Our Summary

This research paper looks at the outcomes of two different surgical treatments for a hereditary form of adrenal gland tumor, known as hereditary pheochromocytoma (hPCC). The two surgeries compared are radical adrenalectomy (RA), which removes the entire adrenal gland, and partial adrenalectomy (PA), which aims to remove only the tumor while leaving some of the adrenal gland intact.

The study involved 256 patients from 12 European centers, who received these treatments between 1974 and 2023. The results showed that those who received the PA had a higher rate of the tumor returning in the same adrenal gland. However, the rates of the cancer spreading and death from the disease did not differ between the two surgery groups.

The study also found that among patients who had tumors in both adrenal glands, nearly half developed adrenal insufficiency (a condition where the adrenal glands can’t produce enough hormones) after receiving the PA.

For patients who initially only had a tumor in one adrenal gland, but later developed a tumor in the other gland, three quarters developed adrenal insufficiency if they had the PA first followed by the RA. However, this rate dropped to 14% if the PA was performed after the RA.

Overall, the study suggests that although PA has a higher risk of the tumor reappearing, it doesn’t increase the risk of the cancer spreading or death. Therefore, PA could be a safe option to preserve adrenal function in patients with hPCC, especially when performed as a second operation.

FAQs

  1. What are the two surgical treatments compared in this study for hereditary pheochromocytoma?
  2. What are the potential risks and outcomes associated with partial adrenalectomy (PA) and radical adrenalectomy (RA)?
  3. Based on this study, can partial adrenalectomy be considered a safe option for patients with hereditary pheochromocytoma?

Doctor’s Tip

A helpful tip a doctor might tell a patient about adrenalectomy is to discuss with their healthcare provider the option of partial adrenalectomy (PA) versus radical adrenalectomy (RA) for hereditary pheochromocytoma (hPCC). While PA may have a higher risk of the tumor returning in the same adrenal gland, it may be a safer option for preserving adrenal function in the long term. Patients should also be aware of the potential for developing adrenal insufficiency after surgery, especially if tumors are present in both adrenal glands. It is important to have open communication with your healthcare team to determine the best treatment approach for your individual case.

Suitable For

Patients who are typically recommended adrenalectomy include those with hereditary pheochromocytoma (hPCC), especially if they have tumors in both adrenal glands. Additionally, patients who have tumors that are causing symptoms such as high blood pressure, sweating, and palpitations may also be recommended for adrenalectomy. Patients with adrenal tumors that are suspicious for cancer, known as adrenal cortical carcinoma, may also be recommended for adrenalectomy to prevent the spread of cancer. Ultimately, the decision to undergo adrenalectomy is made on a case-by-case basis by a healthcare provider in collaboration with the patient.

Timeline

Before adrenalectomy:

  1. Patient is diagnosed with a hereditary form of adrenal gland tumor, such as hereditary pheochromocytoma (hPCC).
  2. Patient undergoes pre-operative evaluation, including imaging tests to determine the size and location of the tumor.
  3. Patient discusses treatment options with their healthcare provider, including the option of radical adrenalectomy (RA) or partial adrenalectomy (PA).
  4. Patient may receive medications to control symptoms of the tumor, such as high blood pressure and sweating, before surgery.

After adrenalectomy:

  1. Patient undergoes either RA or PA surgery to remove the adrenal gland tumor.
  2. In the case of PA, patient may experience a higher risk of the tumor returning in the same adrenal gland compared to RA.
  3. Patients who had tumors in both adrenal glands may develop adrenal insufficiency after receiving PA.
  4. Patients who develop a tumor in the other adrenal gland after initial surgery may have varying rates of adrenal insufficiency depending on the sequence of surgeries.
  5. Overall, PA may be a safe option to preserve adrenal function in patients with hPCC, especially when performed as a second operation.

What to Ask Your Doctor

Some questions a patient should ask their doctor about adrenalectomy include:

  1. What are the potential risks and benefits of radical adrenalectomy (RA) versus partial adrenalectomy (PA) for my specific condition?
  2. What are the chances of the tumor returning in the same adrenal gland after PA compared to RA?
  3. How likely is it that the cancer will spread or lead to death with each type of surgery?
  4. What are the long-term effects on adrenal function after each type of surgery?
  5. Are there any other treatment options available besides adrenalectomy for my condition?
  6. How experienced are you in performing both RA and PA surgeries?
  7. What is the recovery process like for each type of surgery?
  8. Are there any specific lifestyle changes I should make before or after the surgery?
  9. How often will I need follow-up appointments and monitoring after the surgery?
  10. Are there any clinical trials or research studies that I may be eligible for related to adrenalectomy for hPCC?

Reference

Authors: Xu K, Langenhuijsen JF, Viëtor CL, Feelders RA, van Ginhoven TM, Elhassan YS, Bioletto F, Parasiliti-Caprino M, Zandee WT, Kruijff S, Backman S, Åkerström T, Pamporaki C, Bechmann N, Lussey-Lepoutre C, Canu L, Steenaard RV, Driessens N, Velema M, Dreijerink KMA, Engelsman AF, Timmers HJLM, de Laat JM. Journal: Eur J Endocrinol. 2024 Aug 30;191(3):345-353. doi: 10.1093/ejendo/lvae108. PMID: 39171965