Large Adrenal Mass Research Articles

A Mass in Disguise: A Rare Case of High-Attenuation Adrenal Myelolipoma

Abstract Background: Adrenal incidentalomas are common, and imaging studies play a large role in reaching the diagnosis in many cases. Adrenal myelolipomas are classically diagnosed by CT as a hypodense well circumscribed heterogenous mass with low attenuation. Clinical Case: A 51-year-old man with history of resistant hypertension presented with neck pain after a motor vehicle crash. After a cervical X-ray revealed an odontoid fracture, a whole body CT was obtained. The CT incidentally identified a massive heterogeneous left adrenal mass measuring 22.3 × 15.5 × 20.6 cm, with multiple attenuation measurements ranging from 20 to 53 Hounsfield units (HU). The patient’s physical examination and hormonal assessment were both unrevealing, with normal catecholamine, androgen, and an unremarkable renin aldosterone ratio. Inpatient urinary cortisol levels were elevated, however a repeat urinary cortisol as an outpatient weeks after repair of the odontoid fracture showed normal levels, suggesting physiologic stress response. Positron emission tomography showed a metabolically inactive adrenal mass without metastatic diseases. The patient eventually underwent a left adrenalectomy, and pathology revealed a myelolipoma measuring 24.2 × 20.5 × 8.3 cm. Imaging characteristics for adrenal incidentalomas are considered useful diagnostic information according to most clinical guidelines. Lesions with high HU are typically pheochromocytomas, adrenocortical carcinoma, metastatic disease, or lipid poor adenomas. Myelolipomas are identified on CT by their characteristically low HU (often -30). In this case of a massive myelolipoma with high HU, the unique imaging characteristics posed a diagnostic challenge. The patient’s history of resistant hypertension combined with the high HU led to the patient undergoing extensive testing for a functional mass or cancer. Conclusion: This is a rare case of a massive myelolipoma with low attenuation. The role of imaging studies in the diagnosis of adrenal incidentalomas is not definitive, and detailed exam along with hormonal assessment may be warranted in atypical cases. Providers should consider myelolipoma in the differential diagnosis of a large adrenal mass with a negative hormonal evaluation even in the setting of high HU.

An Adrenal Myelolipoma With Hemorrhage

An 80-year-old man with a history of abdominal aortic aneurysm presented after a large left adrenal mass was found on surveillance ultrasonography. A subsequent abdominal and pelvic computed tomography (CT) scan revealed a 15-cm heterogeneous, round mass with a small internal fat component (Fig. 1A) with evidence of focal active extravasation of contrast within the mass consistent with active hemorrhage (Fig. 1B). On his CT 4 years prior, the adrenal lesion measured 4.9 cm and had areas of macroscopic fat and punctate calcifications consistent with a myelolipoma.

Adrenal tumors of different types with or without tumor thrombus invading the inferior vena cava: An evaluation of 33 cases

Background and objectivesAdrenal tumors with/out tumor thrombus (TT) in the inferior vena cava (IVC) pose a challenge to the surgeon due to the potential of massive hemorrhage and tumor thromboembolism. We report our experience in managing different types of adrenal tumors. MethodsFrom 11/1996–5/2015, 33 patients underwent resection of adrenal tumors with/without TT/IVC in 8 and 25 patients, respectively. Transplant-based (TB) techniques were utilized to resect the tumors. Intra-operative as estimated blood loss (EBL) and cardiopulmonary bypass (CPB) use; post-operative as length of hospital stay (LOS); and actuarial survival outcomes were recorded. ResultsMedian EBL was 200 cc (10-8,000), tumor size was 9.0 cm (4–25), and LOS was 7days (5–60). Adrenocortical carcinoma (ACC,11/33) was the commonest type. Three ACC/level IV TT/IVC underwent CPB to extract TT from the right atrium(n = 1), right atrium and right ventricle(n = 1), and right atrium and right pulmonary artery(n = 1), respectively. A complete resection of the adrenal tumors was achieved in all patients, and no deaths were observed in the immediate postoperative period. With a median follow-up of 60 (range: 18–120) months, 4/11 ACC patients have died of their disease. Actuarial survival for ACC patients at 60 months was 57.1 ± 16.4%. ConclusionsAn aggressive surgical approach is the only hope for curing large adrenal tumors with/without TT/IVC. TB techniques provide excellent exposure to the retroperitoneal space and safe removal of large adrenal masses.

Coincidence of Large Adrenal Cyst and Prominent Hyporeninemic Hyperaldosteronism

A 67-year-old Japanese woman who had end-stage renal disease was referred to our hospital for kidney transplantation. Abdominal CT revealed a large adrenal mass with inhomogeneity. She had a history of hospitalization for stroke and heart failure and exhibited prominent hyporeninemic hyperaldosteronism. Histological examination of the resected tumor with anti-CYP11B2 antibody indicated that she had a vascular endothelial cyst with primary aldosteronism (PA) due to multiple adrenocortical micronodules. This report implicates the pathological interaction between adrenal vascular cysts and PA-mediated vascular damage of the adrenal vein.

Is the fear of malignancy in large adrenal masses realistic?

Objectives: Adrenal masses are more frequently detected in autopsy series in recent years and are more frequently detected in clinical practice due to the development of radiological examinations. After the detection of an adrenal mass, the first two important questions come to mind. Does the mass hormonally active (functionally) or not active (non-functional), and this mass is a benign formation or is it malignant? The answer to these two questions is the obligatory questions that clinicians must answer in order to make an operation decision. The decision of operation in non-functioning adrenal masses is directly proportional to the mass's neoplastic potential. If a preoperative histopathological diagnosis is not available; this potential is predicted by the size of the mass in radiological imaging. It is shown that the malignancy rate in adrenal masses is higher in lesions 6 cm and above. In this study, we aimed to determine whether the rate of malignancy is really high in histopathological examination as a result of adrenalectomy operations performed in our clinic between the years of 2010-2012.Methods: Fourteen women and 4 men with 6 cm or higher adrenal masses patients which performed adrenalectomy by Eskişehir Osmangazi University Faculty of Medicine Department of General Surgery between 2010-2012 were included in this study. The results of the final histopathological analysis were classified retrospectively. Results: The rate of malignancy in adrenal masses of 6 cm or more supported by the literature was found to be high in our clinical series. Conclusions: As a result of our clinical retrospective study, we think that the extent of the formation in the related gland is highly effective and significant in making an operation decision before adrenalectomy.

Suspected Primary Adrenal Lymphoma (PAL) Associated With Hemophagocytic Lymphohistiocytosis (HLH).

Adrenal incidentalomas, masses noted on imaging performed for other purposes, are common, with 10% to 15% presenting as bilateral adrenal masses. These cases can be challenging as the differential diagnosis is broad, including metastatic disease, primary adrenal lymphoma (PAL), or infection, and often requiring a biopsy if initial biochemical workup is unrevealing. We present here a relevant case description, laboratory and radiologic imaging studies, and discussion of literature. A 62-year-old Korean woman presented with altered mental status and fevers. She was found to have bilateral adrenal incidentalomas and retained acupuncture needles. Adrenal workup did not show biochemical evidence of hormonal excess. Infectious workup was unrevealing, as was a metal/toxin workup due to retained acupuncture needles. Fevers and episodes of hypotension persisted which prevented the patient from obtaining an adrenal biopsy. Bone marrow biopsy was obtained for pancytopenia and revealed B-cell lymphoma with large cell morphology and few histiocytes with hemophagocytosis, raising concern for lymphoma-induced hemophagocytic lymphohistiocytosis (HLH). PAL associated with HLH was highly suspected in our patient, given the large (7 cm) bilateral adrenal masses and bone marrow biopsy findings of lymphoma. The patient was treated for diffuse large B-cell lymphoma, with clinical improvement. PAL is a rare but aggressive lymphoma with few reported cases. It should be considered in the differential for both unilateral and bilateral adrenal masses. An early diagnosis is crucial as the main treatment is chemotherapy rather than surgery and it confers a significant survival benefit.

Usage of Modified Makuuchi Incision for Surgical Management of Complex Renal and Adrenal Lesions.

Background: Modified Makuuchi (MM) incision is less popular among the urological fraternity as Chevron, subcostal, flank, and midline incisions are commonly used for most of the complex renal and adrenal conditions. We present our experience and report the outcomes of patients operated using this incision.Materials and methods: The records of patients who underwent open surgery for upper abdominal urological conditions using MM incision over the last five years in our department were retrospectively reviewed. Patient demographics, laterality of the lesion, size of the lesion, level of inferior vena caval (IVC) thrombus, intraoperative blood loss, local tumor invasion, need for concomitant hepatectomy, need of diaphragmatic resection, use of self-retaining retractors, operative time, hospital stay, wound-related complications, and readmissions were analyzed.Results: Some 18 patients underwent open surgery by this incision for various complex renal and adrenal conditions during the study period. Patients included those with large upper pole renal and adrenal masses, renovascular conditions like renal artery aneurysm, renal/adrenal masses with liver and diaphragmatic infiltration requiring hepatectomy, diaphragmatic resections, or IVC thrombectomy. The mean size of renal and adrenal masses was 13.8 (±6.3) cm, mean operative time was 370 (±210.6) minutes, mean blood loss was 1124 (±990.3) mL, and mean hospital stay was 11.65 (±13.2) days. Four patients had surgical site infection (SSI) and one had readmission.Conclusion: The MM incision can be widely adapted for complex renal and adrenal surgeries and should become a part of the various commonly used incisions by urologists.

Large incidental calcified adrenal mass.

Large incidental calcified adrenal mass.

SC192 - Study of feasibility and safety in trans-peritoneal laparoscopic adrenalectomy for large adrenal masses

SC192 - Study of feasibility and safety in trans-peritoneal laparoscopic adrenalectomy for large adrenal masses

Pheochromocytoma crisis with cyclic fluctuation in blood pressure mimics acute coronary syndrome.

SummaryPheochromocytoma crisis results from the sudden release of large quantities of catecholamines and leads to progressive multiple organ dysfunction. Here we report a case of pheochromocytoma crisis with symptoms associated with acute coronary syndrome (ACS) and severe fluctuations in blood pressure (BP). A 43-year-old Japanese man with hypertension (240/120 mmHg) visited a general hospital for chest pain. Echocardiogram showed ST segment depression and blood test demonstrated elevated troponin T. However, emergent coronary angiography revealed normal findings. CT showed a large adrenal mass on the left side, which was suspected as the cause of chest pain and BP elevation. After the patient was transported to our hospital, his BP was found to oscillate between 70 and 240 mmHg, and level of consciousness was decreased. After hospitalization, he had a further decrease in consciousness, a rise in body temperature, and a gradual increase in the interval between the upper and lower systolic BP. His systolic BP varied between 30 mmHg and 300 mmHg at the intervals of 20-30 min. After a multimodality therapy, including α-blocker and high dose fluid replacement, the fluctuation in his BP was gradually decreased and got stabilized after approximately 24 h. Approximately 3 weeks later, he underwent left adrenalectomy. This case showed that pheochromocytoma with internal necrosis might be misdiagnosed as ACS. Furthermore, in cases with a large adrenal tumor and severe elevation or fluctuations of BP, pheochromocytoma should be suspected and treated with α-blockers and fluid replacements as soon as possible prior to surgery.Learning points:High catecholamine levels due to pheochromocytoma crisis might cause symptoms associated with acute coronary syndrome.Adrenal tumor with internal necrosis and the elevation or fluctuations of blood pressure should be suspected to be pheochromocytoma.If pheochromocytoma crisis is suspected, the specialist, such as an endocrinologist or a urologist, should intervene, and an α-blocker treatment with adequate fluid replacement therapy should be initiated as soon as possible.Pheochromocytoma multisystem crisis (PMC) is a fatal condition characterized by multiple organ failure, severe blood pressure variability, high fever, and encephalopathy. This is an extremely rare subtype of a very rare disease such as pheochromocytoma. However, because the fatality rate of PMC is high, clinicians should be aware of the symptoms that mark its onset.

52-Year-Old Woman With Fever, Diaphoresis, and Abdominal Pain

52-Year-Old Woman With Fever, Diaphoresis, and Abdominal Pain

Large Adrenal Lesion Series in a Tertiary Care Center in Northern India: Diagnostic and Operative Challenges.

Large adrenal tumors pose varied challenges to surgeons in terms of diagnosis, planning surgical approach, and also intra-operative difficulties in resection. The aim of this study is to discuss challenges in the management of large and difficult adrenal lesions. A retrospective analysis was done on data of all patients with large adrenal lesions/paragangliomas managed from 1 June 2016 to 30 August 2018. Forty-eight patients with adrenal lesions underwent treatment in 2years duration. Pain in the abdomen was the most common presentation. Mean age was 34.4years (range 2-60), female to male (23:25) and right to left side to bilateral tumor 30:16:2. Thirty-one (64.6%) patients had large adrenal masses (size > 6cm). Surgical approaches included open transperitoneal adrenalectomy (n= 20) and laparoscopic transperitoneal (n = 9 and 2 others had conversion to open procedure). Challenges faced are described in the table below: [Table: see text] There were no major peri-operative morbidities, but two patients died in perioperative period. Large adrenal tumors pose a challenge in surgical planning, approach, and resection and need careful planning and multidisciplinary team approach to have the best outcomes.

SUN-179 Oncocytic Adrenocortical Carcinoma: A Case Report

Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Oncocytic variant of adrenocortical carcinoma is considered to be an even more rare malignancy with four reported in the literature in 2002 and thirty-six documented in 2015 i ii. This case report illustrates the case of oncocytic ACC.A 40-year-old woman post-hysterectomy from cervical cancer presented to the ER with severe left flank and back pain for two weeks. There was no weight loss or early satiety. CT scan revealed a large left adrenal mass measuring 12x9x13 cm, displacing the left kidney inferiorly with no metastasis to nearby structures. We ruled out functional adrenal mass through a 24-hour urine collection and blood work, then performed open left adrenalectomy.Grossly, it was a large, tan-colored mass, weighing approximately 2,500 grams. On biopsy, we incidentally found the mass to be oncocytic adrenocortical carcinoma. Using the Lin-Weiss-Bisceglia criteria, it met two major and one minor criteria, when only one major criterion is required to indicate malignancy. Immunohistochemical stains showed that the tumor cells were positive for Mart-1, inhibin, and synaptophysin. Quantitative Ki-67 showed a proliferation index of 10% in average (>5% is considered malignant). Even though adrenocortical carcinoma, specifically the oncocytic variant of ACC, is a very rare occurrence, it should be on the differential diagnosis for a chief complaint of unilateral flank pain with a large adrenal mass on CT due to its poor prognosis.Endnotes i Hoang, M., Ayala, A. & Albores-Saavedra, J. Oncocytic Adrenocortical Carcinoma: A Morphologic, Immunohistochemical and Ultrastructural Study of Four Cases. Mod Pathol 15, 973-978 (2002) doi:10.1038/modpathol.3880638 ii Kalra S, Manikandan R, Srinivas BH. Oncocytic adrenocortical carcinoma--a rare pathological variant. BMJ Case Rep. 2015;2015:bcr2014208818. Published 2015 Apr 24. doi:10.1136/bcr-2014-208818

SAT-221 50-Year-Old Fungus Suddenly Flourishes in Adrenal Glands: A Case of Sudden-Onset Bilateral Adrenal Masses Due to Latent Disseminated Histoplasmosis

Histoplasma capsulatum is endemic to Africa, Asia, Central and South America, and within the US, to the Ohio and Mississippi River Valley. Disseminated histoplasmosis is less commonly seen in immunocompetent individuals, who usually present with asymptomatic self-limited acute pneumonitis. Time to involvement of the adrenals is unknown. Adrenal insufficiency occurs in 45% of cases involving the adrenals, and is thought to be irreversible even in patients in remission.A 76-year-old man with no significant past medical history was incidentally found to have large bilateral adrenal masses during routine surveillance of a 7 mm pulmonary nodule on annual Chest CT, which showed normal adrenal glands the year prior. He was asymptomatic. A lifetime non-smoker native to California, whose only significant travel history was in his 20s to Ecuador and Puerto Rico, areas endemic to Histoplasma.Abdominal CT showed large bilateral adrenal masses with intermediate density and low washout values (right: 4.9 cm, HU 45, absolute washout 30%; left: 4.8 cm, HU 30, absolute washout 25%).On exam, vital signs were stable with normal orthostatics. Labs revealed normocytic anemia, normal chemistry panel, normal cortisol after 1-mg dexamethasone overnight test 2.6 mcg/dL (n<5 mcg/dL), plasma metanephrine <0.10 nmol/L (n <0.50 nmol/L), plasma normetanephrine 0.89 nmol/L (n <0.90 nmol/L), aldosterone 4.0 ng/dL (n <31 ng/dL), PRA 2.0 ng/ml/hr (n 0.5-4 ng/ml/hr) and random free cortisol 0.38 ug/dL (n 0.022-0.254ug/dL). HIV antigen and antibody, and Histoplasma urinary antigen were negative.Left adrenal mass biopsy revealed necrotizing granulomatous inflammation with fungal culture revealing budding yeast morphologically consistent with Histoplasmosis, with DNA probe confirming Histoplasma capsulatum.Treatment with itraconazole was initiated and the patient is tolerating the treatment well.To our knowledge, this is the first case demonstrating rapid development of large bilateral adrenal masses within a year due to latent disseminated histoplasmosis in an asymptomatic individual, which highlights the need for appropriate testing in patients with known exposure or travel history to endemic areas, regardless of time since exposure.1.Singh M, Chandy DD, Bharani T, Marak RSK, Yadav S, Dabadghao P, et al. Clinical outcomes and cortical reserve in adrenal histoplasmosis- a retrospective follow-up study of 40 patients. Clin Endocrinol 2019 Jan 17

SAT-207 Adrenocortical Carcinoma in Untreated Congenital Adrenal Hyperplasia

Background:Congenital adrenal hyperplasia (CAH) is a group of rare inherited autosomal recessive disorders characterized by a deficiency of various enzymes participating in steroid hormone synthesis. It occurs in 1 in 5000 to 1 in 15000 births. The most common (90-95%) cause of CAH is the absence of the enzyme 21-hydroxylase. We are presenting a case of a 34 year old male with untreated congenital adrenal hyperplasia due 21-hydroxylase deficiency and metastatic adrenocortical carcinoma.Case:A 34 year old male with a history of classic congenital adrenal hyperplasia (CAH) untreated since childhood presented with symptoms of right abdominal pain, anorexia and weight loss. His family history was significant for one brother diagnosed with CAH, father with liver and pancreatic cancer and another brother with colon cancer. After 2 weeks of treatment with NSAIDs for the pain, his abdominal pain worsened and patient also reported dyspnea with minimal exertion and dry cough. The patient was admitted to TUMC and his evaluation revealed a large right adrenal mass (17x11cm) with multiple liver and lung lesions suggestive of metastatic cancer. The adrenal mass was needle biopsied and pathology results were diagnostic of adrenal cortical carcinoma.His baseline morning cortisol level was 22.45 mcg/dL, ACTH 13.2 (n=7.2-63.3), androstenedione 291 ng/dL (n=27-152), 17-hydroxy progesterone 10,850 ng/dL (n=27-199), testosterone 140 mg/dL, renin 1.295 ng/ml/h (n=0.167-5.38), aldosterone unable to assay due to interference and LDH 2011 U/L (n=87-241). He failed an overnight 1mg dexamethasone suppression test with cortisol of 20.17 in the morning. During hospitalization, his clinical condition gradually deteriorated with hypotension, altered mental status, acute respiratory failure and acute liver failure with an AST 2787 units/L (n <39), ALT 399 (n=30-65) and ALP 1013 units/L (n=40-120). Oncology decided that the patient was a poor candidate for antineoplastic treatment therefore he was offered hospice care and eventually expired.Discussion:There have been reports of benign and malignant adrenal tumors in patients with CAH. It has been surmised that ACTH is the driver of adrenal tumor transformation in these patients. Our patient with adrenocortical carcinoma presented at a late stage with widespread metastases resulting in death. His elevated cortisol level occurred in association with low normal ACTH and the failed 1mg overnight dexamethasone suppression test are consistent with tumor production of cortisone. Considering that he had untreated CAH since childhood, we assume elevated ACTH levels were present until tumor transformation occurred.

Primary adrenal lymphoma: a case of hiccups.

Approximately 250 cases of primary adrenal lymphoma have been reported. We describe an unusual presentation of this condition. Our patient is a 65-year-old male whom presented to the emergency department with 4 days of severe persistent hiccups. He had lost 26 kg in weight in the preceding 4 months. Computed tomography abdomen revealed large invasive bilateral adrenal masses. Biochemical evaluation confirmed adrenal insufficiency. Hiccups resolved within 24 h of steroid replacement. Adrenal biopsy confirmed a diffuse large B-cell lymphoma. Hypotheses for the aetiology of his hiccups include diaphragmatic irritation and primary adrenal insufficiency. This case is interesting for its rarity involving bilateral adrenal glands, Addison’s disease from the primary adrenal tumour and rapid resolution of hiccups with corticosteroid therapy.

Hepatic hydatid cyst: a masquerader

Liver is the most common organ involved in echinococcosis. Organs affected by E granulosus are the liver (63%), lungs (25%) and muscles (5%). Rest of the organs are rarely affected. Adrenal cysts are uncommon. Their size may range widely and the origin of large adrenal cysts is often difficult to distinguish from other organs, including the kidney, pancreas, spleen, and liver. A large right-sided adrenal cystic mass can rarely be mistaken for a hepatic cyst by imaging. In this report, authors have described an adrenal cyst in a 28 year old lady, who was diagnosed preoperatively to have a hepatic hydatid cyst but intraoperatively it was found to be of adrenal origin. The size of the adrenal cyst can vary from a few millimetres up to 50 cm in diameter. Majority of the adrenal cysts are unilateral, while 8-10% of those cysts have been noted to be present bilaterally. The majority of cases are diagnosed between the 3rd and 6th decades. Although uncommon, Adrenal cyst should be considered as one of the differential diagnosis of upper abdominal cysts. Surgical excision is advisable when the cysts are symptomatic, greater than 5 cm in diameter and in the case of suspecting malignancy.

Case Report: 18F-FDG PET/CT in Nonfunctioning Adrenocortical Carcinoma Relapse

Case report of adrenocortical carcinoma recurrence after adrenalectomy and combined treatment.We report the case of a 46 year old man with silent adrenal mass. Imaging showed a large left adrenal mass with no evidence of distant metastasis. Complete surgical excision of the tumor was performed. After 5 years after surgical treatment, a local relapse of the disease was detected according to the results of CT, followed by PET/ CT with 18F-FDG — no evidence of distant metastases were detected. After complex treatment metastases were diagnosed by follow up PET/CT.This case report demonstrates possibilities of PET/CT with 18F-FDG in determination of adrenal mass malignancy and in cases of recurrent adrenocortical cancer, in cases of metastatic lesions.

Is Laparoscopic Adrenalectomy Safe in Large Adrenal Masses?

Is Laparoscopic Adrenalectomy Safe in Large Adrenal Masses?

Asymptomatic Unilateral Primary Adrenal Lymphoma Mimicking a NonFunctioning Adenoma in Adult Male with a Good Outcome

Primary adrenal lymphoma (PAL) is a type of extranodal lymphoma that is rarely encountered in clinical settings. It is regarded as a high-grade malignancy with a very poor prognosis. Most PALs are bilateral and hardly unilateral. Nevertheless, a rise in the number of cases of bilateral and unilateral PALs has been noted in the past few years, with a wide range of presenting symptoms from fatigue to adrenal insufficiency. Many were found incidentally via radiology imaging or on postmortem examinations. This paper aims to report an unusual case of a unilateral primary adrenal non-Hodgkin’s lymphoma in a previously healthy 53-yearold Bahraini male, who presented with vague yet persistent right flank pain of a few months’ duration. Imaging modalities revealed a large right adrenal mass measuring 11x10x9 cm which was initially thought to be an adenoma on CT scan, but post laparoscopic surgical excision was diagnosed histologically as Diffuse Large B Cell Lymphoma (DLBCL). Cycles of Rituximab-CHOP chemotherapy was given, and on regular follow up for two and a half years, the patient remains disease-free with no signs of recurrence.