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    • The year survival rate for patients with pulmonary EHE is

    2018-10-22

    The 5-year survival rate for patients with pulmonary EHE is about 60% (range, 45–71%). The presence of anemia, weight loss, pulmonary symptoms, and especially hemorrhagic effusion, are associated with poorer prognoses. In our series, Patient 1 had no significant history of poor prognostic factors. He had a smooth postoperative course and returned to work after the operation. In our experience, and according to the literature, however, pleural EHE tends to have a more aggressive clinical course. There were no obvious mitotic figures or tumor necrosis in the pleural tumors in Patients 2 and 3, but Patient 2 developed BTL105 to the thoracic spine, which responded poorly to radiotherapy and chemotherapy. Patient 3 required radiotherapy and chemotherapy with doxorubicin and cisplatin for treatment of a recurrent lesion. The patient’s pleural effusion resolved and there was no new growth after six courses of chemotherapy. Unfortunately, the patient developed repeated episodes of pneumonia and died of septicemia 18 months after the initial diagnosis. In summary, the clinical features of pleural EHE differ from those of pulmonary EHE. Pleural EHE usually develops in men, who are symptomatic at presentation, and has a more aggressive clinical course than EHE arising in the lung or other sites. The clinical, radiological, and histomorphological features of EHE mimic those of metastatic adenocarcinoma and malignant mesothelioma, but the management and prognosis of those tumors are significantly different from the tumors they mimic. EHE must be suspected in cases of pleural epithelioid tumors that do not exhibit cytokeratin. Acute awareness of the morphologic features and immunohistochemical characteristics of EHE will help establish an accurate diagnosis of this tumor.
    Acknowledgments
    Introduction Segmental intestinal dilatation (SID) is a rare entity. For the majority of cases it is discovered incidentally because preoperative diagnosis is quite difficult. Concomitant SID and omphalocele is extremely rare, and only 15 cases have so far been documented in the English literature. We hereby report a premature infant with coincident SID and omphalocele.
    Case report Abdominal radiography revealed a markedly dilated bowel loop in the right lower quadrant (Figure 1). On a barium meal study a hiatal hernia was seen, which was considered to be the cause of the vomiting. In addition, however, there was a segment of dilated bowel with mild contrast stasis but no mechanical obstruction (Figure 2). Four months after the primary abdominal closure, repair of the esophageal hiatus was undertaken with Nissen fundoplication. A segment of dilated ileum was present about 15 cm proximal to the ileocecal valve. There was a predominant feeding vessel resembling a vitelline vessel (Figure 3). Segmental resection of the bowel was performed with end-to-end anastomosis. The baby recovered uneventfully.
    Discussion Swenson and Rathause first reported SID in the colon in 1959. The following diagnostic criteria have been well established: (1) limited dilatation of bowel with threefold or fourfold increased size; (2) sharp conversion between dilated and normal intestine without an obvious obstruction; (3) presence of normal neuronal plexus in submucosa and muscularis propria; (4) clinical presentation of obstruction or sub-obstruction; and (5) recovery after resection of the dilated segment. It is mandatory to exclude Hirschspung disease prior to making the diagnosis of SID. More than 50% of cases are discovered incidentally in the neonatal period during surgery for other anomalies. In addition to the rare association with omphalocele, a number of associated malformations have been documented. Although often asymptomatic, SID may present with intestinal obstruction and hemorrhage. Iron-deficiency anemia, hypoproteinemia, and bowel perforation have also been documented. It is difficult to make a precise preoperative diagnosis. In some cases, a barium study that shows isolated localized intestinal dilation helps to make the diagnosis.