Cases reported "Choristoma"

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1/71. Intrapancreatic accessory spleen. A rare cause of a pancreatic mass.

    CONCLUSION: The clinical significance of intrapancreatic accessory spleens resides in the mimicry of pancreatic cancer. Radionuclide tests (octreotide scan and Tc99m sulfur colloid scan) should be undertaken to distinguish these lesions from neuroendocrine tumors, hypervascular metastases and pancreatic carcinoma. If the tests are equivocal, diagnostic laparotomy or laparoscopy is recommended. BACKGROUND: Despite its relatively common occurrence, intrapancreatic ectopic splenic tissue is rarely detected owing to its asymptomatic nature. methods: We report a case of a clinically asymptomatic patient in which abdominal computed tomography (CT) scans revealed a mass of 1.5 cm in diameter in the distal pancreas. The tumor markers CA 19-9 and carcinoembryonic antigen (CEA) were slightly elevated, and pancreatic neoplasm was suspected. RESULTS: Left pancreatic resection and splenectomy were performed. The removed specimen disclosed the presence of an accessory spleen within the pancreatic tail.
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keywords = neoplasm
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2/71. Intracranial extracerebral neuroglial heterotopia: A case report and review of the literature.

    Heterotopic masses of neuroglial tissue are uncommon and most frequently involve extracranial midline structures. We report an unusual case of an intracranial, extracerebral neuroglial heterotopia involving the middle and anterior cranial fossae of a 5-year-old girl who presented with facial asymmetry. The lesion was composed of mature but disorganized gray and white matter admixed with surrounding soft tissues and exhibited histologic features reminiscent of cortical dysplasia. These rare lesions have been postulated to arise from a protrusion of tissue from the neuraxis through a pial defect, from abnormalities in the migration of embryonic neuroepithelial tissue, or from an accessory evagination of the neural tube inferior to the telencephalic vesicles. Regardless of the underlying pathogenic mechanism, these lesions must be histologically distinguished from both teratomas and primary central nervous system neoplasms.
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keywords = neoplasm
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3/71. Tall cell variant of papillary carcinoma arising from ectopic thyroid tissue in the trachea.

    Ectopic thyroid tissue within the submucosa of the trachea is a rare cause of upper airway obstruction. Primary neoplasms arising from such thyroid nests are rare. This report describes a case of tall cell variant of papillary carcinoma arising from ectopic thyroid tissue in the trachea.
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ranking = 1
keywords = neoplasm
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4/71. A review of heterotopia and associated salivary gland neoplasms of the head and neck.

    Salivary tissue neoplasms may involve normal, accessory and heterotopic salivary gland tissue. A case of Warthin's tumour originating from heterotopic salivary gland tissue of the upper neck is reported. The radioactive uptake of 131I, evidenced in the neck mass in its pre-diagnostic assessment, suggested a diagnosis of cervical node involvement from a primary malignant thyroid neoplasm. A critical review of the literature on heterotopic salivary gland tissue neoplasms of the head and neck is also presented.
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ranking = 7
keywords = neoplasm
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5/71. Orbital ganglioglioma arising from ectopic neural tissue.

    PURPOSE: To report a case of neonatal orbital ganglioglioma originating from ectopic neural tissue. METHOD: Case report. RESULTS: An African-American male presented at birth with proptosis and expansion of the left orbit. A discrete soft-tissue mass was located inferolateral to the globe, which proved to be a ganglioglioma. CONCLUSION: The tumor is presumed to have originated from ectopic neural tissue in the orbit. Although infrequent, this tumor should be included in the differential diagnosis of neonatal orbital neoplasms.
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ranking = 1
keywords = neoplasm
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6/71. 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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ranking = 2
keywords = neoplasm
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7/71. Gliomatosis peritonei as a complication of a ventriculoperitoneal shunt: case report and review of the literature.

    Gliomatosis peritonei, the implantation of neuroglial tissue upon the peritoneal surfaces, is a rare event most often associated with solid or immature teratomas of the ovary in young girls. The authors report a case of a 10-month-old girl with a ventriculoperitoneal shunt (VPS) who presented with bilateral inguinal hernias. herniorrhaphy was uneventful. Microscopic examination of the hernia sacs showed exuberant mesothelial hyperplasia containing multiple nests of differentiated glial tissue. Subsequent computed tomography and laparoscopy disclosed normal ovaries with no evidence of intraabdominal or pelvic abnormalities. Gliomatosis peritonei in this case was attributed to transport of glial tissue from the cerebrospinal fluid into the peritoneal cavity via the shunt. With the exclusion of an ovarian germ cell neoplasm and in the presence of a VPS, the clinical course with regard to the glial implants in these children is uneventful. If it is appreciated that gliomatosis peritonei may be a complication of a VPS, an extensive clinical evaluation generally is unnecessary.
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ranking = 1
keywords = neoplasm
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8/71. Proliferation of eccrine sweat ducts associated with heterotopic neural tissue (nasal glioma).

    The term "nasal glioma" refers to the presence of heterotopic neural tissue, mainly glial in nature, at or near the root of the nose. We describe a case in which all three components of neural tissue, that is, leptomeninges, glia, and neurons, were present, associated with sweat-duct hyperplasia. Proliferation of sweat ducts is a reactive process in some benign and malignant neoplasms, hamartomas, and cysts. This is the first documented case of hyperplasia of eccrine ductal epithelium induced by nasal glioma.
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ranking = 1
keywords = neoplasm
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9/71. 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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ranking = 1
keywords = neoplasm
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10/71. Solid and papillary epithelial neoplasm arising in heterotopic pancreatic tissue of the mesocolon.

    AIM: Solid and papillary epithelial neoplasm (SPEN) is an uncommon pancreatic tumour. Very rarely it has also been described outside the pancreas, usually arising from heterotopic pancreatic tissue. This report summarises all the published extrapancreatic SPENs and documents the sixth such case arising from heterotopic pancreatic tissue of the transverse mesocolon in a 15 year old girl. methods/RESULTS: Histological and immunohistochemical examination revealed typical papillary and solid areas composed of columnar, cuboidal, and round cells, which were focally positive for vimentin, cytokeratin, neurone specific enolase, carcinoembryonic antigen, alpha1-antitrypsin, alpha1-antichymotrypsin, and negative for neuroendocrine markers (neurofilament, PGP 9.5, chromogranin a, synaptophysin, and S100), p53, and oestrogen and progesterone receptors. Electron microscopy showed scant zymogen but no neurosecretory granules. In agreement with the flow cytometric result s of diploidy, comparative genomic hybridisation (CGH) did not reveal loss or gain of genetic material, and the in situ hybridisation analysis of the RB1 and p53 genes revealed no abnormality in the 13q and 17p arms. CONCLUSIONS: Immunohistochemical and electron microscopic data support exocrine differentiation. The CGH and the flow cytometric results suggest a subtle, yet unknown genetic change, rather than a large genetic alteration. RB1 and p53 in situ hybridisation ruled out the role of deletion at these sites in the pathogenesis of SPEN. Interestingly, review of the published and the present heterotopic pancreatic SPENs identified the mesocolon as the most common anatomical site (four of six), despite the very rare occurrence of ectopic pancreatic tissue at this site.
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ranking = 5
keywords = neoplasm
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