Related Subjects:
|Adrenal Physiology
|Addisons Disease
|Phaeochromocytoma
|Adrenal Adenomas
|Adrenal Cancer
|Cushing Syndrome
|Cushing Disease
|Congenital Adrenal hyperplasia
|Primary hyperaldosteronism (Conn's syndrome)
|ACTH
|McCune Albright syndrome
|Male Infertility
|Prolactin
|Prolactinoma
|Sheehan's syndrome
In cases of low 9 am cortisol or inadequate response on short synacthen, always prioritize steroid replacement to manage stress.
About
- Usually benign tumours from the anterior pituitary; incidence of 77 per 100,000.
- Types include hormone-secreting and non-secreting, with macroadenomas (>10mm) and microadenomas (<10 mm).
Aetiology
- Monoclonal benign tumours with various genetic mutations.
Common Tumor Types and Activities
- Prolactinomas (57%), nonfunctioning adenomas (28%), GH-secreting (11%), ACTH-secreting (2%), and TSH-secreting (<1%).
Pathophysiology
- Hormonal effects (e.g., prolactin, GH) often predominate over tumour mass effects.
- Pressure effects may include headache, bitemporal hemianopia, and optic chiasma compression.
- Rarely, may lead to complications like diabetes insipidus and CSF rhinorrhea.
Clinical Manifestations
- Hormonal effects: Elevated prolactin, GH, ACTH, leading to galactorrhea, acromegaly, Cushing's syndrome.
- Pressure symptoms: Bitemporal hemianopia, headache, ophthalmoplegia.
- Hypopituitarism symptoms: Low testosterone, TSH, and cortisol levels, with signs of adrenal insufficiency and hypothyroidism.
Investigations
- Initial hormone levels: 9 am cortisol, PRL, LH, FSH, TSH, testosterone, IGF-1 if acromegaly suspected.
- Imaging: MRI pituitary with sellar views.
- Visual assessment: Goldman perimetry for visual field defects.
Management
- Cushing's Disease:Surgery (trans-sphenoidal), radiotherapy, or medications (ketoconazole, metyrapone).
- Prolactinomas:First-line treatment with dopamine agonists (cabergoline preferred). Surgery reserved for non-responsive cases.
- Acromegaly:Surgery, radiotherapy, and drugs (somatostatin analogues, GH receptor antagonists).
- Nonfunctioning Adenomas:Surgery if compressive; otherwise, observation with regular visual assessments.
Hypopituitarism Management
- Steroids:Hydrocortisone 10 mg morning, 5 mg at noon, and 5 mg evening. Educate on doubling dose during illness.
- Thyroid:Thyroxine 75-150 mcg/day (monitor T4 levels, not TSH).
- Sex Hormones:Testosterone or estrogen/progesterone replacement as needed.
- ADH (for diabetes insipidus):Desmopressin intranasal or oral.
Complications and Follow-Up
- Monitor for CSF leaks, bitemporal hemianopia, and potential for recurrent tumour growth with regular imaging and endocrinology follow-ups.