eMedicine Specialties > Pediatrics: General Medicine > Endocrinology
McCune-Albright Syndrome: Treatment & Medication
Updated: Feb 20, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Precocious puberty
- Precocious puberty in McCune-Albright syndrome is gonadotropin independent and therefore does not respond to the gonadotropin-releasing hormone (GnRH) agonist therapy that is so successful with gonadotropin dependent central precocious puberty.
- The aromatase inhibitor testolactone has been the mainstay of therapy in girls with persistent estradiol elevation. Aromatase inhibitors block the conversion of testosterone to estradiol, thus lowering circulating estrogen levels. Doses beginning at 20 mg/kg/d, divided in 3-4 doses, as much as 40 mg/kg/d are used. Although fairly effective, the large amount of medication required and the frequency of dosing make compliance difficult.
- Fadrozole, a more potent aromatase inhibitor, was shown in a clinical trial to be ineffective in preventing progression of precocious puberty. However, anastrozole, a highly selective aromatase inhibitor, was successful in significantly slowing precocious puberty in one case. It also had the added benefit of once daily dosage.
- Tamoxifen, an antiestrogen that was initially developed for use in estrogen–sensitive breast cancer, has recently been shown to be effective in treating precocious puberty in girls with McCune-Albright syndrome. One multicenter study was performed using 20 mg of tamoxifen once a day. They found significant improvement in growth velocity and rate of skeletal maturation.
- Ketoconazole was used in one study as an alternative therapy in 2 girls. They also showed significant improvement in signs of precocious puberty. Unfortunately, ketoconazole's dosing is 3 times daily, which is a drawback compared to the daily dosing of anastrozole or tamoxifen. Additional clinical studies of newer and more potent aromatase inhibitors, as well as antiestrogens and other therapies, continue to be pursued.
- Fibrous dysplasia: Symptomatic fibrous dysplasia is difficult to treat medically. Currently, no clinically proven medical therapies are available. Studies using both oral and intravenous bisphosphonates are encouraging. Conflicting data regarding the ability of bisphospanates to heal fibrous dysplasia have been reported. Some studies have shown no improvement in bone mineral density, whereas others have shown significant improvement in bone mineral density in the areas of fibrous dysplasia. However, bisphosphanates do make the lesions less painful.
- Hyperthyroidism: Hyperthyroidism due to functional thyroid follicular adenomas can be treated medically. Antithyroid medications, such as propylthiouracil and methimazole, can be used to decrease thyroid hormone production. Unlike Graves disease, hyperthyroidism secondary to an activating mutation of the alpha subunit of g protein (Gsa) is unlikely to go into remission. Therefore, patients probably should use antithyroid drugs indefinitely. Consider a more permanent treatment of the hyperthyroidism, including I131 therapy or thyroidectomy, if a diagnosis of McCune-Albright syndrome is confirmed.
- Hypophosphatemic rickets: The treatment for severe hypophosphatemia is similar to that used in X-linked dominant hypophosphatemic rickets. Phosphate supplementation (approximately 1.5-2.5 g/d divided in 5 doses) is given in addition to 1,25 dihydroxy vitamin D (calcitriol 0.25-0.5 mg/d).
- Infantile Cushing syndrome: No effective medical treatment for adrenocorticotropic hormone (ACTH)-independent Cushing syndrome is available, and the current recommendation for treatment is bilateral adrenalectomy. During the adrenalectomy and afterwards, the patient needs replacement of both glucocorticoid and mineralocorticoid in appropriate amounts. Stress doses of glucocorticoid (approximately 10 times maintenance) should be administered perioperatively and slowly reduced to maintenance replacement levels (hydrocortisone at 12-16 mg/m2/d divided in 3 doses). Mineralocorticoid replacement (Florinef at 0.05-0.1 mg/d) should be started soon after surgery as the hydrocortisone dose is weaned toward maintenance levels.
- Gigantism/acromegaly: Medical treatment is the most effective for growth hormone (GH)-producing adenomas. Radiation therapy may cause sarcomatous changes in the surrounding dysplastic bone if fibrous dysplasia is present. Octreotide, the long-acting somatostatin analogue, has been used with variable success in decreasing GH secretion from these tumors. Octreotide has been successful in lowering GH levels in many cases but rarely has normalized GH secretion. Dopamine agonists, such as bromocriptine and cabergoline, have also been used to decrease GH secretion. They are typically used in conjunction with octreotide. A study showed that cabergoline was able to decrease GH secretion but was unsuccessful in bringing GH secretion down to normal. Combination octreotide and cabergoline therapy has shown additional improvement in GH secretion compared with monotherapy but, in general, was not successful in bringing levels down to normal.
Surgical Care
- Precocious puberty: Historically, wedge resection of the ovary was performed if a single large follicular cyst was found. Unfortunately, this often only temporarily treated the estrogen hypersecretion and other large follicular cysts subsequently formed. Therefore, surgical treatment currently is not recommended to treat precocious puberty in McCune-Albright syndrome.
- Fibrous dysplasia: Fracture is the primary indication for surgical treatment of dysplastic lesions. Most fractures are treated with traction. However, proximal fractures of the femur may require surgically placed fixation devices. Rarely, severe and progressive malformation of the femur can occur. These lesions usually are painful because of the multiple small fractures associated with them and may need to be removed surgically. Routine removal of most polyostotic dysplastic lesions generally is not warranted and can result in the lesion recurring at the same site.
- Hyperthyroidism: Ablative therapy is warranted in treatment of hyperthyroidism due to McCune-Albright syndrome, either by radioactive iodine treatment or by thyroidectomy. A near total thyroidectomy should be considered, because any cells left behind containing activating Gsa mutations result in adenoma formation and recurrence of hyperthyroidism.
- Infantile Cushing syndrome: The current recommendation for treatment is bilateral adrenalectomy. See Medical Care for details on perioperative replacement of hormones.
- Gigantism/acromegaly: Consider surgical removal only if the tumor is threatening vision because removal is rarely curative.
Consultations
- Pediatric endocrine consultation should be considered in any child with McCune-Albright syndrome to evaluate for and manage the myriad potential endocrinopathies. Furthermore, new medical therapies to treat estrogen hypersecretion and fibrous dysplasia are being proposed in clinical studies that are available through some pediatric endocrine programs.
- Consider consultation with a pediatric orthopedic surgeon experienced in the management of polyostotic fibrous dysplasia prior to any major surgical procedure on dysplastic bone lesions. These lesions can be difficult to treat because of the soft nature of the dysplastic bone.
Diet
- No special dietary considerations are noted.
Activity
- Activity is not limited unless the patient has fibrous dysplasia located at critical sites in the skeleton. Because fibrous dysplasia can weaken the bone, the presence of a lesion in a weight bearing bone can increase the risk of a pathologic fracture.
- Therefore, limiting activities (eg, contact sports) in these cases should be considered.
Medication
The focus of therapy in McCune-Albright syndrome is to decrease secretion of the hormones in question. At present, no therapy addresses the underlying molecular problem (ie, the inappropriate activation of the Gsa subunit).
Aromatase inhibitors
Mainstay of therapy in girls with persistent estradiol elevation.
Testolactone (Teslac)
Inhibits steroid aromatase activity, thus blocking the conversion of testosterone to estradiol, decreasing the production of estrogen in the autonomous follicular cyst. Although fairly effective, the large amount of medication required and the frequency of dosing make compliance difficult. Clinical studies using newer and more potent aromatase inhibitors are currently being proposed.
Adult
Not indicated in adult women
Pediatric
20 mg/kg/d PO divided qid; up to 40 mg/kg/d
Possible increase in effects of PO anticoagulants
Documented hypersensitivity; pregnancy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Controlled substance (schedule III); monitor liver function; edema may develop in patients with congestive heart failure, liver, or renal insufficiency; may worsen hypertension; may exacerbate epilepsy and migraine
Anastrozole (Arimidex)
Highly selective aromatase inhibitor that significantly lowers serum estradiol concentrations by inhibiting the conversion of adrenally generated androstenedione to estrone. Daily dosing is convenient; case reports have shown good response; larger studies still needed.
Adult
Not indicated in adult women
Pediatric
Not established, data limited; 1 mg PO daily was used in case reports
Estrogen interferes with aromatase inhibitor efficacy
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Use with caution in patients with hyperlipidemia or hepatic insufficiency; safety not established in premenopausal or pediatric patients; caution with history of thromboembolic disorders or risk of complications from edema; common adverse effects include headache, nausea, dyspnea, cough, pharyngitis, back or bone pain
Glucocorticoids
These agents are used for replacement therapy postadrenalectomy in infantile Cushing syndrome.
Hydrocortisone (Hydrocortone, Cortef)
DOC for glucocorticoid replacement because of mineralocorticoid activity and glucocorticoid effects.
Double or triple dose for febrile illnesses. Possible need for up to 10 times maintenance doses if under severe stress due to trauma, critical illness, or surgery.
Adult
12-16 mg/m2 PO divided bid
Pediatric
12-16 mg/m2 PO divided bid/tid
Corticosteroid clearance may decrease with estrogens; may increase digitalis toxicity secondary to hypokalemia
Documented hypersensitivity; viral, fungal, or tubercular skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Possible growth suppression with overreplacement in children
Mineralocorticoids
These agents are used for replacement therapy postadrenalectomy in infantile Cushing syndrome. They act on fluid and electrolyte balance and enhance sodium reabsorption in the kidney, resulting in expanded extracellular fluid volume. They increase renal excretion of potassium and hydrogen ion.
Fludrocortisone (Florinef)
Partial replacement therapy for primary and secondary adrenocortical insufficiency.
Adult
0.1-0.2 mg/d PO
Pediatric
0.05-0.1 mg/d PO
Antagonizes effects of anticholinergics; rifampin, hydantoins, and barbiturates decrease effects of fludrocortisone; decreases salicylate levels
Documented hypersensitivity; systemic fungal infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Careful monitoring of blood pressure necessary; possible hypertension with overdosage; taper dose gradually when therapy is discontinued; caution in Addison disease, potassium loss, and sodium retention
Somatostatin analogues
These agents inhibit growth hormone (GH) secretion and adenoma growth in somatotroph adenomas. Octreotide, like natural somatostatin, inhibits GH secretion, insulin secretion and glucagon secretion. Following intravenous (IV) administration, basal serum GH, insulin, and glucagon levels are lowered. It also inhibits prolactin secretion via vasoactive intestinal peptide-mediated and thyrotropin releasing hormone–mediated secretion of prolactin. They are used in treatment of patients with acromegaly and hormone-secreting tumors.
Octreotide (Sandostatin)
Potent, long-acting analogue of somatostatin. This drug acts at the somatotroph to inhibit release of GH from the pituitary gland.
Adult
50 mcg SC tid initially; increase as tolerated to achieve decrease in IGF-1 or GH levels; generally not to exceed 300 mcg/d; some reports of maximum effectiveness not achieved until 500 mcg SC tid
Pediatric
1 mcg/kg SC divided bid/tid initially; titrate up to achieve desired effect
May reduce effects of cyclosporine; patients on insulin, oral hypoglycemics, beta-blockers, and calcium channel blockers may require dosage adjustments
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adverse effects primarily related to altered GI motility and include nausea, abdominal pain, diarrhea, and increased incidence of gallstones and biliary sludge; because of alteration in counterregulatory hormones (ie, insulin, glucagon, GH), hypoglycemia or hyperglycemia may be seen; bradycardia, cardiac conduction abnormalities, and arrhythmias have been reported; because of inhibition of TSH secretion, hypothyroidism also may occur; exercise caution in patients with renal impairment; cholelithiasis may occur
Dopamine receptor agonists
Bromocriptine and cabergoline have been used as adjunctive therapy to octreotide in the inhibition of GH release from somatotroph adenomas.
Bromocriptine (Parlodel)
Has been successful in further reducing GH levels in acromegalic patients treated with octreotide, although not generally a first-line therapy.
Adult
2.5 mg PO hs initially with food; may increase by 1.25-2.5 mg/wk, as tolerated, until achievement of decrease in IGF-1 or GH levels
Pediatric
Not established
Toxicity may increase with ergot alkaloids; amitriptyline, butyrophenones, imipramine, methyldopa, phenothiazines, and reserpine may decrease bromocriptine effects
Documented hypersensitivity; ischemic heart disease, peripheral vascular disorders; uncontrolled hypertension; not for use in pregnancy unless risk of renewed tumor growth exists
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
High incidence of adverse effects, including, but not limited to, nausea, vomiting, constipation, headache, orthostatic hypotension, and drowsiness; caution in renal or hepatic disease
Cabergoline (Dostinex)
Has been successful in further reducing GH levels in acromegalic patients treated with or without octreotide, although not generally a first-line therapy.
Adult
0.25 mg PO 2 times/wk initially; may increase at 4 wk intervals by 0.25 mg twice weekly until IGF-1 or GH levels decrease; not to exceed 1 mg twice weekly
Pediatric
Not established
Antipsychotics or metoclopramide may decrease effects (because of dopamine antagonism); coadministration with serotonin agonists (eg, buspirone, SSRIs, TCAs, nefazodone, sibutramine, sumatriptan, trazodone) may increase the risk of serotonin syndrome
Documented hypersensitivity to cabergoline or any component of the formulation, or ergot derivatives; ergot alkaloids are contraindicated with potent inhibitors of CYP3A4 (includes protease inhibitors, azole antifungals, and some macrolide antibiotics); uncontrolled hypertension
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adverse reactions are significant, including headache, dizziness, and nausea; orthostatic hypotension is common when starting doses are 1 mg or higher; other common side effects include, but are not limited to, fatigue, constipation, dyspepsia, abdominal pain, asthenia, and vertigo; caution in hepatic disease
Bisphosphonates
These agents are stable analogs of pyrophosphate and potent inhibitors of bone resorption and bone turnover. They are used to prevent the bone resorption and pain of polyostotic fibrous dysplasia
Pamidronate (Aredia)
Has been successful in treating the pain of polyostotic fibrous dysplasia; may have some benefit in increasing bone mineral density as well.
Adult
30-90 mg IV qmo
Pediatric
0.5-3 mg/kg/d IV for 3 d; may repeat in 4-6 mo intervals; alternatively, 10-30 mg/m2 monthly
May cause additive toxicity with other nephrotoxic drugs (eg, cyclophosphamide, aminoglycosides)
Documented hypersensitivity; hypocalcemia; severe renal impairment
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Use with caution in patients with renal impairment; maintain adequate hydration and urinary output during treatment
Alendronate (Fosamax)
Has been successful in treating the pain of polyostotic fibrous dysplasia; may have some benefit in increasing bone mineral density as well. Has the benefit of PO administration.
Adult
5-10 mg PO daily or 35-70 mg PO qwk; must be taken with plain water (tablets 6-8 oz; PO solution 2 oz) first thing in the morning and 30 min before the first food, beverage, or other medication of the day; to reduce the incidence of esophagitis, must sit or stand upright (do not lie down) for at least 30 min following administration and until after first food of the day is ingested
Pediatric
Not established
Coadministration with aspirin may increase incidence of adverse GI effects (when >10 mg alendronate is used); PO medications (especially those containing multivalent cations, such as aluminum, calcium, iron, or magnesium) may interfere with absorption; wait at least 30 min after taking alendronate before taking any PO medication; ranitidine increases gastric pH and can double the bioavailability of alendronate; all food and beverages interfere with absorption, coadministration with caffeine may reduce alendronate efficacy; coadministration with dairy products may decrease alendronate absorption; beverages (especially orange juice and coffee), food, and medications (eg, antacids, calcium, iron, and multivalent cations) may reduce the absorption of alendronate as much as 60%
Documented hypersensitivity; hypocalcemia; abnormalities of the esophagus (eg, stricture, achalasia); inability to stand upright for 30 min; oral solution should not be used in patients at risk of aspiration
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment; hypocalcemia must be corrected before therapy initiation; ensure adequate calcium and vitamin D intake; may cause irritation to upper gastrointestinal mucosa; esophagitis, esophageal ulcers, esophageal erosions, and esophageal stricture (rare) have been reported; risk increases if unable to comply with dosing instructions; caution with dysphagia, esophageal disease, gastritis, duodenitis, or ulcers (may worsen underlying condition)
Estrogen receptor antagonists
This is a new therapy for girls with persistent estradiol elevation.
Tamoxifen (Nolvadex)
Competitively binds to estrogen receptor, producing a nuclear complex that decreases DNA synthesis and inhibits estrogen effects. Blocks the end-organ effects of abnormal estrogen exposure in prepubertal girls.
Adult
Not indicated in adult women
Pediatric
Not established; 20 mg PO daily was used in multicenter trials
May exacerbate hepatotoxic effects of allopurinol; may increase cyclosporine serum levels; increases anticoagulant effects of warfarin; aminoglutethimide reduces the serum concentration of tamoxifen; cyclophosphamide, methotrexate, and 5-FU increase thrombotic risk of tamoxifen
CYP2C8/9 or CYP3A4 inducers (eg, carbamazepine, phenobarbital, phenytoin, rifampin, rifapentine, secobarbital, aminoglutethimide, carbamazepine, nevirapine, phenobarbital, phenytoin, rifamycins, St. John's Wort) may decrease the levels/effects of tamoxifen
CYP2C8/9, CYP2D6, or CYP3A4 inhibitors (eg, delavirdine, fluconazole, gemfibrozil, ketoconazole, nicardipine, NSAIDs, pioglitazone, sulfonamides, chlorpromazine, delavirdine, fluoxetine, miconazole, paroxetine, pergolide, quinidine, quinine, ritonavir, ropinirole, azole antifungals, clarithromycin, diclofenac, doxycycline, erythromycin, imatinib, isoniazid, nefazodone, nicardipine, propofol, protease inhibitors, quinidine, telithromycin, verapamil) may increase tamoxifen levels/effects
Documented hypersensitivity; concurrent warfarin therapy or history of deep vein thrombosis or pulmonary embolism (when used for cancer risk reduction)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Decreased visual acuity, retinopathy, corneal changes, and increased incidence of cataracts have been reported; liver abnormalities such as cholestasis, hepatitis, and hepatic necrosis have occurred; long-term efficacy and safety has not been established in pediatric patients
Antithyroid agents
Blocks production of thyroid hormone in functional thyroid nodules.
Methimazole (Tapazole)
Inhibits thyroid hormone by blocking oxidation of iodine in thyroid gland. Used to decrease the production of thyroid hormone in functional thyroid nodules associated with McCune-Albright syndrome. Unlike autoimmune-mediated hyperthyroidism, treatment is likely to be long-term. Consider more permanent solutions (ie, radioactive iodine, surgery).
Adult
5-20 mg PO tid initially; once euthyroid, can wean to 5-15 mg PO qd/bid
Pediatric
0.4 mg/kg/d PO divided tid initially; once euthyroid, maintenance dose likely to be approximately 0.2 mg/kg/d
Has antivitamin K activity and may potentiate activity of oral anticoagulants; bone marrow suppressive agents; lithium; potassium iodide
Documented hypersensitivity; bone marrow suppression
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Common adverse effects include rash, urticaria, and arthralgias; mild neutropenia and/or anemia; rarely, fulminant hepatitis and aplastic anemia
Monitor prothrombin time during therapy; agranulocytosis may occur, monitor patients for symptoms (eg, sore throat, fever, bleeding, bruising, malaise, stomatitis) and, if suspected, discontinue drug immediately; may cause hypoprothrombinemia and bleeding; once symptoms of hyperthyroidism have resolved, the presence of elevated serum TSH suggests that a lower maintenance dose of methimazole should be used
More on McCune-Albright Syndrome |
| Overview: McCune-Albright Syndrome |
| Differential Diagnoses & Workup: McCune-Albright Syndrome |
 Treatment & Medication: McCune-Albright Syndrome |
| Follow-up: McCune-Albright Syndrome |
| Multimedia: McCune-Albright Syndrome |
| References |
| « Previous Page | Next Page » |
References
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Further Reading
Keywords
McCune-Albright syndrome, polyostotic fibrous dysplasia, café au lait skin pigmentation, autonomous endocrine hyperfunction, precocious puberty, Albright disease, Albright's disease, Albright syndrome, Albright's syndrome, hyperthyroidism, hypercortisolism, pituitary gigantism, acromegaly, hypophosphatemia, chronic liver disease, tachycardia, cardiac arrhythmias, Cushing syndrome, failure to thrive, osteoporosis, hypertension, glucose intolerance, hypertriglyceridemia, vaginal bleeding, neurofibromatosis, optic gliomas, deafness, vertigo, hyperthermia, fibrous dysplasia, rickets, short stature, jaundice, growth hormone excess
