Cases reported "Esophageal Neoplasms"

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1/14. Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hybridization.

    Two cases of synovial sarcoma that arose in the upper digestive tract are reported. One case was a polypoid mass that arose at the gastroesophageal junction; the other was a large intramural mass that arose in the wall of the stomach. Both cases had a classic biphasic pattern. In the stomach tumor, the biphasic morphology was focal and there was an abrupt transition to poorly differentiated synovial sarcoma. The tumors had immunohistochemical features that were consistent with synovial sarcoma. Ultrastructural evaluation of the gastroesophageal tumor supported the diagnosis. The diagnostic X;18 translocation was demonstrated by fluorescence in situ hybridization on sections from paraffin-embedded tissue in 86% and 50% of interphase nuclei from the gastroesophageal and gastric tumor, respectively. The translocation was present in equal frequency in the epithelial and spindle cells in the biphasic areas and the poorly differentiated areas of the gastric tumor, indicating that the development of the more aggressive subclone was probably due to genetic mutations not encompassing the SYT-SSX gene fusion product. We are aware of only five reported cases of synovial sarcoma arising in the digestive tract, all in the proximal esophagus. These cases are the first reported arising in the gastroesophageal junction and stomach and the only cases of synovial sarcoma of the digestive tract in which the diagnostic translocation was demonstrated. Sarcomatoid carcinoma (carcinosarcoma) and gastrointestinal stromal tumor are the main differential diagnoses for synovial sarcoma in this site. Synovial sarcoma of the digestive tract may be underdiagnosed, and its recognition may have important clinical implications. fluorescence in situ hybridization is helpful in making this distinction.
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2/14. Rapid-growing carcinosarcoma of the esophagus arising from intraepithelial squamous cell carcinoma: report of a case.

    A case of carcinosarcoma arising from the intraepithelial spreading area of esophageal squamous cell carcinoma (SCC) is reported herein. A 64-year-old man was referred to our hospital for investigation of a sore throat and dysphagia. An endoscopic examination revealed a 2. 5-cm polypoid mass in the mid-esophagus. Esophagograms taken 1 month prior to consultation by our hospital and just before surgery revealed marked change within a period of less than 2 months from a 2-cm sessile elevated mass to a 4-cm polypoid mass with a lobular appearance. The resected specimen contained two lesions in the esophagus. The larger one measured 4.0 x 2.0 x 2.0 cm and had a pedunculated polypoid shape, while the smaller one, measuring 1 cm in diameter, was a plateau-type elevated lesion located 3 cm distal from the larger mass. Histologically, the distal smaller lesion was diagnosed as primary SCC associated with a high frequency of intraepithelial spread, while the larger polypoid mass was composed of spindle-shaped sarcomatous cells, arising from the intraepithelial spread of SCC. To our knowledge, this is the only reported case of esophageal carcinosarcoma arising from an area of intraepithelial spread of SCC that showed such surprisingly rapid growth.
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3/14. Laparoscopic intragastric surgery for gastric tube cancer following esophagectomy.

    As a result of the recent improvement of the prognosis of esophageal cancer, the reporting frequency of gastric tube cancer following esophageal cancer has increased. Gastric tube total resection following median sternotomy, a highly invasive surgical procedure, is applied to the cases of advanced gastric tube cancer, whereas endoscopic mucosal resection is selected for the cases of early gastric tube cancer. If endoscopic mucosal resection is not applicable for some reason, partial or total resection of the gastric tube following median sternotomy has been selected. We applied laparoscopic intragastric surgery to such a case: The patient, a 59-year-old man with esophageal cancer, had undergone subtotal esophagectomy followed by gastric tube reconstruction through the retrosternal route 6 years before. Since endoscopy revealed early gastric cancer in the body of the stomach, we tried to perform mucosal resection but failed because of anastomotic stenosis. However, we successfully performed intragastric surgery, in which a camera and forceps were inserted directly into the gastric tube. Thus, laparoscopic intragastric surgery is a useful technique in cases to which endoscopic mucosal resection is not applicable.
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4/14. A case of small cell carcinoma of the esophagus detected incidentally by FDG-PET.

    Small cell carcinoma (SmC) of the esophagus is rare, and is sometimes impossible to detect by macroscopic inspection using an endoscope or histological examination of biopsied specimens. A 73-year-old man received F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) to evaluate the response to radiofrequency thermal ablation therapy for lung cancer. FDG-PET showed abnormal accumulation in the posterior mediastinum. endoscopy disclosed ulcerous lesions with marginal elevation in the middle segment of the esophagus, but the biopsy specimen taken concurrently was not malignant histologically. FDG-PET, performed two months later, revealed abnormal accumulation in the suspect area, and the extent of accumulation was wider than previously. Histological examination of the specimen biopsied during the endoscopy led to a diagnosis of SmC. FDG-PET thus proved useful in the early detection of SmC.
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5/14. Isolation and characterization of the human homologue of rig and its pseudogenes: the functional gene has features characteristic of housekeeping genes.

    rig (rat insulinoma gene) was first isolated from a cDNA library of rat insulinomas and has been found to be activated in various human tumors such as insulinomas, esophageal cancers, and colon cancers. Here we isolated the human homologue of rig from a genomic dna library constructed from a human esophageal carcinoma and determined its complete nucleotide sequence. The gene is composed of about 3000 nucleotides and divided into four exons separated by three introns: exon 3 encodes the nuclear location signal and the dna-binding domain of the RIG protein. The transcription initiation site was located at -46 base pairs upstream from the first ATG codon. The 5'-flanking region of the gene has no apparent TATA-box or CAAT-box sequence. However, two GC boxes are found at -189 and -30 base pairs upstream from the transcription initiation site and five GC boxes are also found in introns 1 and 2. The gene is bounded in the 5' region by cpg islands, regions of dna with a high GC content and a high frequency of CpG dinucleotides relative to the bulk genome. Furthermore, the human genome contains at least six copies of RIG pseudogenes, and four of them have the characteristics of processed pseudogenes. From these results together with the finding that RIG is expressed in a wide variety of tissues and cells, we speculate that RIG belongs to the class of "housekeeping" genes, whose products are necessary for the growth of all cell types.
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6/14. A double-crossover study comparing conventional ventilation with high frequency ventilation in a patient with tracheoesophageal fistula.

    Respiratory distress, from severe gastric aspiration pneumonitis and abdominal distention in the patient with tracheoesophageal fistula frequently requires mechanical ventilatory support. Bulk flow ventilation can lead to enlargement of the fistulous tract, elevation of gastric intraluminal pressures, raised airway pressures with hemodynamic instability, and retained secretions. We report a case of tracheoesophageal fistula, secondary to perforation of a squamous cell carcinoma of the esophagus, with temporary improvement in gas exchange on high frequency ventilation after failing on a conventional ventilator. The patient initially failed to improve on an Engstrom ventilator (Engstrom-Gambro, Inc., Barrington, IL) at 13 l/minute ventilation. Instituting high frequency jet ventilation with a VS 600 Jet Ventilator (Instrument Development Corporation, Pittsburgh, PA) at initial settings of 35 psi, rate 150, inspiratory time 40%, FiO2 0.8 and 12 cm H2O positive end expiratory pressure (PEEP), provided incremental improvement in gas exchange and oxygenation up to 26 cm H2O PEEP. However, in view of progressive multi-organ failure we terminated the jet ventilation after 48 h and returned the patient to conventional ventilation. We were unable to provide life-sustaining ventilation and oxygenation with either an Engstrom ventilator at 13 l/-minute ventilation or an MA-1 ventilator (Puritan-Bennett, kansas City, MO) at a tidal volume of 800 cc and a ventilator rate of 30. Terminal respiratory failure occurred. Based on the period of improvement using high frequency jet ventilation, we believe this mode of ventilatory support is beneficial in the management of tracheoesophageal fistula.
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7/14. Transthoracic resection of esophageal cancer in patients with pulmonary dysfunction. Usefulness of high frequency ventilation during thoracotomy.

    Although curative resection of esophageal cancer has become a safe procedure, in patients with pulmonary dysfunction, postoperative complications remain a serious problem. Of 122 patients who had transthoracic resection of esophageal cancer, 27 had pulmonary dysfunction; in six, the forced vital capacity was less than 70% (minimum of 42.8%, mean /- SD of 56.6 /- 8.9%); in 18, forced expiratory volume for one second (FEV1%) was less than 70% (minimum of 34.6%, mean /- SD of 60 /- 10%); and in three, both forced vital capacity and forced expiratory volume was less than 70%. Two patients had undergone hemipneumonectomy before receiving resection of the esophagus. During the intrathoracic operative procedure, high frequency ventilation was used, providing good surgical exposure and contributing to a decrease of postoperative pulmonary complications. There were no deaths during the month after surgery. The survival curve of these patients was not significantly different from that of other patients who had had esophagectomy for cancer of the esophagus. These patients survived for an average of 24 months. The patient who survived the longest has been alive for more than 11 years.
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8/14. Extrapulmonary oat cell carcinoma.

    Primary extrapulmonary tumors with histologic features indistinguishable from bronchogenic oat cell carcinoma are appearing with increasing frequency in the literature. These tumors have been described in the esophagus, stomach, pancreas, larynx, hypopharynx, salivary glands, nasal cavity and paranasal sinuses, thymus, small and large bowel, uterine cervix, endometrium, breast, prostate, urinary bladder, and skin. It is now widely believed that oat cell carcinoma is a poorly differentiated counterpart of carcinoid tumor and that both originate from an endocrine cell system. In this article, the authors review all cases of extrapulmonary oat cell carcinomas, which they were able to find in the English literature, and report personally studied examples of these tumors, occurring in the esophagus, stomach and urinary bladder. A closely related, if not identical, tumor arising in the skin is also described. It is emphasized that a wider recognition of these tumors is likely to lead to their more frequent diagnosis and possible treatment.
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9/14. leiomyosarcoma of the esophagus.

    This report analyzes classification, frequency, and history of esophageal sarcomas and introduces a clinical case of leiomyosarcoma of the thoracic esophagus. The clinical, radiologic, endoscopic, pathologic, and therapeutic aspects of this unique case are presented and discussed.
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10/14. High frequency jet ventilation in the management of intra-operative tracheal injury.

    A patient undergoing pharyngo-oesphagectomy with gastric transposition sustained a large tracheal tear during the blind oesophageal dissection. High frequency jet ventilation was successfully employed in the acute airway management and continued postoperatively.
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