Top 5 Things to Know About Adrenal Tumors and Pregnancy
April is Adrenal Disease Awareness Month. Adrenal disease and adrenal tumors occur in both genders and all age groups. This means that occasionally adrenal tumors occur during pregnancy.
Top 5 Things to Know About Adrenal Tumors and Pregnancy
It is very important for women to know about the symptoms of adrenal tumors, as they can occur and be diagnosed during pregnancy. In fact, sometimes the adrenal tumor is “unmasked” during pregnancy. This is partly due to the stress of pregnancy leading to signs and symptoms such as high blood pressure, headache, anxiety, low potassium levels, and migraines. The other reason adrenal tumors are often diagnosed during pregnancy is that women are more closely monitored, and will have their blood pressure, heart rate and sometimes laboratory tests performed more commonly.
Here, we discuss the top 5 things you should know about adrenal tumors and pregnancy
- Pheochromocytoma can lead to death of both the baby and the mother if not diagnosed and treated properly during pregnancy
- Conn’s syndrome often leads to bad high blood pressure during pregnancy
- Cushing’s syndrome can cause many complications during pregnancy
- Adrenal surgery during the second trimester is best if surgery needs to be performed during pregnancy
- The Mini Back Scope Adrenalectomy is the best adrenal operation during pregnancy
1) Pheochromocytoma can lead to death of both the baby and the mother if not diagnosed and treated properly during pregnancy
Pheochromocytoma is an adrenal tumor arising from the medulla of the adrenal gland. All symptoms of a pheochromocytoma are due to the very toxic effects of too much adrenaline hormones (catecholamines). Symptoms from a pheochromocytoma are often characterized by paroxysm (“spells”). This means patients develop symptoms from the pheochromocytoma during an attack, a “spell”, which often last 5 to 20 minutes. The typical spell is associated with very high blood, headache, palpitations (heart racing), and sweating. Chest or abdominal pain is also common as is a sense of impending doom. The patient may turn pale although sometimes flushing may occur. These spells (paroxysms) typically last 5 min to an hour but occasionally last even longer. Not all paroxysms are typical, and many variations are seen.
Read more about pheochromocytoma symptoms.
Patients and expecting mothers can become very sick from a pheochromocytoma. If the patient has any of the suspected symptoms lab test both via the blood and urine are very important to be performed to see if the catecholamines (adrenaline hormones) are elevated. Elevated catecholamines establishes the diagnosis of pheochromocytoma. These tests can easily be performed normally during pregnancy.
The next step would be to perform an imaging study. We typically avoid a CT scan during pregnancy and an MRI is the preferred imaging modality for pheochromocytomas during pregnancy.
Occasionally we see doctors that want to put a biopsy needle into an adrenal mass. This should absolutely not be performed and especially not in that patient who is pregnant with a pheochromocytoma.
If the patient indeed has a pheochromocytoma, the mother needs to be treated with medications (alpha-blocker such as phenoxybenzamine, doxazosin, prazosin, etc, and sometimes metyrosine) and then a decision has to be made whether to perform the adrenal surgery during the pregnancy or treat with medications until after delivery of the baby and then remove the pheochromocytoma.
There are pros and cons to both approaches, and it has to be done in an individualized manner. Nonetheless, it is rare for almost all doctors except Dr. Carling to ever operate on patients with pheochromocytoma during pregnancy (remember, the average number of adrenal operations performed by US adrenal surgeons is one per year! So, almost no American surgeon except Dr. Carling has ever operated on an adrenal tumor in a pregnant patient before). Expectant mothers and their families need to take this into account.
Just treating the mother with medications until delivery also present some downsides and the later stages of the pregnancy can lead to severe release of catecholamines with significant problems and risk to both the mother and the fetus. The outcomes for pheochromocytoma during pregnancy have improved in recent decades. This is partly due to the diagnosis of genetic predisposition in carriers of germ line mutations of the susceptibility genes (in brackets) which predispose to von Hippel–Lindau syndrome (VHL), Multiple Endocrine Neoplasia type 2 (RET), Neurofibromatosis type 1 (NF1), Paraganglioma Syndromes type 1–5 (SDHD, SDHAF2, SDHC, SDHB, SDHA), MYC-associated factor X (MAX), hypoxia-inducible factor 2A (HIF2A), fumarate hydratase (FH), and transmembrane protein 127 (TMEM127).
However, undiagnosed pheochromocytoma during pregnancy is still associated with a high death rate both for the baby and the mother.
Figure 1. Ultrasound demonstrating a perfectly healthy baby following Mini-Back Scope Adrenal Operation (MBSA) for a pheochromocytoma by Dr. Carling in a patient with a large pheochromocytoma. The baby is even giving a thumbs up!
2) Conn’s syndrome often leads to bad high blood pressure during pregnancy
Primary Hyperaldosteronism (also known as Conn’s syndrome) is due to an aldosterone producing tumor of the adrenal gland. Too much aldosterone is very toxic for the body and leads to high blood pressure. Primary hyperaldosteronism often occurs in young women and is not uncommon that it occurs or is diagnosed during pregnancy. The most common symptom is high blood pressure during pregnancy. High blood pressure during pregnancy is not uncommon and the most common causes preeclampsia.
However, primary hyperaldosteronism is common and often is detected as high blood pressure and sometimes low potassium during the pregnancy. Significantly high blood pressure during the pregnancy should prompt a workup for primary hyperaldosteronism including measurements of aldosterone which is typically high and renin which is low or suppressed. Sometimes patients also have low potassium. Low potassium together with high blood pressure is often due to primary hyperaldosteronism.
Similar as in patients with pheochromocytoma the most important part is to diagnose the patient. Again, CT scans are avoided during pregnancy and thus an MRI of the adrenal is a good way to identify the adrenal tumor. It should be individualized whether the patient should have adrenal surgery (Mini-Back Scope Adrenal Adrenalectomy) during the second trimester of the pregnancy or to be treated with medications until after delivery and then have the aldosterone producing adrenal adenoma removed.
The following patients with high blood pressure during pregnancy needs to be screened for primary hyperaldosteronism and Conn’s syndrome:
- Resistant hypertension (i.e. poor response to medications)
- Hypertension requiring two or more blood pressure medications
- Hypertension requiring being on Spironolactone (Aldactone), Eplerenone (Inspra) or Amiloride
- Hypertension with low blood potassium (hypokalemia) (this is classic for Conn's Syndrome).
- Hypertension with an adrenal incidentaloma (adrenal tumor found when x-raying for something else)
- Hypertension and sleep apnea
- Hypertension in a family member with an adrenal tumor
Read more about primary hyperaldosteronism and Conn’s syndrome symptoms, diagnosis, and treatment.
Figure 2. High blood pressure is relatively common during pregnancy. In most cases it is not due to an adrenal tumor, but primary hyperaldosteronism (Conn’s syndrome) sometimes needs to be checked for.
3) Cushing’s syndrome can cause many complications during pregnancy
Cushing’s and subclinical Cushing’s syndromes can be caused by cortisol over-production from an adrenal tumor. Subclinical Cushing’s (less severe than overt disease) is more common.
Too much cortisol is very toxic to the expectant mother and the baby. Cortisol has many functions on various cells of the body and can cause a myriad of symptoms.
Learn more about the typical symptoms of Cushing's syndrome or review the top myths about adrenal Cushing's syndrome.
In pregnancy, almost 50% of Cushing’s syndrome is due to adrenal adenoma, 30% to Cushing’s Disease (an ACTH producing pituitary tumor), and 1–3% due to ectopic ACTH syndrome, which is typically associated with malignancy. Recognition of cortisol excess is crucial, as untreated CS during pregnancy has severe consequences.
Figure 3. Cushing’s syndrome, whether due to an adrenal or pituitary tumor can lead be significant complications during pregnancy.
Similar as in pheochromocytoma and Conn’s syndrome, an MRI of the adrenal glands are preferred over a CT scan during pregnancy. Surgical management is a safe and effective option for treatment of both pituitary and adrenal Cushing’s syndrome. Regardless of etiology, surgery should be performed during the second trimester, preferably before the 24th week of gestation.
Learn about the best surgery and treatment for Cushing’s Disease.
4) Adrenal surgery during the second trimester is best if surgery needs to be performed during pregnancy
Medical therapy for Cushing’s 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
Figure 4. The best time to perform adrenal surgery during the 2ndd trimester of pregnancy.
The second trimester occurs between week 13 and week 26 of gestation. This is the best time to remove the adrenal tumor if it needs to be removed during the pregnancy. The risk of miscarriage or preterm labor and delivery slowest during the second press trimester.
In addition, the uterus is not as big as it will become during the third trimester which means that any surgical procedures are easier to be performed. A large pregnant uterus squishes all other organs to some extent making less room for surgery to be performed during later stages of the pregnancy. Also, surgery during later stages of the pregnancy leads to higher risk of the anesthesia.
5) The Mini Back Scope Adrenalectomy is the best adrenal operation during pregnancy
The best operation for adrenal surgery is the Mini Back Scope Adrenalectomy (MBSA). This is true for almost all adrenal tumors and is especially true during the pregnancy. Some of the reasons the MBSA is the best is summarized here:
- The operation is performed through the back, where the adrenals are located = faster and better
- The operation is performed away from the uterus (and baby) = safer
- The operation requires less mobilization of other organs = safer, faster, and better
- The operation takes minutes instead of hours = safer and better
- The operation leads to less pain = better
You definitely do not want to have either a laparoscopic adrenalectomy or a robotic adrenalectomy during pregnancy. Do not have your surgeons go through the belly to get to the adrenal glands. Guess what!? The adrenal glands are not in the belly. They are behind the belly in the retroperitoneum.
Learn more about why robotic adrenal surgery and laparoscopic adrenalectomy surgeries are not recommended by Dr. Carling.
Read about adrenal surgery and the Mini-Back Scope Adrenal Operation (MBSA).
Additional Resources:
- Learn more about the Carling Adrenal Center
- Learn more about Dr. Tobias Carling
- Learn more about our sister surgeons at the Norman Parathyroid Center, Clayman Thyroid Center and Scarless Thyroid Surgery Center
- Learn more about the Hospital for Endocrine Surgery
Reference:
Affinati AH, Auchus RJ. Endocrine causes of hypertension in pregnancy. Gland Surg. 2020 Feb;9(1):69-79.