Inflammatory pseudotumor of the spleen with expression of follicular dendritic cell markers is an extremely rare lesion with only a few cases reported previously. The present study reports on an inflammatory pseudotumor of the spleen 10 × 8 × 7 cm in size that was incidentally found in a 61-year-old man and increased gradually in size during a period of 3 months. Abdominal ultrasonography revealed a well-circumscribed splenic mass, and abdominal computed tomography confirmed the presence of a well-circumscribed isodense lesion in the splenic hilum with inhomogenous enhancement in the early-phase images and no enhancement on delayed-phase contrast-enhanced images. Magnetic resonance imaging of the abdomen showed a well-defined isodense tumor on T1-weighted images with mildly increased signal intensity on T2-weighted images, and this is only the second study that provides MRI findings of this entity. The patient underwent an uncomplicated open splenectomy for definitive histologic diagnosis. Under microscopic examination, the lesion was an admixture of lymphocytes, plasma cells and spindle cells. In situ hybridization analysis for Epstein-Barr virus (EBV) revealed that most of the spindle cells were positive for EBV, and immunochemistry showed the expression of the follicular dendritic cell markers CD21, CD35 and CD23 within the tumor. The diagnosis of inflammatory pseudotumor-like follicular dendritic cell tumor was established.
Esophageal perforation is a life-threatening situation and represents a major therapeutic challenge. Results have improved in recent years particularly as a result of progress in antibiotic therapy and the use of total parenteral nutrition. Surgical management retains a predominant role, involving early primary closure and thoracic drainage. We have made an addition to the surgical management by applying an absorbable mesh and fibrin glue to the repaired site. Seven patients (ages 38-79 years) were treated as described. The mean interval from leak to surgery was 28 hours. Six patients had an uneventful postoperative course with a mean hospital stay of 34 days (range 26-45 days). In one case the technique failed and the patient required an exclusion-diversion procedure. All 7 patients recovered without mortality. We believe that this technique provides a real improvement for this precarious esophageal repair.
Intestinal neuronal dysplasia (IND) is a well-defined entity which raises controversy among authors, described as a congenital malformation of gastrointestinal innervation and caused by dysplastic embryonal development of the enteric nervous system. It is potentially associated with mild and chronic gastrointestinal motility disturbances. IND is rarely reported in adults and especially elderly patients. The present study reports on the case of a 71-year-old man suffering from longstanding idiopathic constipation and who was misdiagnosed for more than 60 years, despite several hospital admissions and a sigmoidectomy in the meantime. On the last admission, the patient presented with megacolon, abdominal pain and X-ray finding of bowel obstruction. Due to massive large bowel dilatation, an exploratory laparotomy failed to reveal any obvious mechanical cause, and a subtotal colectomy and Hartmann's procedure was performed. Bowel continuity was performed 3 months later. Analysis of full-thickness biopsies revealed enlarged myenteric and submucosal neurons as well as an increased number of giant cells and increased acetylcholinesterase activity in the mucosa. The diagnosis of IND was established. The main diagnostic criteria, the underlining pathophysiology and the recommended therapeutic approach of this rare entity are extensively reviewed.
Presacral ganglioneuroma is an extremely rare tumor of neural crest origin. To the best of our knowledge, less than 20 cases have been reported previously. The present study reports on a presacral ganglioneuroma, 10.5 × 8 × 4 cm in size, that was found incidentally in a 35-year-old man with prior history of diverticulitis. He was admitted to our hospital due to lower left abdominal pain. Abdominal computed tomography and magnetic resonance imaging confirmed the extension of the lesion from the S2 level to the coccyx. The mass had low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images with no intraspinal or rectal extension. T2-weighted images demonstrated a compartmentalized solid tumor with cystic components. Complete tumor resection with free surgical margins was achieved using an abdominal approach. The patient remains asymptomatic 2 years after surgery. We emphasize on clinical features, radiologic appearance and surgical treatment of this rare entity. The clinical and pathologic features of previously reported studies are also briefly reviewed.
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