Adrenal specimens are an infrequent but often challenging spectrum of tumors where the determination of malignant potential could be especially a challenge. This simplified diagnostic approach outlines comprehensive yet easy-to-follow guidelines.

.

Chapters

0:00 Introduction
0:51 Causes of Benign adrenal tumors
1:30 Symptoms of Benign adrenal tumors
2:20 Diagnosis of Benign adrenal tumors
2:42 Treatment of Benign adrenal tumors

An adrenal tumor or adrenal mass[2] is any benign or malignant neoplasms of the adrenal gland, several of which are notable for their tendency to overproduce endocrine hormones. Adrenal cancer is the presence of malignant adrenal tumors, and includes neuroblastoma, adrenocortical carcinoma and some adrenal pheochromocytomas. Most adrenal pheochromocytomas and all adrenocortical adenomas are benign tumors, which do not metastasize or invade nearby tissues, but may cause significant health problems by unbalancing hormones. Metastasis to one or both adrenal glands is the most common form of malignant adrenal lesion, and the second most common adrenal tumor after benign adenomas.[4] Primary tumors in such cases are most commonly from lung cancer (39%), breast cancer (35%), malignant melanoma, gastrointestinal tract cancer, pancreas cancer, and renal cancer.[4]
Tumors of the adrenal cortex

The adrenal cortex is composed of three distinct layers of endocrine cells which produce critical steroid hormones. These include the glucocorticoids which are critical for regulation of blood sugar and the immune system, as well as response to physiological stress, the mineralcorticoid aldosterone, which regulates blood pressure and kidney function, and certain sex hormones. Both benign and malignant tumors of the adrenal cortex may produce steroid hormones, with important clinical consequences.[citation needed]
Adrenocortical adenomas are benign tumors of the adrenal cortex which are extremely common (present in 1-10% of persons at autopsy). They should not be confused with adrenocortical "nodules", which are not true neoplasms. Adrenocortical adenomas are uncommon in patients younger than 30 years old, and have equal incidence in both sexes.[citation needed] The clinical significance of these neoplasms is twofold. First, they have been detected as incidental findings with increasing frequency in recent years, due to the increasing use of CT scans and magnetic resonance imaging in a variety of medical settings. This can result in expensive additional testing and invasive procedures to rule out the slight possibility of an early adrenocortical carcinoma. Second, a minority (about 15%) of adrenocortical adenomas are "functional", meaning that they produce glucocorticoids, mineralcorticoids, and/or sex steroids, resulting in endocrine disorders such as Cushing's syndrome, Conn's syndrome (hyperaldosteronism), virilization of females, or feminization of males. Functional adrenocortical adenomas are surgically curable.[citation needed]

Most of the adrenocortical adenomas are less than 2 cm in greatest dimension and less than 50 gram in weight. However, size and weight of the adrenal cortical tumors are no longer considered to be a reliable sign of benignity or malignancy. Grossly, adrenocortical adenomas are encapsulated, well-circumscribed, solitary tumors with solid, homogeneous yellow-cut surface. Necrosis and hemorrhage are rare findings.[citation needed]

Prof Alfredo Berruti talks to ecancer about his talk at ASCO GU 2022, First randomised trial on adjuvant mitotane in adrenocortical carcinoma patients: The Adjuvo study.

He begins by explaining that adrenocortical carcinoma is an extremely rare and aggressive disease with estimated incidents in western countries being around 0.72 per 1,000,000 per year. Even after surgery, patients are at high risk of relapse, so adjuvant therapy is recommended by international guidelines for patients with high-risk overlap. The ADIUVO study was undertaken to show whether mitotane administered in an adjuvant setting is efficacious in the subset of patients with low intermediate risk of relapse.

Prof Berruti then goes on to explain the most important finding of this study was that patients meeting the eligibility criteria of the trial depicted a relatively good prognosis with a relapse free survival (RFS) rate after 5 years of 75%. This was unexpected but is good news for the patients. In this patient subset mitotane therapy failed to demonstrate a significant advantage in terms of RFS or overall survival (OS) with respect to observation. However, the number of events were low, as only 19 relapses were observed during a followup of 48 months. 7 deaths were recorded.

He concludes by saying it’s important to continue followup of these patients because this is the first randomised clinical trial ever conducted in an adjuvant setting for this extremely rare disease.

Sign up to ecancer for free to receive tailored email alerts for more videos like this.
ecancer.org/account/register.php

Matthew Campbell, MD, The University of Texas MD Anderson Cancer Center, Houston, TX, provides an overview of the current treatment options in advanced adrenocortical carcinoma. As this form of cancer is relatively rare and no standard of care exists. Dr Campbell additionally highlights the potential role of immunotherapy, with several Phase II trials assessing pembrolizumab have shown promising results. However, Phase III studies are needed to accurately evaluate their efficacy. This interview took place at the European Society for Medical Oncology (ESMO) 2022 Congress in Paris, France.

Here is a short video infographic on Adrenocortical Cancer.
Brought to you by #CoMICs team:
Dwi Delson, 3rd-year Medical Student, University of Dundee, #UK
Meri Davitadze, Endocrinologist, Georgian-American Family Medicine Clinic "Medical House", Tbilisi, #Georgia
Nia Evans, Foundation Year 1 Doctor, Royal Glamorgan Hospital, #Wales
Punith Kempegowda, Clinical Research Fellow, Institute of Metabolism and Systems Research, University of Birmingham, #UK

The CoMIC was prepared in collaboration with Dr Vasileios Chortis, Clinical Lecturer in Endocrinology, University of Birmingham, #UK who has a special interest in Adrenocortical cancer and other adrenal pathologies.

The content of the CoMICs was critically reviewed by Professor Wiebke Arlt, William Withering Chair of Medicine, University of #Birmingham, #UK and
Prof. Dr. med. Martin Fassnacht, Head, Division of Endocrinology, University Hospital #Würzburg, #Germany. Both are world-leading experts in Adrenocortical cancer.

#birmingham #uniofbirmingham #IMSR #SIMBA #medicalstudents #medstudent #medicaleducation #futureleaders #endocrinology #adrenal #cancer #adrenocorticalcancer #ACC

The patient is a 69-year-old woman with an incidentally noted right adrenal tumor.

She had a CT scan back in September 2020 to evaluate a kidney cyst on the left side. The scan picked up on a nodularity on the right adrenal gland measuring 2.1 cm.

Fortunately, her doctors did not ignore her adrenal tumor. She was also sent to a medical endocrinologist who did a complete hormone work up. They made sure she did not have aldosterone-, cortisol-, or adrenaline- overproduction.

One year later they repeated the CT scan. Now the tumor had grown to 2.5 cm. On the CT scan there the tumor had an “atypical imaging phenotype”. This means that the tumor has some concerning features. The tumor was lipid-poor. This means that it does not have a lot of fat in it. This is a concerning sign.

At this point the patient was referred to me. When I reviewed all her scans, I was sufficiently concerned about this tumor that I recommended surgery. I recommended surgery even though the patient did not have any symptoms.

I review all the scans myself in great detail. I have looked at thousands of adrenal tumors on CT, PET and MRI scans. Her scan was concerning to me.

The patient underwent an uncomplicated right mini back scope adrenalectomy (MBSA). The operation lasted 21 minutes.

Two days later our specialist adrenal pathologist called me. Indeed, the suspicion I had about the scans was verified. The patient had an adrenocortical cancer (also known simple as adrenal cancer, or ACC).

Fortunately, because it was detected early it was still Stage 1. Importantly, all the margins were negative. This means the cancer was completely removed. It is likely that the patient will have a surgical cure and do very well.

We have already set up a team of doctors who will keep a very close eye on her for many years to come.

#adrenal #adrenalsurgery #carlingadrenalcenter #adrenalsurgeon #adrenaltumor #adrenalgland #adrenalcancer #adrenocorticalcancer #adrenalglands #tampabay