Cases reported "Choristoma"

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1/42. Pseudocapsulorrhexis in a patient with iridocorneal endothelial syndrome.

    We describe a patient with Chandler's syndrome variant of the iridocorneal endothelial syndrome in whom ectopic Descemet's membrane was found intraoperatively on the anterior surface of the lens. Initially, the membrane was confused with the anterior lens capsule during extracapsular cataract extraction, leading to the performance of a pseudocapsulorrhexis. Electron microscopy disclosed that the epilenticular membrane was composed of multiple layers of abnormal basement membrane consistent with the iridocorneal endothelial syndrome.
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2/42. Gastric adenocarcinomas in displaced mucosal glands.

    Although gastric carcinoma is one of the most common cancers worldwide, only a few histologic proximate precursors have been demonstrated. Several authors have found that foci of misplaced gastric mucosa with cystic dilatations (s.c. heterotopic mucosa) are often associated with gastric adenocarcinomas. However, adenocarcinomas originating within heterotopic gastric mucosa have never been reported. In present work, the review of 213 consecutive gastrectomy specimens in Japanese patients showed heterotopic gastric mucosa in 20.1% (n = 43). Up to 18 foci per gastrectomy were present. The heterotopic mucosa was surrounded by invaginations of the muscularis mucosae which showed strong positivity for smooth muscle actin. In 3 of the 213 specimens, an adenocarcinoma was found within a focus of heterotopic gastric mucosa. All 3 adenocarcinomas had cystic dilatations lined by neoplastic columnar epithelium with polymorphic nuclei, irregular nuclear membrane, large irregular nucleoli and pathological mitosis. The tumors had lateral bundles of smooth muscle (smooth muscle actin positive), regarded as invaginations of the muscularis mucosae. The p53 protein was strongly overexpressed in all 3 tumors. The heterotopic gastric mucosa may be one mucosal locus from which gastric adenocarcinomas may originate.
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3/42. 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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4/42. Phakomatous choristoma: a case report and review of the literature.

    Phakomatous choristoma is a rare congenital lesion of the eyelid that can be clinically and/or histologically mistaken for a cyst, cutaneous adnexal neoplasm, or an ocular adnexal oncocytoma. Only 13 such cases have been previously described, mostly in the English language ophthalmic literature. Zimmerman reported the first case in 1971 and proposed the lesion to be of lenticular anlage origin, a theory that has been widely accepted. We report an additional case occurring in an 8-week-old male infant with a firm nodule of the right lower eyelid that was present since birth. A 15 x 12 x 2 mm circumscribed solid nodule with a homogenously white cut surface was surgically excised. Histologically, this lesion was comprised of cuboidal cells forming cystically dilated and irregularly branched ducts and cords within a densely fibrotic stroma. Also present were eosinophilic basement membranelike material, psammoma body-like calcifications and intraluminal degenerated ghost cells. The immunohistochemical profile of the epithelial cells included strong immunoreactivity for vimentin, focal weak staining for S-100, and negative staining for cytokeratin, epithelial membrane antigen, synaptophysin, and chromogranin. The irregularity of the ducts and cords of epithelial cells within the densely fibrotic stroma resembled an infiltrative neoplasm of cutaneous adnexal or lacrimal duct origin. However, the site of involvement, the peculiar basement membrane material, ghost cells, and immunohistochemical profile were features that helped to distinguish phakomatous choristoma from an infiltrative carcinoma. The correct identification of this lesion is essential to avoid an aggressive surgical excision, thus sparing the eyelid and lacrimal system. The purpose of this article is to bring attention to this rare entity, because it has not been described in either the dermatology or dermatopathology literature and furthermore, is not mentioned in any of the major dermatopathology texts.
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5/42. Ectopic thyroid tissue in the left ventricular outflow tract.

    Ectopic thyroid tissue in the heart (struma cordis) is rare. The only report from a hemodynamically significant obstruction of the left ventricular outflow tract (LVOT) by a heterotopic thyroid gland was published in 1988. In our patient, a 42-year-old woman with recurrent chest pain, two spheric cardiac tumors were diagnosed by transthoracal echocardiography. One of the tumors, obstructing the LVOT, could successfully be resected under cardiopulmonary bypass. The pathologic examination showed a colloid-filled ectopic thyroid gland. The second tumor, which was entirely located in the submembraneous part of the interventricular septum, had no hemodynamic influence, and was left in situ to avoid surgical damage of adjacent intraseptal structures. The midterm follow-up showed no recurrence.
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6/42. Phakomatous choristoma of the eyelid.

    This article describes the first report of phakomatous choristoma of the eyelid in korea. A six-month-old boy underwent excision of a congenital inferonasal orbital mass arising from the left lower lid. A dermoid cyst was suspected, however a diagnosis of phakomatous choristoma was made following conventional histology. An immunohistochemical study of this rare benign congenital tumor was conducted. The cuboidal epithelial cells comprising this choristoma showed strongly positive cytoplasmic staining with S-100 protein and vimentin. They also showed focally positive staining with a neuron-specific enolase, while they showed no immunoreactivity to cytokeratin or epithelial membrane antigen. The results of the immunohistochemical study support the conclusion that this tumor is of lenticular anlage origin.
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7/42. Power Doppler sonographic diagnosis of torsion in a wandering spleen.

    A wandering spleen is a rare clinical entity resulting from congenital maldevelopment or acquired laxity of the spleen's suspensory ligaments; it may result in torsion of the spleen. We report the gray-scale sonographic, power Doppler sonographic, and CT findings in a case of wandering spleen torsion. The gray-scale sonograms showed a displaced spleen that appeared as a homogeneous, hypoechoic mass suggestive of an enlarged, ectopic spleen in the central abdomen. Power Doppler sonograms showed no blood flow in the parenchyma or hilum of the spleen and were consistent with torsion and infarction in the spleen. Contrast-enhanced CT scans showed a homogeneous, unenhanced mass that was diagnosed as a torsioned wandering spleen. The hilar vessels of the spleen were also unenhanced.
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8/42. Two cases of ectopic hamartomatous thymoma.

    Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm. Since it was named by Rosai et al. in 1984, 24 cases have been reported. We herein report two cases of EHT, one of which presented with massive myoid cells, and review the literature related to EHT. Both of our cases displayed the typical features of EHT: (1) nests of epithelial cells, including solid, cystic, or glandular epithelial islands; (2) spindle cells dominating the microscopic picture; and (3) adipose cells which intermingle haphazardly to impart a hamartomatous quality to the tumor. In this paper, we observed massive myoid cells and the transition from spindle epithelial cell to myoid cell in one of our cases. Immunohistochemical examinations showed that the main component of EHT, spindle cells, was positive for cytokeratin and epithelial membrane antigen (EMA). Intriguingly, the myoid cells simultaneously expressed cytokeratin, EMA, myoglobin, and creatine kinase-mm, suggesting that myoid cells may originate from epithelial cells and are an intermediate state between epithelial cells and muscular cells.
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9/42. An autopsy case of pancreatic and ectopic nesidioblastosis.

    We report an autopsy case of pancreatic and ectopic nesidioblastosis. A five-month-old Japanese girl was born at 35 weeks gestation, and showed clinical symptoms of hyper-insulinemic hypoglycemia before death. At autopsy a tumorous nodule was observed at the portion of the jejunum, 90 cm from Treitz's ligament. The nodule measured 30 x 20 x 20 mm. The ectopic pancreas, also revealed nesidioblastosis histologically. Immunohistologically, both nesidioblastoses were stained positive for chromogranin a, insulin, glucagon and somatostatin. The proliferating cell nuclear antigen (PCNA) and Ki-67 indices were less than 4% in the nesidioblastosis. To our knowledge, this is the first reported case of nesidioblastosis demonstrating proliferating activity with PCNA and Ki-67, and is the third reported case of nesidioblastosis arising in the pancreas and ectopic pancreas.
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ranking = 2.7611940669043
keywords = ligament
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10/42. Massive gastrointestinal bleeding caused by ectopic pancreas mimicking jejunal tumor.

    BACKGROUND: Ectopic pancreas is usually an incidental finding at surgery or autopsy and rarely produces clinical symptoms. But it is capable of producing symptoms, depending on its location, size and involvement of the overlying mucosa. CASE REPORT: We report a case with massive gastrointestinal bleeding from ectopic pancreas mimicking jejunal tumor, confirmed by emergency operation. A 40-year-old male was admitted to Chonnam National University Hospital with a 2-day history of melena. A technetium-99m-labeled RBC scan showed massive radioactivity in loops of small bowel due to active bleeding. Superior mesenteric angiography revealed a hypervascular stained mass supplied by proximal jejunal branch. A computed tomographic scan of abdomen revealed an enhancing mass in the proximal jejunum. At emergency operation, bleeding from the center of the mass was found situated approximately 30 cm from the Treitz ligament. Segmental resection of the involved jejunum and end-to-end anastomosis were performed. Histologic examination of resected specimen revealed an ectopic pancreas. CONCLUSION: So far, there have been no case reports of massive gastrointestinal bleeding from ectopic pancreas mimicking jejunal tumor as described in our case. In every patient in whom ectopic pancreas can definitely be seen to cause clinical symptoms including gastrointestinal bleeding, the lesion should be excised.
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keywords = ligament
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