Ординатура / Офтальмология / Английские материалы / Neuro-Ophthalmology_Kidd, Newman, Biousse_2008
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9 Primary and Secondary Tumors of the Optic Nerve and Its Sheath |
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unless the infiltration is bilateral and symmetric. In addition, there are often peripapillary and peripheral retinal hemorrhages.145 Neuroimaging in such cases typically reveals a diffusely enlarged optic nerve that usually enhances
after intravenous injection of contrast material.146 In other patients there may be a paucity of findings on both ophthalmoscopy and MRI.136,141
The response of leukemic infiltration of the optic nerve to radiation therapy is usually rapid and dramatic. In almost all cases, visual function returns to normal
or near normal, and the disc elevation, if present, resolves.145 Even though some patients do not respond to this treatment,146,147 radiation therapy is the treat-
ment of choice for optic nerve infiltration in the setting of acute leukemia.135,137,148 In addition, Brown et al132 successfully treated a patient with
an infiltrative optic neuropathy in the setting of acute promyelocytic leukemia with trans-retinoic acid. This form of acute leukemia results from a chromosome translocation that is associated with the retinoic acid receptor gene,149 and transretinoic acid works by stimulating the terminal differentiation of the malignant cells so that they become normal lymphoid cells.
It must be emphasized that swelling of the optic disc can occur in patients with acute leukemia when CNS involvement by the disease results in increased intracranial pressure. Optic disc swelling and neovascularization also occur as a
local phenomenon in the setting of the diffuse retinopathy of acute leukemia.145,150 Thus, one must consider a number of pathologic mechanisms in
addition to infiltration in any patient with acute leukemia and apparent optic disc swelling.
The acute leukemias are responsible for most of the reported cases of infiltrative optic neuropathies caused by lymphoreticular disorders; however, patients with chronic forms of leukemia, particularly chronic lymphocytic leukemia, may also develop optic nerve infiltration.151 Patients with optic nerve infiltration in the setting of CLL and other chronic leukemias have a more indolent clinical course than patients with the acute leukemias. Visual loss is less severe, and retinal changes of the type seen in acute leukemia are rare. Optic disc swelling, however, is indistinguishable from that which occurs in patients with infiltration in the setting of acute leukemia.
Summary
Most primary tumors of the optic nerve and its sheath are benign and produce slowly progressive visual loss associated with evidence of an anterior or posterior optic neuropathy and variable proptosis. The diagnosis of the most common tumors—glioma and meningioma—can be made with neuroimaging. However, in some cases, the diagnosis is not made until the orbit is explored and a biopsy of the nerve is obtained or the affected nerve is excised. The treatment of optic nerve tumors depends on the nature of the lesion. In some cases, excision of the nerve is appropriate; however, in most, observation, radiation, or chemotherapy is a better choice. The visual prognosis of primary optic nerve tumors depends on the nature of the lesion. Secondary tumors of the optic nerve are rare but much more serious, not only because of the poor visual prognosis but also because they are often associated with a poor systemic prognosis.
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10 Disorders of the Sella and
Parasellar Region
MICHAEL POWELL
Overview
Clinical Presentation
Endocrine Presentation and
Investigation
Visual Symptoms and
Measurement
Headache and Symptoms of
Raised Intracranial Pressure
Incidental Discovery
Tumor Types
Pituitary Adenomas
Meningiomas
Rathke’s Cleft Cysts
Craniopharyngiomas
Other Tumors
Cysts
Arachnoid Cysts
Metastatic Tumors
Infections and Inflammations
Hypophysitis
Langerhans Cell Histiocytosis
Vascular Lesions
References
Key Points
The parasellar region may be host to a wide range of pathologies, from embryologic remnants and tumors to inflammatory, infective, and vascular disorders.
Only one in 20 tumors in this region is not a pituitary adenoma.
Patients present with endocrine symptoms or visual dysfunction because of pressure effects from a mass lesion and in an asymptomatic phase when
the lesion is discovered on imaging for investigation for an unrelated problem.
Overview
The pituitary and parasellar region is host to a wide range of pathologies, as the tissues that are normally found there derive from every part of the embryo, from the Rathke’s cleft elements of the primitive stomatodeum through mesenchyme to neuroectodermal elements of the brain itself.1 Not only is there a vast number of tumors that are derived from these structures but inflammatory disorders,
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infections, and aneurysms and other vascular malformations may cause symptoms in this region. Most lesions in the area are pituitary adenomas, in fact only 1 in 20 is not, and of the remaining 5%, most are planum sphenoidale meningiomas, craniopharyngiomas, or Rathke’s cleft cysts.
For clinicians, the good news is that whatever the lesion, there are only four basic ways in which these tumors can be discovered. These are with endocrine dysfunction, visual symptoms, pressure symptoms of a mass lesion, and now, with the frequency of intracranial imaging for every imaginable symptom, by chance in the asymptomatic patient. These presentations are discussed in more detail in the following section.
Clinical Presentation
ENDOCRINE PRESENTATION AND INVESTIGATION
Endocrine symptoms can be the result of both underproduction and overproduction of pituitary hormones.2 Pituitary function is vital to the normal body, and without two of the five anterior pituitary hormones, serious morbidity and even death can rapidly follow. These are adrenocorticotropin-stimulating hormone (ACTH) as the central part of the hypothalamic-pituitary-adrenal axis and thyroid-stimulating hormone (TSH). These are the two hormones that are responsible for the majority of symptoms in patients presenting with hypopituitarism: weight loss, lethargy, and general underperformance. Many patients will have suffered from decreased sexual function (libido, failed menses, etc.) for some time resulting from lack of the gonadotrophins (luteinizing hormone [LH] and follicle-stimulating hormone [FSH]) and on occasions from the symptoms of general lethargy from adult growth hormone (GH) deficiency.
The fifth anterior pituitary hormone, prolactin, has a more complex role. In males, its existence has no known biologic function, and in women its only role is in lactation; however, when elevated, it has a powerful action in suppressing gonadotroph cell activity, resulting again in symptoms of lack of libido, sexuality, and menses. The lactotroph cells from which the hormone is secreted are, unusually, under mainly negative feedback control from the hypothalamus; dopamine inhibits hormone release. Therefore, any tumor upsetting the hypothalamus, median eminence, or stalk can have the effect of leading to a paradoxical rise in prolactin. The rise may be up to 5000 mU/L (upper limit of normal 500 to 700 mU/L); it is unwise, therefore, to treat all moderate hyperprolactinemias with dopamine agonists as they may restore gonadotroph function, allowing the tumor causing the hyperprolactinemia to continue to grow unrecognized. True macroprolactinomas have levels in excess of 5000 mU/L as a rule, although not always.
Pituitary endocrine overfunction is well recognized in a number of diseases. Acromegaly (4 to 8 per million new cases per annum) from somatotroph cells, Cushing’s disease (2 to 4 per million per annum) from corticotroph cells, and prolactinomas all have well-recognized clinical syndromes and symptoms. Each of these has protocols for treatment. For some reason, TSH-secreting adenomas causing high TSH thyrotoxicosis are rare. Many of the so-called nonfunctioning tumors (or null cell tumors) contain LH, FSH, or both on careful study, although they never seem to express their hormones in a recognized clinical syndrome.
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Figure 10–1 Coronal magnetic resonance imaging, T1 with enhancement. Thyrotroph hyperplasia in a 16-year-old girl who presented with delayed puberty.
Two rarities of anterior function syndromes are also worth mentioning. Primary thyroid failure may lead to exuberant thyrotroph hyperplasia (Fig. 10–1), which because of its rarity is seldom thought of in the differential diagnosis. The patient is particularly vulnerable if thyroid function is not fully checked. Second, hypothalamic lesions in children, although usually presenting with reduced stature from lack of GH, can on occasions produce the opposite, precocious puberty.
Posterior lobe function is equally important; the presence of diabetes insipidus from a lack of antidiuretic hormone (ADH) almost precludes the diagnosis of a pituitary adenoma. It is, however, not uncommon in craniopharyngioma and other lesions that involve the hypothalamus. Overproduction of ADH (syndrome of inappropriate antidiuretic hormone [SIADH]) usually occurs in the early postoperative period, but hyponatremia may on occasion be part of the presentation of other large pituitary-based lesions as part of hypopituitarism.
Investigations for pituitary function should always include prolactin and thyroid function. Complex dynamic testing of the hypothalamic-pituitary axis is suggested by some, although for others a morning cortisol level of more than 300 nmol/L will suffice. Sex hormone levels are of interest, although a single level of FSH and LH in a young woman will only be of use if both are very low, and in men testosterone levels will be of more value than the gonadotroph levels. Both acromegaly and Cushing’s disease require complex investigation with a barrage of special endocrine tests. Posterior lobe function seldom requires investigation, but if diabetes insipidus is suspected, urine output, serum and urine osmolarity, and fluid restriction investigations may be required.
VISUAL SYMPTOMS AND MEASUREMENT
Most of the patients seen with sella and parasellar pathology in a clinical neuroscience center have problems with their vision. Although visual symptoms are theoretically a less common presentation than those of hyperprolactinemia, compression of the optic chiasm with its attendant symptom of bitemporal visual field loss by a macroadenoma remains a regular problem leading to a neurosurgical admission. Although 1 in 20 macroadenomas are associated with acromegaly and even Cushing’s disease can present as a large tumor, the majority