Ординатура / Офтальмология / Английские материалы / Neuro-Ophthalmology_Kidd, Newman, Biousse_2008
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Figure 10–13 Sagittal magnetic resonance imaging, T1 with enhancement. Clival chordoma compressing brainstem. The astute may notice the enlarged pituitary fossa; the patient had had surgery and radiotherapy for acromegaly 20 years previously.
worth considering because of their benign behavior and radiation insensitivity.42 It is important, therefore, to resist the temptation to offer radiotherapy as it has no known benefit and makes further surgery extremely difficult.
Chordomas
Intracranial chordomas occasionally involve the cavernous sinus and pituitary fossa at presentation (Fig. 10–13) but are found more commonly in the clivus where the lower cranial nerves are more likely to be affected. Unlike the chondrosarcoma, the behavior is usually far from benign, and relentless progression despite heroic surgery and radiotherapy is the norm.64–68 Fast neutrons and other hard to source forms of radiation are said to be worth considering.
Schwannomas
Schwannomas of the trigeminal nerve arising in the cavernous sinus are uncommon. It might be expected that they would present with facial sensory loss or pain, but those seen at the National Hospital have all presented with ophthalmoplegia. Slow growing, they can be seen as bony scalloping on CT scans of the skull base but are as usual best imaged with gadolinium enhanced MRI (Fig. 10–14).
These tumors are usually considered surgical challenges (access is along the skull base using extradural approaches)54–57 but may become a group of tumors,
which when moderate to small, are best treated with single fraction radiotherapy stereotactic techniques such as gamma knife, like vestibular schwannomas.58
Germinomas
The pituitary is a well-recognized if uncommon site for germinoma, a rare tumor; it presents with panhypopituitarism, often with diabetes insipidus.
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Figure 10–14 Axial magnetic resonance imaging, T1 with enhancement. Temporal fossa trigeminal schwannoma presenting with double vision. Only a small component extends from the mouth of Meckel’s cave into the posterior fossa.
When found, their treatment is with minimal surgery, usually by the trans-
sphenoidal route, followed by radiotherapy, to which they are exquisitely sensi- tive.37–53 Usually, however, by the time the histologic diagnosis is made, the
surgery has been radical.
Cysts
Epidermoids and dermoids arise only rarely in the sella region. Their incidence
is very low and, because these developmental anomalies grow insidiously and very slowly, they may be massive at discovery.120–123 The patient illustrated in
Figure 10–15 had no visual problems but a mild hypothalamic syndrome and went on to develop seizures.
ARACHNOID CYSTS
An incredible amount of surgical discussion time is given to arachnoid cysts without much clarity of resolve. Mostly these are incidental findings; occasionally they
may present with hyperprolactinemia. What one does about them is another matter.113–119 They are probably best left alone; I have been persuaded to explore
them and have been left with a dramatic cerebrospinal fluid leak as a consequence.
Metastatic Tumors
The pituitary fossa is not a common place for carcinomatous metastasis. Metastases from lung and breast carcinoma are more common. There may be neuroepithelial differentiation, and often many of the histologic features of a simple
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Figure 10–15 Sagittal magnetic resonance imaging, T1 with enhancement. Large sella epidermoid presenting with variable headache and nonspecific symptoms, possibly epilepsy, in a 40-year-old woman.
adenoma are seen. Although the imaging features are usually those of a typical benign adenoma, they almost always have headache and panhypopituitarism with diabetes insipidus, most uncommon in benign pituitary adenoma, and therefore a warning sign.
Surgery and oncologic management, usually radiotherapy, is required.139–166 Other metastases may occur, for example, lymphoma and melanoma, but are
very rare; I have also seen a choriocarcinoma in the pituitary.
Infections and Inflammations
Infection within the sphenoid is rare, even with extensive sinus disease. It is one of the fortunate coincidences of life that however potentially dirty the nose is, transsphenoidal surgery, at least when not complicated by cerebrospinal fluid leak, tends to have a very low prevalence of postoperative infection. Mucoceles
in the sphenoid sinus do occur, however (Fig. 10–16), as well as sphenoid sinusitis.153–156 Usually there has been previous surgery. Infected trapped
mucoceles can trigger pituitary apoplexy.
Of primary infections of the pituitary, only tuberculosis has been seen in my
practice (Fig. 10–17). It is painless but causes panhypopituitarism with diabetes insipidus.177–180 Pyogenic abscesses may occur,146–152 and fungal and parasitic infections have also been reported.180–185
HYPOPHYSITIS
Hypophysitis is an autoimmune disease in which the pituitary, usually the adenohypophysis, is involved. It typically occurs in the last trimester of pregnancy or in the immediate postpartum period. Patients present with pituitary failure and visual loss from chiasmal compression. Prolactin should be extremely high in the postpartum period, so if this and other pituitary hormones are low, it is reasonable to make the diagnosis without histology.
High-dose steroids cause a prompt resolution of the imaging abnormalities, and the visual loss usually recovers well.157–164
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Figure 10–16 A, Sagittal magnetic resonance imaging (MRI), T1 with enhancement. Large prolactinoma with two entrapment mucoceles in the sphenoid sinus. Compare with Figure 10–16B. B, Sagittal MRI, T1 with enhancement. Florid enhancement in infected mucocele caused by large macroadenoma.
Figure 10–17 Sagittal magnetic resonance imaging, T1 with enhancement. Tuberculosis of the hypophysis. Note that the enhancing mass replaces the normal gland. There was pituitary failure, diabetes insipidus, and headache. The observer could be forgiven for thinking this looks like an adenoma. Compare with Figure 10–19.
The condition may, however, occur in postmenopausal women and even in men.161,162 It may involve the cavernous sinus and so present with pain and
ophthalmoparesis. Although the majority needs only a relatively short period of high-dose steroids, some have a more relapsing course and may need plasma exchange or radiotherapy for control.
LANGERHANS CELL HISTIOCYTOSIS
Langerhans cell histiocytosis, the most common variant of an extremely rare condition, is found in children usually presenting with diabetes insipidus proceeding on to anterior pituitary failure. It usually involves the pituitary
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Figure 10–18 Axial magnetic resonance imaging, T1 with enhancement. Langerhans cell histiocytosis. The patient had a 5-year history of diabetes insipidus progressing to anterior pituitary failure.
infundibulum (Fig. 10–18) and can be managed on an imaging diagnosis, by hormone replacement and steroids. Biopsy, via a transsphenoidal route, can be
achieved with care and some difficulty.173
Granular cell tumor (Fig. 10–19), sarcoidosis,165–171 Rosai-Dorfman disease,186 and Chester-Erdheim disease187 may also present with pituitary involvement.
Figure 10–19 Sagittal magnetic resonance imaging, T1 with enhancement. Granular cell tumor. Compare with Figure 10–17; similar story, although no diabetes insipidus. This image is indistinguishable from an adenoma.
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Figure 10–20 A, Axial computed tomography. Aneurysm presenting with bitemporal visual failure. B, Carotid angiogram of the same lesion, anterior-posterior view.
Vascular Lesions
I have only seen one aneurysm presenting as a pituitary tumor, with bitemporal hemianopia. It was in the pre-MRI days. Luckily, there were some suspicious features on the CT and an angiogram was requested (Fig. 10–20). Horror stories of surgeons biopsying aneurysms through the transsphenoidal route do exist, but modern MR is so powerful a diagnostic tool, the stories are drifting into legend. Nowadays, such a lesion would probably be coiled.
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