Large Adrenal Myelolipoma Research Articles

SAT326 Massive Adrenomyelolipomas Due To Longstanding CAH

Abstract Disclosure: Q. Aziz: None. A. Champion: None. Introduction: Congenital Adrenal Hyperplasia (CAH) is an autosomal recessive disorder caused by deficiency in adrenal corticosteroid biosynthesis. 21 Hydroxylase Deficiency is common in 90-95% of CAH cases and have a defect in converting 17 OH Progesterone to 11 deoxycortisol. Therefore, they have an increase in ACTH secretion due to the decrease negative feedback drive along with excess adrenal androgens. Adrenal myelolipoma are common in patients with CAH and the tumors are benign and lipomatous. Clinical Case: Patient is a 47 year old female with past medical history of CAH presents to the clinic for evaluation of generalized fatigue and abdominal pain. She was diagnosed with CAH- 21 hydroxylase deficiency at birth and required vaginal reconstruction surgery. She was on hydrocortisone and fludrocortisone until age 18, but thereafter stopped taking medications or seeing physicians for nearly 30 years. CT abdomen/pelvis showed large bilateral adrenal myelolipomas, right adrenal gland measuring 23.2 cm and left adrenal gland measuring 20 cm. MRI confirmed enlarged adrenal glands. Initial labs included ACTH 299, 17-OH Progesterone >2000, 11 deoxycorticosterone 23.6, total testosterone 160, free testosterone 30.3, DHEA-S 192, Cortisol 2.8. She was started on fludrocortisone 50 mcg and Hydrocortisone 10 mg in the morning and 5 mg in the evening and was referred to an endocrine surgeon out of state, but surgery was not offered due to concerns of medication nonadherence. She returned to clinic 6 months after presentation, she admitted to missing multiple doses of her hydrocortisone. Repeat CT Abdomen and Pelvis showed severe compression of the IVC and right lobe of the liver along with mild compression of the kidneys and pancreas. To improve adherence, she was switched to prednisone, and eventually dexamethasone 0.75mg and fludrocortisone 100mcg. She complains of fatigue but improved abdominal pain. After a period of good adherence with medication administration, she was referred to a local surgical oncologist who agreed to do bilateral adrenalectomy due to mass effect. Conclusion: CAH is a challenging chronic disease with variable penetrance in phenotype, ranging from mild hirsutism to frank adrenal insufficiency. Massive adrenomyelolipomas are thought to be ACTH driven, especially seen in undertreated and longstanding CAH. Without the 21 hydroxylase enzyme to convert 17 OH Progesterone to 11 deoxycortisol, levels of ACTH rise. While these myelolipomas are benign and can generally be monitored, surgery should be considered if the size impairs function of surrounding organs. Bilateral adrenalectomy can also be considered in patients with CAH for refractory hypertension, hyperandrogenism and virilization, Cushing syndrome, infertility, or mass effect. Presentation: Saturday, June 17, 2023

A case of bilateral adrenal myelolipoma in congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency: A rare and uncommon finding

Abstract Congenital adrenal hyperplasia (CAH) is a common endocrine disorder that can lead to poor quality of life and the development of adrenal and extra-adrenal mass lesions when poorly controlled. Adrenal myelolipomas are rare, benign neoplasms that have been observed in long-standing, uncontrolled CAH cases. In this report, we describe a 31-year-old patient with ambiguous genitalia, hypokalemia, and normotension. The hormonal profile showed elevated 11-deoxy cortisol and 17-hydroxy progesterone. The clinical and biochemical profile was suggestive of CAH due to 11-beta-hydroxylase deficiency. On evaluation for pain abdomen, bilateral large adrenal myelolipomas were found for which surgery was done. Presently the index case is on steroid replacement therapy. This case emphasizes the importance of timely diagnosis adherence to treatment and appropriate follow-up of CAH cases in order to prevent the development of adrenal myelolipomas.

A large adrenal myelolipoma: case report and review of the literature

Abstract Adrenal myelolipoma is a rare benign neoplasm composed of mature adipose tissue and myeloid tissue with a variable amount of hematopoietic elements. Most patients are asymptomatic although some present with pain or even endocrine dysfunction. The rising use of CT and MRI scans has led to an increase of the detection of adrenal myelolipomas in recent years. The indications for surgery are symptomatic patients and lesions bigger than 5 cm or suspicious for malignancy. A case of a 50-year-old woman is presented here who was referred for surgical resection of a large nonfunctioning right adrenal mass. The neoplasm was resected through a midline laparotomy. Histopathology revealed a lesion consisting predominantly of fatty issue containing all types of hematopoietic stem cells and confirmed the diagnosis of myelolipoma.

Adrenal Myelolipoma Associated with Contralateral Secretory Adenoma: A Case Report

Adrenal myelolipomas are benign lipomatous tumors containing myeloid cell elements, most of which present as adrenal incidentalomas and account for 6% of all adrenal masses. Adrenal myelolipomas are usually unilateral in size, most often discovered in midlife, and affect both sexes almost equally. On imaging, adrenal myelolipomas have pathognomonic imaging features consistent with the presence of gross fat. Large adrenal myelolipomas may cause symptoms of mass effect and may occasionally be complicated by hemorrhage. If there is a concomitant adenoma or adrenal hyperplasia, excess adrenal hormones may be detected in patients with adrenal myelolipoma. We report the case of an adrenal myelolipoma associated with a contralateral secretory adenoma, responsible for Cushing syndrome in the patient.

Abstract #1185183: Large Bilateral Adrenal Myelolipoma in a patient with Congenital Adrenal Hyperplasia

Adrenal myelolipoma is a benign and biochemically non-functioning tumor, containing adrenal, adipose, and myeloid tissue. There are some reports of its association with endocrine disorders such as Congenital Adrenal Hyperplasia and Cushing's disease. They are usually smaller than 7 cm but rarely they can grow into giant size and cause compressive symptoms. Here, we present a case with development of giant myelolipoma in a patient with history of congenital adrenal hyperplasia.

Large Bilateral Adrenal Myelolipomas in the Setting of Congenital Adrenal Hyperplasia

Adrenal myelolipomas (AMLs) are rare benign adrenal tumors containing adipose and hematopoietic tissue, with a reported incidence of 0.08 to 0.4% on autopsy. AMLs are the second most common primary adrenal incidentaloma. Congenital adrenal hyperplasia (CAH) is associated with 10% of analyzed AML cases, half of which are bilateral in CAH patients.This is a 40-year old male with CAH diagnosed shortly after birth, due to 21-hydroxylase deficiency. He was doing well on a maintenance dose of hydrocortisone 20mg PO qAM and 10mg PO qPM and fludrocortisone 0.2mg PO daily until two years ago when he was incidentally found to have large bilateral AMLs while undergoing abdominal MRI and CT scans. These measured 6.6x3.6x7.7cm on the right (R) and 12.3x8.4x6.8cm on the left (L) at the time. He was asymptomatic, denying flank and abdominal pain. Follow up adrenal CT a year later revealed his AMLs increased in size to 8.7x4.2x6.6cm (R) and 13.9x6x8cm (L). Repeat CT another year later showed further rapid enlargement of his AMLs, measuring 11.1x6.1x7.9cm (R) and 17.1x7.8x10.8cm (L). He also exhibited a rising 17-hydroxyprogesterone level of 11,547ng/dL, despite an increased hydrocortisone dose (20mg BID). Although he remained asymptomatic, due to the precipitous growth of the masses and his increasing steroid requirement, a surgical approach was recommended. Open bilateral adrenalectomy was performed by an experienced endocrine surgeon and patient was discharged from the hospital with maintenance hydrocortisone and fludrocortisone therapy as well as strict sick day instructions.AMLs were first described in 1905 by Gierke. In the past, they were often discovered on autopsies, but more recently, due to the increase in imaging, have been incidentally diagnosed on more patients. Mostly, they occur unilaterally and are small (<4 cm) in size. Individuals with hormonal dysfunction such as those with Cushing’s Syndrome, Conn’s syndrome and CAH, particularly with difficult-to-control corticotropin levels, may be at a greater risk of developing AMLs concurrently, however this phenomenon is still not well understood. Small asymptomatic AMLs can be monitored with serial imaging over time. Spontaneous rupture of AMLs was found in 4.5% of cases, mostly occurring in tumors > 10cm, some resulting in retroperitoneal hemorrhage or even hemorrhagic shock. Although there is no clear consensus on surgery, development of symptoms or significant growth (to >10cm), as in our case, is a reason to pursue surgical evaluation especially in a young, otherwise relatively healthy adult.

Development of a large adrenal myelolipoma in the context of long-term elevated ACTH

Adrenal myelolipomas are the second most common benign tumours of the adrenal gland. Despite their frequent occurrence, there is uncertainty around their aetiology. We present the case of a giant...

Diagnosis and Management of a Large Adrenal Myelolipoma

AbstractAdrenal myelolipoma is a rare condition. Although benign in nature, these tumors can increase in size and cause a mass effect to nearby structures presenting as abdominal distension and pain. In 90% of cases, adrenal myelolipomas are unilateral. However, the real challenge in management is when bilateral adrenal myelolipoma is present. Herein, we present a case of a 51-year-old male Caucasian patient with bilateral adrenal myelolipoma. The patient had a large left adrenal myelolipoma (23 × 13 cm) and a small one on the right (4.4 × 4 cm). We opted for an open left adrenalectomy due to classical features on computed tomography scan and the mass effect of the tumor.

Laparoscopic excision of a large symptomatic and kidney-displacing adrenal myelolipoma: A case report

IntroductionAdrenal myelolipoma is a rare tumor. Traditionally, open surgical adrenalectomy was the standard treatment for symptomatic myelolipoma. However, laparoscopic excision is a promising alternative approach. ObservationA 38-year-old obese male patient presented with right loin pain of many months duration. Imaging studies revealed a large well-defined high fat content right adrenal mass displacing the right kidney. Adrenal tumor markers were within normal suggesting a non-functioning lesion. In spite of some technical difficulties and adjustments, laparoscopic excision was done, successfully, with uneventful recovery and short convalescence. Histopathological examination described mature adipose tissue with myeloid cells and confirmed the diagnosis of adrenal myelolipoma. ConclusionsLaparoscopic excision of large adrenal myelolipoma may indicate some technical adjustments, but it seems to be a feasible and advantageous approach even in obese patients.

Radiological and Pathological Findings in a Case of Large Left Adrenal Myelolipoma

Adrenal myelolipomas are rare and uncommon benign tumours of the adrenal gland. They contain mature adipose tissue and a variable amount of haemopoietic tissue. Most lesions are small, asymptomatic and discovered incidentally. However larger lesions may be symptomatic. Here we describe the imaging and pathological findings in a case of large left adrenal myelolipoma.

Hypertensive Emergency Following Embolization of a Large Adrenal Myelolipoma–Adrenal Medullary Infarction, Pheochromocytoma, or Acute Sympathetic Discharge? A Case Report and Literature Review

ABSTRACT Objective: To describe a case of hypertensive emergency following embolization of a large myelolipoma. Methods: Using the case report format to present clinical, laboratory, imaging, and dynamic testing data supporting clinical decision making. This is accompanied by a literature review. Results: A 40-year-old male with a history of hypertension presented with acute right upper abdominal pain and shortness of breath. Imaging completed at the transferring facility revealed a large right adrenal mass encapsulating the inferior vena cava. The blood hemoglobin was 7.8 g/dL because of internal hemorrhage into the mass. He underwent embolization of arterial flow to the right adrenal mass. A hypertensive emergency developed, and endocrinology was consulted to evaluate for pheochromocytoma. The plasma and urine normetanephrine were elevated, but the corresponding metanephrines remained normal. Surgery was imminent, and a clonidine suppression test in the surgical intensive care unit resulted in 60% suppr...

A 10-Year Single-Center Experience with Surgical Management of Adrenal Myelolipoma

The purpose is to report our 10-year experience with surgical management of large or symptomatic adrenal myelolipoma. Patients receiving surgical treatment for adrenal myelolipoma between December 2001 and September 2011 in our institution were retrospectively reviewed. Patients were divided into two groups: open surgery and laparoscopic surgery. Patient demographic data, lesion size evaluated by computed tomography scan or magnetic resonance imaging, operation time, blood loss, time of returning to diets, perioperative complications, and length of hospital stay were collected and analyzed. Forty patients (14 received open surgery and 26 received laparoscopic surgery) were enrolled in our study. Both procedures were successful and no patient in the retroperitoneal laparoscopic group required conversion to open surgery. The mean age of the patients was 52.7 years. The median size of the tumor was 5.0 cm. Forty-three percent of patients suffered from lumbago. There was no statistical difference in perioperative complications between the two groups (p>0.05). Retroperitoneal laparoscopic adrenalectomy patients had a shorter operation time (90.66±37.97 min vs 141.82±62.78 min, p=0.017), less blood loss (150, 100-200 mL vs 450, 300-525 mL, p=0.000), earlier time of returning to diets (2, 2-3 days vs 3, 2-4.5 days, p=0.036), and a shorter hospital stay (6, 5-7 days vs 10, 8-11.25 days, p=0.000) when compared with open surgery patients. Both open and laparoscopic surgeries are efficient and safe treatments for large or symptomatic adrenal myelolipoma, and retroperitoneal laparoscopic surgery has the advantages of minimal invasion and rapid postoperative recovery.

Retroperitoneal Laparoscopic Liposuction for Large Adrenal Myelolipomas: A Report of Nine Cases

Removing relatively large adrenal myelolipomas using retroperitoneal laparoscopy is difficult and carries risk of intraoperative injury to adjacent organs and vessels. We aimed to introduce a new method of retroperitoneal laparoscopic liposuction with suction units for resection of large adrenal myelolipomas. From June 2005 to October 2011, 8 patients (nine lesions, including bilateral lesions in 1 patient) with adrenal myelolipoma more than 8 cm in maximum diameter underwent retroperitoneal laparoscopic liposuction with suction units. Patients included 3 males and 5 females with a mean age of 47 years (range, 35-62 years). Tumor resection was performed after deflation and shrinkage of tumor. The mean maximum diameter was 10.5 cm, ranging from 8 to 14 cm. Five tumors were located on the right side, two were on the left side, and one was bilateral. Adrenal computed tomography was done in each patient preoperatively. Round or oval masses were found in dense fat in the adrenal area, and all cases were diagnosed as adrenal myelolipoma. Five patients had backache or abdominal discomfort; three patients had no symptoms. The mean operation time was 75 minutes, and the mean intraoperative blood loss was 30 mL. The mean postoperative length of stay was 3 days. No significant intraoperative or postoperative complications occurred. The follow-up length ranged from 8 to 77 months. No tumor recurrence was observed. Retroperitoneal laparoscopic liposuction with suction units is safe and effective for resection of relatively large adrenal myelolipomas. Deflation and shrinkage of the tumor make the operation safer and easier without affecting its pathological diagnosis.

Laparoscopic surgery for adrenal myelolipoma: Report of two cases

Adrenal myelolipoma, consisting of mature fat and hematopoietic tissue, is a rare, benign and biochemically inactive tumor. Most lesions are small and asymptomatic, discovered incidentally at autopsy or on imaging studies performed for other reasons. We report two cases of adrenal myelolipoma which were large and causing symptoms and successfully removed by laparoscopic lateral transabdominal adrenalectomy. We conclude that large and symptomatic adrenal myelolipomas can be treated with laparoscopic adrenalectomy.

Left-sided giant adrenal myelolipoma secreting catecholamine

Adrenal myelolipoma (AML) is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA), a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

Adrenal Myelolipoma And Membranoproliferative Glomerulonephritis: Case Report

Adrenal myelolipoma is a rare neoplasm typically <4 cm. It is usually discovered as an incidental finding and is asymptomatic. Currently, no known association exists between adrenal myelolipomas and membranous glomerulonephritis. This case demonstrates the coexistence of a large adrenal myelolipoma with a history of membranous glomerulonephritis (MGN) in the ipsilateral kidney.

Giant adrenal myelolipoma: Incidentaloma with a rare incidental association

Adrenal myelolipoma is an unusual, benign and biochemically inactive tumor that is composed of mature adipose and hematopoietic tissue. It is usually diagnosed accidentally and nowadays much more frequently because of widespread use of ultrasonography, computed tomography (CT) and magnetic resonance imaging. Adrenal myelolipoma is usually unilateral and asymptomatic, though known to be associated with obesity, hypertension, endocrinological disorders and some malignancies. We report herein two cases of right-sided giant adrenal myelolipoma diagnosed by multidetector-row CT. One patient was symptomatic because of a large mass in the right upper abdomen, which on imaging with CT was seen to be right adrenal myelolipoma. Another patient had a large left side Bochdalek hernia and right adrenal myelolipoma was incidentally discovered on CT.

Laparoscopic resection of a large adrenal myelolipoma: a case report

A 53 year man presented with pain right upper quadrant for seven months. On evaluation he was found to have a large right adrenal myelolipoma. Laparoscopically by transperitoneal right flank approach this was removed. Postoperative recovery was uneventful. Operative specimen's histopathological examination confirmed the diagnosis of adrenal myelolipoma.

Management of an incidentally found large adrenal myelolipoma: a case report

Adrenal myelolipoma is a rare benign neoplasm composed of mature adipose and hematopoietic tissue. Most lesions are small, unilateral and asymptomatic, discovered incidentally at autopsy or on imaging studies performed for other reasons. We would like to present a case report of this rare tumour. Cross-sectional imaging is helpful in making a pre-operative diagnosis. The size of the lesion should be a criterion for surgical intervention.

Large Adrenal Myelolipoma- an unusual occurrence

Fatty adrenal tumors are of rare occurrence and have been increasingly reported due to advanced imaging techniques. Although asymptomatic in majority of cases they should be excised to avoid complications like rupture or malignant transformation