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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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bruit that may be audible to the patient ( self-audible bruit ) Other neurologic manifestations are phrenic nerve palsy, numbness of the face, a Horner syndrome, cerebellar ataxia, and temporal lobe epilepsy The jugular foramen is eroded (visible in basilar skull lms and CT) and the CSF protein may be elevated Women are affected more than men, and the peak incidence is during middle adult life The tumor grows slowly over a period of many years, sometimes 10 to 20 or more Treatment has consisted of radical mastoidectomy and removal of as much tumor as possible, followed by radiation The combined intracranial and extracranial two-stage operation has resulted in the cure of many cases (Gardner et al) A detailed account of this tumor will be found in the article by Kramer Carotid Body Tumor (Paraganglioma) This is a generally benign but potentially malignant tumor originating in a small aggregate of cells of neuroectodermal type The normal carotid body is small (4 mm in greatest diameter and 10 mg in weight) and is located at the bifurcation of the common carotid artery The cells are of uniform size, have an abundant cytoplasm, are rich in substance P, and are sensitive to changes in PO2, PCO2, and pH (ie, they are chemoreceptors, not to be confused with baroreceptors) The tumors that arise from these cells are identical in appearance with tumors of other chemoreceptor organs (paragangliomas) Interestingly, they are 12 times more frequent in individuals living at high altitudes Clinically the usual presentation is of a painless mass at the side of the neck below the angle of the jaw; thus it must be differentiated from the branchial cleft cyst, mixed tumor of the salivary gland, and carcinomas and aneurysms in this region As the tumor grows (at an estimated rate of 20 cm in diameter every 5 years), it may implicate the sympathetic, glossopharyngeal, vagus, spinal accessory, and hypoglossal nerves (syndrome of the retroparotid space; see Chap 47) Hearing loss, tinnitus, and vertigo are present in some cases Tumors of the carotid body have been a source of transient ischemic attacks in 5 to 15 percent of the 600 or more reported cases One of the most interesting presentations has been with sleep apnea, particularly with bilateral tumors (see below); respiratory depression as well as lability of blood pressure are common postoperative problems Malignant transformation occurs in 5 percent of cases A similar paraganglioma of the vagus nerve has been reported; it occurs typically in the jugular or nodose ganglion but may arise anywhere along the course of the nerve These tumors may also undergo malignant transformation, metastasize, or invade the base of the skull A carotid body tumor, separate in nature from the glomus jugulare tumor, has been seen in combination with von Recklinghausen neuro bromatosis Familial cases are known, especially with bilateral carotid body tumors (about 5 percent of these tumors are bilateral) The treatment should be surgical excision with or without prior intravascular embolization; radiation therapy is not advised Pituitary Adenomas (See also page 486) Tumors arising in the anterior pituitary are of considerable interest to neurologists because they often cause visual and other symptoms related to involvement of structures bordering upon the sella turcica before an endocrine disorder becomes apparent Pituitary tumors are agelinked; they become increasingly numerous with each decade; by the 80th year, small adenomas are found in more than 20 percent of pituitary glands In some cases, an apparent stimulus to adenoma
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