Adrenal Myelolipoma Research Articles

Rare huge bilateral adrenal myelolipoma confused with retroperitoneal liposarcoma

IntroductionAdrenal myelolipomas are uncommon benign adrenal tumors, which mostly occur unilaterally. We describe a rare case of giant bilateral adrenal myelolipoma mistaken for retroperitoneal liposarcoma.Case presentationA 49‐year‐old man developed fever, left flank pain, and a large mass in his left abdomen. Plain computed tomography showed a 23‐cm mass with fat‐containing components in the retroperitoneal cavity, suggestive of retroperitoneal liposarcoma. A similar 9‐cm mass was observed in the right adrenal gland, suggesting adrenal metastasis. With a clinical diagnosis of retroperitoneal liposarcoma, we administered preoperative radiation therapy and performed a bilateral tumor resection. On postoperative day 9, a surgical site infection occurred, necessitating incisional drainage and the administration of intravenous antibiotics. The histopathological diagnosis was bilateral myelolipoma of the adrenal glands.ConclusionIn cases of bilateral retroperitoneal tumors with a large fat component, adrenal myelolipoma should be considered in the differential diagnosis to avoid overtreatment.

8571 Co existence of aldosterone excess in adrenal myelolipomas

Abstract Disclosure: I. Mishra: None. A. Baliarsinha: None. Co existence of aldosterone excess in adrenal myelolipomas Abstract: Adrenal myelolipomas are benign, lipomatous tumours, most of which present as adrenal incidentalomas.Co existence of cortisol excess states and aldosterone producing carcinomas is reported with adrenal myelolipomas,however isolated aldosterone excess without imaging features of adrenal malignancy is rare.We describe 3 cases presenting for evaluation of hyperaldosteronism but with imaging features consistent with myelolipomas. Case 1;58 year female presented with hypertension and spontaneous hypokalemia.On evaluation BMI was 25.3kg/m2,Gr 2 hypertensive retinopathy with systemic examination within normal limits.Biochemical evaluation revealed; Pl.Aldo(ng/dl)32.5; PRA(ng/ml/hr)0.54; ARR 60.5; SIT Pl.Aldo(ng/ml)27.8; AVS- not done; IMAGING(CT) Left adrenal fat attenuating 16×13mm lesion baseline HU < - 20 ?adrenal myelolipoma; Mx Left adrenalectomy H/P Adrenocortical hyperplasia;post op-resolution of hypokalemia with persistence of hypertension controlled with decreased number of drugs. Case 2;38 year male presented with resistant hypertension ,cerebro vascular accident,right hemiparesis and diuretic induced hypokalemia.On evaluation BMI was 31 kg/m2,Gr 1 hypertensive retinopathy with right hemiparesis.Biochemical evaluation revealed; Pl.Aldo(ng/dl)12.3; PRA(ng/ml/hr) 0.82; ARR 15; SIT Pl.Aldo(ng/ml)7.79; AVS- not done; IMAGING(CT) Left adrenal fat attenuating 28×30mmlesion baseline HU < -20? adrenal myelolipoma; Mx Left adrenalectomy H/P adrenal myelolipoma;post op-resolution of hypokalemia ,recovery of weakness and normalization of hypertension without drugs. Case 3;49 year male presented with hypertension ,incidental detection of adrenal lesion on ultrasound for pain abdomen and normokalemia.On evaluation BMI was 27 kg/m2,no significant findings on general examination with systemic examination within normal limits. Biochemical evaluation revealed; Pl.Aldo(ng/dl)12.2; PRA(ng/ml/hr) 0.58; ARR 20.2; SIT Pl.Aldo(ng/ml)11; AVS- not done; IMAGING(CT) Left adrenal fat attenuating 22x 26 mm lesion baseline HU < -20? adrenal myelolipoma; Mx drug therapy for hypertension due to refusal for surgical therapy with monitoring of blood pressure. In surgically treated adrenal myelolipomas, adrenal hormone excess is reported in 5-6% of cases, with autonomous cortisol secretion being most common .Even on the background of CT findings consistent with myelolipomas, patients fulfilling the screening and confirmatory criterias of primary hyperaldosteronism must be subjected to apt therapeutic modality.The presence of aldosterone clusters with excessive aldosterone synthase activity in these lesions could be reason for occurrence of primary hyperaldosteronism in myelolipomas. Presentation: 6/3/2024

6963 A Rare Case Presentation of Untreated Congenital Adrenal Hyperplasia with Bilateral Giant Myelolipoma

Abstract Disclosure: F. Omran: None. S. Bromley: None. M. Ilahi: None. M. Zena: None. W. Al-Refaie: None. J. Delto: None. A. Singh,: None. Background: Adrenal myelolipoma is a rare benign neoplasm predominantly composed of mature adipose tissue and intermixed myeloid tissue, often associated with conditions like Congenital Adrenal Hyperplasia. The etiology of this neoplasm is not definitively known although there are numerous hypotheses, including research suggesting that excess ACTH could be responsible for the pathogenesis. Clinical Case: A 40-year-old female with a history of congenital adrenal hyperplasia noncompliant with treatment. She presented with cholelithiasis (s/p laparoscopic cholecystectomy), abdominal imaging revealed incidental finding of a large right retroperitoneal mass. She was noted to have generalized excessive hirsutism in male distribution, small breasts, enlarged clitoris, male body habitus, and amenorrhea after stopping steroids years prior. Laboratory workup showed the following hormonal abnormalities: elevated ACTH (1080 pg/ml), elevated testosterone (243 ng/dl) and free testosterone (31.3 pg/ml), elevated androstenedione (23.431 ng/ml), and low LH (<0.02 miu/ml), low FSH (<0.02 miu/ml). CT abdomen showed right lobulated retroperitoneal mass of 4x14.3x20cm with internal fat and soft tissue components, left lobulated retroperitoneal mass of 13.7x4.7x7.5cm with soft tissue and fat components. CT guided-biopsy showed pathology consistent with adrenal myelolipoma: fatty areas with interspersed hematopoietic tissue components, peripheral rim of normal adrenal cortical tissue. Given the large size of the mass and compressive effects on surrounding organs, patient underwent exploratory laparotomy with resection of right retroperitoneal mass, nephrectomy, and adrenalectomy. Discharged on hydrocortisone and fludrocortisone, instructed to follow up by endocrinologist, always wear medical alert bracelet, and provided with hydrocortisone emergency kit. Conclusion: This case serves as a classic presentation of a rare disease state. Adrenal myelolipomas, a rare benign adrenal neoplasm, have been found in several disorders associated with chronic ACTH hypersecretion including congenital adrenal hyperplasia. Most CAH patients with myelolipomas have untreated or poorly treated CAH. Adrenal myelolipomas that are asymptomatic and <5cm in size are usually monitored if one is certain there is no occult malignancy. If these tumors are symptomatic or >7cm, patients should undergo surgical excision. These patients should be followed by endocrinology. Importantly, further guidelines should be further developed for management. Presentation: 6/3/2024

7446 Primary Adrenal Mature Teratoma Initially Diagnosed As An Adrenal Myelolipoma

Abstract Disclosure: A. White-Cotsmire: None. B. Kodali: None. A.M. Avram: None. S.S. Shah: None. Background: Teratomas are a type of germ cell tumor containing different types of tissue (e.g., hair, teeth, bone adipose, muscle). Most teratomas are benign (i.e., mature), but they may also be malignant (i.e., immature). Both immature and mature teratomas contain derivatives of ectoderm, endoderm, and mesoderm; however, immature teratomas are differentiated by the presence of embryonic, usually neuroectodermal, tissue. Mature teratomas have the potential for malignant transformation (1–2% of cases), usually squamous cell carcinoma within the teratoma. Extra-gonadal teratomas are rare (only 1–5%); reported locations include sacrococcygeal, head and neck, retroperitoneum, mediastinum, brain and spine, liver, and rarely adrenal glands. (1) Only 18 cases of adult primary adrenal teratomas have been reported since 1952. (2)Clinical Case: A 48-year-old woman with 4 days of worsening abdominal pain underwent CT imaging, which demonstrated a 6cm complex left adrenal mass containing peripheral and scattered internal-coarse calcifications with internal soft tissue and fat components, suggestive of adrenal myelolipoma with probable sequela of previous hemorrhage. Subsequent endocrine follow-up ruled out a cortisol secreting tumor with a negative dexamethasone suppression test and pheochromocytoma with normal serum free metanephrines and normetanephrines. Dedicated adrenal protocol CT scan further characterized the mass as a mature adrenal teratoma containing a rudimentary mandible and two teeth within the soft tissue associated with internal microscopic fat. The left renal vein was anteriorly displaced by this left adrenal mass and severely narrowed by the large adrenal mass, with development of left retroperitoneal collateral venous drainage from the left kidney into the lumbar venous system. The patient opted for conservative management with active clinical surveillance. Conclusion: This is a rare case of adrenal mature teratoma in an adult, originally diagnosed as an adrenal myelolipoma. This case highlights the use of more dedicated adrenal imaging for the characterization of adrenal tumors and the broad differential of benign non-functional adrenal tumors, including myelolipomas, lipomas, angiomyolipomas, and nonfunctional adenomas. However, a definitive diagnosis can only be confirmed by surgical removal of the tumor, usually through a laparoscopic approach, with histopathologic evaluation.

Adrenal Myelolipoma with Synchronous Ipsilateral Clear Cell Type Renal Cell Carcinoma: A Case Report with Review of Literature

Adrenal myelolipomas, typically benign and nonfunctional, are uncommon tumors found incidentally in the adrenal gland, unlike the more prevalent renal cell carcinoma in the kidney. The occurrence of synchronous renal cell carcinoma with adrenal myelolipomas is exceptionally rare, with only a few sporadic case reports documented in the literature spanning over six decades. Here, we present a case of a 67-year-old obese male who complained of colicky left upper abdominal pain, accompanied by a significant medical history of type 2 diabetes and hypertension. Ultrasonography revealed a large space-occupying lesion in the middle pole of the left kidney. Subsequently, left radical nephrectomy and adrenalectomy were performed, and the specimens were sent for histopathological examination, confirming the diagnosis of adrenal myelolipoma with synchronous renal cell carcinoma

7428 Clinical and Radiological Characterization and Outcomes of Lipid-Poor Adrenal Masses- A Multi-Center Retrospective Study

Abstract Disclosure: V. Balderrama-Brondani: None. R. Al-ward: None. K. Kiseljak-Vassiliades: None. L.M. Fishbein: None. D.A. Kirelik: None. O. Hamidi: None. R. Pishdad: None. H.K. Ghayee: None. M. Sukkari: None. S.S. Bedrose: None. A.H. Hamrahian: None. M.A. Habra: None. Background: Lipid-poor adrenal masses (LPAMs) with pre-contrast radiodensity >10 Hounsfield Units (HU) on computed tomography (CT) represent ∼25% of adrenal tumors. There is limited data about the natural history of LPAMs; therefore, we evaluated a large series of patients with LPAMs to describe the radiological and clinical features and outcomes of these cases. Methods: We conducted a multi-center retrospective cohort study of patients with LPAMs with a baseline diameter of 10-39 mm and derived from 6 institution members of the American-Australian-Asian Adrenal Alliance (A5). We excluded suspected or proven adrenal metastasis, pheochromocytoma, sarcoma, lymphoma, myelolipoma, infiltrative lesions, and adrenal hemorrhage, as well as patients with personal/familial history of genetic predisposition to adrenal neoplasms. The data is presented in percentage and median (range). Results: A total of 242 adrenal masses from 218 patients were evaluated, but only 225 (93%) masses met the inclusion criteria and were analyzed. The age was 58 (23-92), and 61% were women. LPAMs were incidentally discovered in 155 (71.1%) cases, 32 (14.7%) cases were diagnosed due to hormonal overproduction, and 17 (7.8%) due to pain/mass effect. Forty-six (21.1%) patients were on surveillance for a history of extra adrenal malignancy. Masses were unilateral in 89.9% (left side, 57.1%), with size 21 (10-38) mm, with a pre-contrast density of 24 (11-136) HU. Absolute washout was reported in 107 LPAMs and was >60% in 73 (68.2%) cases. The follow-up time was 44.4 (0.9-241) months. Hormonal overproduction developed during the follow-up in 23 (10.5%) cases as follows: cortisol in 15 (6.9%), aldosterone in 4 (1.8%), and sex hormones in 3 (1.4%) cases. LPAM growth rate was 0.03 (-2.18-11.59) cm/year. Seventy-one masses (31.5%) underwent diagnostic procedures (surgery/biopsy) with adrenocortical adenoma (n=37; 52.1%) and adrenocortical carcinoma (ACC) (n=25; 35.2%) being the two most common diagnoses. The tumor growth rate of pathologically confirmed adenomas was 0.03 (-0.30-10.50) cm/year, and of ACC was 1.31 (0.03-11.59) cm/year. A total of 154 (68.4%) LPAMs remained under surveillance at the end of the study, with a follow-up time of 51.8 (6-241) months and a tumor growth rate of 0.02 (-2.18-1.36) cm/year. Forty-five (20.6%) LPAMs decreased by 2 mm (1-13) during the study period. Cases that proved to be ACC had the most significant tumor growth rate over the study (p<0.001). The median time between the initial discovery of LPAM and the surgical resection of ACC cases was 34 (6-128) months. Conclusion: Our study showed that LPAMs carry significant malignant potential, and close long-term follow-up is warranted when surgical resection is not pursued. Prospective studies are critically needed to validate our findings and to establish high-risk molecular, imaging, and biochemical features associated with malignancy. Presentation: 6/2/2024

9294 Clinical and Radiological Characterization And Outcomes of Lipid-Poor Adrenal Masses- A Multi-Center Retrospective Study

Abstract Disclosure: V. Balderrama-Brondani: None. R. Al-ward: None. K. Kiseljak-Vassiliades: None. L.M. Fishbein: None. D.A. Kirelik: None. O. Hamidi: None. R. Pishdad: None. H.K. Ghayee: None. M. Sukkari: None. S.S. Bedrose: None. A.H. Hamrahian: None. M.A. Habra: None. Background: Lipid-poor adrenal masses (LPAMs) with pre-contrast radiodensity >10 Hounsfield Units (HU) on computed tomography (CT) represent ∼25% of adrenal tumors. There is limited data about the natural history of LPAMs; therefore, we evaluated a large series of patients with LPAMs to describe the radiological and clinical features and outcomes of these cases. Methods: We conducted a multi-center retrospective cohort study of patients with LPAMs with a baseline diameter of 10-39 mm and derived from 6 institution members of the American-Australian-Asian Adrenal Alliance (A5). We excluded suspected or proven adrenal metastasis, pheochromocytoma, sarcoma, lymphoma, myelolipoma, infiltrative lesions, and adrenal hemorrhage, as well as patients with personal/familial history of genetic predisposition to adrenal neoplasms. The data is presented in percentage and median (range). Results: A total of 242 adrenal masses from 218 patients were evaluated, but only 225 (93%) masses met the inclusion criteria and were analyzed. The age was 58 (23-92), and 61% were women. LPAMs were incidentally discovered in 155 (71.1%) cases, 32 (14.7%) cases were diagnosed due to hormonal overproduction, and 17 (7.8%) due to pain/mass effect. Forty-six (21.1%) patients were on surveillance for a history of extra adrenal malignancy. Masses were unilateral in 89.9% (left side, 57.1%), with size 21 (10-38) mm, with a pre-contrast density of 24 (11-136) HU. Absolute washout was reported in 107 LPAMs and was >60% in 73 (68.2%) cases. The follow-up time was 44.4 (0.9-241) months. Hormonal overproduction developed during the follow-up in 23 (10.5%) cases as follows: cortisol in 15 (6.9%), aldosterone in 4 (1.8%), and sex hormones in 3 (1.4%) cases. LPAM growth rate was 0.03 (-2.18-11.59) cm/year. Seventy-one masses (31.5%) underwent diagnostic procedures (surgery/biopsy) with adrenocortical adenoma (n=37; 52.1%) and adrenocortical carcinoma (ACC) (n=25; 35.2%) being the two most common diagnoses. The tumor growth rate of pathologically confirmed adenomas was 0.03 (-0.30-10.50) cm/year, and of ACC was 1.31 (0.03-11.59) cm/year. A total of 154 (68.4%) LPAMs remained under surveillance at the end of the study, with a follow-up time of 51.8 (6-241) months and a tumor growth rate of 0.02 (-2.18-1.36) cm/year. Forty-five (20.6%) LPAMs decreased by 2 mm (1-13) during the study period. Cases that proved to be ACC had the most significant tumor growth rate over the study (p<0.001). The median time between the initial discovery of LPAM and the surgical resection of ACC cases was 34 (6-128) months. Conclusion: Our study showed that LPAMs carry significant malignant potential, and close long-term follow-up is warranted when surgical resection is not pursued. Prospective studies are critically needed to validate our findings and to establish high-risk molecular, imaging, and biochemical features associated with malignancy. Presentation: 6/2/2024

5179 Uncommon Benign Mimics Of Suspicious Adrenal Nodules

Abstract Disclosure: L. Zhu: None. L. Loh: None. V. Gan: None. P. Kek: None. Introduction: Adrenal incidentaloma is often detected in imaging studies. In addition to biochemical testing, imaging plays a key role in guiding the management of such lesions. We describe two uncommon cases of benign lesions masquerading as suspicious adrenal nodules. Case 1. A 54-year-old man with hypertension, diabetes, dyslipidaemia, ischaemic heart disease and atrial fibrillation (on warfarin) underwent CT scan to exclude malignancy when he presented with thigh DVT, and was found to have a right 3.2cm adrenal nodule. Adrenal hormone profile was unremarkable. CT contrast characterization showed attenuation of 30, 35, and 32 HU on pre-contrast, venous and 10-minute delayed phase respectively. Given the minimal enhancement in high density areas, possible haemorrhage was considered and he was put on imaging surveillance. CT scans over the following 5 years showed stability of the lesion, resolution of high-density areas, and pre-contrast attenuation of -35HU, suggestive of myelolipoma. Further follow-up over the next 2 years then revealed tumour enlargement to 8.5cm, progressive predominance of dense areas (58 HU), and development of compressive symptoms. In view of mass effect, growing heterogeneity and possibility of a collision tumour, right adrenalectomy was performed. Histopathology revealed myelolipoma with extensive haemorrhage and no evidence of malignancy. Case 2. A 65-year-old woman had hypertension, ischaemic heart disease, dialysis-dependent end-stage renal disease secondary to glomerulonephritis, and oncological history of left renal cell carcinoma (RCC) and low-grade papillary urothelial carcinoma, both surgically resected 10 years ago. A new 1.6cm right adrenal nodule was seen on her surveillance CT scan for RCC. It was not present in a CT scan 7 years ago. The nodule displayed suspicious features such as nodular avid rim enhancement, persistent enhancement on delayed phase of contrast washout and central cystic change. Biochemical screening revealed plasma free metanephrine of 0.33 nmol/L (NR: <0.33) and plasma free normetanephrine of 0.99 nmol/L (NR: <0.71). Other adrenal hormone profiles were unremarkable. Cystic phaeochromocytoma was suspected and she underwent right adrenalectomy and nephrectomy following alpha blockade. Histopathology revealed a well-circumscribed nodule in the supra-renal adipose tissue, with vascular proliferation and features of anastomosing haemangioma. The right kidney was reported to have acquired cystic disease associated RCC. She was well post-operatively and remained on follow-up with the urologist for her RCC. Discussion: Haemorrhagic adrenal myelolipoma and supra-renal anatomosing haemangioma are benign lesions that may have CT characteristics similar to those of sinister lesions. Pre-operative diagnosis can be challenging. Surgical excision may be required to confirm diagnosis and exclude malignancy. Presentation: 6/3/2024

Management of patients with adrenal myelolipoma: experience from a tertiary referral centre.

Adrenal myelolipomas are rare, benign, tumours of the adrenal cortex. This study reports the experience of a tertiary adrenal surgery referral centre's approach to the management of patients with adrenal myelolipoma. A retrospective observational cohort study was conducted on all adult patients (> 18years age) diagnosed with adrenal myelolipoma from January 1, 2014, to December 30, 2022. Demographics, imaging characteristics, histological diagnosis (where applicable) and follow-up data were compared between patients undergoing surgery and those referred to surveillance. Indications for operative intervention were recorded at the time of multidisciplinary team discussion, consisting of surgeons, endocrinology physicians, radiologists, pathologists and specialist nursing representatives. Of the 522 patients with an adrenal lesion discussed in adrenal tumour meeting between 2014 and 2022, n = 15 (2.8%) were diagnosed with adrenal myelolipoma. Of the 15 patients, 4 underwent adrenalectomy at first presentation (27%), while 1 patient underwent adrenalectomy after interval follow-up. Indications for operative intervention were as follows: 'indeterminate lesion' (n = 3), 'abdominal pain and size (> 4cm)' (n = 1) and 'mass effect on adjacent organs' (n = 1). The mean rate of lesion growth in patients referred for surveillance (n = 10) was 0.13cm/year. Histology confirmed adrenal myelolipoma as the diagnosis in all resected tumours. For patients with adrenal myelolipoma, the presence of symptoms and/or indeterminate features on imaging may be more clinically useful indications for operative intervention over size alone. The surveillance of adrenal myelolipomas, even in patients with adrenal lesions > 4cm, is a safe clinical strategy, provided the imaging characteristics are benign and patients remain asymptomatic.

A case of adrenal myelolipoma: A rare encounter requiring specific approach in diagnosis and management

A case of adrenal myelolipoma: A rare encounter requiring specific approach in diagnosis and management

Large bilateral non-functional adrenal myelolipomas: A rare case report

Myelolipomas are rare, benign, and non-functional neuroendocrine tumors that are usually discovered incidentally. The widespread use of imaging modalities has increased the detection of incidental tumors over the last decades. Most myelolipomas are small, unilateral, and asymptomatic. Occasionally, tumors grow over time and become symptomatic due to the mass effect on adjacent structures. As a therapeutic approach, surgery is known as the standard treatment for symptomatic or large lesions. We report a patient presented with persisted abdominal pain which had been initiated two months ago. Ultrasonography examination showed large, bilateral, and well-defined hyperechoic lesions without calcification in both adrenal glands. Tumors were non-functional with hormone secretion-wise. After laparoscopic resection, on the macroscopic examination, two adrenal lesions with 60×50×40 mm and 35×30×10 mm size, respectively, were observed. The subsequent microscopic assessment also confirmed the diagnosis of bilateral adrenal myelolipomas.

Inducible CXCL12/CXCR4–dependent extramedullary hematopoietic niches in the adrenal gland

AbstractAdult hematopoietic stem and progenitor cells (HSPCs) reside in the bone marrow (BM) hematopoietic niche, which regulates HSPC quiescence, self-renewal, and commitment in a demand-adapted manner. Although the complex BM niche is responsible for adult hematopoiesis, evidence exists for simpler, albeit functional and more accessible, extramedullary hematopoietic niches. Inspired by the anecdotal description of retroperitoneal hematopoietic masses occurring at higher frequency upon hormonal dysregulation within the adrenal gland, we hypothesized that the adult adrenal gland could be induced into a hematopoietic-supportive environment in a systematic manner, thus revealing mechanisms underlying de novo niche formation in the adult. Here, we show that upon splenectomy and hormonal stimulation, the adult adrenal gland of mice can be induced to recruit and host functional HSPCs, capable of serial transplantation, and that this phenomenon is associated with de novo formation of platelet-derived growth factor receptor α/leptin receptor (PDGFRα+/LEPR+/–)–expressing stromal nodules. We further show in CXCL12–green fluorescent protein reporter mice that adrenal glands contain a stromal population reminiscent of the CXCL12-abundant reticular cells, which compose the BM HSPC niche. Mechanistically, HSPC homing to hormonally induced adrenal glands was found dependent on the CXCR4–CXCL12 axis. Mirroring our findings in mice, we found reticular CXCL12+ cells coexpressing master niche regulator FOXC1 in primary samples from human adrenal myelolipomas, a benign tumor composed of adipose and hematopoietic tissue. Our findings reignite long-standing questions regarding hormonal regulation of hematopoiesis and provide a novel model to facilitate the study of adult-specific inducible hematopoietic niches, which may pave the way to therapeutic applications.

Synchronous adrenal myelolipoma with renal cell carcinoma: A case report

Synchronous adrenal myelolipoma and Renal Cell Carcinoma (RCC) have been rarely reported in the literature. Cases occurring on the ipsilateral side are even rarer. We report a case of a 65-year-old male who presented with hematuria, lithuria, and suprapubic pain

Acromegaly and adrenal myelolipoma - Is there a connection?

Acromegaly and adrenal myelolipoma - Is there a connection?

Mature Cystic Teratoma of Left Adrenal Gland Diagnosed as Myelolipoma

A 32 year old woman who presented with fever, sore throat and abdominal pain that was evolving from last 7 months. A huge mass was palpable in left hypochondrium and left lumbar region. All baselines were within normal range. On ultrasonography, it appeared as well defined diffusely echogenic area in epigastric region likely arising from adrenal/pancreas, while CT scan findings reported a large heterogenous fat containing mass in left supra renal region. On the basis of CT findings, diagnosis of adrenal myelolipoma was made, exploratory laparotomy was done, the adrenal mass was excised and sent for histopathology. The biopsy report has shown that microsection of lesion were composed of benign squamous and pseudostratified epithelium, fat cartilage and glial tissue. Postoperative recovery of patient was uneventful.Keywords: extragonadal teratoma, retroperitoneal teratoma, adrenal myelolipoma, mature cystic teratoma of adrenal gland

Approach to the Patient With Bilateral Adrenal Masses.

Bilateral adrenal masses, increasingly encountered in clinical practice, manifest across diverse contexts, including incidental discovery, malignancy staging, and targeted imaging after hormonal diagnosis of adrenal disorders. The spectrum encompasses various pathologies, such as cortical adenomas, macronodular adrenal disease, pheochromocytomas, myelolipomas, infiltrative disorders, and primary and secondary malignancies. Notably, not all masses in both adrenal glands necessarily share the same etiology, often exhibiting diverse causes. Recently, the European Society of Endocrinology and the European Network for the Study of Adrenal Tumors updated guidelines, introduced a 4-option schema based on imaging, aiding in targeted hormonal testing and management. This "Approach to the Patient" review delves into the latest advancements in imaging, biochemical, and genetic approaches for the diagnostic and management nuances of bilateral adrenal masses. It provides insights and a contemporary framework for navigating the complexities associated with this clinical entity.

Evidence of the Role of Inflammation and the Hormonal Environment in the Pathogenesis of Adrenal Myelolipomas in Congenital Adrenal Hyperplasia.

Adrenal myelolipomas (AML) are composed of mature adipose and hematopoietic components. They represent approximately 3 percent of adrenal tumors and are commonly found in patients with congenital adrenal hyperplasia (CAH). CAH provides a unique environment to explore AML pathogenesis. We aimed to evaluate the role of the immune system and hormones that accumulate in poorly controlled CAH in the development of AML. When compared to normal adrenal tissue, CAH-affected adrenal tissue and myelolipomas showed an increased expression of inflammatory cells (CD68, IL2Rbeta), stem cells (CD117) B cells (IRF4), and adipogenic markers (aP2/FABP4, AdipoQ, PPARγ, Leptin, CideA), and immunostaining showed nodular lymphocytic accumulation. Immunohistochemistry staining revealed a higher density of inflammatory cells (CD20, CD3, CD68) in CAH compared to non-CAH myelolipomas. In vitro RNA-sequencing studies using NCI-H295R adrenocortical cells with exogenous exposure to ACTH, testosterone, and 17-hydroxyprogesterone hormones, showed the differential expression of genes involved in cell cycle progression, phosphorylation, and tumorigenesis. Migration of B-lymphocytes was initiated after the hormonal treatment of adrenocortical cells using the Boyden chamber chemotaxis assay, indicating a possible hormonal influence on triggering inflammation and the development of myelolipomas. These findings demonstrate the important role of inflammation and the hormonal milieu in the development of AML in CAH.

Adrenal Myelolipoma: Case Report

Introduction: Myelolipomas were first described in 1905 as an adrenal tumor composed of mature fat mixed with myeloid and erythroid cells. Adrenal myelolipomas are the second most common benign type of tumor in the adrenals, following after adrenocortical adenomas. It is mostly discovered incidentally on imaging of abdomen done for non-adrenal related reasons or at autopsy. Symptomatic tumors, growing tumors or tumors larger than 10 cm should be excised surgically. We report the case of 1 male patient presenting with flank pain and upper pole renal masse, treated surgically with adrenalectomy. Myelolipoma was confirmed on histology. Case presentation: a 46-years-old male, with a history of blood hypertension, presented with persistent abdominal discomfort and occasional pain in the right flank region. Physical examination revealed a non-tender palpable mass in the right upper quadrant of the abdomen. The abdominal CT scan revealed a large well-defined retroperitoneal tumor, which compressed and displaced the right kidney downward, the mass was heterogeneous with areas of soft-tissue attenuation. The patient underwent open left adrenalectomy. The pathological study confirmed the diagnosis of adrenal myelolipoma. The differential diagnosis of fat-containing retroperitoneal masses would include retroperitoneal lipoma or liposarcoma and renal angiomyolipoma. Discussion: Adrenal myelolipomas are the second most common adrenal incidentalomas comprising 6 to 16% of adrenal incidentalomas. The size of adrenal myelolipomas is variable and can range from a few millimeters to greater than 10 cm. These are called giant adrenal myelolipomas. The largest adrenal myelolipoma reported to date weighed 6 kg. Extra-adrenal sites for myelolipomas include the retroperitoneum, thorax, and pelvis. Usually unilateral however they can also involve both adrenals. The most common symptoms observed are abdominal discomfort/pain, hypochondrial pain, and flank pain. Ultrasonography (USG), ......

Bilateral giant adrenal myelolipoma associated with congenital adrenal hyperplasia

Adrenal myelolipomas are rare benign tumors, often non-functioning, located in the adrenal cortex, consisting mainly of mature adipose tissue and hematopoietic tissue. Although uncommon, the number of reported cases has increased due to the greater use of diagnostic imaging techniques. This tumor is usually unilateral and found as an adrenal incidentaloma, although there is a predominance of bilaterality in patients with congenital adrenal hyperplasia (CAH). In this study, we report the case of a 33-year-old male patient with CAH due to 21-hydroxylase deficiency, in non-regular use of the control medication, with bilateral giant adrenal myelolipoma and subsequent evolution of bilateral testicular adrenal rest tumors. He underwent bilateral adrenalectomy by video laparoscopy. The anatomopathological analysis, which confirmed myelolipomas’ diagnosis, revealed the right adrenal with 430 g and 12.5 x 9.3 cm and the left with 257 g and 11.5 x 10.4 cm. This tumor may be accompanied by adrenocortical adenoma and carcinoma, ganglioneuroma, pheochromocytoma, Addison’s disease, Cushing’s syndrome, or CAH.Among the hypotheses of its pathogenesis, we highlight an association between the development of adrenal myelolipoma and chronic hormonal stimulation by the adrenocorticotrophic hormone (ACTH), especially in CAH. The non-regular treatment of CAH with glucocorticoids may have contributed to the chronic and elevated secretion of ACTH and, consequently, to the development of bilateral giant adrenal myelolipoma.

Surgical management of giant adrenal myelolipoma using a modified Makuuchi incision: a case report

Giant adrenal myelolipomas are rare, benign, and hormonally inactive tumors. We present the case of a 53-year-old man with a 19-cm retroperitoneal mass, initially suspected to be a retroperitoneal liposarcoma, angiomyolipoma, or adrenal myelolipoma. After conducting endocrine assessments, which were within normal ranges, we decided to perform surgical excision using a modified Makuuchi incision. The tumor was successfully removed, and the final pathological examination confirmed the diagnosis of adrenal myelolipoma. The patient was discharged with no complications and remained without disease recurrence or distant metastasis as of 1 year postoperatively. In conclusion, giant myelolipomas are rare and cause symptoms owing to their large size. Surgical removal is recommended for large or symptomatic myelolipomas. The modified Makuuchi incision allows efficient and safe tumor removal in open surgery for giant myelolipomas.