What Are Rare Adrenal Problems?

Certain adrenal tumors and adrenal diseases are common. In contrast, here is a list of rare adrenal problems:
  • Rare tumors/masses of the adrenal gland
  • Rare tumors of the retroperitoneum which may mimic an adrenal tumor
  • Rare genetic variants of Primary hyperaldosteronism
  • Rare genetic variants of Cushing syndrome (Primary hypercortisolism)
  • Rare genetic variants of Pheochromocytoma and Paraganglioma
  • Rare genetic variants of Adrenocortical cancer

There are also a number of rare adrenal masses and the differential diagnosis may include adrenolipoma, amyloidosis, ganglioneuroma, granuloma, hamartoma, hematoma, hemangioma, leiomyoma, lipoma, neurofibroma, adrenal pseudocyst, lymphoma and teratoma.

Infections of the Adrenal Gland

Although rare in the Western world and developed countries, various infectious processes may cause an adrenal mass. These include fungal, tuberculosis, echinococcosis, and cryptococcosis. Adrenal cysts can be infectious, lymphangiomatous, or angioma¬tous endothelial, cystic degenerative adenomas or embryonal retention cysts. Sometimes, the imaging (x-rays and scans) characteristics of these particular lesions are helpful. Surgical resection may be needed due to mass effect where the mass pushes on nearby organs, or to prevent rupture, hemorrhage or infection. Additionally, when malignancy cannot be excluded based on imaging, surgical resection (adrenalectomy) is warranted.

Is it Ever Appropriate to Biopsy an Adrenal Mass?

As mention there are a few exceptions to the rule: DO NOT PERFORM A BIOPSY OF AN ADRENAL MASS: There are only a few exceptions to this rule! One exception to perform an adrenal fine-needle-aspiration-biopsy is to distinguish adrenal tissue from metastatic tissue (cancer that spread from some other organ like the breast into the adrenal gland) and less commonly to determine if an adrenal mass is due to an infection. Even respected physicians such as endocrinologists or radiologists may erroneously send you for a biopsy. Unfortunately, we see this all the time, including at the most prestigious hospitals around the world. As a general rule, however, adrenal glands should not be biopsied!

You can review images from scans and pathology in our picture gallery of a number of rare tumors that are rare that arises from the adrenal gland. Some of these lesions require surgery whereas others can safely be left alone. You certainly want an experienced surgeon and team who have seen these cases before and know whether you would benefit from treatment and if so what kind of treatment.

Tumors that are Adjacent to the Adrenal

It is also very important to identify and distinguish adrenal tumors from the rare tumors that arise in the retroperitoneum (the space behind the abdomen) and next to the adrenal glands. These tumors on a scan (i.e. radiological imaging) can mimic an adrenal tumor. It is very important to distinguish these rare tumors from more common adrenal tumors since the treatment may be completely different. For instance, some of these tumors that are next to the adrenal gland do not require surgery, while others can be very agressive and cancerous. These rare tumors of the retroperitoneum that do not arise from the adrenal gland include retroperitoneal sarcoma, lymphoma and other type of tumors. If you have a tumor in this area of the body, you must get in your car, or on an airplane, and see an expert!

There are rare genetic susceptibility syndromes that can cause adrenal disease, such as rare genetic variants of causing Primary hyperaldosteronism, Cushing’s syndrome (Primary hypercortisolism), Pheochromocytoma and Paraganglioma and Adrenocortical cancer. Please review each of these entities in their own sections of this large website.