Cases reported "Splenic Diseases"

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1/23. Epithelial splenic cysts in an intrapancreatic accessory spleen and spleen.

    A rare case of cysts simultaneously occurring in the intrapancreatic accessory spleen and spleen in a 49-year-old female is reported. The patient underwent distal pancreatomy for a cystic tumor of the pancreas, and a splenectomy. A multilocular cyst (4.3 x 2.6 cm) in an accessory spleen at the pancreas tail, and a solitary cyst (1.2 x 0.9 cm) of the spleen were found. The cyst in the intrapancreatic accessory spleen was lined by non-keratinizing stratified squamous epithelium, and the spleen cyst by a single layer of flat epithelium; these lining cells were positive for alcian blue stain and periodic acid-schiff reaction, and were immunohistochemically positive for cytokeratin, HBME-1 and Sialyl-Tn. Epithelial membrane antigen, carcinoembryonic antigen and CA19-9 were positive in the accessory spleen cyst but were negative in the spleen cyst. An electron microscopic examination of the flat epithelium of the spleen cyst revealed numerous microvilli on the surface, cytoplasmic microfilaments, and a number of tight junctions between adjacent cells. These features suggested that the two cysts differ in nature, and origin; the accessory spleen cyst may be an embryonic inclusion of the pancreas duct while the spleen cyst may be an inclusion cyst of the mesothelium.
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2/23. Pitfall of the accessory spleen.

    Two patients, one with insulinoma and one with Cushing's syndrome, are presented. Biochemical evaluation readily suggested the correct diagnosis. During radiologic imaging, the anatomic abnormality giving rise to these diseases, i.e. a pancreatic islet cell tumor, and an adrenal adenoma, at first were mistakenly interpreted as an accessory spleen on the basis of specific computed tomography and magnetic resonance imaging appearances. The insulinoma was identified as such during laparotomy, whereas additional jodo-cholesterol scintigraphy revealed the real nature of the lesion in the patient with Cushing's syndrome. Both patients were operated successfully.
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3/23. A true splenic cyst producing carbohydrate antigen 19-9 and cancer antigens 50 and 125, but not interleukin 10.

    A giant cystic lesion of the left upper abdomen associated with a smaller ovarian cyst in a young female patient is reported. Laboratory data revealed elevated serum levels of carbohydrate antigen 19-9 (CA 19-9), carcino-embryonic antigen (CEA), cancer antigens 50 and 125, and tissue polypeptide antigen. In contrast, the serum levels of interleukin 10, a cytokine involved in modulating immune responses and produced by many cancer histotypes, were normal. Since ovarian cancer or cystic adenocarcinoma of the tail of the pancreas were not ruled out, the patient underwent laparotomy. After splenectomy and ovariectomy, the tumour marker serum levels normalized. histology and immunohistochemical analysis revealed a true splenic cyst with the inner epithelium strongly positive for CA 19-9 and CEA and high levels of cancer antigens in the fluid. The ovarian lesion was a serous cystadenoma. The inner epithelium showed no immunoreactivity for tumour markers which were not measurable in the fluid. True cysts of the spleen are rare; in a few cases, high serum levels of CA 19-9 and CEA have been reported. In such instances, cyst resection or splenectomy is indicated to rule out malignant lesions and to remove the cancer antigen producing epithelium. The reported case shows that the epithelium lining true splenic cysts may produce, besides CA 19-9 and CEA, other tumour markers, in particular cancer antigens 50 and 125. In addition, normal serum values of interleukin 10 correctly predicted the benign nature of the lesion.
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4/23. Normal interleukin-10 serum level opposed to high serum levels of carbohydrate antigen 19-9 and cancer antigens 125 and 50 in a case of true splenic cyst.

    True cysts of the spleen are rare. In a few cases, high serum levels of carbohydrate antigen (CA) 19-9 and carcinoembryonic antigen (CEA) have been reported. It has been suggested that they are produced by inner epithelium of the cyst. In such instances, cyst resection or splenectomy is indicated to rule out malignant lesions and to remove cancer antigen-producing epithelium. Furthermore, a high serum level of interleukin (IL)-10, an immunosuppressive cytokine, has been described in many neoplastic diseases, suggesting it as a potential new diagnostic method. Giant cystic lesion of the left upper abdomen associated with ovarian tumor was diagnosed in a young patient. Laboratory data revealed elevated serum levels of several tumor markers [CA 19-9, CEA, cancer antigens (CA) 125 and 50, and tissue polypeptide antigen]. In contrast, IL-10 serum level was normal. After splenectomy and ovariectomy, tumor marker serum levels normalized. histology and immunohistochemical analysis revealed true splenic cyst with inner epithelium strongly positive for CA 19-9, CEA, and high levels of cancer antigens in fluid. Ovarian lesion was a serous cystoadenoma; its inner epithelium showed no immunoreactivity for tumor markers that were not measurable in fluid. The reported case showed that epithelium lining true splenic cysts may produce, in addition to CA 19-9, CEA, and other tumor markers, in particular CA 125 and CA 50. When malignant disease is suspected, IL-10 serum level could be useful to correctly predict the nature of the lesion.
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5/23. Inflammatory pseudotumor of the spleen.

    We report a case of a patient with inflammatory pseudotumor (IPT) of the spleen. IPTs can appear at many sites and represent rear lesions of uncertain etiopathogenesis. Usually they present as mass lesions, so the clinical and radiologic features often suggest malignancy. However, the microscopic findings are quite characteristic, and the diagnosis can be made readily by identifying the reactive nature of the cells. Diagnostic problems can arise when these lesions occur in lymphoid organs or the spleen.
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6/23. Inflammatory pseudotumor of the spleen concomitant with renal cell carcinoma: case report.

    CONTEXT: Inflammatory pseudotumor is a rare benign lesion that can occur at a wide variety of primary sites. It is usually worrisome for the patient and the medical staff, since it cannot be clinically or radiologically distinguished from malignant entities. CASE REPORT: We report on a case of splenic inflammatory pseudotumor presenting with concomitant renal cell carcinoma. Despite the alarming macroscopic appearance of pseudotumors, their microscopic features usually confirm the inflammatory nature of such lesions. Evidence regarding the etiology of pseudotumors is still lacking, but hypotheses have been created.
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7/23. Mri findings in primary non-Hodgkin's lymphoma of the liver.

    Primary lymphoma of the liver is a very rare malignancy. Most often, these lesions consist of diffuse large B-cell non-Hodgkin's lymphoma that occurs mostly in immunodeficient patients. To prove the primary nature of a hepatic lesion, a systemic lymphoproliferative disease should be ruled out. Secondary liver involvement during Hodgkin's and non-Hodgkin's lymphoma is frequent. In advanced cases the incidence varies from 25% to 50%.The present case describes the MRI features of a primary lymphoma of the liver presenting as a solitary nodule. The primary lymphoma presents as a T2-hyperintense homogeneous nodule, with a signal intensity comparable with the signal intensity of the spleen. Signal intensity is comparable on in and out of phase imaging. The nodule is slightly T1-hypointense and doesn't show any arterial contrast uptake. In the late venous phase a slight increase in signal intensity is noted. Two and a half minutes after the administration of contrast agent, the lesion is iso-attenuating with the liver parenchyma.This case is rare because of the concomitant presence of heterozygous sickle cell anaemia and the presence of Gamna-Gandy bodies in the splenic parenchyma. It remains uncertain whether the presence of the Gamna-Gandy bodies is associated with the liver lymphoma or with the underlying sickle cell anaemia, or with a combination of both.
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8/23. Postoperative recurrence of splenic epithelial cyst; treatment with subtotal splenectomy.

    Three children were operated upon for congenital epithelial cysts of the spleen in the five-year period 1983-1987. Their mean age was 9.8 /- 2.3 years. Two of them were boys. They comprise 1.6% of the surgical pathology of the spleen in our hospital, which is a Reference Centre for congenital haemolytic diseases. Two of the children had been suffering from intermittent colic pain in the left hypochondrium with a tender palpable mass for a short while to the present day. The third patient was asymptomatic; a splenic cyst with calcification of its wall was accidentally revealed by a plain x-ray of the abdomen. Contrast gastrointestinal studies and intravenous pyelography helped us with the diagnosis; they indicated a splenic mass that caused visceral displacement adjacent to the spleen. ultrasonography proved the cystic nature of the enlargement of the spleen in all the cases. Marsupialization was performed in one patient and removal of the cysts in the other two. All three patients were followed up and re-checked 1-5 years postoperatively (May-June 1988). In one boy who underwent removal of the cyst four years ago, recurrence of the splenic cyst was revealed by ultrasonography, CT scan and radionuclide scan. Subtotal splenectomy was performed. The remaining upper pole is supplied with blood by the short gastric arteries. The recurrence was attributed to coexistence of invisible tiny cysts that had remained in the splenic tissue after the dissection of the major cyst during the previous operation. Subtotal splenectomy is an acceptable alternative procedure for splenic cysts.
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9/23. Functional asplenia and portal hypertension in a patient with primary splenic hemangiosarcoma.

    A 60-year-old man with primary splenic hemangiosarcoma (PSH) presented with weakness, weight loss, abdominal pain, and anemia. physical examination revealed hepatomegaly, ascites, and firm, huge splenomegaly. ultrasonography showed many nodular structures characterized by hypoechogenic and hyperechogenic areas. The patient also had portal hypertension, which was confirmed by physical findings and by measurement of portal vein pressure during operation. A liver-spleen scan using Tc-99m sulfur colloid and Tc-99m labeled heat denatured erythrocytes failed to demonstrate any splenic uptake, a reliable feature of functional asplenia. Although on a total body scan with Ga-67 citrate there was no splenic uptake, there was gallium uptake in the liver, where the presence of the metastatic lesion was histopathologically verified and confirmed by operation. There was also uptake in the middle zones of the lungs. Ga-67 citrate imaging appears to be helpful in the diagnosis of metastasis of PSH, and PSH can rarely cause portal hypertension.
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10/23. Systemic lupus erythematosus with multiple calcified fibrous nodules of the spleen.

    An autopsy case of systemic lupus erythematosus (SLE) in a 39-year-old woman with peculiar multiple splenic nodules is reported. Multiple calcific nodular shadows were incidentally found in the left hypochondrial region on chest and abdominal X-ray films taken at admission. The patient died of chronic heart failure due to massive pericardial effusion as one of the manifestations of SLE with 2 and a half years' clinical course. lupus nephritis and terminal miliary tuberculosis were the other conspicuous autopsy findings. The splenic nodules were almost evenly distributed on each cut-surface of the spleen at the density of about 5/cm2. Each nodule was spherical in shape and 1 to 3 mm in diameter. Most of the nodules were calcified in variable degrees. Semi-serial sectionings and reconstruction procedure of the nodules disclosed that they were formed around the central or penicillary arteries and had a close relation to so called "onion-skin lesion" of the spleen in SLE. The true nature, pathogenesis, and relation of the nodules to SLE are discussed.
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