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children, but the tumor is not infrequent in adults, and we have encountered patients up to 60 years of age The presenting syndrome may be one of increased intracranial pressure, but more often it takes the form of a combined pituitaryhypothalamic-chiasmal derangement The symptoms are often subtle and of long standing In children, visual loss and diabetes insipidus are the most frequent ndings, followed by adiposity, delayed physical and mental development (Froehlich or Lorain syndrome see page 486), headaches, and vomiting The visual disorder takes the form of dim vision, chiasmal eld defects, optic atrophy, or papilledema, as emphasized long ago by Kennedy and Smith In adults, waning libido, amenorrhea, slight spastic weakness of one or both legs, headache without papilledema, failing vision, and mental dullness and confusion are the usual manifestations One of the most remarkable cases in our experience was a middle-aged nurse who became distractible and ineffective at work and was thought for many months to be simply depressed Often it is observed that drowsiness, ocular palsies, diabetes insipidus, and disturbance of temperature regulation (indicating hypothalamic involvement) occur later Spontaneous rupture of the cystic lesion can incite a severe aseptic meningitis, at times with depressed glucose in the CSF In the differential diagnosis of the several craniopharyngioma syndromes, a careful clinical analysis is more informative than laboratory procedures Among the latter, MRI is likely to give the most useful information Often, because of the cholesterol content, the tumor gives an increased signal on T1-weighted images Usually, the cyst itself is isointense, like CSF, but occasionally it may give a decreased T2 signal Treatment Modern microsurgical techniques, reinforced by corticosteroid therapy before and after surgery and careful control of temperature and water balance postoperatively permit successful excision of all or part of the tumor in the majority of cases While smaller tumors can be removed by a transsphenoidal approach, attempts at total removal require craniotomy and remain a challenge because of frequent adherence of the mass to surrounding structures (Fahlsbusch et al) Partial removal practically assures recurrence of the tumor mass, usually within 3 years, and the surgical risks of reoperation are considerable (10 percent mortality in large series) In 21 of our 35 patients, only partial removal was possible; of these, 8 died, most in the rst postoperative year Stereotaxic aspiration is sometimes a useful palliative procedure, as are focused radiation therapy and ventricular shunting in patients with solid, nonresectable tumors Endocrine replacement is necessary for an inde nite time following tumor resection Glomus Jugulare Tumor This tumor is relatively rare but of particular interest nonetheless It is a purplish red, highly vascular tumor composed of large epithelioid cells, arranged in an alveolar pattern and possessing an abundant capillary network The tumor is thought to be derived from minute clusters of nonchromaf n paraganglioma cells (glomus bodies) found mainly in the adventitia of the dome of the jugular bulb (glomus jugulare) immediately below the oor of the middle ear but also in multiple other sites in and around the temporal bone These clusters of cells are part of the chemoreceptor system that also includes the carotid, vagal, ciliary, and aortic bodies The typical syndrome consists of partial deafness, facial palsy, dysphagia, and unilateral atrophy of the tongue combined with a vascular polyp in the external auditory meatus and a palpable mass below and anterior to the mastoid eminence, occasionally with a.

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