Cases reported "Choristoma"

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1/200. Glial choristoma of the tongue: a case report and review of the literature.

    Heterotopic brain tissue is an extremely rare developmental malformation. It is considered to be one of the very rare choristomatous lesions involving the oral cavity. We present a case of glial choristoma located on the dorsal surface of the tongue, midline area, in an 8-month-old African-American female baby. The clinical, histologic, and immunohistochemical features are presented. In addition, a review of previously reported cases and their probable embryogenesis are analyzed.
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2/200. Progression of myelinated retinal nerve fibers.

    PURPOSE: To present a case demonstrating progression of retinal nerve fiber myelin and to suggest that myelinated retinal nerve fibers be considered among the peripapillary choristomas. METHOD: Case report. A 46-year-old woman demonstrated progression of myelinated retinal nerve fibers and associated retinal vascular anomalies. RESULTS: Myelinated retinal nerve fibers can be progressive. We confirm earlier reports of vascular anomalies associated with retinal nerve fiber myelin and show that they can also be progressive. CONCLUSIONS: The rarely progressive nature of myelinated retinal nerve fibers and the histopathologic studies published to date suggest that myelinated retinal nerve fibers represent a striking ophthalmoscopic feature resulting from ectopic oligodendrocytes; thus, the entity may be described as an oligodendrocytic choristoma.
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3/200. Epileptogenic mineralization: pathological variants with good prognosis.

    Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy.
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4/200. Unusual choristoma of the parotid gland in a girl. A possible trichoadenoma.

    An 8-year-old girl had a painless circumscribed nodule in the right parotid gland for 4 weeks. A tumour (1.3 cm diameter) within the salivary gland parenchyma showed small cystic spaces with horn-like material macroscopically. The tumour tissue contained solid squamous cell formations and cystic spaces limited by multilayered squamous epithelium and covered by layers of ortho- and parakeratotic cells. The cystic spaces contained keratotic lamellae. In some areas pin-like epithelial proliferations were seen. All epithelial cells were characterized by uniform nuclei, and no atypical mitoses were seen. Keratinizated masses with partial calcification were occasionally located in the interstitial tissue and replaced by multinucleated giant cells. The tumour was classified as a choristoma and resembled a trichoadenoma. The ectodermally derived oral and salivary gland epithelium may be the source of skin-like or adnexal tumours.
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5/200. Phakomatous choristoma may be located in the eyelid or orbit or both.

    PURPOSE: Phakomatous choristoma is a rare congenital tumour of lenticular origin. The exact location at clinical presentation is controversial, but herein we provide evidence that phakomatous choristoma may be located in the lower eyelid or orbit or both. methods: Case report of an infant presenting with a mass in the lower eyelid at birth, a systematic review of previous cases of phakomatous choristoma and an outline of the histopathological features in normal mid-facial embryonic development at different gestational ages. RESULTS: The histopathological features of the present case were consistent with those of a phakomatous choristoma. The preoperative imaging studies and clinical findings at surgery suggested that the tumour occupied parts of both the lower eyelid and anterior orbit. Histopathological sections of a normal human embryo showed that at the 26-mm stage of development the embryonic lens is formed but the bony walls defining the orbit are not yet present. CONCLUSION: Phakomatous choristoma arises in a setting of undifferentiated mesenchymal tissue which later may develop into the lower eyelid or orbit depending on the choristomatous elements being deposited superficial or deep to the embryonic surface.
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6/200. Salivary gland choristoma in the middle ear: a case report.

    Salivary gland choristoma (ectopic or heterotopic salivary gland tissue) is a rare condition that occurs in various locations within the head and neck regions. We present a 13-year-old boy with a salivary gland choristoma in the middle ear associated with congenital alopecia around the auricle in addition to facial nerve and ossicular chain abnormalities. Because the lesions commonly involve the facial nerve, intraoperative facial nerve monitoring is helpful in allowing safe biopsy without facial nerve damage.
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7/200. Ectopic brain tissue in the orbit.

    PURPOSE: The authors report findings in a 9-month-old male infant with heterotopic brain tissue in the orbit, and compare and contrast the characteristics in this patient with the few other descriptions of such lesions in the literature. methods: Excisional biopsy of the growth was undertaken by means of an anterior orbitotomy. RESULTS: A 9-month-old male infant had a history of congenital left 'anophthalmia' and a slowly growing mass in the left orbit. An MRI scan revealed an orbital mass with solid and cystic components. Histological study of the excised tissue was performed and revealed a choristomatous arrangement of dysplastic brain tissue with intermixed primitive retina including pigmented epithelium. There was no connection between the orbit and cranial cavity. CONCLUSIONS: The mass must be considered a rare example of heterotopic brain tissue in the orbit and is the only instance we could find in the literature in which a formed eye was absent but in which a scattered primitive ocular structure could be identified.
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8/200. Pituitary adenoma with neuronal choristoma: a report of two rare cases.

    Two rare cases of pituitary adenoma with neuronal choristoma are described. Both patients were female and presented with features of acromegaly with elevated growth hormone and prolactin levels. Radiologically, both lesions were predominantly intrasellar in location with extension into suprasellar region, but hypothalamus was not involved. Histopathological examination revealed a mixture of chromophobe pituitary adenoma cells and neuronal cells. In both cases, the adenoma component was positive for growth hormone and prolactin. Interestingly, immunopositivity for alpha-subunit, cytokeratin and prolactin was seen in the adenoma and neuronal cells in one case. Our findings support the hypothesis that the neuronal cells possibly arise from adenoma cells as a result of metaplasia.
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9/200. Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

    PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. methods: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.
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10/200. 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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