Primary hepatic leiomyosarcoma is exceedingly rare accounting for less than 1% of the hepatic tumors. Close to 45 cases have been reported in the English literature. Presentation is usually nonspecific and diagnosis is often delayed until tumors reach a large size. This leads to a dismal prognosis. The tumors are not yet fully understood, hence the standard of care is not well defined. Curative resection remains the mainstay of management. Close association of Epstein Barr virus (EBV) induced soft tissue sarcomas is proven, especially in the presence of immunosuppression encountered in HIV/AIDS patients and in posttransplant patients. We herein present a case report of a 54-year-old man diagnosed to have HIV/AIDS and EBV infection admitted to our hospital with complaints of intractable hiccups for more than a week. Extensive workup revealed primary leiomyosarcoma of the liver.
Inflammatory fibroid polyp (IFP) is a rare benign polypoid lesion of the gastrointestinal tract. Most IFPs occur in the stomach and colonic occurrence is very rare. Histologically IFP is characterized by a mixture of numerous small vessels, fibroblasts and edematous connective tissue associated with marked inflammatory infiltration by eosinophils. We present a rare case of a pedunculated IFP in the hepatic flexure of the colon treated successfully with a combination of argon plasma coagulation, endoclipping and polypectomy. A 74-year-old asymptomatic female underwent a screening colonoscopy in our hospital. A 12-mm pedunculated polyp was found at the hepatic flexure of the colon. After saline injection, we attempted to remove the polyp with a hot snare. However the polyp stalk was extremely difficult to resect despite several attempts with the hot snare. We placed an endoclip at the base of the stalk and then applied argon plasma coagulation at 1.0 l/min and 40 W. After these measures we were able to resect the stalk and the polyp was retrieved. Histologically the polyp was located in the submucosa of the gastrointestinal tract. Proliferation of spindle cells and infiltration of inflammatory cells such as plasma cells and eosinophils were observed. The spindle cells were positive for CD34 and S100 but negative for c-kit and muscle markers. These findings are consistent with a histopathological diagnosis of IFP.
Pneumatosis of the gastrointestinal tract is a rare condition characterized by the presence of air filled cavities in the gastrointestinal tract wall. Its occurrence has been described throughout the gastrointestinal tract from the esophagus to the rectum, however it is most commonly reported in the small intestine. Despite multiple case reports in literature, its pathogenesis still remains unclear. Pneumatosis may be idiopathic or associated with a variety of disorders namely peptic ulcer disease, jejunoileal bypass, intestinal obstruction and non-gastrointestinal disorders like asthma, chronic obstructive pulmonary disease, systemic lupus erythematosus, infectious enteritis, etc. We here present a rare case of pneumatosis of the esophagus diagnosed incidentally at an esophagogastroduodenoscopy (EGD). A 78-year-old asymptomatic woman underwent EGD and colonoscopy at our hospital for evaluation of anemia. Few months prior to EGD, she had undergone excision of laryngocele at our hospital. EGD revealed extensive submucosal blebs distributed throughout the esophagus, otherwise unremarkable stomach and duodenum. Colonoscopy showed a tubular adenomatous polyp. Since our patient was asymptomatic she did not require any surgical intervention. Management of pneumatosis depends on the underlying cause.
Neurofibromatosis is a genetic disorder manifested by characteristic cutaneous lesions called neurofibromas. There are two distinct neurocutaneous syndromes named neurofibromatosis type 1 (also called von Recklinghausen disease or NF1) and neurofibromatosis type 2 (NF2). NF1 is by far the most common presentation and is caused by an autosomal dominant mutation in the NF1 gene mapped to chromosome 17q11.2. The literature shows that gastrointestinal involvement is noted in systemic neurofibromatosis in up to 25% of patients, but isolated intestinal neurofibromatosis is a very rare manifestation. We herein present the case of a 70-year-old woman who was diagnosed with an isolated colonic neurofibroma without any systemic signs of neurofibromatosis; only a few case reports of this condition have been published to date.
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