Volume 74 - 2011 - Fasc.2 - Case reports
Splenic cystic lymphangioma in a young woman : case report and literature review
Splenic cystic lymphangioma is extremely rare, with very few cases reported until now. Here, we report a case of cystic lymphangioma of the spleen in a young woman who was admitted for evaluation of abdominal pain and a mass lasting for two years. We present this case with emphasis on the problem of differential diagnosis and the difficulties of diagnostic certainty in the absence of histologic features. (Acta gastroenterol. belg., 2011, 74, 334-336).
Primary duodenal follicular lymphoma: 6-years complete remission after combined radio-immunotherapy
Primary gastrointestinal lymphoma (PGL) is known to account for 40% of all extranodal non-Hodgkin's lymphomas (NHLs) and between 4% to 12% of all NHLs. The small intestine is the site of presentation in 20-30% of cases, with the terminal ileum usually involved. Duodenal localizations have always been thought to be rare, but are presently growing in incidence. We herein report on a case of Stage IV primary duodenal FCL, located to the second por- tion of the duodenum with concomitant minimal bone marrow involvement. The patient was frontline approached with a conser- vative combined modality treatment consisting of 4 weekly infu- sions of the chimeric human-murine IgG1 mono-clonal antibody against the B-cell surface antigen CD-20, Rituximab (375 mg/m2) and consolidation 3D conformal external beam radiotherapy up to a total dose of 36 Gy given into 20 fractions to the involved duode- nal portion. Six years after treatment has been completed, the patient is free from disease with no treatment-related toxicity. (Acta gastroenterol. belg., 2011, 74, 337-342).
Lymphoepithelial cyst of the pancreas : case report and review of the literature
Background and study aims: Lymphoepithelial cyst of the pancreas (LCP) is a rare, benign cyst mimicking pseudocyst or cystic neoplasm. Literature describing LCP is limited to case or brief series reports, and the natural history of this condition is largely unknown. A literature review was carried out in order to elucidate the clinical, pathological and biochemical features of LCP. The aim of this study was to define diagnostic criteria and treatment.
Methods : A Medline and Pubmed search was conducted by using the key-words "lymphoepithelial cyst" and "pancreas". The articles found were accurately examined and all details regarding clinical and pathological features were included in a data-base. Furthermore, a case recently observed in our unit was added to the review.
Results : Ninety-two cases of LCP were found in the worldwide literature, including the case that we observed. LCP occurs more frequently in males (M:F = 5.5:1), its preferred site is the tail of the pancreas, and its size ranges between 2 and 10centimetres. Histologically, it is a true cyst delineated by a keratinizing squamous epithelium surrounded by lymphoid tissue. LCP is asymptomatic in the majority of cases and preoperative diagnosis is complicated by a lack of specific radiological features of the disease. An accurate preoperative diagnosis can only be made by obtaining cytological specimens and placing them in the hands of a pathologist who is familiar with the cytological appearances of the disease.
Conclusions: LCP is a rare lesion worldwide, without any prevalence in different countries or in different ethnic groups. Understanding the features of LCP, making an accurate diagnosis and differentiating it from cystic neoplasm preoperatively is vital, as when it is diagnosed certainly, a conservative treatment is justi- fied. Otherwise, radical surgery in the form of pancreatic resection is required to exclude the diagnosis of pancreatic cystic neoplasm. (Acta gastroenterol. belg., 2011, 74, 343-346).
An unusual cause of upper gastrointestinal bleeding : duodenal GIST. A case report and literature review
A duodenal GIST is an unusual cause of upper gastrointestinal bleeding. Duodenal GISTs are rare and constitute 5% of all GISTs. A significant percentage of duodenal GISTs are located in the third and fourth portion of the duodenum and may not be detected on routine upper endoscopy. Push enteroscopy is necessary to locate these lesions. It is extremely important to differentiate a duodenal GIST from other submucosal tumors like leiomyomas, leiomyosar- comas or leiomyoblastomas which may present in a similar man- ner, because the treatment and prognosis differ significantly. Appropriate histological and immunohistiochemical staining is required to confirm the diagnosis. Surgical resection is the treat- ment of choice and may involve limited resection or a pancreatico- duodenectomy. Adjuvant therapy with Imatinib has been shown to prolong survival in patients with GIST in general. (Acta gastro- enterol. belg., 2011, 74, 347-351).
A case of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) with multiple polyps arising in the cecum and appendix
We present the case of a 32-year-old female with cecal and appendiceal polyps that were removed by laparoscopy-assisted surgery. She also had recurrent nosebleeds due to telangiectases in the nasal mucosa and arteriovenous malformations in the lung, all of which contributed to the diagnosis of hereditary hemorrhagic telangiectasia. (Acta gastroenterol. belg., 2011, 74, 352-354).
Necrotizing acute pancreatitis following therapeutic plasmapheresis in HCV-related cryoglobulinemia
Mixed cryoglobulinemia and hepatitis C virus infection are strongly connected and the therapeutic approach is standardized according to the severity of the symptoms.
We report the difficult management of 59 year old female HCV patient presenting cutaneous lesions and arthralgia due to mixed cryoglobulinemia. No therapy was able to achieve a complete remission and during the six years of active disease we observed several clinical recurrences. The intensive plasmapheresis regimen led to a complete remission of the symptoms but it was associated with severe complications. In this case report we describe an episode of acute necrotizing pancreatitis due to intravascular haemolysis following therapeutic plasmapheresis.
To the best of our knowledge the association between plasma- pheresis and acute pancreatitis has not been previously described. (Acta gastroenterol. belg., 2011, 74, 355-358).