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1/9. Multifocal epithelioid angiosarcoma of the small intestine.

    A 67-year-old man presented with weight loss, intermittent severe abdominal pain and melaena. Initial radiology (including abdominal ultrasonography), gastroscopy and colonoscopy did not demonstrate any lesions that could explain the complaints. Three weeks later, upper gastrointestinal and small-bowel barium studies revealed two areas in the small intestine with an abnormal mucosal pattern. Explorative laparotomy revealed three tumoral lesions. Three partial enterectomies were performed. Gross examination showed centrally depressed dark reddish tumoral lesions extending from the mucosa throughout the full thickness of the bowel wall (diameter varying between 1.6 cm and 2.2 cm). The tumours, composed of large, plump, polygonal cells showing little architectural differentiation, were mainly situated in submucosa and muscularis propria. The growth pattern appeared rather solid. The epithelioid cells showed pronounced nuclear pleomorphism and atypia with central large nucleoli. There were several small blood vessels with occasional anaplastic endothelial cells. Immunohistochemical staining demonstrated an intense expression of CD 31, CD 34, factor viii related antigen and keratin. This supported the diagnosis of an epithelioid angiosarcoma. The patient died 3 months after diagnosis. Tumours of the small intestine are very rare, and angiosarcomas of the small intestine are even more rare. Epithelioid variants have only been described in two patients and only one of these had a multifocal presentation. The prognosis is very poor. Because of the epithelioid growth pattern and the cytokeratin expression, these tumours may erroneously be diagnosed as a carcinoma.
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2/9. Atypical presentation of churg-strauss syndrome: another "forme fruste" of the disease?

    Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels. A broad and heterogenous group of syndromes may result from this process, because any type, size, and location of blood vessel may be involved. The cause of these conditions remains unclear, but an autoimmune inflammatory process, characterized by involvement of both neutrophils and endothelial cells, seems to play an important role. In 1951, Churg and Strauss described a clinical syndrome of severe asthma, hypereosinophilia with eosinophilic infiltrates, eosinophilic vasculitis, and granulomata in various organs. asthma may precede this vasculitis by many years. We report a case of anti-neutrophil cytoplasmic antibody-positive, pauci-immune, crescentic, necrotizing glomerulonephritis with peripheral and interstitial eosinophilia but without asthma. This is very unusual in churg-strauss syndrome.
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3/9. superior mesenteric artery syndrome: case report.

    superior mesenteric artery syndrome (SMAS) is caused by trapping of the third part of the duodenum between Superior Mesenteric Artery (SMA) and aorta as result of narrowing of the angle between the two vessels due to acute loss of mesenteric fat which is secondary to rapid weight loss. A fifteen year old caucasian female on a mountain climbing expedition with an acute on chronic upper gastrointestinal obstruction is presented. The diagnosis was confirmed with a barium meal and at surgery, a bypass procedure performed to relieve the obstruction.
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4/9. weight loss-induced calciphylaxis: potential role of matrix metalloproteinases.

    calciphylaxis is an uncommon and often devastating syndrome of calcification of small vessels, leading to tissue infarction. The mechanism of how calcium deposits on small vessels is unknown. Recently, metalloproteinase digestion of elastin has been shown to enhance deposition of calcium, suggesting a possible mechanism of calciphylaxis. We describe a case of a patient who developed calciphylaxis after rapid weight loss, but had normocalcemia and normal renal function. She was found to have high levels of matrix metalloproteinases, which may have chemically altered elastin, allowing deposition of calcium on small vessels. Inhibitors of matrix metalloproteinases may be useful in the prevention and treatment of calciphylaxis.
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5/9. Critical intestinal ischaemia in a patient with patent mesenteric vasculature.

    We present a case of a 62-year-old man with known coeliac disease who was admitted for investigation of abdominal pain and weight loss. He underwent multiple biochemical, haematological, radiological and endoscopic investigations (which were all normal) and also had a normal laparoscopy. Abdominal computerized tomography angiography, however, suggested significant mesenteric stenosis. Mesenteric angiography confirmed superior mesenteric artery stenosis and reproduced the patient's abdominal pain when the catheter crossed the lesion. Balloon angioplasty successfully dilated the stenosis, and since then the patient has gained 19 kg in weight (returning his body mass index from 17 to 23) and has been symptom free. Symptomatic single vessel mesenteric ischaemia (other than coeliac artery stenosis in median arcuate syndrome) is not previously well described. The symptom reproduction on catheterization highlights how useful angiography can be in diagnosis of disease significance. Symptom resolution after angioplasty demonstrated clearly how even single vessel disease can cause significant compromise to the mesenteric circulation.
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6/9. Celiomesenteric trunk compression and absence of collateral vessels in the large intestine--a case report.

    The authors report the case of a rare mesenteric anomaly in a 71-year-old man who presented with a preexisting abdominal aortic aneurysm (AAA) and a progressive history of postprandial abdominal pain and 10-lb weight loss. aortography revealed a common celiomesenteric trunk, an absent middle colic artery, and a stenotic inferior mesenteric artery. At operation, neural fibers compressing the common celiomesenteric trunk were lysed, the AAA was repaired, and the inferior mesenteric artery was subjected to endarterectomy and then reimplanted. The patient remains well and free of symptoms 1 year after operation. This rare case demonstrates the many different causes of intestinal angina and its surgical relief.
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7/9. systemic vasculitis: a cause of indeterminate intestinal inflammation.

    OBJECTIVES: Indeterminate intestinal inflammation may result from a variety of inflammatory conditions in addition to ulcerative colitis and crohn disease. The primary systemic vasculitides may present with intestinal inflammation and an indeterminate colitis. We set out to describe a series of children with primary systemic vasculitis who initially presented with clinical features suggestive of inflammatory bowel disease (IBD) to establish criteria that might help discriminate between IBD and primary systemic vasculitis. methods: Ten children (6 boys, median age at presentation 8.9 years, range 0.9-14.5 years) satisfied inclusion criteria. RESULTS: All had abdominal pain, weight loss, diarrhea (6 of 10 bloody) and laboratory evidence of a severe acute phase response. Extraintestinal clinical features included vasculitic rash, renal impairment, myalgia, testicular pain and polyarthritis. endoscopy showed vascular changes or other macroscopic findings suggestive of vasculitis in 5 of 10 patients. Gut histology revealed indeterminate chronic inflammatory mucosal changes and one patient with small artery fibrinoid necrosis in the submucosal vessels. Extraintestinal biopsy was performed in 6 patients and had a higher yield for the demonstration of vasculitis than intestinal biopsy. The results of selective visceral angiography was suggestive of vasculitis in all patients, but was normal in 7 cases of treatment-unresponsive classic IBD. Treatment comprised corticosteroid and azathioprine in all patients. cyclophosphamide was given to 7 of 10 patients. CONCLUSIONS: Extraintestinal manifestations and inflammatory responses that may be disproportionate to the degree of intestinal inflammation provide clues to the presence of an underlying primary systemic vasculitis, and these data suggest that selective visceral angiography plays a key role in the diagnosis of vasculitis in this context. It is important to identify and treat any vasculitic component because failure to do so may result in consequential morbidity or mortality.
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8/9. Appleby operation for carcinoma of the body and tail of the pancreas.

    Long-term survival of carcinomas in the body and tail of the pancreas after surgery is still rare. One of the major reasons for unresectability is cancerous invasion to major vessels, such as the common hepatic and splenic arteries. Resection of the involved arteries can increase resectability and thus might increase post-operative survival. The aim of this study was to clarify the importance of the Appleby operation for carcinoma of the body and tail of the pancreas. A Case Report was carried out with a 54 year-old man, had suffered back pain and loss of body weight for six months. Imaging procedures such as US, CT or angiography showed a carcinoma in the body of the pancreas, about 3 cm in size, and both the common hepatic and splenic arteries were invaded by the tumor. The Appleby operation was used for this patient, since firstly there was no invasion to the head of the pancreas, secondly neither the proper hepatic artery nor the SMA was involved, thirdly the root of the CA was free of carcinoma, and finally because clear pulsation of the proper hepatic artery could be felt one or two minutes after occlusion of the CHA, which indicated that resection of the CHA would not lead to hepatic ischemia. The postoperative course was uneventful. His appetite recovered well and his body weight increased to the level before the disease. The patient was relieved from back pain and has returned to work 18 months after the operation, although he had a local recurrence eight months after the operation. In addition, eleven cases with carcinoma of the body and tail of the pancreas were used for a literature review. The average survival time after the Appleby operation is 6.6 months, and four patients are still alive. One patient has survived 13 years after the operation. It was concluded that although the prognosis after Appleby procedure is still not satisfactory that this operation can at least offer patients a better quality of life.
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9/9. Polyneuropathy with endoneurial immune complex deposition as the first manifestation of systemic lupus erythematosus.

    A 72-year-old male presented with progressive sensorimotor polyneuropathy. Later weight loss, proteinuria and deteriorating renal function were noted. The electrophysiological examinations revealed extensive, symmetrical demyelinating and axonal polyneuropathy. frozen sections obtained from sural nerve biopsy sample showed the presence of immune complexes and complements in the walls of the epi- and endoneurial blood vessels, and perineurium suggestive of systemic lupus erythematous (SLE). IgG and Clq deposits were also present along the basement membranes of schwann cells. The electron microscopy confirmed the presence of immune complex deposition. diagnosis of SLE was proven by positive serology (anti-nuclear antibodies, anti-Sm, anti-RNP, anti-double-stranded dna) and renal biopsy showing membranous lupus nephritis with extensive immune complex deposition in the tubular basement membranes. Despite combined immunosuppressive treatment for 10 months, the patient died of complications of generalized immune complex vasculitis. The manifestation of SLE in elderly patients, especially in males, is very rare. Moreover, the polyneuropathy is an unusual initial symptom of SLE. Immune complex deposition in Schwann cell basement membrane probably plays an important role in the pathomechanism of sensorimotor polyneuropathy in SLE.
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