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Cureus Feb 2024Adrenal myelolipoma is a rare, benign tumor of the adrenal gland, typically non-functional, asymptomatic and unilateral. With the increased use of radiological imaging,...
Adrenal myelolipoma is a rare, benign tumor of the adrenal gland, typically non-functional, asymptomatic and unilateral. With the increased use of radiological imaging, it has been discovered more frequently as incidental mass. It is common to occur concurrently with hormonal dysfunction conditions like congenital adrenal hyperplasia. However, there are few previous reported cases of malignancy concomitant with adrenal myelolipoma. We present a case of a 33-year-old patient diagnosed with congenital adrenal hyperplasia since birth. She was diagnosed with giant bilateral adrenal myelolipoma incidentally during the investigation done for staging her breast cancer. To the best of our knowledge, this is the second reported case of breast cancer concomitant with adrenal myelolipoma. Although this entity is very rare, physicians should be familiar with such rare adrenal masses and their associations in order to manage them appropriately.
PubMed: 38529452
DOI: 10.7759/cureus.54784 -
Journal of Surgical Case Reports Mar 2024Adrenal myelolipomas are rare, benign, nonfunctional tumors composed of mature adipose tissue and hematopoietic elements. Hemorrhage within an adrenal myelolipoma is an...
Adrenal myelolipomas are rare, benign, nonfunctional tumors composed of mature adipose tissue and hematopoietic elements. Hemorrhage within an adrenal myelolipoma is an uncommon occurrence, and when it happens, it can present with various clinical manifestations. Here, we report a case of a hemorrhagic giant adrenal myelolipoma in a 45-year-old female that was discovered incidentally. We discuss the clinical presentation, radiological findings, surgical intervention, and postoperative outcomes in this case report.
PubMed: 38524672
DOI: 10.1093/jscr/rjae169 -
International Journal of Molecular... Feb 2024Adrenal myelolipomas (AML) are composed of mature adipose and hematopoietic components. They represent approximately 3 percent of adrenal tumors and are commonly found...
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 (), stem cells () B cells (), and adipogenic markers (), 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.
Topics: Humans; Adrenal Hyperplasia, Congenital; Myelolipoma; Adrenal Gland Neoplasms; Lipoma; Leukemia, Myeloid, Acute
PubMed: 38473790
DOI: 10.3390/ijms25052543 -
International Journal of Surgery Case... Mar 2024Myelolipoma, a benign tumor characterized by mature fat cells and hematopoietic cells, is predominantly found in the adrenal glands, accounting for 6-16 % of all...
INTRODUCTION
Myelolipoma, a benign tumor characterized by mature fat cells and hematopoietic cells, is predominantly found in the adrenal glands, accounting for 6-16 % of all adrenal tumors. These tumors are often asymptomatic and discovered incidentally during imaging. We present a rare case of concurrent adrenal and extra-adrenal myelolipomas, contributing to the limited research in this area.
CASE PRESENTATION
A 65-year-old female with a history of Steven-Johnson syndrome presented with epigastric pain, initially diagnosed with emphysematous cholecystitis. Imaging revealed unexpected lesions near the left kidney. During surgery for presumed cholecystitis, significant hemorrhaging occurred following an attempted biopsy of the left adrenal lesion. This complication necessitated a complete adrenalectomy. Pathological examination confirmed the presence of myelolipomas in the left adrenal gland, para-aortic, and left para-iliac regions.
DISCUSSION
The simultaneous occurrence of adrenal and extra-adrenal myelolipomas is exceptionally rare, posing diagnostic and management challenges. This case highlights the complexity of managing patients with multiple comorbidities and the critical importance of differentiating myelolipomas from other fat-containing retroperitoneal masses. The incidental discovery of these tumors and their potential for significant intraoperative complications, as seen in our case, underscores the need for careful surgical planning and thorough preoperative assessment.
CONCLUSION
This case emphasizes the diagnostic challenges and management complexities in patients with incidental findings of myelolipoma, particularly when accompanied by significant medical histories. The occurrence of unexpected intraoperative complications highlights the importance of cautious decision-making in surgical interventions. This report provides valuable insights into the unpredictable nature of medical practice and the management of rare pathologies.
PubMed: 38430892
DOI: 10.1016/j.ijscr.2024.109398 -
Journal of Cancer Research and... Oct 2023A 57-year-old male had abdominal pain and distension for 6-7 months with a palpable swelling in the right lumbar region. Contrast-enhanced computed tomography abdomen...
A 57-year-old male had abdominal pain and distension for 6-7 months with a palpable swelling in the right lumbar region. Contrast-enhanced computed tomography abdomen showed a large heterogeneous lesion with fat density measuring 22 cm ´ 16.5 cm in the right suprarenal region. Laparotomy was done which showed an encapsulated mass measuring 21 cm ´ 14 cm ´ 5 cm. Cut section revealed yellowish areas admixed with hemorrhage and large areas of necrosis. Microscopy revealed adrenal myelolipoma. The patient was found to have sickling positive. High-performance liquid chromatography showed double heterozygous for HbS and beta-thalassemia trait. The association of giant adrenal myelolipoma with double heterozygous for HbS and beta-thalassemia trait is rare, and as clinically it simulates retroperitoneal sarcoma, awareness of this rare entity is critical for its accurate diagnosis and proper management.
Topics: Male; Humans; Middle Aged; beta-Thalassemia; Myelolipoma; Adrenal Gland Neoplasms; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms; Lipoma
PubMed: 38376325
DOI: 10.4103/jcrt.jcrt_2127_21 -
Urology Case Reports Mar 2024Adrenal myolipoma is a benign adrenal tumor which contains macroscopic amount of adipose tissue and usually hormonally inactive. However, functional adrenal myolipoma...
Adrenal myolipoma is a benign adrenal tumor which contains macroscopic amount of adipose tissue and usually hormonally inactive. However, functional adrenal myolipoma has also been reported in the literature. In this article, we present an interesting case of hormone secreting adrenal myelolipoma in an asymptomatic pregnant woman.
PubMed: 38362362
DOI: 10.1016/j.eucr.2024.102672 -
International Journal of Surgery Case... Jan 2024Adrenal myelolipomas are uncommon benign tumors of the adrenal gland. It has various other sites of origin.
Unusual discovery: Incidental diagnosis of adrenal myelolipoma, initially confused with liposarcoma, following bee sting bite, resolving chronic right hypochondrial discomfort - A case report.
INTRODUCTION AND IMPORTANCE
Adrenal myelolipomas are uncommon benign tumors of the adrenal gland. It has various other sites of origin.
CASE PRESENTATION
A 51-year-old male with elevated serum creatinine and an incidentally discovered adrenal mass underwent right adrenalectomy for an adrenal myelolipoma, a benign tumor with fat and haematopoietic components after bee string bite. As the patient was experiencing right hypochondrial pain surgery was planned. After surgery, he developed a surgical site infection, which was treated with antibiotics, and his wound healed well. Excision biopsy confirmed the non-malignant nature of the tumor.
CLINICAL DISCUSSION
Adrenal myelolipomas are often unilateral, non-functional, and diagnosed using imaging (CT/MRI). While radiological findings can be specific, inconclusive cases may require biopsy for confirmation. Surgical intervention is reserved for hormonally active, symptomatic, or rapidly growing myelolipomas, particularly if they exceed 5 cm.
CONCLUSION
Adrenal myelolipomas are common in adrenal glands, typically diagnosed in midlife with no gender bias. Imaging, especially CT, is effective in detecting fat components. In cases of diagnostic uncertainty, fine needle biopsy is crucial for confirmation, and surgery is considered when tumors are hormonally active, grow substantially, or cause symptoms, highlighting personalized care based on patient and imaging data.
PubMed: 38086131
DOI: 10.1016/j.ijscr.2023.109130 -
Cureus Oct 2023Adrenal myelolipomas (AMs) are rare and benign neoplasms, consisting of adipose and mature hematopoietic tissue. They are commonly discovered incidentally with increased...
Adrenal myelolipomas (AMs) are rare and benign neoplasms, consisting of adipose and mature hematopoietic tissue. They are commonly discovered incidentally with increased use of radiologic imaging. A small number of giant bilateral adrenal masses are reported, particularly in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 36-year-old male with a history of CAH on steroids since childhood, self-discontinued shortly after diagnosis, presenting mainly with abdominal distension and pain besides infertility. Imaging revealed giant bilateral adrenal masses. Subsequently, he underwent bilateral adrenalectomy, and the surgical pathology report revealed myelolipomas measuring 39×17×8 cm on the left and weighing 4050 grams and 28×16×5 cm on the right and weighing 1702 grams. AMs are found to coexist with many other conditions such as Cushing's syndrome, pheochromocytoma, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as a stimulator leading to myelolipomas. This case report highlights the proper history taking and biochemical evaluation for early detection and intervention to avoid catastrophic consequences.
PubMed: 38021675
DOI: 10.7759/cureus.47266 -
Biomedicines Nov 2023Our aim is to update the topic of adrenal tumours (ATs) in congenital adrenal hyperplasia (CAH) based on a multidisciplinary, clinical perspective via an endocrine... (Review)
Review
Our aim is to update the topic of adrenal tumours (ATs) in congenital adrenal hyperplasia (CAH) based on a multidisciplinary, clinical perspective via an endocrine approach. This narrative review is based on a PubMed search of full-length, English articles between January 2014 and July 2023. We included 52 original papers: 9 studies, 8 case series, and 35 single case reports. Firstly, we introduce a case-based analysis of 59 CAH-ATs cases with four types of enzymatic defects (CYP21A2, CYP17A1, CYP17B1, and HSD3B2). Secondarily, we analysed prevalence studies; their sample size varied from 53 to 26,000 individuals. AT prevalence among CAH was of 13.3-20%. CAH prevalence among individuals with previous imaging diagnosis of AT was of 0.3-3.6%. Overall, this 10-year, sample-based analysis represents one of the most complex studies in the area of CAH-ATs so far. These masses should be taken into consideration. They may reach impressive sizes of up to 30-40 cm, with compressive effects. Adrenalectomy was chosen based on an individual multidisciplinary decision. Many tumours are detected in subjects with a poor disease control, or they represent the first step toward CAH identification. We noted a left lateralization with a less clear pathogenic explanation. The most frequent tumour remains myelolipoma. The risk of adrenocortical carcinoma should not be overlooked. Noting the increasing prevalence of adrenal incidentalomas, CAH testing might be indicated to identify non-classical forms of CAH.
PubMed: 38002081
DOI: 10.3390/biomedicines11113081 -
JCEM Case Reports Mar 2023We report a case of a 58-year-old woman with a history of hypertension diagnosed at aged 35 years, on 5 antihypertensive agents and a history of intermittent spontaneous...
We report a case of a 58-year-old woman with a history of hypertension diagnosed at aged 35 years, on 5 antihypertensive agents and a history of intermittent spontaneous hypokalemia, was found to have a 6-cm left adrenal mass on computed tomography scan of the abdomen. The unenhanced computed tomography attenuation of the adrenal mass was -16 Hounsfield units (HU). The biochemical evaluation showed potassium of 2.8 mEq/L (SI unit, mmol/L) (reference range, 3.5-5.0), plasma aldosterone concentration of 61.3 ng/dL (SI unit, 1701 pmol/L) with plasma renin activity of 0.4 ng/mL/h (SI unit, μg/L/h). An overnight 1-mg dexamethasone suppression test showed nonsuppressible serum cortisol of 10.8 μg/dL (SI unit, 298 nmol/L). Dehydroepiandrosterone sulfate and ACTH were measured at 24.5 μg/dL (age-adjusted, 26-200) (SI unit, 0.66 μmol/L; 0.70-5.43) and <5 pg/mL (SI unit, < 1.1 pmol/L), respectively. Left adrenalectomy was performed and hydrocortisone therapy was initiated. Postoperatively and thereafter, her blood pressure was controlled with no antihypertensive agent. Seven months later, hydrocortisone therapy was stopped once her cortisol level had normalized. Pathology showed adrenal cortical neoplasm of uncertain malignant potential with associated lipomatous and myelolipomatous metaplasia. This is a rare case of aldosterone and cortisol co-secreting adrenal cortical neoplasm of uncertain malignant potential with lipomatous and myelolipomatous metaplasia. Although the majority of cases of myelolipoma are benign and nonfunctioning, this case emphasizes the importance of thorough hormonal and morphologic evaluation of the tumor.
PubMed: 37908480
DOI: 10.1210/jcemcr/luad012