1/12. Ruptured epidermoid cyst and haematoma of spleen: a diagnostic clue of high levels of serum carcinoembryonic antigen, carbohydrate antigen 19-9 and Sialyl Lewis x.Splenic epidermoid cyst is a rare disease and that with haematoma is even more rare. The case of epidermoid cyst of the spleen is described, in a 36-year-old Japanese female, manifesting as left hypochondralgia and rupture of the cyst. Clinical features were splenic lesion 14 cm in diameter and consisting of round-hypovascular and crescent-hypervascular sublesions. Extravasation of cystic fluid was detected in abdominal cavity Preoperative diagnosis was difficult due to such uncommon features, however high levels of serum tumour markers (carcinoembryonic antigen, carbohydrate antigen 19-9, Sialyl Lewis x) strongly suggested epidermoid cyst. Laparotomic splenectomy and cholecystectomy were performed for splenic lesion and gallstones, and serum tumour markers decreased following surgery. Pathological diagnosis of the round-hypovascular lesion was epidermoid cyst and crescent-hypervascular lesion was haemorrhage (haematoma).- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/12. On the pathogenesis and clinical course of mesenteric lymph node cavitation and hyposplenism in coeliac disease.BACKGROUND: Coeliac disease is a disorder characterised by malabsorption related to abnormal small bowel structure and intolerance to gluten. There are several reports of an increased risk for malignancy in coeliac disease and its relation to gluten-free, reduced gluten, or normal diet. While a normal diet is associated with an excess of cancer of the mouth, pharynx, oesophagus, and also of lymphoma, treatment with a gluten-free diet restores the cancer risk back to normal. PATIENT: In the present study, we report on a 63-year-old female patient with a history of coeliac disease for twenty years who presented with persistent diarrhoea, weight loss, and an abdominal mass. RESULTS: The gastroenterological work-up revealed small bowel mucosal atrophy, absence of functional splenic tissue, and evidence for an involution of a mesenteric lymph node, termed cavitation. DISCUSSION: This triad has been previously described to represent a rare disease entity related to coeliac disease. We report a two-year follow-up and a review of the literature on the pathogenesis, prognosis, and therapeutical implications of this disease entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/12. Isolated peliosis of the spleen: report of 2 autopsy cases.Isolated peliosis of the spleen, characterized by the gross appearance of multiple cystlike, blood-filled cavities on cut surfaces of the organ, is a very rare pathologic entity that has been reported in 33 cases to date. We present 2 additional cases of isolated peliosis of the spleen observed at autopsy and confirmed by histology. In both cases investigated, cirrhosis of the liver was present. We hypothesize that local microcirculatory disturbances manifesting under altered local intravascular pressure conditions in the spleen may, at least to a certain degree, be responsible for the peliosis-associated vascular lesions. For the forensic pathologist, isolated peliosis of the spleen represents more than just another morphologic curiosity. The significance of peliosis lienalis lies in the potential of (1) overlooking the correct diagnosis as the cause of a spontaneous splenic rupture with intraabdominal hemorrhage, thus leading to the false conclusion of a violent death; and (2) misinterpreting the macromorphological appearance of this rare disease, also in the absence of splenic rupture, as a result of blunt force trauma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/12. Epidermoid cyst in an intrapancreatic accessory spleen: a case report.Ectopic splenic tissue in the abdominal cavity is a common entity, with a reported incidence of 10% in the general population. However, an intrapancreatic accessory spleen is a rare disease, and moreover cyst formation in it is exceedingly rare. A 58-year-old woman with a 25-mm multilocular cyst in the tail of the pancreas detected incidentally by ultrasonography was admitted for further evaluation. Because malignancy could not be ruled out, a spleen-preserving distal pancreatectomy was performed. The cut surface of the surgical specimen showed a multilocular cyst surrounded by brown solid tissue resembling normal spleen. Pathological examination revealed it was stratified squamous epithelium and was surrounded by splenic tissue. The final pathological diagnosis was epidermoid cyst in an accessory spleen in the pancreas. This cyst has no characteristic features on diagnostic imaging. Consequently, it is not possible to make a definite preoperative diagnosis in most cases. Epidermoid cyst in intrapancreatic splenic tissue is another lesion to be considered in the differential diagnosis of pancreatic tail tumors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/12. Primary pulmonary hypertension and functional hyposplenism.Primary pulmonary hypertension is a rare disease of unknown case. Functional hyposplenism is characterized by the appearance of abnormal circulating erythrocytes despite the presence of a spleen and is associated with a variety of disease states. A case of primary pulmonary hypertension associated with functional hyposplenism is described in this report.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/12. Non-parasitic splenic cysts: a report of three cases.Primary splenic cyst is a relatively rare disease, and the majority of cases are classified as epithelial cysts. Three cases with nonparasitic splenic cysts are presented: two epithelial and one pseudocyst. All cases had an atypical symptomatology, consisted mainly of fullness in the left upper abdomen and a palpable mass. Preoperative diagnosis was established with ultrasonography and computerized tomography. Two cases with large cysts located in the splenic hilum were treated with open complete splenectomy. The most recent case, a pseudocyst, was managed laparoscopically with partial cystectomy. All cases did not have any problems or recurrence during follow-up. Laparoscopic partial cystectomy is an acceptable procedure for the treatment of splenic cysts, because it cures the disease preserving the splenic tissue. Complete splenectomy is reserved for cases in which cyst excision cannot be done otherwise.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/12. Two cases of calcified cysts of the spleen.Presented in this paper are two cases of calcified cyst of the spleen. The first case is an epidermoid cyst of a 20-year-old male who visited our hospital because of vomiting and epigastric discomfort. The second case is a pseudocyst of a 38-year-old male who was referred to our hospital for evaluation of an abdominal mass. Both patients had no history of abdominal trauma. Linear or curvelinear calcification was revealed in the left epigastric region in the plain X-ray films of the abdomen. ultrasonography, computed tomography and selective celiac angiography led to the diagnosis of a cyst of the spleen, and surgical operations were performed because the cysts were large and symptomatic. Splenic cyst is a relatively rare disease. However, the cases including asymptomatic cases with small cyst have been increasing in number with the improvement of diagnostic methods and the common use of ultrasonography and computed tomography. In Japanese literatures, 332 cases had been reported by 1983.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/12. Torsion of the spleen with incomplete infarction: case report.Torsion and infarction of a "wandering spleen" is a rare disease which is often confused with other acute abdominal crises. A correct preoperative diagnosis, when made, has usually been determined by arteriographic studies. A child is described in whom changes in the TcSC scan made a correct diagnosis possible by non-invasive methods.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/12. Peliosis of the spleen with intraperitoneal hemorrhage.We encountered a patient with steroid-related peliosis of the spleen, a rare disease characterized by multiple blood-filled cavities in the splenic parenchyma, with spontaneous intraperitoneal hemorrhage. The ultrasonographic, computed tomographic, and angiographic images were compared with pathologic findings of the material obtained surgically.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/12. Inflammatory pseudotumor of the spleen in a young child.We describe a case of an inflammatory pseudotumor of the spleen in a 5-year-old boy, found incidentally during a physical examination. The boy underwent a hemisplenectomy. The problems in differentiating this disease from lymphoma of the spleen before surgery and the advantages of hemisplenectomy are discussed. This rare disease has previously been described in the spleen in only 28 cases, the youngest being a 16-year-old patient.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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