extirpation was performed with left thoracotomy. Microscopically, a tumoral structure composed of uniform cells with a round centrally located nucleolus and narrow eosinophilic cytoplasm was seen. Thin-walled vessels lined with endothelium were interspersed between tumoral structures. The cells were stained chromogranin and cytokeratin negative and strongly vimentin positive. The pathological diagnosis for the thoracotomy specimen was pulmonary glomus tumour. Follow-up chest CT was negative for recurrent tumour and the patient remains free of disease 17 months after surgery.     

Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen＇s disease

Von Recklinghausen’s disease is an autosomal dominant hereditary disease associated with a wide number of neoplasms. We report a case of a 47-year-old Caucasian male affected by Von Recklinghausen’s disease who developed a malignant somatostatinoma of the papilla major and minor associated with jejunal gastrointestinal stromal tumour with uncertain behaviour. At laparotomy, multiple hepatic metastases were evident. Whipple pancreaticoduodenectomy, jejunal resection, extensive lymphadenectomy and multiple hepatic wedge resections were performed. The patient was alive without recurrence after 24 mo. This is the fourth case reported in the world literature of a patient with Von Recklinghausen’s disease associated with periampullary somatostatinomas and jejunal stromal tumor. In patients with Von Recklinghausen’s disease who complain of gastrointestinal symptoms, a high suspicion index for periampullary endocrine tumours and/or gastrointestinal stromal tumour is required. An aggressive surgical approach seems to give long term survival also in metastatic patients.     

Peripheral primitive neuroectodermal tumour after allogeneic bone marrow transplantation

Summary. We report a case of peripheral primitive neuroectodermal tumour (PNET) in a 36-year-old man who 10 years earlier received an allogeneic bone marrow transplantation (BMT) for a non-Hodgkin's lymphoma. Immunohistochemistry proved definitive elimination of a relapse of the original disease and confirmed the diagnosis of PNET. The role of total body irradiation pre-BMT in the genesis of this secondary tumour is discussed. The importance of registering secondary malignant solid tumours after BMT is emphasized. To our knowledge, an Ewing family tumour following BMT has not previously been reported.     

泰素帝治疗晚期恶性实体瘤疗效观察

目的观察泰素帝联合卡铂治疗晚期恶性肿瘤的疗效及其副反应。方法经病理学或细胞学证实的64例、期恶性肿瘤患者,泰素帝75mg/m2静滴,第1天,卡铂6mg·ml-1·min-1静滴第2天,3周为一周期,每例患者治疗2周期以上。结果全组CR0例,PR24例,SD36例,PD4例,总有效率37.5%,初治组有效率38.7%,复治组有效率36.8%,两组间比较有效率差异无显著性(P>0.05),主要毒副反应为骨髓抑制, 度白细胞下降为46.9%,其它毒副反应较轻微,可耐受。结论泰素帝联合卡铂一线治疗或二线治疗晚期恶性实体瘤均有较好的疗效,毒性可耐受,值得临床进一步推广。