Materiały źródłowe

• #1 Treatment of Cushing’s Syndrome: An Endocrine Society Clinical Practice Guideline

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4525003/

  The objective is to formulate clinical practice guidelines for treating Cushing’s syndrome. […] Treatment of Cushing’s syndrome is essential to reduce mortality and associated comorbidities. Effective treatment includes the normalization of cortisol levels or action. It also includes the normalization of comorbidities via directly treating the cause of Cushing’s syndrome and by adjunctive treatments (eg, antihypertensives). Surgical resection of the causal lesion(s) is generally the first-line approach. The choice of second-line treatments, including medication, bilateral adrenalectomy, and radiation therapy (for corticotrope tumors), must be individualized to each patient. […] In patients with overt Cushing’s syndrome (CS), we recommend normalizing cortisol levels or action at its receptors to eliminate the signs and symptoms of CS and treating comorbidities associated with hypercortisolism.

• #1 Treatment of Cushing’s Syndrome: An Endocrine Society Clinical Practice Guideline

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4525003/

  We recommend initial resection of primary lesion(s) underlying Cushing’s disease (CD), ectopic and adrenal (cancer, adenoma, and bilateral disease) etiologies, unless surgery is not possible or unlikely to significantly reduce glucocorticoid excess. […] We recommend that hypocortisolemic patients receive glucocorticoid replacement and education about adrenal insufficiency after surgical remission. […] In patients with ACTH-dependent CS who underwent a noncurative surgery or for whom surgery was not possible, we suggest a shared decision-making approach because there are several available second-line therapies (eg, repeat transsphenoidal surgery, radiotherapy, medical therapy, and bilateral adrenalectomy). […] We recommend confirming that medical therapy is effective in normalizing cortisol before administering radiation therapy (RT)/radiosurgery for this goal because this will be needed while awaiting the effect of radiation.

• #1 Treatment of Cushing’s Syndrome: An Endocrine Society Clinical Practice Guideline

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4525003/

  We recommend steroidogenesis inhibitors under the following conditions: as second-line treatment after TSS in patients with CD, either with or without RT/radiosurgery; as primary treatment of EAS in patients with occult or metastatic EAS; and as adjunctive treatment to reduce cortisol levels in ACC. […] We recommend treating the specific comorbidities associated with CS (eg, cardiovascular risk factors, osteoporosis and psychiatric symptoms) in all patients with CS throughout their lives until resolution. We also recommend testing for recurrence throughout life, except in patients who underwent resection of an adrenal adenoma with a computerized tomography (CT) density of 10 Hounsfield units. […] We recommend urgent treatment (within 24-72 h) of hypercortisolism if life-threatening complications of CS such as infection, pulmonary thromboembolism, cardiovascular complications, and acute psychosis are present.

• #2 Endogenous Cushing Syndrome Treatment & Management: Approach Considerations, Pharmacotherapy, Surgical Therapy

  https://emedicine.medscape.com/article/2233083-treatment

  Treatment of Cushing syndrome is directed by the primary cause of the syndrome. In general, therapy should reduce the cortisol secretion to normal to reduce the risk of comorbidities associated with hypercortisolism. The treatment of choice for endogenous Cushing syndrome is surgical resection of the causative tumor. The primary therapy for Cushing disease is transsphenoidal surgery, and the primary therapy for adrenal tumors is adrenalectomy. […] When surgery for Cushing syndrome is not successful or cannot be undertaken, as often occurs with ectopic ACTH or metastatic adrenal carcinoma, control of hypercortisolism may be attempted with medication. However, medication failures are common, and adrenalectomy may be indicated in ACTH-mediated Cushing syndrome. Pituitary radiation may be useful if surgery fails for Cushing disease.

• #3 Medical Treatment of Cushing’s Syndrome

  https://www.e-enm.org/journal/view.php?number=2566

  Endogenous Cushings syndrome refers to the manifestations of chronic cortisol excess. Surgical resection of the tumor is typically the first-line and preferred treatment. However, when surgery is not feasible or has been unsuccessful, medical therapies may be employed to control Cushings syndrome. This mini-review provides an overview of the main medical therapies currently used to treat Cushings syndrome, including their efficacy, safety, and management. […] The management of CS remains challenging. Regardless of the underlying cause, surgical resection of the tumor is typically the first-line and preferred treatment. However, when surgery is not feasible or has been unsuccessful, medical therapies may be employed to control CS. […] The aim of this mini-review is to provide an overview of the main drugs used to treat CS, including their efficacy, safety, and management.

• #4 Cushing syndrome: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000410.htm

  Treatment depends on the cause. […] Cushing syndrome caused by corticosteroid use: Your provider will instruct you to slowly decrease the medicine dosage. Stopping the medicine suddenly can be dangerous. […] If you cannot stop taking the medicine because of the disease it is treating, your high blood sugar, high cholesterol levels, and bone thinning or osteoporosis should be closely monitored and treated. […] With Cushing syndrome caused by a pituitary tumor that releases too much ACTH (Cushing disease), you may need: Surgery to remove the tumor, Radiation after removal of the pituitary tumor (in some cases), Cortisol replacement therapy after surgery, Medicines to replace pituitary hormones that become deficient, Medicines to prevent the body from making too much cortisol. […] With Cushing syndrome due to a pituitary tumor, adrenal tumor, or other tumors: You may need surgery to remove the tumor. If the tumor cannot be removed, you may need medicines to help block the release of cortisol or the ability of cortisol to signal.

• #5 Endogenous Cushing Syndrome Treatment & Management: Approach Considerations, Pharmacotherapy, Surgical Therapy

  https://emedicine.medscape.com/article/2233083-treatment

  Optimal treatment of Cushing syndrome involves direct surgical removal of the causal tumor, except in cases unlikely to cause a drop in glucocorticoids or in patients who are not candidates for surgery. Second-line therapy should be individualized. Other first-line treatments include surgical resection of ectopic ACTH-secreting tumors; transsphenoidal selective adenomectomy; blocking hormone receptors in bilateral micronodular adrenal hyperplasia; and surgical removal in cases of bilateral adrenal disorders. […] The choice of second-line treatments include medication, bilateral adrenalectomy, and radiation therapy (for corticotrope tumors). Effective treatment includes the normalization of cortisol levels or action. It also includes the normalization of comorbidities (eg, hypertension, diabetes) by adjunctive treatments (eg, antihypertensives). Lowering cortisol levels improves hypertension, insulin resistance, dyslipidemia, and obesity.

• #6 Cushing syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cushing-syndrome/diagnosis-treatment/drc-20351314

  Treatments for Cushing syndrome are designed to lower the amount of cortisol in the body. The best treatment for you depends on the cause of the syndrome. Options include: […] If Cushing syndrome is caused by taking glucocorticoid medicine for a long time, your health care provider may be able to control your symptoms by lowering how much medicine you take. This is done carefully over time, while still managing the condition for which you take it. Don’t reduce the dose of glucocorticoid drugs or stop taking them on your own. Do so only with help from your health care provider. […] If Cushing syndrome is caused by a tumor, your health care provider may recommend removing the tumor with surgery. Pituitary tumors are often removed by a neurosurgeon, who may do the operation through your nose. ACTH-producing tumors in other parts of the body may be removed with regular surgery or using less-invasive approaches with smaller incisions.

• #6 Cushing syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cushing-syndrome/diagnosis-treatment/drc-20351314

  Medicines to control cortisol production at the adrenal gland include ketoconazole, osilodrostat (Isturisa), mitotane (Lysodren), levoketoconazole (Recorlev), and metyrapone (Metopirone). […] Mifepristone (Korlym, Mifeprex) is approved for people with Cushing syndrome who have type 2 diabetes or high blood sugar. Mifepristone does not lower the amount of cortisol the body makes, but it blocks the effect of cortisol on tissues. […] Pasireotide (Signifor) is given as a shot two times a day. It works by lowering the amount of ACTH from the tumor, which lowers cortisol levels. Other medicines are being developed. […] Side effects from these medicines may include tiredness, upset stomach, vomiting, headaches, muscle aches, high blood pressure, low potassium and swelling. Some have more-serious side effects, such as brain and nervous system side effects and liver damage. […] Sometimes, the tumor or its treatment causes the pituitary or adrenal gland to make too little of other hormones. If this happens, your health care provider can recommend hormone replacement.

• #6 Cushing syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cushing-syndrome/diagnosis-treatment/drc-20351314

  If an ACTH-producing tumor isn’t found, or if one can’t be fully removed and Cushing syndrome continues, your health care provider may recommend removing the adrenal glands. This is called a bilateral adrenalectomy. This procedure immediately stops the body from making too much cortisol. After both adrenal glands are removed, you may need to take medicines to replace cortisol and another adrenal hormone called aldosterone for the rest of your life. […] If the surgeon can’t totally remove a pituitary tumor, radiation therapy may be needed along with surgery. Radiation also may be used for people who can’t have surgery. […] Medicines can be used to control cortisol levels when surgery and radiation don’t work or aren’t an option. Medicines also might be used before surgery in people who are very sick with Cushing syndrome. This can improve symptoms of the disease and reduce the risks of surgery. Medical therapy for Cushing syndrome is not a cure and may not completely improve all of the symptoms of too much cortisol.

• #7 Cushing Syndrome: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5497-cushing-syndrome

  Cushing syndrome is a condition caused by too much of the hormone cortisol in the body. There are treatments for Cushing syndrome. Treatment may last for some time. […] The type of treatment depends on the underlying cause of the high cortisol levels. If you use glucocorticoids, your healthcare provider will likely lower the dosage or prescribe a non-glucocorticoid medication. […] If a tumors causing Cushing syndrome, your provider may suggest surgery or radiation. […] Another option is for your healthcare provider to prescribe a medication such as ketoconazole thatll slow down the production of cortisol. […] Chemotherapy is necessary if a tumors cancerous and has spread to other parts of your body. […] Adding drugs that reduce cortisol or taking away drugs that can cause Cushing syndrome. […] Surgery: Surgically removing pituitary tumors, adrenal tumors and ectopic tumors is effective, but youll have to adjust to new, lower cortisol levels. […] If Cushing syndrome is properly treated, the disease may go away after two to 18 months.

• #8 Is there a cure for Cushing syndrome?

  https://www.medicalnewstoday.com/articles/can-cushings-syndrome-be-cured-faqs

  If long-term glucocorticoid use is the cause of a person’s Cushing syndrome, their doctor may slowly reduce the dosage to the lowest level needed to manage their condition. In some cases, doctors may manage the condition with alternative non-glucocorticoid medications. […] Treatment of Cushing syndrome requires addressing the underlying cause of high cortisol levels. However, lifestyle measures may help manage symptoms and improve overall health. […] With proper diagnosis and treatment, many people with Cushing syndrome achieve full recovery or long-term remission. Early intervention and close medical supervision are key to managing the condition and preventing complications.

• #9 Iatrogenic Cushing Syndrome Treatment & Management: Medical Care

  https://emedicine.medscape.com/article/117365-treatment

  The appropriate treatment for exogenous Cushing syndrome is gradual withdrawal of the causative drug, with the aim of discontinuing this agent if possible. This can be a lengthy process that may take several months to a year, depending on the duration and dosages of the glucocorticoids used. It is important to remember that during this process, an individual with HPA-axis suppression cannot increase steroid production appropriately during a medical illness or other stress and should receive stress-dose steroids to avoid adrenal crisis. […] See Glucocorticoid Therapy and Cushing Syndrome.

• #10 Cushing’s Syndrome Information & Treatment

  https://www.columbiadoctors.org/health-library/condition/cushing-s-syndrome/

  If long-term use of steroid medicine is the cause of Cushing’s syndrome: […] Your doctor will help you lower your dose or gradually stop taking it. It may take a while for the symptoms to go away. […] Do not stop taking steroid medicine on your own. That can be very dangerous. Your doctor will help you change your medicine or lower the dose slowly. […] If a pituitary tumor is the cause: […] Surgery to remove the tumor offers the best chance for recovery. This surgery requires great skill and should be done at a major medical center where doctors specialize in pituitary surgery. […] If surgery to remove the tumor isn’t possible or hasn’t worked, you may have other options to consider such as medicine, radiation, or surgery to remove the adrenal glands. You and your doctor can talk about the pros and cons of each option.

• #11 Cushing Disease / Cushing Syndrome | Brain Institute | OHSU

  https://www.ohsu.edu/brain-institute/cushing-disease-cushing-syndrome

  Treatment for Cushing disease/syndrome has changed in the last decade, thanks to several breakthroughs for treating pituitary adenomas with medication. […] The OHSU Pituitary Center was one of the leading contributors to clinical trials that led to medications pasireotide and mifepristone to treat Cushing disease/syndrome and acromegaly. […] Surgery to remove a tumor causing the problem is often the first treatment for Cushing disease. At a specialized pituitary center such as ours, surgery to remove a small pituitary tumor has a success rate of 80-85%. […] We use a precise, minimally invasive procedure called transsphenoidal surgery to remove pituitary tumors through the nose. This approach leaves no visible external scars and has few side effects and risks. You will most likely go home two or three days after surgery.

• #11 Cushing Disease / Cushing Syndrome | Brain Institute | OHSU

  https://www.ohsu.edu/brain-institute/cushing-disease-cushing-syndrome

  For some patients, surgery isn’t an option or doesn’t fully solve the problem. You may need medication for Cushing disease/syndrome: Before surgery to control cortisol levels. If you can’t have surgery because of other health reasons or risks. If you had surgery, but the neurosurgeon couldn’t remove the entire tumor or the removal didn’t solve the problem. If you have too much ACTH, but a tumor doesn’t show up on imaging. […] Some patients may need a combination of medications. All drugs used for Cushing disease/syndrome can potentially cause adrenal insufficiency, or a low level of adrenal hormones. This can cause fatigue and muscle weakness, among other symptoms. Our team will monitor you closely and provide treatment if this happens. […] Medications for Cushing disease/syndrome include: Central-acting inhibitors of ACTH: Pasireotide (a somatostatin receptor ligand, or SRL) reduces ACTH to normal levels for about a quarter of patients. It lessens symptoms for others. Most see results within two months. This medication is injected and can cause side effects, including high blood sugar and diabetes, in more than half of patients. Your doctor will talk with you about risks and benefits. Adrenal-directed inhibition of steroidogenesis: Medications in this category are used off-label for Cushing disease. This means the doctor uses a safe and FDA-approved medication for a reason other than its approved use. Our team has extensive experience with off-label uses for Cushing disease. Medications include ketoconazole, metyrapone, mitotane and etomidate. They target the adrenal glands to lower cortisol levels. Glucocorticoid receptor blockade: OHSU participated in the clinical trial that led to FDA approval for this medication, called mifepristone. It’s used to treat high blood sugar associated with Cushing syndrome. Mifepristone may improve other conditions brought on by too much cortisol. It can also cause low potassium levels, called hypokalemia, and vaginal bleeding. It’s not recommended for women who wish to become pregnant.

• #11 Cushing Disease / Cushing Syndrome | Brain Institute | OHSU

  https://www.ohsu.edu/brain-institute/cushing-disease-cushing-syndrome

  Some patients have an aggressive form of Cushing disease, and surgery and medication don’t help. In these cases, we may recommend a type of targeted radiation therapy called stereotactic radiosurgery to control hormone levels. […] After surgery and if you are in remission, your body no longer produces cortisol. You will need medication to maintain normal levels. Symptoms can take time to go away. During this time, you’ll receive follow-up care from our experts. […] Cushing disease comes back, even after decades, in about a quarter of cases. We will monitor you closely and recommend further treatment if needed.

• #12 Cushing’s Disease | Pacific Pituitary Disorders Center

  https://www.pacificneuroscienceinstitute.org/pituitary-disorders/conditions/pituitary-adenomas/cushings-disease/

  For the great majority of patients with Cushings disease transsphenoidal tumor removal (via the nose) is the initial treatment of choice. […] Endoscopic Endonasal Surgery Surgical removal is the primary means to achieve long term remission in Cushings disease; at Pituitary Centers of Excellence, remission rates range from 80-90% for microadenomas and 30-70% for invasive adenomas or macroadenomas; for non-visible adenomas, the surgical success rate ranges from 40-60%. […] In patients who fail to have remission of their Cushings disease or syndrome state after surgery, adrenal-directed medications can be used such as ketoconazole which inhibits steroid (cortisol) production in the adrenal glands. […] For patients with persistent Cushings disease after surgery, include Signifor (pasireotide) and Korlym (mifepristone). […] Removal of the adrenal glands was at one time a preferred method for treating Cushings disease and lowering cortisol levels. Currently however, this treatment is reserved for patients who have failed prior endonasal transsphenoidal surgery.

• #13 Frequently Asked Questions about Transsphenoidal Surgery for Cushing’s Disease

  https://www.massgeneral.org/neurosurgery/treatments-and-services/faq-transsphenoidalsurgery-cushing-disease

  A. The best treatment is almost always transsphenoidal surgery performed by a surgeon with extensive experience in pituitary tumor removal. […] A. If the endocrine tests indicate a pituitary source for the excess ACTH, transsphenoidal surgery is recommended even if the MRI does not show a tumor. […] A. The overall cure rate for Cushing’s disease is between 80-90% when the operation is performed by an experienced pituitary surgeon. […] A. If residual tumor remains in an accessible location, your surgeon may recommend a second operation. […] This treatment is planned by a radiation oncologist. […] In the meantime, drugs to suppress cortisol secretion by the adrenal glands are used to control the effects of excess cortisol, while waiting for the radiation to become effective. […] In some cases, removal of both adrenal glands, termed bilateral adrenalectomy, may be advised.

• #14 Cushing’s Syndrome – NIDDK

  https://www.niddk.nih.gov/health-information/endocrine-diseases/cushings-syndrome

  How do doctors treat Cushings syndrome? Treatment depends on the cause and may include surgery, radiation, chemotherapy, or cortisol-reducing medicines. If the cause is long-term use of glucocorticoids to treat another disorder, your doctor will gradually reduce your dosage to the lowest dose that will control that disorder. Sometimes disorders that doctors treat with glucocorticoids can be treated with a non-glucocorticoid medicine instead. […] The most common treatment for pituitary tumors is surgery to remove the tumor. Using a special microscope and fine instruments, a surgeon approaches the pituitary gland through a nostril or an opening made below the upper lip. Your doctor will probably refer you to a center specializing in this type of surgery. […] If surgery fails or the cure doesn’t last, surgery can be repeated, often with good results.

• #14 Cushing’s Syndrome – NIDDK

  https://www.niddk.nih.gov/health-information/endocrine-diseases/cushings-syndrome

  Surgery to remove the adrenal gland with the tumor is the most common treatment. Some rare diseases cause many nodules in both adrenal glands and require surgery to remove both glands. If you have both adrenal glands removed, you will need to take medicine for life to replace cortisol and other hormones the adrenal glands make.

• #15 Endogenous Cushing Syndrome Treatment & Management: Approach Considerations, Pharmacotherapy, Surgical Therapy

  https://emedicine.medscape.com/article/2233083-treatment

  An orally administered steroidogenesis inhibitor, osilodrostat (Isturisa) acts on 11-beta-hydroxylase, an enzyme that catalyzes the last step of cortisol synthesis in the adrenal cortex. It is indicated for adults with Cushing disease who cannot undergo pituitary surgery or in whom the operation has not been curative. […] Mifepristone (Korlym) is an antiprogestational agent, which, at high doses, competitively binds to the glucocorticoid and progesterone receptors. In February 2012, the FDA approved mifepristone to control hyperglycemia secondary to hypercortisolism in adult patients with endogenous Cushing syndrome who have type 2 diabetes mellitus or glucose intolerance and have failed surgery, or are not candidates for surgery. […] Treatment options for patients with persistent or recurrent Cushing disease have included pituitary irradiation and repeat surgery. A small study found that a percentage of such patients respond to cabergoline therapy.

• #16 Cushing’s Syndrome – NIDDK

  https://www.niddk.nih.gov/health-information/endocrine-diseases/cushings-syndrome

  After the surgeon removes the tumor, your pituitary won’t make enough ACTH for a while. Your doctor will prescribe cortisol medicine since there’s not enough ACTH to tell the adrenal glands to make enough cortisol. You may be able to stop cortisol medicine in 6 to 18 months. […] If surgery fails or isn’t possible, radiation therapy is an option. One type of radiation therapy uses small doses of radiation to the pituitary over a 6-week period. Another type uses a single, high dose of radiation. Cortisol levels may not return to normal after radiation therapy for several years. Your doctor may prescribe medicines to bring cortisol levels back to normal until radiation therapy takes effect. […] The first choice of treatment for ectopic tumors is to remove them surgically. If the tumor is cancerous and has spread, you may need chemotherapy, radiation therapy, or other cancer treatments. Medicines to reduce cortisol levels may also be part of your treatment. If other treatments fail, surgeons might have to remove the adrenal glands to control Cushings syndrome.

• #17 Cushing’s Disease – Causes, Symptoms & Treatment | MedStar Health

  https://www.medstarhealth.org/services/cushings-disease

  In some individuals, the tumor does not respond to surgical intervention, radiation, or medications that prevent excess cortisol production. If this occurs, surgical intervention to remove the adrenal glands may be recommended in order to inhibit the body from producing cortisol altogether. […] In order to successfully treat Cushing’s disease, it is important to seek care from a team of experts who are highly experienced in the diagnosis and treatment of this condition. If left untreated it can lead to serious health complications, long-term health complications like hypertension, and even premature death. Removal of the entire tumor may result in a full recovery. However, there is a risk that the tumor can return; therefore, careful monitoring is necessary.

• #18

  https://www.healio.com/news/endocrinology/20200205/new-treatments-offer-glimmers-of-optimism-possible-cure-for-cushings-disease

  Most think that surgical intervention for the majority of individuals with significant cortisol excess of pituitary/adrenal origin or an ectopic tumor is certainly the initial treatment of choice, and the guidelines reflect this, Findling told Endocrine Today. […] Second-line options are repeat surgery, radiation, adrenalectomy or medical therapy, each with its own sets of pros and cons, Melmed said. […] Currently, there are two FDA-approved treatments for Cushings disease. In June 2018, the FDA approved pasireotide for the treatment of people with Cushings disease for whom pituitary surgery is not an option or has not been curative. In 2012, the FDA approved mifepristone (Korlym, Corcept Therapeutics) as a once-daily oral medication to treat adults with Cushings syndrome who had elevated blood glucose due to type 2 diabetes.

• #18

  https://www.healio.com/news/endocrinology/20200205/new-treatments-offer-glimmers-of-optimism-possible-cure-for-cushings-disease

  Bilateral adrenalectomy is reserved as a third-line treatment option for patients with uncontrolled hypercortisolism despite pituitary surgery, appropriate medical therapy and pituitary radiation, Fleseriu said. […] Several new medical therapies are in development, some in phase 3 trials, Fleseriu said. […] Emerging pituitary-targeted drug treatments not yet in clinical trials include heat shock protein inhibitors, histone deacetylase inhibitors and monoclonal ACTH antibodies, among others, Fleseriu said. […] Now, we can look at novel, targeted molecules and add those to our armamentarium and at least offer our patients the opportunity to participate in trials, or at least offer the optimism that, over the coming years, there will be a light at the end of the tunnel for their disorder.

• #19 Endogenous Cushing Syndrome Treatment & Management: Approach Considerations, Pharmacotherapy, Surgical Therapy

  https://emedicine.medscape.com/article/2233083-treatment

  In cases of benign unilateral adrenal adenoma, adrenalectomy is associated with a high cure rate in both children and adults. Adrenal carcinoma is associated with a poor prognosis; therefore, complete resection, and possibly medical treatment to stabilize cortisol levels, are necessary. […] Long-term follow-up is recommended for osteoporosis, cardiovascular disease, and psychiatric conditions. […] Medications used in the management of Cushing syndrome include the following: 11-beta-hydroxylase inhibitor: Osilodrostat; Somatostatin analogs: Pasireotide; Adrenal steroid inhibitors: Metyrapone, ketoconazole, etomidate; Glucocorticoid receptor antagonist: Mifepristone; Adrenolytic agents: Mitotane. […] Pasireotide (Signifor) is a somatostatin analog that binds and activates human somatostatin receptors, resulting in inhibition of ACTH secretion, which leads to decreased cortisol secretion. It is indicated for treatment of adults with Cushing disease in whom pituitary surgery is not an option or has not been curative.

• #20 Cushings Syndrome – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/conditions-treated/adrenal/adrenal-tumors/cushings-syndrome

  Treatment depends upon the cause of the disorder. In Cushing’s syndrome caused by drug therapy with corticosteroids, the drug dose must be slowly decreased under medical supervision. […] In Cushing’s disease caused by a pituitary tumor, surgery to remove the tumor is recommended. Radiation is sometimes needed as well. Hydrocortisone (cortisol) replacement therapy is needed after surgery. In some cases, life-long cortisol-replacement therapy becomes necessary. […] Cushing’s syndrome caused by an adrenal tumor is usually treated by surgical removal of the tumor. If the tumor cannot be removed, certain medications can suppress the secretion of cortisol. […] In Cushing’s syndrome caused by a tumor secreting ACTH, removal of the tumor is the best way to treat the Cushing’s syndrome. Cortisol replacement therapy is needed after surgery until cortisol production resumes. In some cases, life-long therapy with cortisone drugs becomes necessary.

• #21 Cushing’s Syndrome | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/cushings-syndrome

  Patients with Cushing’s disease (i.e. a pituitary tumor) typically have benign tumors that are removed by a neurosurgeon. This operation is usually performed through the patient’s nose in an operation called transsphenoidal surgery. […] In cases where surgery cannot cure the patient, other therapies like directed radiation treatment (i.e. Gamma Knife) may be successful. If surgery, radiation, and medications fail to control Cushing’s disease, removal of both adrenal glands may be needed. […] Patients with Cushing’s syndrome caused by an adrenal tumor can usually be cured by removal of either one or both adrenal glands, depending on which adrenal glands are involved. […] Almost all of these operations can be done using minimally invasive techniques such as laparoscopy (i.e. making 3 or 4 very small incisions and using a small camera and special instruments). […] During and after the operation to remove one diseased adrenal gland, patients may receive extra steroids through the IV and pills until the other adrenal starts working again. This steroid dose will be slowly lowered over time until it can be stopped safely. Patients having both adrenal glands removed will also receive steroids in the operating room and will need steroid pills for the rest of their lives.

• #22 Cushing’s Syndrome (Cushing’s Disease) | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/cushings-syndrome.html

  If an adrenal tumor is the cause: Surgery to remove the tumor is usually done if the tumor is not cancer (benign). If the tumor is cancer, the whole gland is removed. […] Medicine may be tried if surgery isn’t an option. […] If a tumor of the lungs or another organ is the cause, the tumor will be removed or treated with radiation or medicines. […] Treatment for Ectopic Cushings Syndrome: If the evaluation suggests the presence of ectopic Cushing’s syndrome, then the treatment is directed to the source. For example, if a carcinoid tumor in the lung is detected, then there are several options for therapy, including surgical excision. […] Treatment for Adrenal Growth: If an adrenal growth is the underlying cause, then surgical removal of the abnormal adrenal gland is necessary. […] Clinical trials evaluate new approaches, devices, or medications in the treatment of Cushings Disease. Ask your doctor or clinical trials coordinator about available trials that may be additional options for your care.

• #23 Cushing’s Syndrome | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/cushings.html

  If a tumor of the lungs or another organ is the cause, the tumor will be removed or treated with radiation or medicines. […] If the patient has Cushing’s syndrome due to excess ACTH levels and the primary tumor has not been resected, there are several next step options. Repeat surgery can be considered to try again to remove the tumor. Surgical removal of both adrenal glands (bilateral adrenalectomy) can also be performed, as this will result in normalization of the effects of Cushings syndrome. This therapy will lead to required replacement of adrenal gland hormones, including cortisol and a medication that controls sodium and potassium balance. […] Another option is the use of medical therapy. There are several medical therapies that work by reducing adrenal gland production of cortisol. These medications include ketoconazole and aminoglutethamide.

• #23 Cushing’s Syndrome | Brain Tumor Center | Stanford Medicine

  https://med.stanford.edu/brain-tumor/conditions/cushings.html

  There are several new medical agents recently available that may help further with medical control of Cushings syndrome. Korlym (mifepristone) is a cortisol blocker. This medication, given as a pill, blocks cortisol wherever it goes, and can lead to improvement in appearance and medical consequences in the majority of people. […] Signifor (pasireotide) is a new medicine that works like somatostatin at the primary tumor and reduces ACTH in approximately one-third of patients. […] Another new medication, Isturisa (osilodrostat) is a medication that blocks the adrenal gland to lower cortisol levels in most people. It is tolerated very well.

• #24 Medical Treatment of Cushing’s Syndrome

  https://www.e-enm.org/journal/view.php?number=2566

  Medical therapies may be employed in the following situations: (1) as an adjuvant therapy in cases of pituitary surgical failure or recurrence, assuming repeat surgery is not indicated, as well as while awaiting the effectiveness of pituitary radiotherapy in CD; (2) when surgery is not feasible for occult or metastatic endocrine tumors with EAS, in cases of locally advanced or metastatic adrenocortical carcinoma with hypercortisolism, or for patients who have inoperable disease or decline surgery; or (3) as first-line therapy in patients with severe hypercortisolism and life-threatening complications, while reserving rescue bilateral adrenalectomy as the potential treatment of last resort. […] Medical therapy should be individualized, incorporating numerous factors that influence the choice of drug(s) and administration strategy. […] The therapeutic strategy is founded on established scientific knowledge, the expertise of a multidisciplinary team, and the patients preferences, which are informed by detailed information on the available options.

• #25 Systemic therapy of Cushing’s syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-014-0122-8

  If damage to the surrounding normal pituitary tissue occurs during surgery, the patient may require lifelong pituitary hormone replacement. […] Pharmacological therapy applies when these two options are not amenable or refused. […] In cases when pharmacological therapy becomes necessary, Pasireotide should be used in first-line in ACTH-dependent CS. […] However, systemic pharmacotherapy of CS often shows sometimes dose-limiting side effects. […] Pituitary irradiation is usually reserved for patients who have tumor remaining after surgery, for patients who are poor candidates for surgery, and for patients who do not respond adequately to surgery and/or medication. […] The main disadvantages of radiotherapy are that i) normalization of ACTH secretion may take extended periods of time (eventually years) to occur demanding for medication while success of radiation is awaited, and ii) that patients may develop generalized anterior pituitary insufficiency.

• #25 Systemic therapy of Cushing’s syndrome | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-014-0122-8

  Despite its efficacy in lowering cortisol blood levels, Metyrapone is not constantly effective and therefore does not cover the entire CS-population. […] Clinical and/or biochemical improvements can be achieved in 60 to 80% of patients and are usually associated with improvements in symptoms. […] Mifepristone is not approved in the indication CS. […] However, all cell lines under investigation in this study express GR and derivatives of mifepristone with lower GR affinity are less effective. […] Mitotane might be of clinical benefit in some patients, adjuvant to radical surgery. […] Pasireotide exhibits at least a subtype-prevalence for subtype-5 of the SSTR in the pituitary gland. […] Therefore, first-line pharmacological therapy is Pasireotide injection (usually while definitive therapy is awaited). […] However, it should be noticed that Pasireotide is indicated only in a subgroup of CD-patients and shows satisfactory efficacy only in~30% of patients.

• #26 The Best Cushing’s Disease Surgery and Treatment

  https://www.adrenal.com/blog/the-best-cushing-s-disease-surgery-and-treatment

  Medical therapy for Cushings disease includes three categories of drugs: 1) adrenal-directed drugs, which block cortisol production from the adrenal gland; 2) pituitary-directed drugs, which inhibit ACTH secretion from the tumor and, secondarily, cortisol production; and 3) glucocorticoid receptor-directed drugs, which peripherally block the activation of the glucocorticoid receptors. […] Medical treatment is presently used in different situations, including: Presurgical treatment aimed at improving the clinical picture of patients before surgery, Postsurgical treatment in case of unsuccessful pituitary surgery, Bridging treatment before, bilateral adrenal surgery, Bridging treatment before, during, and after the administration of radiotherapy, while awaiting its definitive effects, Primary treatment in case of severe disease when no other options are available.

• #26 The Best Cushing’s Disease Surgery and Treatment

  https://www.adrenal.com/blog/the-best-cushing-s-disease-surgery-and-treatment

  Many recent studies have shown that bilateral adrenalectomy is an increasingly used and essential treatment option when pituitary surgery is no longer an option. […] Bilateral adrenalectomy is associated with definitive disease cure in the vast majority of patients with Cushings Disease. […] The major advantage of bilateral adrenalectomy is the rapid and definitive control (100% in Dr. Carlings experience) of the clinical syndrome of Cushings Disease together with the rapid reversal of the comorbidities. […] Patients having undergone bilateral adrenalectomy (BLA) require special attention and must have a booked follow up appointment with their endocrinologist before surgery. […] The results of the studies on radiotherapy for Cushings Disease demonstrate that it is associated with disease control in about 60% of patients.

• #27 Medical therapy of hypercortisolism (Cushing syndrome) – UpToDate

  https://www.uptodate.com/contents/medical-therapy-of-hypercortisolism-cushings-syndrome

  Medical therapy of hypercortisolism (Cushing syndrome) […] The hypercortisolism of Cushing syndrome is primarily treated surgically whenever possible, regardless of its cause (ie, whether due to a corticotropin [ACTH]-producing pituitary tumor [Cushing disease], ectopic ACTH secretion by a nonpituitary tumor, or cortisol secretion due to an adrenal adenoma, bilateral adrenal hyperplasia, or adrenal carcinoma). However, when surgery is delayed, contraindicated, or unsuccessful, medical therapy is often required. Medical therapies for Cushing syndrome can be divided into three broad categories based on the following mechanisms of action: […] Decrease cortisol production […] Antagonize the action of cortisol at the glucocorticoid receptor, or […] Target the specific etiology of Cushing syndrome

• #28 New Trends in Treating Cushing’s Disease – touchENDOCRINOLOGY

  https://touchendocrinology.com/pituitary/journal-articles/new-trends-in-treating-cushings-disease/

  Levoketoconazole and osilodrostat have been approved for controlling cortisol excess and comorbidities. The somatostatin analogue pasireotide is the only currently approved tumour-targeted therapy. […] The selection of the agent depends not only on individual patients and tumour characteristics, availability and cost, but also on highly specific molecular patterns.

• #28 New Trends in Treating Cushing’s Disease – touchENDOCRINOLOGY

  https://touchendocrinology.com/pituitary/journal-articles/new-trends-in-treating-cushings-disease/

  Rates of recurrence and/or persistence of Cushings disease after surgical treatment are high. Recently, advances in molecular insights and a better understanding of pathophysiology have enabled the development of potential therapeutic targets that could control adrenocorticotropic hormone (ACTH) and cortisol secretion or even reduce tumour cell proliferation. At the pituitary level, pasireotide is an approved somatostatin receptor ligand, and compounds targeting cell cycle regulation, cell signalling and epigenetics are now under investigation. Levoketoconazole and osilodrostat are novel steroid inhibitors, and relacorilant overcomes the adverse effects of mifepristone. Adrenal ACTH receptor blockage and immunotherapy could also play a role. […] Although transsphenoidal pituitary surgery (TSS) is the mainstay of treatment for this disease, up to one-third of the patients eventually relapse and are required to be treated with a variety of drugs, as none can achieve optimal disease control. Drugs may aim directly at the corticotroph adenoma (pasireotide and cabergoline), act on the adrenal steroidogenesis pathway mitigating the cortisol synthesis (such as ketoconazole, etomidate, metyrapone and mitotane) or block the glucocorticoid receptor (mifepristone).

• #29 Management of Cushing’s syndrome – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/management-of-cushings-syndrome

  Metyrapone is a competitive inhibitor of 11β-hydroxylation. […] It inhibits the conversion of pregnenolone to cortisol in the adrenal cortex. […] Ketoconazole inhibits 11β-hydroxylase and so the final conversion of pregnenolone to cortisol. […] Mitotane selectively inhibits the activity of the adrenal cortex. […] It has a direct cytotoxic effect in adrenal carcinoma, shrinking adrenal tumours and decreasing steroid production. […] Mifepristone is licensed as an abortifacient. […] It is a glucocorticoid and progestin receptor antagonist. […] Etomidate is an imidazole-derived anaesthetic agent which was reported to have an adverse effect on adrenocortical function in the early 1980s.

• #29 Management of Cushing’s syndrome – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/ld/management-of-cushings-syndrome

  This article explains treatment strategies for Cushing’s syndrome, steroid replacement therapy and how pharmacists can support patients. […] Cushing’s syndrome can be treated with surgery, radiotherapy, chemotherapy and cortisol-inhibiting drugs or, in iatrogenic Cushing’s syndrome, steroid withdrawal. […] Some patients may need corticosteroid replacement therapy. They should carry a steroid card and wear a Medic-Alert bracelet. […] Patients on steroid replacement therapy will need special counselling related to timing of doses, symptoms of adrenal insufficiency and when emergency doses are required. […] Treatment is usually a combination of the options, including surgery. […] Pituitary tumours can be removed with transsphenoidal surgery and this is usually the mainstay of treatment.

• #30 FDA Approves New Treatment for Adults with Cushing’s Disease | FDA

  https://www.fda.gov/news-events/press-announcements/fda-approves-new-treatment-adults-cushings-disease

  The U.S. Food and Drug Administration today approved Isturisa (osilodrostat) oral tablets for adults with Cushings disease who either cannot undergo pituitary gland surgery or have undergone the surgery but still have the disease. […] Isturisa is the first FDA-approved drug to directly address this cortisol overproduction by blocking the enzyme known as 11-beta-hydroxylase and preventing cortisol synthesis. […] By helping patients achieve normal cortisol levels, this medication is an important treatment option for adults with Cushings disease. […] Isturisas safety and effectiveness for treating Cushings disease among adults was evaluated in a study of 137 adult patients (about three-quarters women) with a mean age of 41 years. […] At the end of this 24-week period, about half of patients had cortisol levels within normal limits.

• #31 Cushing’s Syndrome: Causes and Treatment | Doctor

  https://patient.info/doctor/cushings-syndrome-pro

  Metyrapone and ketoconazole are enzyme inhibitors and have rapid onset of action; however, control of hypercortisolism is often lost with corticotropin oversecretion in Cushing’s disease. These drugs are not usually effective as long-term treatment and are used mainly for preparation for surgery or as adjunctive treatment after surgery or pituitary radiotherapy. […] Mitotane acts as an adrenolytic drug with delayed onset but long-lasting action, but control of corticotropin oversecretion in Cushing’s disease is maintained. […] Medical treatment can also be used in patients who are unwilling or unfit for surgery. Treatment can be used long-term for patients with ectopic corticotropin secretion; however, adrenalectomy may be preferred. Multiple agents are more effective than monotherapy.

• #31 Cushing’s Syndrome: Causes and Treatment | Doctor

  https://patient.info/doctor/cushings-syndrome-pro

  Etomidate can be used for acute control of severe hypercortisolaemia. […] Osilodrostat is a novel potent oral steroidogenesis inhibitor recently approved for the treatment of adult patients with endogenous Cushing’s syndrome, and Cushing’s disease not cured by pituitary surgery or in whom pituitary surgery is not an option. […] Surgical treatment is the treatment of choice for the following conditions: Pituitary tumours: trans-sphenoidal microsurgery. Radiation therapy may be used as an adjunct for patients who are not cured. Bilateral adrenalectomy may be necessary to control toxic cortisol levels. […] Adrenocortical tumours: require surgical removal. Laparoscopic surgery is now the treatment of choice for unilateral adrenal adenomas. […] Removal of neoplastic tissue is indicated for ectopic ACTH production. Metastatic spread makes a surgical cure unlikely or impossible. Bilateral adrenalectomy is indicated if necessary to control toxic cortisol levels. […] Persisting hypercortisolaemia after trans-sphenoidal surgery can be treated with pituitary radiotherapy. Conventional fractionated radiotherapy is very effective but is associated with long-term hypopituitarism and can be very delayed in effectiveness.

• #32 New Cushing’s syndrome treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=75

  Effective management of Cushing’s syndrome involves a multidisciplinary approach, including endocrinologists, surgeons, radiologists, and other specialists. […] The primary and most common treatment for Cushing’s syndrome is surgical intervention. […] Radiation therapy is often considered when surgery is unsuccessful, incomplete, or unsuitable due to patient health conditions. […] Medical therapy is frequently used when surgery and radiation are not viable options, or as adjunctive therapy to control cortisol levels preoperatively or postoperatively. […] The likelihood of achieving a cure in Cushing’s syndrome depends on multiple factors, including the underlying cause, tumor size and location, surgical expertise, and patient-specific factors such as age, overall health, and the presence of comorbidities. […] In summary, the potential for a definitive cure for Cushing’s syndrome exists and is highly dependent on the underlying cause and timely intervention.

• #33 Endogenous Cushing Syndrome Treatment & Management: Approach Considerations, Pharmacotherapy, Surgical Therapy

  https://emedicine.medscape.com/article/2233083-treatment

  The treatment of choice for endogenous Cushing syndrome is surgical resection of the causative tumor. The primary therapy for Cushing disease is transsphenoidal surgery, and the primary therapy for adrenal tumors is adrenalectomy. […] Patients with endogenous Cushing syndrome who undergo resection of pituitary, adrenal, or ectopic tumors should receive stress doses of glucocorticoid in the intraoperative and immediate postoperative period. Typically, hydrocortisone is infused intravenously, either continuously (10 mg/h) or in boluses (80-100 mg q8h) starting prior to surgery and for the first 24 hours afterward. If the patient does well, intravenous glucocorticoid replacement may be tapered over 1-2 days and replaced with an oral formulation. […] The more extensive the mass and the resulting resection, the greater the risk for loss of pituitary function. Successful amelioration of hypercortisolism occurs in 60-80% of cases. […] Bilateral adrenalectomy is an option if transsphenoidal surgery, pituitary irradiation, and medical therapy fail or if rapid normalization of cortisol levels is required. The patient then requires lifelong glucocorticoid and mineralocorticoid therapy.

• #34 Treatment for Cushing’s – Pituitary Foundation

  https://www.pituitary.org.uk/information/cushings-treatment/

  Cushings disease is a condition caused by having too much of a hormone called cortisol in your body. It is often treated through an operation. […] Treatment for Cushings disease often includes an operation and drugs may be used to reduce the amount of cortisol produced. […] If your Cushings is caused by a pituitary tumour (Cushings disease), you will usually need an operation. […] This is called transsphenoidal surgery. […] For a few days after the operation some patients feel very thirsty and need to pass urine more than normal. This condition, called Arginine Vasopressin Deficiency (previously Diabetes Insipidus), is usually temporary but occasionally can become permanent. It can be treated by using a drug called desmopressin. […] You will need further hormone tests, either immediately and/or four to six weeks after the operation. […] You may have to take replacement cortisol (which is called hydrocortisone when it is in tablet form) or another steroid tablet such as prednisolone, for some time after your operation to compensate for a temporary reduction in your bodys ACTH production.

• #35 Cushing’s Disease Causes, Symptoms and Treatments

  https://www.upmc.com/services/neurosurgery/brain/conditions/brain-tumors/cushings-disease

  Once your doctor knows the cause, they can treat it. In the case of a tumor, surgery is usually the treatment. […] If medication is causing Cushing’s syndrome, the treatment may include reducing the dose or switching medications. For Cushing’s disease caused by a pituitary tumor, the best treatment is to remove it with surgery. In rare cases with residual tumor that is not removable, other options include Gamma Knife radiosurgery and medications. […] At UPMC, doctors treat Cushing’s disease by taking out the tumor with surgery. The least invasive and most complete option for this surgery is the endoscopic endonasal approach (EEA). […] You may need radiation for your tumor after surgery if there are areas that are too stuck to an artery or nerve to be safely removed. This treatment can begin soon after surgery. Your surgeon may recommend Gamma Knife radiosurgery. […] A number of medications can treat Cushing’s. There are medications that act at the pituitary gland that can be used for Cushing’s disease. There also are medications that act at the adrenal gland (to prevent cortisol from being made) and medications that block the action of cortisol.

• #36 Subclinical Cushing’s Syndrome Treatment and Surgery

  https://www.adrenal.com/blog/subclinical-cushings-syndrome-treatment-and-surgery

  Adrenal surgery has been demonstrated to decrease the cardiovascular risk factors associated with subclinical Cushings syndrome. […] We do know that those individuals with subclinical Cushings syndrome treated with surgery will typically show improvement in their hypertension, obesity, diabetes, and cholesterol levels. […] Adrenal surgery can be performed via the Mini Back Scope Adrenalectomy (MBSA) operation, which is by far the best adrenal operation, suing small incisions on the patients flank. […] The best treatment is surgery on the adrenal gland in which the benign tumor is growing. […] In patients undergoing adrenalectomy, studies have proven that they demonstrate an improvement in their blood pressure (reducing number of medications needed), diabetes, obesity, and cholesterol levels, as well as glucose metabolism and lowered body weight. […] The goal of treatment with Korlym is to decrease the symptoms of excess cortisol activity. Korlym does not decrease cortisol levels, [surgery does]. […] Clinical and hormonal features improve after adrenalectomy making a difficult diagnosis imperative to save your life.

• #37 Cushing Syndrome | Condition | UT Southwestern Medical Center

  https://utswmed.org/conditions-treatments/cushing-syndrome/

  After surgery, the adrenal glands might not work as they should right away. Because of this, patients take medications to replace their cortisol. Over time the adrenal glands usually go back to making the correct amount of cortisol, meaning it is likely patients can eventually taper off their use of the replacement drugs. This process can take up to a year or longer. […] Some tumors that secrete ACTH are too small to be found and removed. In those cases, treatment for Cushing syndrome involves removing both adrenal glands. This procedure is called bilateral adrenalectomy. Patients might need this surgery if they have severe Cushing syndrome and other treatments have not worked. […] If a surgeon cannot remove all of the pituitary tumor, the patient might need radiation therapy after surgery. This treatment can destroy what is left of the tumor. If it is not possible to take out any of the tumor, the patient might receive radiation therapy alone. […] Medications might be part of your treatment plan for Cushing syndrome. Patients might receive medication before surgery if Cushing syndrome is very severe. The doctor might also recommend medication before surgery to improve signs and symptoms and to lower the risks of surgery.

• #38 Cushing’s Disease | UVA Health

  https://uvahealth.com/services/benign-brain-tumor/cushings-disease

  Cushing’s disease is caused by your medications. Changing your medications can improve your symptoms. […] Our UVA Health experts can pinpoint the source of your symptoms. They can get your hormone levels back to normal. And they can treat your tumor with surgery or radiation. […] Medication can get your adrenal glands under control. It can stop them from making too much cortisol. We’ll adjust your dose to get it just right. […] If you have Cushing’s disease because of a tumor, the best treatment is to remove it. Our neurosurgeons can remove the tumor through your nose. […] We can use radiation to treat the tumor if surgery isn’t right for you. We’ll also use it along with surgery if we can’t fully remove the tumor. […] Gamma Knife radiosurgery is another option. This procedure can target the tumor directly. It also uses less radiation than conventional radiation treatment, which can last 5 weeks.

• #39 Cushing’s Syndrome/Disease – AANS

  https://www.aans.org/patients/conditions-treatments/cushings-syndrome-disease/

  Treatment of Cushings syndrome depends on the underlying cause of excess cortisol but may perhaps include surgery, radiation, chemotherapy or the use of cortisol-inhibiting drugs. […] Microsurgical resection of an ACTH-secreting pituitary adenoma is the optimum treatment for Cushings disease with cure rates of 80-90 percent if a tumor is found. […] In patients to whom a remission is not obtained after surgery, the use of various medications that can inhibit cortisol production may be used, such as: Ketoconazole (Nizoral), Mitotane (Lysodren), Metyrapone (Metopirone), Mifepristone (Korlym). […] One additional treatment option in patients who have failed surgical procedures or are too high risk medically to bring to surgery is radiation therapy. […] Stereotactic radiosurgery has also been found to be an effective treatment for patients with ACTH-secreting pituitary adenomas.

• #40 Get Cushing Disease Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/cushing-disease-treatment

  If surgery for your pituitary tumor isnt possible, we might also recommend Gamma Knife radiosurgery. This highly focused radiation technique can reduce or eliminate your tumor noninvasively. We were one of the first three medical centers in the nation to start using Gamma Knife. Our surgeons have been doing these procedures for more than 30 years, giving us a high level of expertise and skill. […] Gamma Knife is one type of radiosurgery. Depending on your location and recommendation of your provider, you may get a different type of radiosurgery. Talk to your provider about the best option for you.

• #41 Cushing Syndrome – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/adrenal-gland-disorders/cushing-syndrome

  Surgery or radiation therapy is often needed to remove a tumor. […] Cushing disease is treated with surgery or radiation to remove the pituitary tumor. If removal of the pituitary tumor can’t be done or is unsuccessful, the adrenal glands can be removed surgically, or medications can be given to reduce ACTH production or block the production or effects of the excess cortisol on tissues. […] Treatment depends on whether the problem is in the adrenal glands, the pituitary gland, or elsewhere. […] The first step people can take in the treatment of Cushing syndrome is to support their general condition by following a diet that is high in protein and potassium. […] Surgery or radiation therapy (including proton beam therapy, if available) may be needed to remove or destroy a pituitary tumor.

• #42

  https://link.springer.com/article/10.1007/s40265-016-0539-6

  Severe Cushings syndrome presents an acute emergency and is defined by massively elevated random serum cortisol [more than 36 g/dL (1000 nmol/L)] at any time or a 24-h urinary free cortisol more than fourfold the upper limit of normal and/or severe hypokalaemia (3.0 mmol/L), along with the recent onset of one or more of the following: sepsis, opportunistic infection, intractable hypokalaemia, uncontrolled hypertension, heart failure, gastrointestinal haemorrhage, glucocorticoid-induced acute psychosis, progressive debilitating myopathy, thromboembolism or uncontrolled hyperglycaemia and ketocacidosis. Treatment focuses on the management of the severe metabolic disturbances followed by rapid resolution of the hypercortisolaemia, and subsequent confirmation of the cause. Emergency lowering of the elevated serum cortisol is most rapidly achieved with oral metyrapone and/or ketoconazole; if parenteral therapy is required then intravenous etomidate is rapidly effective in almost all cases, but all measures require careful supervision.

• #42

  https://link.springer.com/article/10.1007/s40265-016-0539-6

  The optimal order and combination of drugs to treat severe hypercortisolaemia mostly in the context of ectopic ACTH-secreting syndrome, adrenocortical carcinoma or an ACTH-secreting pituitary adenoma (mainly macroadenomas) is not yet established. Combination therapy may be useful not only to rapidly control cortisol excess but also to lower individual drug dosages and consequently the possibility of adverse effects. If medical treatments fail, bilateral adrenalectomy should be performed in the shortest possible time span to prevent the debilitating complications of uncontrolled hypercortisolaemia.

• #43 Cushing Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/cushing-syndrome

  Treatment of Cushing Syndrome includes high protein intake and potassium administration (or potassium-sparing drugs such as spironolactone) […] Adrenal inhibitors such as metyrapone or ketoconazole and rarely mitotane, or medications such as osilodrostat and levoketoconazole […] Surgery or radiation therapy to remove pituitary, adrenal, or ectopic ACTH-producing tumors […] Sometimes somatostatin analogs or dopamine agonists to block ACTH secretion, or the glucocorticoid receptor antagonist mifepristone […] Sometimes parenteral etomidate to inhibit 11-beta hydroxylase and reduce adrenal steroidogenesis […] Initially, the patients general condition should be supported by high protein intake and appropriate administration of potassium. If clinical manifestations of hypercortisolism are severe, it may be reasonable to block corticosteroid secretion with metyrapone 250 mg to 1 g orally 3 times a day or ketoconazole 400 mg orally once a day, increasing to a maximum of 400 mg 3 times a day

• #43 Cushing Syndrome – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/cushing-syndrome

  Bilateral adrenalectomy is reserved for patients with pituitary hyperadrenocorticism who do not respond to both pituitary exploration (with possible adenomectomy) and irradiation, or in patients in whom surgery was unsuccessful and radiotherapy is contraindicated […] Adrenocortical tumors are removed surgically. Patients must receive cortisol during the surgical and postoperative periods because their nontumorous adrenal cortex will be atrophic and suppressed […] Ectopic ACTH syndrome is treated by removing the nonpituitary tumor that is producing the ACTH […] Mifepristone also may be useful for treating ectopic ACTH syndrome; however, because it blocks the action of cortisol but does not lower serum levels, monitoring its use can be problematic […] In an emergency situation, parenteral etomidate can produce a rapid fall in serum cortisol, but its use requires careful monitoring

• #44 Treatment of Cushing’s Syndrome Guideline Resources | Endocrine Society

  https://www.endocrine.org/clinical-practice-guidelines/treatment-of-cushing-syndrome

  The Endocrine Society recommends that the first-line treatment for endogenous Cushings syndrome be the removal of the tumor unless surgery is not possible or unlikely to address the excess cortisol. Surgical removal of the tumor is optimal because it leaves intact the hypothalamic-pituitary-adrenal axis, which is integral to the bodys central stress response. […] We recommend initial resection of primary lesion(s) underlying Cushing’s disease (CD), ectopic and adrenal (cancer, adenoma, and bilateral disease) etiologies, unless surgery is not possible or is unlikely to significantly reduce glucocorticoid excess. […] We recommend additional treatments in patients with persistent overt hypercortisolism. […] We recommend steroidogenesis inhibitors under the following conditions: as second-line treatment after TSS in patients with CD, either with or without RT/radiosurgery; as primary treatment of EAS in patients with occult or metastatic EAS; and as adjunctive treatment to reduce cortisol levels in adrenocortical carcinoma (ACC). […] We recommend treating the specific comorbidities associated with CS (eg, cardiovascular risk factors, osteoporosis and psychiatric symptoms) in all patients with CS throughout their lives until resolution.

• #45 Cushing’s Disease Symptoms, Diagnosis | Froedtert & the Medical College of Wisconsin

  https://www.froedtert.com/endocrinology/cushings-disease

  The best course of treatment for Cushings disease, ectopic ACTH syndrome and other forms of Cushings syndrome depends on the cause of the condition. […] When Cushings syndrome is caused by an excessive level of cortisol due to medications, reducing the dosage may be an option. If not, treatment involves symptom relief and medication to help treat complications of cortisol excess. […] Patients with Cushings disease or syndrome may also receive treatment for specific complications, such as medication for high blood pressure, diabetes and depression. […] When Cushings disease or syndrome is caused by a pituitary tumor, the most effective treatment is surgery. Tumors may recur, however, so an annual assessment of cortisol secretion is important. If pituitary surgery does not resolve the symptoms or if the tumor recurs, several treatment options are available, including a repeat operation, radiation therapy and medication. […] If an adrenal gland tumor is the cause, the most effective treatment is the removal of the gland through minimally invasive surgery.

• #46 Recovery From Cushing’s and Coping With Recovery – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/coping-with-cushings/recovery-from-cushings-and-coping-with-recovery/

  I advise my patients that they didnt develop symptoms overnight, and wont recover from the signs/symptoms quickly. It may take a year or more, but that they should improve little by little. Expect the first month or so to be the most difficult. […] For coping and helping themselves recover, I tell them to get some exercise and make some recommendations about how to do that and how much. I recommend that they push themselves a little more every week, but to not expect big improvements quickly. Healthy food and drink intake and stress reduction techniques are also helpful. I also advise them to make sure that any other hormone deficiencies are addressed and to consider psych evaluation and intervention for the emotional issues that surface during recovery. […] Recovery and coping are tough questions to address; every patient is different.

• #47 Recovery From Cushing’s and Coping With Recovery – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/coping-with-cushings/recovery-from-cushings-and-coping-with-recovery/

  My advice: patience, patience, patience. […] I tell my patients BEFORE surgery that they will probably feel worse after successful surgery than before the operation. […] The muscle aches and joint pains can be helped with medication (Aleve, aspirin). I also tell patients that it usually takes 6 to 12 months (and dieting to lose the weight) to recover from Cushings. […] I point out that it took a long time to develop loss of muscle mass and it takes a long time for this to improve. […] I also tell patients that it usually takes 6 to 12 months (and dieting to lose the weight) to recover from Cushings. […] My advice to my patients: (a) expect that recovery will be very gradual and slow; do not have unrealistic expectations, which result in frustration and anxiety; (b) try to increase exercise gradually, start slow and increase activity gradually; (c) do pay attention to diet and food intake, count calories; (d) know that things will improve, but improvement will be gradual; (e) if depression is a problem, continue treatment for depression; (f) again: patience, patience and patience: there is no way around this; (g) testing for growth hormone (GH) deficiency: if a patient is growth hormone deficient, GH replacement may improve the rate of recovery from Cushings.

• #48 Recovery From Cushing’s and Coping With Recovery – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/coping-with-cushings/recovery-from-cushings-and-coping-with-recovery/

  Recovery can be difficult and takes time but, recovery will happen. […] Gradual, progressive increases in exercise duration and intensity will help regain muscle function, and assistance with emotional issues as needed are very important. […] A healthy diet, to help with weight loss along with being sure to get the recommended daily amounts of calcium and vitamin D for bone health is important. […] While recovery requires time, the good news is that with patience, exercise, a healthy diet, and support from others, most patients recover very well within 12-24 months after surgery. […] Recovery was very difficult at first, but gradually became easier over the course of the year. […] I credit the success to the slow tapering, and to some personal emotional work I did. […] I had to learn how to function without being driven by cortisol.

• #49 Adrenal Cushing’s Syndrome | Saint John’s Cancer Institute

  https://www.saintjohnscancer.org/endocrine/conditions/cushings-syndrome-adrenal/

  After you have surgery for Cushings syndrome, you will meet with your surgeon two weeks after the operation. You will have regular appointments with your endocrinologist to monitor your blood tests and manage your medications. You will be slowly weaned off of steroids, a process that can take up to two years in some patients. […] Many Cushing syndrome symptoms will go away after treatment, but this will most likely happen over time up to 18 months. Weight loss, changing face shape, and reduced bruising are usually first. Additionally, if you have had long-standing issues caused by Cushings syndrome, there may be some long-term effects that require monitoring. Lastly, increased cortisol levels may have suppressed some autoimmune disorders. Once cortisol levels are normal, the disorders may come back.

• #49 Adrenal Cushing’s Syndrome | Saint John’s Cancer Institute

  https://www.saintjohnscancer.org/endocrine/conditions/cushings-syndrome-adrenal/

  Understanding how to identify and manage Cushings Syndrome is crucial for effective treatment and management. […] Treatment for Cushings Syndrome depends on the underlying cause. Surgery is often the first-line treatment for tumors. For pituitary tumors, transsphenoidal surgery is typically performed to remove the tumor. Adrenal tumors may require adrenalectomy, while ectopic tumors necessitate surgical removal of the tumor producing ACTH. Medication is an alternative or adjunct to surgery, particularly if surgery is not feasible. Medications such as ketoconazole, mitotane, or metyrapone can inhibit cortisol production. For patients with endogenous Cushings Syndrome who are not surgical candidates, medications like mifepristone may help control symptoms. […] In managing Cushings Syndrome, lifestyle modifications are also beneficial. Patients should maintain a balanced diet, engage in regular physical activity, and manage stress effectively. Regular follow-up with healthcare providers is crucial for monitoring cortisol levels and adjusting treatment plans as needed. Early diagnosis and tailored treatment strategies are essential for reducing complications and improving the quality of life for individuals with Cushings Syndrome.

• #50 Cushing’s Disease: Clinical Manifestations and Diagnostic Evaluation | AAFP

  https://www.aafp.org/pubs/afp/issues/2000/0901/p1119.html/

  Transphenoidal removal of the tumor is the treatment of choice for Cushing’s disease. Cure is likely if the patient develops hypocortisolism in the first few days to weeks after surgery. Most patients are rendered hypoadrenal for months to years after the procedure. During this period, they require glucocorticoid replacement therapy. […] Pituitary irradiation can induce remission of disease in more than one half of patients with recurrence after surgery. Patients who are not cured may require total bilateral adrenalectomy to control their symptoms. […] Ketoconazole (Nizoral) and aminoglutethimide (Cytadren), which are inhibitors of adrenal steroid biosynthesis, may be used in the perioperative period to decrease the clinical effects of hypercortisolism. […] Patients who have been surgically treated for Cushing’s disease require careful long-term follow-up and monitoring for signs and symptoms of tumor recurrence. The pituitary adrenal axis must be evaluated six to 12 months after surgery to determine the potential need for lifetime exogenous steroid replacement therapy. Patients with panhypopituitarism subsequent to surgery require lifetime monitoring and titration of hormone therapy. […] All patients who need glucocorticoid replacement therapy should be given careful instructions about the effects of stress and illness on glucocorticoid dosages. In addition, these patients should wear appropriate medical alert labels.

• #51 Recovery From Cushing’s and Coping With Recovery – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/coping-with-cushings/recovery-from-cushings-and-coping-with-recovery/

  I had to realize that recovery is a process that takes a long time. […] I had to go into a new phase of my life and quit focusing so much on getting back to normal. […] I had to learn to appreciate the person I was becoming. […] I had lots of withdrawal symptoms and not a very good disposition, either, as my husband and daughter would gladly attest to. […] Mild exercise such as walking every day, reducing stress as much as possible and lots of quiet relaxation time helped considerably. […] I did MANY things to try to help myself including physical therapy so that my body wouldn’t completely atrophy. […] I joined a gym with a pool and took a lot of Arthritis classes in the pool to help my joints. […] You must be patient and positive. […] Get lots of rest. […] What I found helpful were breathing exercises, being patient with myself, venting with family members and friends, distracting myself with fun things like buying and watching seasons of Greys Anatomy, making jewelry, listening to music, and getting massages when I could afford them really helped with the aching muscles. […] Recovery impacts not only every aspect of my life but also that of my entire family. […] Recovery was the biggest challenge of my life, but worth every minute.

• #52 What Is Cushing Syndrome? An Expert Explains | Henry Ford Health – Detroit, MI

  https://www.henryford.com/blog/2024/04/what-is-cushing-syndrome-an-expert-explains

  If surgery was not successful in removing the whole pituitary tumor, radiation therapy may be utilized following surgery, or as an option for those who are not a surgical candidate. In patients who cannot undergo surgery or radiation, medication can be used to reduce or control cortisol levels. This can be used temporarily in patients who are very sick to help improve their symptoms and reduce risks associated with surgery. […] There are many complexities in diagnosis and management of Cushing syndrome, says Dr. Athimulam. This condition can affect multiple bodily functions at once and require doctors in many fields (including endocrinology, neurosurgery, endocrine surgery, and radiation oncology) to treat completely. That is why it is important to be evaluated and treated by a multi-disciplinary team with expertise in managing this condition.

• #53 AACE Patient Guide to Rare Endocrine Diseases: Cushing’s Syndrome | American Association of Clinical Endocrinology

  https://www.aace.com/disease-and-conditions/cushings-syndrome/aace-patient-guide-rare-endocrine-diseases-cushings-syndrome

  If surgery does not result in remission, then a repeat of surgery, medications, and/or radiation therapy may be considered. […] Most likely, the treatment plan that your health care professional creates will include a combination of options to help reduce your cortisol levels and manage your symptoms.

• #54 Cushing syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cushing-syndrome/symptoms-causes/syc-20351310

  Treatments for Cushing syndrome can lower the body’s cortisol levels and improve symptoms. The sooner treatment starts, the better the chances for recovery. […] Without treatment, Cushing syndrome can cause complications, including: […] Cushing syndrome care at Mayo Clinic.

• #55 Cushing’s disease treatment options | Cushing’s Disease NewsEnvelope icon

  https://cushingsdiseasenews.com/cushings-disease-treatment/

  One therapeutic strategy for Cushing’s disease, involving the removal of one or both of the adrenal glands, is a procedure referred to as an adrenalectomy. This surgery can be used to reduce cortisol levels in patients with persistent or recurrent Cushing’s disease for whom other forms of treatment have failed. […] Cushing’s disease can be treated and cured, with 100% remission, although lifelong monitoring might be required to check for disease recurrence and related health problems, including high blood pressure and diabetes. […] The optimal treatment for Cushing’s disease depends on a patient’s particular clinical situation, the severity of the symptoms, and the characteristics of the underlying pituitary tumor that causes the condition. Usually the first line treatment for Cushing’s disease is the surgical removal of the pituitary tumor — a procedure called transsphenoidal adenomectomy. Other treatment options include radiation therapy and specific medications, all with the goal of reducing the high levels of cortisol that characterize the rare disorder.

• #56 What is Cushing syndrome? – Harvard Health

  https://www.health.harvard.edu/diseases-and-conditions/what-is-cushing-syndrome

  Without treatment, Cushing syndrome can be life-threatening. […] If Cushing syndrome is caused by glucocorticoids you are taking to treat another medical condition, you may be able to work with your doctor to gradually and carefully reduce your dose. […] If a tumor is causing the disorder, you may need surgery to remove it. Medical therapies such as cortisol-blocking drugs may also be needed. […] Dr. Nachtigall coauthored a research study on Cushing syndrome, which found that about 10% of people with Cushing syndrome develop an autoimmune disease after they are surgically treated and in remission. Some people have problems with memory and mood afterward as well. These problems may require additional medical therapy, says Dr. Nachtigall, but most symptoms improve after treatment. […] Cushing syndrome may come back, even years later. Being aware of the symptoms of Cushing syndrome, and getting treated early, can help prevent long-term effects.

• #57 Cushing’s syndrome – symptoms, treatments and causes | healthdirect

  https://www.healthdirect.gov.au/cushings-syndrome

  Cushing’s syndrome is a condition where you have high levels of a hormone called cortisol. […] Often Cushing’s syndrome can be cured, but you will need long term follow up to make sure it doesn’t come back. […] The treatment depends on the cause. […] If you have a tumour causing Cushing’s syndrome, your doctor may recommend treatment such as: surgery to remove the tumour, radiotherapy if your tumour cannot be fully removed with surgery, medicine to stop your body making too much cortisol. […] After surgery, it may take some time for your body to make the right amount of cortisol. […] Cushing’s disease can come back after treatment. After you have recovered, you should have your cortisol levels checked regularly to make sure you stay healthy.

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