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mous, heteronymous, and sometimes bitemporal type and progressing to blindness and optic atrophy with or without papilledema Ocular proptosis from the orbital mass is the other main feature Hypothalamic signs (adiposity, polyuria, somnolence, and genital atrophy) occur occasionally as a result of proximal tumor extension CT scanning, MRI, and ultrasound will usually reveal the tumor, and radiographs will show an enlargement of the optic foramen (greater than 70 mm) This nding and the lack of ballooning of the sella or of suprasellar calci cation will exclude pituitary adenoma, craniopharyngioma, Hand-Schuller-Christian disease, and sarcoidosis In adolescents and young adults, the medial sphenoid, olfactory groove, and intraorbital meningiomas (optic nerve sheath meningioma) are other tumors that cause monocular blindness and proptosis If the entire tumor is prechiasmatic (the less common con guration), surgical extirpation can be curative For tumors that have in ltrated the chiasm or are causing regional symptoms and hydrocephalus, partial excision followed by radiation is all that can be offered Both gliomas and nontumorous gliotic (hamartomatous) lesions of the optic nerves may occur in von Recklinghausen disease; the latter are sometimes impossible to distinguish from optic nerve gliomas Chordoma This is a soft, jelly-like, gray-pink growth that arises from remnants of the primitive notochord It is located most often along the clivus (from dorsum sellae to foramen magnum) and in the sacrococcygeal region It affects males more than females, usually in early or middle adult years, and is one of the rare causes of syndromes involving multiple cranial nerves or the cauda equina About 40 percent of chordomas occur at each of these two ends of the neuraxis; the rest are found at any point in between The tumor is made up of cords or masses of large cells with granules of glycogen in their cytoplasm and often with multiple nuclei and intracellular mucoid material Chordomas are locally invasive, especially of surrounding bone, but they do not metastasize The cranial neurologic syndrome is remarkable in that all or any combination of cranial nerves from the second to twelfth on one or both sides may be involved Associated signs in the series of Kendell and Lee were facial pain, conductive deafness, and cerebellar ataxia, the result of pontomedullary and cerebellar compression A characteristic sign is neck pain radiating to the vertex of the skull on neck exion The tumors at the base of the skull may destroy the clivus and bulge into the nasopharynx, causing nasal obstruction and discharge and sometimes dysphagia Extension to the cervical epidural space may result in cord compression Thus, chordoma is one of the lesions that may present both as an intracranial and extracranial mass, the others being meningioma, neuro broma, glomus jugulare tumor, and carcinoma of the sinuses or pharynx Plain lms of the base of the skull, in addition to MRI, are important in diagnosis Midline (Wegener) granulomas, histiocytosis, and sarcoidosis also gure in the differential diagnosis Chondrosarcoma of the clivus produces a similar syndrome Treatment of the chordoma is surgical excision and radiation (proton beam or focused gamma radiation) This form of treatment has effected a 5-year cure in approximately 80 percent of patients Nasopharyngeal Growths that Erode the Base of the Skull (Nasopharyngeal Transitional Cell Carcinoma, Schmincke Tumor) These are rather common in a general hospital; they arise from the mucous membrane of the paranasal sinuses or the nasopharynx near the eustachian tube, ie, the fossa of Rosenmuller In addition to symptoms of nasopharyngeal or sinus disease, which may not be.

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prominent, facial pain and numbness (trigeminal), abducens, and other cranial nerve palsies may occur Diagnosis depends on inspection and biopsy of a nasopharyngeal mass or an involved cervical lymph node and radiologic evidence of erosion of the base of the skull Bone and CT scans are helpful in diagnosis (Fig 47-5, page 1188) The treatment is surgical resection (in some cases) and radiation Carcinoma of the ethmoid or sphenoid sinuses and postradiation neuropathy, coming on years after the treatment of a nasopharyngeal tumor, may produce similar clinical pictures and are dif cult to differentiate Special imaging techniques, such as the diffusion-weighted MRI sequence, may be useful in separating them The several syndromes resulting from nasopharyngeal tumors are discussed in Chap 47, under Diseases of the Cranial Nerves Other Tumors of the Base of the Skull In addition to meningioma, nasopharyngeal tumors, and the other tumors enumerated above, there are a large variety of tumors, rare to be sure, that derive from tissues at the base of the skull and paranasal sinuses, ears, etc, and give rise to distinctive syndromes Included in this category are osteomas, chondromas, ossifying bromas, giant-cell tumors of bone, lipomas, epidermoids, teratomas, mixed tumors of the parotid gland, and hemangiomas and cylindromas (adenoid cystic carcinomas of salivary gland origin) of the sinuses and orbit; sarcoid produces the same effect Most of these tumors are benign, but some have a potential for malignant change To the group must be added the esthesioneuroblastoma (of the nasal cavity) with anterior fossa extension and, perhaps most common of all of these, the systemic malignant tumors that metastasize to basal skull bones (prostate, lung, and breast being the most common sources) or involve them as part of a multicentric neoplastic process, eg, primary lymphoma, multiple myeloma, plasmacytoma, and lymphocytic leukemia Suprasellar arachnoid cysts also occur in this region CSF ows upward from the interpeduncular cistern but is trapped above the sella by thickened arachnoid (membrane of Liliequist) As the CSF accumulates, it forms a cyst that invaginates the third ventricle; the dome of the cyst may intermittently block the foramina of Monro and cause hydrocephalus (Fox and Al-Mefty) Children with this condition exhibit a curious to-and-fro bobbing and nodding of the head, like a doll with a weighted head resting on a coiled spring This has been referred to as the bobble-headed doll syndrome by Benton and colleagues; it can be cured by emptying the cyst See-saw and other pendular and jerk types of nystagmus may also result from these suprasellar lesions Details of the pathology, embryogenesis, and symptomatology of these rare tumors are far too varied to include in a textbook devoted to principles of neurology Table 31-4, and Table 47-1 on page 1180, adapted from Bingas s large neurosurgical service in Berlin, summarizes the known facts about the focal syndromes of the skull base; his authoritative article and the more recent one by Morita and Piepgras, both in the Handbook of Clinical Neurology, are recommended references Modern imaging techniques now serve to clarify many of the diagnostic problems posed by these tumors MRI is particularly helpful in delineating structures at the base of the brain and in the upper cervical region CT is also capable of determining the absorptive values of the tumor itself and the sites of bone erosion When the lesion is analyzed in this way, an etiologic diagnosis often becomes possible For example, the absorptive value of lipomatous tissue is different from that of brain tissue, glioma, blood, and calcium Bone scans (technetium and gallium) display active.

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