Medical Care
See the list below:
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Prolactinomas: The majority of these lesions respond to dopamine receptor agonists. Improvement in visual field abnormalities, resolution of symptoms associated with hyperprolactinemia, and visible diminution of the actual mass can result with treatment.
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Acromegaly: Somatostatin analogues (octreotide) can be helpful in the treatment of increased postoperative levels of GH. In some cases, the tumor may shrink modestly. Gallstones are a frequent complication of somatostatin-analogue therapy. Dopamine agonists also have been used.
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Replacement therapy for decreased or absent hormones should be instituted as needed.
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All hormone-based treatment should be directed by a consulting endocrinologist.
Surgical Care
Pituitary surgery has undergone quite an evolution since the days of Harvey Cushing’s research and his pioneering development of the sublabial and transcranial methods of accessing the sella. Besides the transsphenoidal/translabial approaches, an endoscopic transnasal approach has become an additional an increasingly favored option with a wider surgical field. For intrasellar tumors the transsphenoidal or endonasal endoscopic techniques show similar results but for larger extrasellar tumors the endonasal approach may be preferred. [14]
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Transsphenoidal surgery
Transsphenoidal microscopic surgery is the most frequent surgical approach for the resection of pituitary tumors. With larger lesions, a transfrontal approach may become necessary to decompress the visual pathways.
Minimally invasive endoscopic surgery using a 4-mm endoscope through a nostril is a possibility in selective cases. A 2013 review examined the transnasal endoscopy approach for resection of giant adenomas with profound mass effect. In 54 cases, the lesions had the greatest diameters, measuring 4 cm; roughly 80% of them had visual-field defects. Near-total resection was achieved in 67% of cases, demonstrating efficacy of the approach in larger tumors. The endonasal approach was concluded to be effective, not only in tumor removal, but had limited complication rates. CSF leak, residual tumor, postoperative diabetes insipidus, and apoplexy in residual adenoma were listed as complications.
Open low-field intraoperative MRI monitoring for transsphenoidal surgical resection is gaining acceptance to monitor the precise extent of tumor resection.
Null cell tumors and gonadotrophinomas are best treated with transsphenoidal surgery.
The main complication after transsphenoidal surgery (from the endocrine standpoint) is hypopituitarism.
Low- and high-field intraoperative monitoring is used to minimize resectable tumor. [15]
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Prolactinomas
Microprolactinomas: Transsphenoidal resection of the tumor offers a chance for a cure without the need for long-standing dopamine agonist therapy; however, many patients choose dopaminergic therapy.
Macroadenomas that secrete prolactin are best treated with dopamine agonists.
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Acromegaly
Transsphenoidal surgery decreases GH levels to less than 5 mcg/L in 60% of cases.
Normal pulsatile secretion of GH is not always regained, and 20% of patients continue to have increased GH levels in response to TRH.
Radiotherapy is an alternative, although GH levels may not decrease for 2-4 years.
Elevated GH levels may be treated with somatostatin analogues and dopamine agonists, if tolerated.
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Cushing disease
Transsphenoidal tumor resection is the first line of treatment in patients with basophilic adenomas of the pituitary gland. It is curative in 80% of cases.
Pituitary irradiation is required in the remaining cases to prevent the development of Nelson syndrome.
In children, pituitary irradiation and adrenalectomy are highly effective.
Immediate postoperative biochemical remission of Cushing syndrome, evidenced by cortisol levels less than 2 micrograms/dL, was associated with sustained postoperative remission lasting 68.4 months. MRI evidence of a microadenoma was also a predictor of successful tumor resection via a transsphenoidal microsurgical approach.
Consultations
See the list below:
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The treatment team should consist of any or all of the following specialists: ophthalmologist or neuro-ophthalmologist, neuroradiologist, endocrinologist, gynecologist, neurosurgeon, neuropathologist, and radiation medicine specialist.
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Different specialists may be involved as indicated by the patient's specific symptoms.
Diet
See the list below:
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Dietary factors are important in patients with acromegaly or Cushing disease.
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Patients with hypothyroidism, hypoadrenalism, or hypopituitarism have specific dietary needs.
Activity
See the list below:
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Activities of daily living (ADLs) generally are not restricted in these patients.
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Exercise tolerance may be limited in some cases.
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This is a characteristic bitemporal hemianopic visual field defect.
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This contrast-enhanced coronal MRI was obtained in a patient who complained of visual loss.
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This visual field was plotted using a Goldman perimeter (ie, kinetic perimetry). It was obtained from a patient who reported visual loss and had a normal endocrine workup. The dark areas correspond to the impaired peripheral visual field. This visual field defect is consistent with an intrasellar lesion.
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Coronal T1 precontrast MRI A (left panel), B postcontrast (middle panel) and T2 (right panel) showing a sellar mass causing obvious left and upward displacement of the optic chiasm. The mass is a histologically proven pituitary macroadenoma, which presented initially with a large cystic subfrontal extension that was successfully resected in April of 2006. This patient has been observed closely for 2.5 years and despite obvious mass effect, he has no visual complaints and the neuro-ophthalmologic evaluation is normal. Although infrequent, clinicians should be aware of this possibility. Close follow-up is required.
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Axial, sagittal, and coronal MRI of the sellae in a patient with a severe headache, normal neuro-ophthalmologic examination, and no evidence of endocrine failure. A hyperintense mass is observed in the sella with suprasellar extension. This case illustrates the clinical spectrum of pituitary apoplexy. Transsphenoidal resection confirmed the diagnosis of pituitary apoplexy.
