Cases reported "Choristoma"

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1/20. Glial heterotopia in the subcutaneous tissue overlying T-12.

    Heterotopic glial nodules occur most commonly in the head and neck area, and are theorized to arise following abnormalities in the development of the facial and skull bone plates. However, in spite of the fact that some of these lesions are associated with communication with the central nervous system (CNS), the lack of a meningeal component, argues against simple herniation and separation of brain tissue through a defect in the skull. We present an infant with a nodule directly over the spine present in the T-12 region with no skin abnormalities. magnetic resonance imaging (MRI) and computerized axial tomography (CT) showed no spinal abnormalities with an overlying fibrotic soft tissue mass. The patient had no other associated clinical findings. Histologic findings showed a cellular component arising within the reticular dermis with a deep circumscribed margin. The nodule contained irregularly shaped cells containing abundant cytoplasm and indistinct cellular margins with bland nuclei. These cells were clustered around and between a fibro-mucinous stroma. Immunohistochemical stains showed positive staining for S-100 protein, vimentin, GFAP, NSE, and CD57, and negative staining for Ki-67, CD34, Neurofilament protein, cytokeratin, and EMA. The spindle cells showed positive staining for CD34 and vimentin. The clinical and histologic features and immunohistochemical profiles are used to separate this lesion from the closely related, ependymal rests, ependymomas, and primary cutaneous chorodomas.
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2/20. Aetiology of nasopharyngeal glioma.

    A case of nasopharyngeal glioma is presented in which the postnatal scans clearly show intracranial communication, whilst subsequent scans and surgical exploration could not demonstrate this finding. This case seems to confirm that the aetiology of these lesions is due to an encephalocele that subsequently loses its connection with the brain.
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3/20. Heterotopic brain tissue in the upper lip of a neonate mimicking bilateral cleft lip features.

    Heterotopic brain tissue is an extremely rare developmental anomaly frequently diagnosed in the newborn period. This entity has been described in various sites of the head and neck, most commonly in the nasal area. Computed tomography and magnetic resonance imaging are necessary for exclude associated cranial communications. Heterotopic brain tissue is an extremely rare developmental anomaly frequently diagnosed in the newborn period. This entity has been described in various sites of the head and neck, most commonly in the nasal area. Computed tomography and magnetic resonance imaging are necessary for exclude associated cranial communications.
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4/20. Nasal cerebral heterotopia: nasal atretic cephalocele.

    We report the case of a 4-year-old boy who presented with a congenital mass on the bridge of his nose. A magnetic resonance study failed to rule out a communication of the tumor with the cranial cavity. The lesion was totally removed. Histopathological study of the excised mass showed a peripheral zone of fibrous-connective tissue with a core of glial and neuronal elements. We discuss the origin of these masses and their relation to nasal cephaloceles. We suggest that this type of lesions should be included within the broader spectrum of atretic cephaloceles.
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5/20. Ectopic growth hormone-releasing adenoma in the cavernous sinus--case report.

    A 55-year-old woman presented a rare ectopic pituitary adenoma in the right cavernous sinus manifesting as acromegaly. The tumor was removed via transsphenoidal approach. Intraoperative observation showed the adenoma was located entirely within the right cavernous sinus, and separated from the normal pituitary gland by the medial wall of the cavernous sinus. There was no communication between the tumor and the pituitary. Histological examination showed a growth hormone-releasing adenoma. Including our case, only eight of 86 reported ectopic adenomas have occurred in the cavernous sinus. Such ectopic presentation may be responsible for failed transsphenoidal surgery for endocrinologically active tumors.
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6/20. Lumbosacral ectopic nephrogenic rest unassociated with spinal dysraphism.

    Nephrogenic rests (NRs) are thought to originate from persistent nephrogenic blastema and are considered precursor lesions of Wilms' tumor (WT). These rests usually occur as perilobar and intralobar lesions in the kidney and, rarely, in ectopic sites. We report a midline lumbosacral ectopic NR in a healthy full-term newborn male with no family history of WT or WT-associated syndromes. The NR presented as a soft polypoid mass covered by normal skin. An MRI study revealed no lumbosacral spine abnormalities and no communication with the vertebral canal. The resected mass measured 3 cm and contained fat and had a central 1.2-cm solid nodule. The nodule was composed of blastema, epithelial elements (mature tubules and nephrons), and abundant stroma. No other somatic tissue elements were identified after complete microscopic examination. There are 4 cases of NRs reported in the lumbosacral area associated with spinal dysraphism, and only 2 cases, in addition to our report, unassociated with spinal abnormalities. The pathogenesis of heterotopic immature nephrogenic tissue remains a source of conjecture and speculation. If these lesions are heterotopic rests, their potential for neoplastic progression is probably quite limited, but if a monodermal teratoma, then more scrupulous clinical follow-up is warranted.
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7/20. Heterotopic brain presenting as a cystic mass of the palate.

    We report a case of heterotopic brain which presented as a cystic mass in the palate and which clinically was thought to be a cystic hygroma. Histologically, there was a remarkable proliferation of choroid plexus-like structures which we believe to have been responsible for the production of cerebrospinal fluid. We believe heterotopic brain to result from early displacement of multipotential cells and that the presence of cerebrospinal fluid within extracranial brain tissue does not imply an intracranial communication.
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8/20. Complex choristoma of the eyelid containing ectopic cilia and lacrimal gland.

    A 2-year-old girl was born with an aggregate of ectopic cilia in the right upper eyelid that intermittently produced tears. During elective excision, the cilia were found to be clustered tightly with prominent bulb-like follicles. A separate lobe of lacrimal gland tissue was adjacent to the roots of the cilia just posterior to the orbital septum with no direct communication with the lacrimal gland. Histopathologically, the lesion contained multiple large hair follicles in the dermis with accompanying adnexal structures. Large ectopic lobules of lacrimal gland tissue also were present. Postoperatively, the child had a good cosmetic result and has remained asymptomatic over an 18-month follow-up period. To our knowledge, this is the first reported case of an eyelid choristoma containing ectopic cilia and lacrimal gland. Ectopic cilia alone is a very rare anomaly of the lashes, with only nine cases reported in the literature.
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9/20. Heterotopic brain in the middle ear: CT findings.

    A soft tissue mass was found by CT in the mesotympanum of the left middle ear of a 4-year-old boy. This lesion was without radiologic or surgical evidence of associated bony defect or communication with the intracranial cavity. Pathological examination revealed heterotopic brain tissue. Although heterotopic brain tissue in the middle ear is rare, it should be considered in the differential diagnosis of soft tissue middle ear masses.
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10/20. Gastrointestinal duplications causing relapsing pancreatitis in children.

    Two cases of children with relapsing pancreatitis due to intramural gastrointestinal duplications with ductal communication to the pancreas are reported. A gastric duplication with ectopic pancreatic tissue was detected by endoscopic cholangiopancreatography in the gastric antrum of a 6-yr-old girl. A periampullary duodenal duplication was visualized preoperatively by duodenoscopy and computed tomography in a 10-yr-old boy. Resection of the duplication was curative in each case.
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