Cases reported "Craniopharyngioma"

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1/15. Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases.

    Generally accepted contraindications to using a transsphenoidal approach for resection of tumors that arise in or extend into the suprasellar region include a normal-sized sella turcica, normal pituitary function, and adherence of tumor to vital intracranial structures. Thus, the transsphenoidal approach has traditionally been restricted to the removal of tumors involving the pituitary fossa and, occasionally, to suprasellar extensions of such tumors if the sella is enlarged. However, conventional transcranial approaches to the suprasellar region require significant brain retraction and offer limited visualization of contralateral tumor extension and the interface between the tumor and adjacent structures, such as the hypothalamus, third ventricle, optic apparatus, and major arteries. In this paper the authors describe successful removal of suprasellar tumors by using a modified transsphenoidal approach that circumvents some of the traditional contraindications to transsphenoidal surgery, while avoiding some of the disadvantages of transcranial surgery. Four patients harbored tumors (two craniopharyngiomas and two hemangioblastomas) that arose in the suprasellar region and were located either entirely (three patients) or primarily (one patient) within the suprasellar space. All patients had a normal-sized sella turcica. Preoperatively, three of the four patients had significant endocrinological deficits signifying involvement of the hypothalamus, pituitary stalk, or pituitary gland. Two patients exhibited preoperative visual field defects. For tumor excision, a recently described modification of the traditional transsphenoidal approach was used. Using this modification, one removes the posterior portion of the planum sphenoidale, allowing access to the suprasellar region. Total resection of tumor was achieved (including absence of residual tumor on follow-up imaging) in three of the four patients. In the remaining patient, total removal was not possible because of adherence of tumor to the hypothalamus and midbrain. One postoperative cerebrospinal fluid leak occurred. Postoperative endocrinological function was worse than preoperative function in one patient. No other new postoperative endocrinological or neurological deficits were encountered. This study demonstrates the feasibility of using a modified transsphenoidal approach for resection of certain suprasellar, nonpituitary tumors.
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2/15. Suprasellar squamous papillary craniopharyngioma: a case report.

    BACKGROUND: Craniopharyngiomas are benign neoplasms that are usually suprasellar in origin. They are considered a type of pituitary tumor that can cause hyposecretion of anterior pituitary hormones or hypopituitarism. Clinically, they may cause visual and endocrine deficiencies similar to pituitary adenoma. Histopathologically, they are distinct from pituitary adenoma and are generally classified as either adamantinomatous or squamous papillary. Adamantinomatous tumors are more common in children, whereas the squamous papillary type is found almost exclusively in adults. CASE REPORT: A 45-year-old white man came to our clinic with a history of decreased vision in his left eye for the previous five months that fluctuated. magnetic resonance imaging of the brain revealed a suprasellar multilobular mass, which was distinguishable from the pituitary gland. The patient underwent a right frontotemporal craniotomy for resection of the tumor. Histopathological analysis showed a benign squamous epithelial-lined mass consistent with squamous papillary craniopharyngioma. CONCLUSION: Craniopharyngiomas are generally considered to be adamantinomatous or squamous papillary in origin. Recently, magnetic resonance imaging has been used to distinguish between these two types of tumors, although clinically no significant difference has been found with respect to resectability. Clinical presentation, treatment options, recurrence, and differential diagnosis of craniopharyngiomas are discussed.
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3/15. craniopharyngioma and bardet-biedl syndrome. A case report.

    BACKGROUND: bardet-biedl syndrome is a rare disorder and associated with a variety of anomalies. CASE: An 18-year-old woman was referred with primary amenorrhea. Following physical, ophthalmologic, psychiatric, hormonal and radiologic examinations, the diagnosis of both craniopharyngioma and bardet-biedl syndrome was established. CONCLUSION: Although the pathogenesis of hypogonadism in a woman with bardet-biedl syndrome remains unclear, cranial structures, especially the hypothalamus and pituitary gland, should be investigated to reveal any possible abnormalities.
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4/15. Endoscope-assisted microsurgery for treatment of a suprasellar craniopharyngioma presenting precocious puberty.

    Being the most common childhood tumor to involve the hypothalamus and pituitary gland, craniopharyngioma, a histologically benign tumor, is usually related to growth retardation and hypogonadism. The presentation of precocious puberty is very rare for a hypothalamic craniopharyngioma. Here, we report such a case. The female patient had presented with symptoms of menarche and breast development since she was 6 years old. Hormonal therapy with a gonadotropin-releasing hormone analogue was instituted to cease precocious puberty but was unsuccessful. magnetic resonance imaging of the sella showed a 1-cm tumor in the hypothalamic area. Through a pterional approach, the tumor was removed en bloc with endoscope-assisted microsurgery. After the operation, our patient's symptoms improved, so the hormone therapy was discontinued. This extremely unique association of craniopharyngioma and precocious puberty, the causes and mechanisms involved along with the advantages of endoscope-assisted microsurgery are discussed.
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5/15. Intracranial adenoid cystic carcinoma of suprasellar region.

    Adenoid cystic carcinoma (ACC) is a relatively common head and neck tumor which grows slowly but locally aggressive and prone to recurrence. ACC most commonly arises in the major and minor salivary glands. Some rare cases of intracranial ACC with an unknown primary site have been reported. The authors present the first case of primary intracranial ACC of the suprasellar region. A 34 year-old female presented with one month's duration of visual disturbance and galactorrhea. Magnetic resonance image (MRI) showed intra- and suprasellar mass mimicking a craniopharyngioma. There was no evidence of invasion from an extracranial site. The operative findings showed the mass existed under the arachnoid membrane and seemed to originate from the pituitary stalk. Pathological diagnosis was ACC. After the first operation, local recurrence and CSF dissemination to the lower clivus occurred within two months. recurrence repeatedly treated by Gamma knife radiosurgery 10 times and 4 times by surgery during a 3 year follow-up period. But total removal of the tumor at the first operation along with radiosurgery may control the lesion and prevent further recurrence.
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6/15. Unilateral exophthalmos. Occurrence after treatment for perichiasmatic neoplasms.

    Unilateral exophthalmos developed shortly after treatment in three patients with chromophobe adenoma of the pituitary gland and craniopharyngioma. In two patients, the exophthalmos followed surgery, while in the third it did not appear until after radiation treatment. The pathogenesis of the exophthalmos in these cases in obscure. However, the other findings and subsequent course indicate that the exophthalmos is not due to recurrence of the neoplasm nor to a complication of therapy.
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7/15. An entirely suprasellar symptomatic Rathke's cleft cyst: case report.

    An entirely suprasellar symptomatic Rathke's cleft cyst in a 21-year-old woman is reported. An unusual feature of this cyst was the fact that the subepithelial tissues were composed of pituitary gland cells. High resolution magnetic resonance imaging is sensitive in the detection of subtle suprasellar abnormalities. We review the literature regarding the embryological pathogenesis of Rathke's cleft cyst, with special reference to the entirely suprasellar type.
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8/15. Craniopharyngiomas.

    Craniopharyngiomas are rare, benign, suprasellar brain tumors that account for 1%-3% of all intracranial tumors. pituitary gland involvement causes loss of pituitary function, which makes endocrine evaluation necessary before and after surgery. Symptoms of intracranial mass effect, including visual field loss, obstructive hydrocephalus, and extraocular palsy of the cranial nerves, may occur. Lifelong follow-up is crucial because of the risk of recurrence and the need for ongoing hormone replacement therapy. Surgical resection is the primary therapy. However, radiotherapy may be indicated. nurses play a major role in caring for patients with a craniopharyngioma by preventing and identifying complications, educating patients and families about the importance of long-term follow-up, and collaborating with multidisciplinary teams.
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9/15. magnetic resonance imaging of symptomatic Rathke's cleft cyst: report of a case.

    A case of symptomatic Rathke's cleft cyst is described. In spite of the intrasellar component and radiological and clinical evidence of involvement of the pituitary gland, the sella turcica was minimally involved. MRI afforded optimal visualization and localization of the intra- and suprasellar components vis-a-vis the pituitary gland, optic chiasm, infundibular stalk, and carotid arteries.
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10/15. Transitional cell tumor of the pituitary gland developing from a Rathke's cleft cyst.

    A tumor of the pituitary gland is reported which developed from the wall of a Rathke's cleft cyst. This was an incidental autopsy finding in a 79-year-old diabetic woman. The cyst wall was lined by ciliated, mucus producing columnar epithelium with areas of squamous metaplasia, the solid portion of the tumor was composed of cells with light and electron microscopic characteristics of squamous cells, mucus producing cells and anterior lobe cells of the pituitary: they displayed intercellular bridges, intracellular mucus droplets and on electron microscopy varying numbers of intracytoplasmic secretory granules in the 220-450 millimicron range. The cells thus correspond to an early developmental stage of the pituitary anterior lobe, when the still squamous and columnar Rathke's cleft cells begin to develop their endocrine granulation. For this reason it was felt that "transitional cell tumor of the pituitary" would best characterize this hitherto undescribed neoplasm.
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