Cases reported "Choristoma"

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1/41. 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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ranking = 1
keywords = sella
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2/41. Pharyngeal pituitary: development, malformation, and tumorigenesis.

    The development of the pharyngeal pituitary (PhP) in the fetal period was morphologically and, for the first time, immunohistochemically examined. PhP, found in every individual, begins its hormone production at the 17-18th week of gestation, that is, 4-8 weeks later than that of sellar pituitary (SP). Only 1 of 25 examined fetuses without any stigmata of developmental anomalies showed a residual pituitary fragment in the craniopharyngeal canal (craniopharyngeal pituitary, CPhP). An adult case of a rare clivus pituitary adenoma that we examined is demonstrated in discussing its relationship to PhP. Extracranial ectopic pituitary adenomas in the literature describe an exclusively sphenoid sinus/nasopharyngeal/clivus location of the tumor. Their location corresponded exactly with that of PhP, so that the origin of the tumors can be reasonably speculated as PhP, although another origin, e.g., CPhP, can not be excluded. A variety of malformations of PhP, although very rare, have been described for the fist time during the systemic examination of 16 fetuses with different cranioneural malformations, such as agenesis, unseparated PhP from SP (pharyngosellar pituitary), fragmentation, and residual pituitary tissue in the open craniopharyngeal canal. However, developmental anomaly of PhP was not specifically associated with cranioneural malformations except in cases of chromosomal aberrations. The hormone production in PhP in malformation cases tended to be retarded. Absence of SP was recorded in 50% of anencephalics in the literature; however, PhP was identified in all anencephalics in our series, independent of the existence of SP. This supports the opinion that agenesis of SP in anencephalics seems to be false information.
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ranking = 1
keywords = sella
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3/41. MR imaging in children with ectopic pituitary gland and anterior hypopituitarism.

    Posterior pituitary ectopia refers to an absent normal posterior pituitary bright spot within the sella with ectopic bright signal at another site (such as the median eminence) on a weighted magnetic resonance. We describe two children with idiopathic anterior hypopituitarism who showed an ectopic posterior pituitary and absent pituitary stalk on imaging. We emphasize the association of the absent pituitary stalk in ectopic pituitary gland and low growth hormone levels.
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ranking = 0.5
keywords = sella
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4/41. A heterotopic cerebellum presenting as a suprasellar mass with associated nasopharyngeal teratoma.

    We present a case of nasopharyngeal teratoma that was discovered in association with a suprasellar heterotopic cerebellum in a newborn. Well-differentiated, heterotopic, cerebellar masses have been reported in the orbits, spine, and frontal encephalocele but not, to our knowledge, in the suprasellar region. In this report, we describe the imaging findings and discuss the possible origins of the two masses discovered in this case.
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ranking = 3
keywords = sella
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5/41. Ectopic cerebellum presenting as a suprasellar mass in infancy: implications for cerebellar development.

    A 4-month-old infant with a history of nasopharyngeal teratoma developed progressive optic neuropathy. neuroimaging studies demonstrated a solid, isointense, suprasellar mass impinging on optic nerves and chiasm superiorly. The mass was subtotally resected. No attachment of the mass to brain stem or cerebellar structures was noted. Histological examination identified the tissue as developing cerebellum. The cytoarchitecture and cellular constituents of the cerebellar tissue were only slightly distorted. All cerebellar cortical constituents were arranged anatomically, and an external granular cell layer was present superficially. The latter was actively proliferating and appropriately cellular for the infant's age. The clinical presentation of ectopic cerebellum as a suprasellar mass in an infant is highly unusual. Moreover, this example illustrates the ability of cerebellar tissue to mature appropriately in a site distant from the posterior fossa, removed from ascending and descending afferent projections. Intrinsic signaling mechanisms appear sufficient to direct histogenesis in developing cerebellar cortex.
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ranking = 3
keywords = sella
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6/41. Ectopic pituitary adenoma with malignant transformation.

    We report here a case of ectopic pituitary adenoma with malignant transformation after repeated relapses. First, an ectopic pituitary adenoma producing follicle-stimulating hormone was found in the nasal cavity extending to the frontal cranial fossa. Despite repeated surgical resections of the tumor, it recurred three times in 2 years. The tumor gradually showed cellular atypia, mitosis, and necrosis. Immunohistochemical analyses revealed that the expressions of proliferating cell nuclear antigen and MIB-1 increased progressively. Moreover, the expression of p53 was detected at the second recurrence. Finally, at the third recurrence the tumor showed dissemination to the subarachnoid space and multiple metastases in the brain. The patient died of the tumor 10 months after the last resection. These findings indicate that the ectopic pituitary adenoma became malignant. To our knowledge, this is the first report on malignant transformation of ectopic pituitary adenoma. It is important to know that ectopic pituitary adenomas show malignant transformation and that the above parameters (proliferating cell nuclear antigen, MIB-1, and p53) may be useful indicators of the malignant potential of both ectopic and sellar pituitary tumors.
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ranking = 0.5
keywords = sella
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7/41. Ectopic clival craniopharyngioma.

    Ectopic craniopharyngioma is a rare entity. The authors present a very rare case of an ectopic clival craniopharyngioma completely separate from the sella turcica.A 44-year old woman presented with abducens palsy. A MR imaging study and a CT scan revealed a cystic clival lesion separate from the sella turcica. Surgical resection was performed successfully with flexible endoscope-assisted procedure using an endonasal transsphenoidal approach. No evidence of involvement of the sellar region was found according to radiological, intra-operative, and clinical findings.A review of the literature revealed no other such cases. The discussion includes the formation of craniopharyngioma from the ectopic Rathke's pouch remnants and the surgical approach for clival lesions. We believe that our approach provides good results with minimal invasiveness for some clival lesions.
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ranking = 16.038218118374
keywords = sella turcica, turcica, sella
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8/41. Ectopic pituitary adenoma in the cavernous sinus causing oculomotor nerve paresis--case report.

    A 24-year-old woman presented with a rare adrenocorticotropic hormone (ACTH)-positive pituitary adenoma in the cavernous sinus, manifesting as sudden onset of oculomotor nerve paresis. neuroimaging revealed a tumor in the cavernous sinus bulging into the subdural space. There was no continuity between the tumor and the pituitary gland in the sella turcica. Gross total removal of the tumor was performed through the orbitozygomatic approach followed by stereotactic radiosurgery. The oculomotor nerve paresis was resolved. Histological examination revealed an adenoma positive for ACTH. Ectopic pituitary adenoma occurs mostly in the sphenoid sinus or the suprasellar region. This extremely rare case of ectopic macroadenoma in the cavernous sinus manifested as oculomotor nerve paresis without signs of Cushing's syndrome.
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ranking = 8.2691090591868
keywords = sella turcica, turcica, sella
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9/41. Suprasellar ectopic pituitary adenoma presenting as cranial diabetes insipidus.

    We describe the occurrence of a supracellar ectopic pituitary adenoma in a 34 year old woman who presented with cranial diabetes insipidus and subsequently developed galactorrhoea-amenorrhoea. The tumour was demonstrated by both contrast computed tomography scan and magnetic resonance imaging with gadolinium enhancement and was confirmed at operation. Histological examination showed that the suprasellar lesion consisted of a pituitary adenoma while the pituitary biopsy revealed an unrelated pituitary microadenoma embedded in normal pituitary tissues. A review is made of the reported cases of ectopic pituitary adenomas.
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ranking = 2.5
keywords = sella
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10/41. A rare expression of neural crest disorders: an intrasphenoidal development of the anterior pituitary gland.

    congenital abnormalities of the pituitary gland are rare and may be associated with midline cranial, orbital, and facial anomalies and with hormonal insufficiency. Here we report a case of asymptomatic, abnormal migration of the adenohypophysis. The normally developed adenohypophysis was located in the sphenoid bone and developed on the intersphenoidal septum, extending from the superior pharyngeal wall to the floor of the sella turcica (craniopharyngeal canal). The abnormal migration of the pituitary gland was isolated without hormonal deficit, brain, or facial developmental anomalies.
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ranking = 7.7691090591868
keywords = sella turcica, turcica, sella
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