Pituitary Tumor Apoplexy: Characteristics, Treatment, and
Pituitary Tumor Apoplexy: Characteristics, Treatment, and
Pituitary tumor apoplexy is an uncommon event heralded by abrupt onset of severe headache, restriction of visual fields, deterioration of visual acuity, and weakness of ocular motility frequently coupled with clinical indications of decreased endocrine function. Hemorrhage into or necrosis of a preexisting sellar mass, usually a pituitary macroadenoma, produces an expansion of sellar contents. Compression of adjacent structures elicits the variable expression of symptoms referable to displacement of the optic nerves and chiasm and impingement of the third, fourth, and sixth cranial nerves. Damage to or destruction of the anterior pituitary leads to multiple acute and/or chronic hormone deficiencies in many patients. Medical management may be used in rare cases in which the signs and symptoms are mild and restricted to meningismus or ophthalmoplegia deemed to be stable. In patients with visual or oculomotor lability or an altered level of consciousness, expeditious surgical decompression, accomplished most commonly through a transsphenoidal approach, should be performed to save life and vision and to optimize the chance of regaining or maintaining pituitary function.
Pituitary tumor apoplexy (the second syllable of the suffix "-plexy," originating from the Greek term meaning to strike or to have a stroke) refers to the abrupt onset of a severe headache frequently coupled with nausea, vertigo, meningismus, and/or a decreased level of consciousness. Ophthalmoplegia, deterioration of visual acuity, and restriction of visual fields are also frequently encountered ( Table 1 ). The episode is most often obvious and discrete, but may have a more subtle onset, or even be clinically silent.
Signs and symptoms are attributed to the rapid expansion of an infarcted and/or hemorrhagic pituitary adenoma that extends laterally into the cavernous sinus or reaches superiorly to displace the optic nerves and chiasm. Computerized tomography or MR imaging indicate sellar and/or suprasellar abnormalities that are consistent with hemorrhage and necrosis.
Bailey described the first case of pituitary tumor associated with hemorrhage in 1898; in 1950, Brougham and colleagues examined five patients and called the collection of signs and symptoms due to necrosis and/or hemorrhage "pituitary apoplexy." We believe that the term "pituitary tumor apoplexy" is more suitable because the syndrome is associated with a pituitary adenoma in the overwhelming number of cases and is linked with healthy glands in only a few isolated instances.
Pituitary adenomas comprise approximately 10% of intracranial tumors; the reported incidence of apoplexy in these lesions ranges from 0.6 to 27.7%. Values quoted at the high end of this range may reflect the inclusion of cases in which blood discovered during surgical or histopathological examination was obtained from specimens of asymptomatic patients. Most reports of series indicate that the incidence of apoplexy among pituitary adenomas is 2 to 7% when clinical signs coupled with surgical or histopathological evidence are considered. Recurrence following apoplexy is rare and has only been documented in a few cases. The clinical syndrome usually evolves from within a few hours to 3 days.
All types of tumors are at similar risk for developing apoplexy. Nearly 50% of apoplectic events occur in patients who were not previously known to harbor a pituitary lesion. Men are affected more commonly than women and in some series the ratio of male to female patients is as divergent as 2:1. The age range of affected individuals stretches from the first through the eighth decade, with most cases presenting among individuals in the fifth or sixth decade.
Abstract
Pituitary tumor apoplexy is an uncommon event heralded by abrupt onset of severe headache, restriction of visual fields, deterioration of visual acuity, and weakness of ocular motility frequently coupled with clinical indications of decreased endocrine function. Hemorrhage into or necrosis of a preexisting sellar mass, usually a pituitary macroadenoma, produces an expansion of sellar contents. Compression of adjacent structures elicits the variable expression of symptoms referable to displacement of the optic nerves and chiasm and impingement of the third, fourth, and sixth cranial nerves. Damage to or destruction of the anterior pituitary leads to multiple acute and/or chronic hormone deficiencies in many patients. Medical management may be used in rare cases in which the signs and symptoms are mild and restricted to meningismus or ophthalmoplegia deemed to be stable. In patients with visual or oculomotor lability or an altered level of consciousness, expeditious surgical decompression, accomplished most commonly through a transsphenoidal approach, should be performed to save life and vision and to optimize the chance of regaining or maintaining pituitary function.
Background and Epidemiological Considerations
Pituitary tumor apoplexy (the second syllable of the suffix "-plexy," originating from the Greek term meaning to strike or to have a stroke) refers to the abrupt onset of a severe headache frequently coupled with nausea, vertigo, meningismus, and/or a decreased level of consciousness. Ophthalmoplegia, deterioration of visual acuity, and restriction of visual fields are also frequently encountered ( Table 1 ). The episode is most often obvious and discrete, but may have a more subtle onset, or even be clinically silent.
Signs and symptoms are attributed to the rapid expansion of an infarcted and/or hemorrhagic pituitary adenoma that extends laterally into the cavernous sinus or reaches superiorly to displace the optic nerves and chiasm. Computerized tomography or MR imaging indicate sellar and/or suprasellar abnormalities that are consistent with hemorrhage and necrosis.
Bailey described the first case of pituitary tumor associated with hemorrhage in 1898; in 1950, Brougham and colleagues examined five patients and called the collection of signs and symptoms due to necrosis and/or hemorrhage "pituitary apoplexy." We believe that the term "pituitary tumor apoplexy" is more suitable because the syndrome is associated with a pituitary adenoma in the overwhelming number of cases and is linked with healthy glands in only a few isolated instances.
Pituitary adenomas comprise approximately 10% of intracranial tumors; the reported incidence of apoplexy in these lesions ranges from 0.6 to 27.7%. Values quoted at the high end of this range may reflect the inclusion of cases in which blood discovered during surgical or histopathological examination was obtained from specimens of asymptomatic patients. Most reports of series indicate that the incidence of apoplexy among pituitary adenomas is 2 to 7% when clinical signs coupled with surgical or histopathological evidence are considered. Recurrence following apoplexy is rare and has only been documented in a few cases. The clinical syndrome usually evolves from within a few hours to 3 days.
All types of tumors are at similar risk for developing apoplexy. Nearly 50% of apoplectic events occur in patients who were not previously known to harbor a pituitary lesion. Men are affected more commonly than women and in some series the ratio of male to female patients is as divergent as 2:1. The age range of affected individuals stretches from the first through the eighth decade, with most cases presenting among individuals in the fifth or sixth decade.
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