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1
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Jain V, Aggarwal A, Kulshreshtha B, Singh P, Goel H. A Rare Case of Catecholamine-Secreting Adrenal Myelolipoma. J ASEAN Fed Endocr Soc 2025; 40:104-107. [PMID: 40416490 PMCID: PMC12097973 DOI: 10.15605/jafes.040.01.15] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2024] [Accepted: 08/30/2024] [Indexed: 05/27/2025] Open
Abstract
Adrenal myelolipoma (AML) is a rare, benign, asymptomatic, nonfunctioning tumor of the adrenal cortex detected incidentally. AML can be accompanied by several other endocrine disorders simultaneously. Here, we report a case of a 36-year-old female with primary hypothyroidism and metabolic syndrome accompanied by severe hypertension and pheochromocytoma. However, the histopathological examination of the excised adrenal gland confirmed myelolipoma. Following surgery, her plasma nor-metanephrine levels decreased to normal values and the patient became normotensive, which suggested that the mass was functioning.
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Affiliation(s)
- Veenu Jain
- Department of Endocrinology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
| | - Anshita Aggarwal
- Department of Endocrinology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
| | - Bindu Kulshreshtha
- Department of Endocrinology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
| | - Preeti Singh
- Department of Pathology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
| | - Hemant Goel
- Department of Urology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, India
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2
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Ito I, Takehara K, Miyazaki Y, Tsuchiyama A, Mukae Y, Hayakawa K, Sakamoto I, Irie J, Watanabe J. Spontaneous hemorrhage in adrenal myelolipoma treated with elective laparoscopic adrenalectomy following selective arterial embolization. IJU Case Rep 2025; 8:114-117. [PMID: 40034912 PMCID: PMC11872213 DOI: 10.1002/iju5.12821] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2024] [Accepted: 12/05/2024] [Indexed: 03/05/2025] Open
Abstract
Introduction Adrenal myelolipoma is a benign adrenal tumor that is typically asymptomatic and is rarely associated with hemorrhage or rupture. Here, we present a case of adrenal myelolipoma with spontaneous hemorrhage. Case presentation A 72-year-old man with a history of obesity and hypertension visited the Department of Emergency Medicine with a sudden onset of severe left flank pain. Enhanced computed tomography showed a left adrenal tumor containing a fat component with a focus of contrast medium visualized extravasation. The patient was diagnosed with adrenal myelolipoma with spontaneous hemorrhage. Selective adrenal arterial embolization was performed to manage the severe pain, and the condition immediately improved. Four months later, laparoscopic left adrenalectomy was performed via a transperitoneal approach. Histopathological examination confirmed the diagnosis of adrenal myelolipoma. Conclusion Urgent transarterial embolization followed by elective laparoscopic adrenalectomy is a safe and minimally invasive treatment option for managing adrenal myelolipomas with hemorrhage.
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Affiliation(s)
- Itsuho Ito
- Department of UrologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Kosuke Takehara
- Department of UrologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Yuya Miyazaki
- Department of UrologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Ayaka Tsuchiyama
- Department of UrologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Yuta Mukae
- Department of UrologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Koichi Hayakawa
- Coordination Office for Emergency Medicine and International Response, Acute and Critical Care Center, Nagasaki University HospitalNagasakiJapan
- Department of Emergency MedicineNagasaki Harbor Medical CenterNagasakiJapan
| | - Ichiro Sakamoto
- Department of RadiologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Junji Irie
- Department of PathologyNagasaki Harbor Medical CenterNagasakiJapan
| | - Junichi Watanabe
- Department of UrologyNagasaki Harbor Medical CenterNagasakiJapan
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3
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Paul A, Toale C, Egan M, Whelan M, Feeney J, Crowther S, Gibney J, Conlon K. Management of patients with adrenal myelolipoma: experience from a tertiary referral centre. Ir J Med Sci 2024; 193:2941-2947. [PMID: 39230649 PMCID: PMC11666644 DOI: 10.1007/s11845-024-03779-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2024] [Accepted: 08/02/2024] [Indexed: 09/05/2024]
Abstract
BACKGROUND Adrenal myelolipomas are rare, benign, tumours of the adrenal cortex. AIMS This study reports the experience of a tertiary adrenal surgery referral centre's approach to the management of patients with adrenal myelolipoma. METHODS A retrospective observational cohort study was conducted on all adult patients (> 18 years 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. RESULTS 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 (> 4 cm)' (n = 1) and 'mass effect on adjacent organs' (n = 1). The mean rate of lesion growth in patients referred for surveillance (n = 10) was 0.13 cm/year. Histology confirmed adrenal myelolipoma as the diagnosis in all resected tumours. CONCLUSIONS 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 > 4 cm, is a safe clinical strategy, provided the imaging characteristics are benign and patients remain asymptomatic.
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Affiliation(s)
- Anant Paul
- Department of Surgery, Tallaght University Hospital, Dublin, Ireland
- School of Medicine, Trinity College Dublin, The University of Dublin, Dublin, Ireland
| | - Conor Toale
- Department of Surgery, Tallaght University Hospital, Dublin, Ireland.
- School of Medicine, Trinity College Dublin, The University of Dublin, Dublin, Ireland.
- Trinity Centre for Health Sciences at Tallaght University Hospital, Dublin 24, Dublin, Ireland.
| | - Marie Egan
- Department of Surgery, Tallaght University Hospital, Dublin, Ireland
| | - Maria Whelan
- Department of Surgery, Tallaght University Hospital, Dublin, Ireland
| | - John Feeney
- Department of Radiology, Tallaght University Hospital, Dublin, Ireland
| | - Stephen Crowther
- Department of Histopathology, Tallaght University Hospital, Dublin, Ireland
| | - James Gibney
- Department of Endocrinology, Tallaght University Hospital, Dublin, Ireland
- School of Medicine, Trinity College Dublin, The University of Dublin, Dublin, Ireland
| | - Kevin Conlon
- Department of Surgery, Tallaght University Hospital, Dublin, Ireland
- School of Medicine, Trinity College Dublin, The University of Dublin, Dublin, Ireland
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4
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Sweeney AT, Hamidi O, Dogra P, Athimulam S, Correa R, Blake MA, McKenzie T, Vaidya A, Pacak K, Hamrahian AH, Bancos I. Clinical Review: The Approach to the Evaluation and Management of Bilateral Adrenal Masses. Endocr Pract 2024; 30:987-1002. [PMID: 39103149 DOI: 10.1016/j.eprac.2024.06.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/07/2024] [Revised: 06/25/2024] [Accepted: 06/26/2024] [Indexed: 08/07/2024]
Abstract
OBJECTIVE This white paper provides practical guidance for clinicians encountering bilateral adrenal masses. METHODS A case-based approach to the evaluation and management of bilateral adrenal masses. Specific clinical scenarios presented here include cases of bilateral adrenal adenomas, hemorrhage, pheochromocytomas, metastatic disease, myelolipomas, as well as primary bilateral macronodular adrenal hyperplasia. RESULTS Bilateral adrenal masses represent approximately 10% to 20% of incidentally discovered adrenal masses. The general approach to the evaluation and management of bilateral adrenal masses follows the same protocol as the evaluation of unilateral adrenal masses, determined based on the patient's clinical history and examination as well as the imaging characteristics of each lesion, whether the lesions could represent a malignancy, demonstrate hormone excess, or possibly represent a familial syndrome. Furthermore, there are features unique to bilateral adrenal masses that must be considered, including the differential diagnosis, the evaluation, and the management depending on the etiology. Therefore, considerations for the optimal imaging modality, treatment (medical vs surgical therapy), and surveillance are included. These recommendations were developed through careful examination of existing published studies as well as expert clinical opinion consensus. CONCLUSION The evaluation and management of bilateral adrenal masses require a comprehensive systematic approach which includes the assessment and interpretation of the patient's clinical history, physical examination, dynamic hormone evaluation, and imaging modalities to determine the key radiographic features of each adrenal nodule. In addition, familial syndromes should be considered. Any final treatment options and approaches should always be considered individually.
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Affiliation(s)
- Ann T Sweeney
- Division of Endocrinology, Department of Medicine, St Elizabeth's Medical Center, Brighton, Massachusetts.
| | - Oksana Hamidi
- Division of Endocrinology and Metabolism, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Prerna Dogra
- Division of Endocrinology, Diabetes and Metabolism, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin
| | - Shobana Athimulam
- Division of Endocrinology, Diabetes, Bone and Mineral Disorders, Henry Ford Health, Detroit, Michigan
| | - Ricardo Correa
- Division of Endocrinology, Cleveland Clinic, Cleveland, Ohio
| | - Michael A Blake
- Division of Abdominal Imaging, Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts
| | - Travis McKenzie
- Division of Endocrine Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota
| | - Anand Vaidya
- Center for Adrenal Disorders, Division of Endocrinology, Diabetes, Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Karel Pacak
- Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland
| | - Amir H Hamrahian
- Division of Endocrinology, Diabetes, and Metabolism, Johns Hopkins University, Baltimore, Maryland
| | - Irina Bancos
- Division of Endocrinology, Joint appointment Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
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5
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Kethireddy M, Lee T, Rodrigues M, Munir I, Kim DI. A Rare Case of Giant Bilateral Adrenal Myelolipomas in a Patient With Classical Congenital Hyperplasia. Cureus 2024; 16:e56953. [PMID: 38665713 PMCID: PMC11044845 DOI: 10.7759/cureus.56953] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/26/2024] [Indexed: 04/28/2024] Open
Abstract
Congenital adrenal hyperplasia (CAH) is caused by genetic defects in the enzymes involved in cortisol biosynthesis in the adrenal gland and, in more than 90% of cases, due to a deficiency in the 21-hydroxylase enzyme. Classical CAH due to 21-hydroxylase deficiency is a severe form of the disease that presents with cortisol deficiency and is further categorized into salt-wasting or simple-virilizing types. Appropriate steroid replacement has been shown to effectively treat patients with classical CAH and prevent complications. Individuals who receive inadequate treatment or fail to comply with their prescribed steroid hormone regimen are susceptible to the development of adrenal myelolipomas. Myelolipomas are benign tumors composed of both adipose and hematopoietic tissues. While documented cases of adrenal myelolipomas exist in medical literature, instances of large bilateral myelolipomas remain exceedingly rare. This case report highlights a 40-year-old female patient with a known history of classical congenital adrenal hyperplasia who presented with unusually large bilateral adrenal myelolipomas. A diagnostic CT scan of the abdomen and pelvis revealed a 13.4 x 10.8 cm myelolipoma on the left adrenal gland and a 10 x 8.6 cm myelolipoma on the right adrenal gland. Prior to her presentation, the patient experienced recurrent nausea and vomiting, along with left upper quadrant pain, over five months. Hormonal assessments indicated significantly elevated serum androgen levels, suggesting inadequate management of her CAH. In this report, we present a rare case of symptomatic bilateral large adrenal myelolipomas, underscoring the significance of adhering to treatment regimens, diagnostic assessments, and management for adrenal myelolipomas in individuals diagnosed with CAH.
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Affiliation(s)
- Meghana Kethireddy
- Internal Medicine, Rocky Vista University College of Osteopathic Medicine, Saint George, USA
| | - Taejun Lee
- Internal Medicine, Loma Linda University School of Medicine, Loma Linda, USA
| | - Medora Rodrigues
- Internal Medicine, Loma Linda University Medical Center, Loma Linda, USA
| | - Iqbal Munir
- Endocrinology, Riverside University Health System Medical Center, Moreno Valley, USA
| | - Daniel I Kim
- Internal Medicine, Riverside University Health System Medical Center, Moreno Valley, USA
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6
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Basiri A, Torabi A, Rabani S. A hormone secreting adrenal myolipoma in an asymptomatic woman. Urol Case Rep 2024; 53:102672. [PMID: 38362362 PMCID: PMC10867577 DOI: 10.1016/j.eucr.2024.102672] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2024] [Revised: 01/28/2024] [Accepted: 01/30/2024] [Indexed: 02/17/2024] Open
Abstract
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.
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Affiliation(s)
- Abbas Basiri
- Urology and Nephrology Research Center (UNRC), Shahid Labbafinejad Hospital, Shahid Beheshti University of Medical Sciences (SBMU), Tehran, Iran
| | - Ala Torabi
- Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, Shariati Hospital, AAL Ahmad Highway, Tehran, Iran
| | - Seyedhossein Rabani
- Urology and Nephrology Research Center (UNRC), Shahid Labbafinejad Hospital, Shahid Beheshti University of Medical Sciences (SBMU), Tehran, Iran
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7
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Alhassan L, Nasser H, Ali MA, Sagher HG, Al-janabi MH. Hemorrhagic giant adrenal myelolipoma discovered incidentally: a case report. J Surg Case Rep 2024; 2024:rjae169. [PMID: 38524672 PMCID: PMC10960941 DOI: 10.1093/jscr/rjae169] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2024] [Revised: 02/17/2024] [Accepted: 02/29/2024] [Indexed: 03/26/2024] Open
Abstract
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.
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Affiliation(s)
- Luma Alhassan
- Department of Nephrology, Tishreen University Hospital, Lattakia, Syria
| | - Hassan Nasser
- Department of Urologic Surgery, Tishreen University Hospital, Lattakia, Syria
| | - Mohammed A Ali
- Department of Radiology, Tishreen University Hospital, Lattakia, Syria
| | | | - Moatasem H Al-janabi
- Department of Pathology, Cancer Research Center, Tishreen University Hospital, Lattakia, Syria
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8
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Kolli V, Frucci E, da Cunha IW, Iben JR, Kim SA, Mallappa A, Li T, Faucz FR, Kebebew E, Nilubol N, Quezado MM, Merke DP. Evidence of the Role of Inflammation and the Hormonal Environment in the Pathogenesis of Adrenal Myelolipomas in Congenital Adrenal Hyperplasia. Int J Mol Sci 2024; 25:2543. [PMID: 38473790 DOI: 10.3390/ijms25052543] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2024] [Revised: 02/16/2024] [Accepted: 02/19/2024] [Indexed: 03/14/2024] Open
Abstract
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.
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Affiliation(s)
- Vipula Kolli
- National Institutes of Health Clinical Center, 10 Center Drive, Bethesda, MD 20892, USA
| | - Emily Frucci
- National Institutes of Health Clinical Center, 10 Center Drive, Bethesda, MD 20892, USA
| | - Isabela Werneck da Cunha
- Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA
- D'Or Institute for Research and Education (IDOR), São Paulo 05403, Brazil
| | - James R Iben
- The Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD 20892, USA
| | - Sun A Kim
- Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA
| | - Ashwini Mallappa
- National Institutes of Health Clinical Center, 10 Center Drive, Bethesda, MD 20892, USA
| | - Tianwei Li
- The Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD 20892, USA
| | - Fabio Rueda Faucz
- The Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD 20892, USA
| | - Electron Kebebew
- Division of General Surgery, Department of Surgery, Stanford University School of Medicine, Palo Alto, CA 94304, USA
| | | | - Martha M Quezado
- Laboratory of Pathology, National Cancer Institute, Bethesda, MD 20892, USA
| | - Deborah P Merke
- National Institutes of Health Clinical Center, 10 Center Drive, Bethesda, MD 20892, USA
- The Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD 20892, USA
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9
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Balagobi B, Gobishangar S, Theepan JMM, Priyatharsan K, Heerthikan K, Vaishnavi T. Unusual discovery: Incidental diagnosis of adrenal myelolipoma, initially confused with liposarcoma, following bee sting bite, resolving chronic right hypochondrial discomfort - A case report. Int J Surg Case Rep 2024; 114:109130. [PMID: 38086131 PMCID: PMC10758857 DOI: 10.1016/j.ijscr.2023.109130] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2023] [Revised: 12/04/2023] [Accepted: 12/05/2023] [Indexed: 01/05/2024] Open
Abstract
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.
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Affiliation(s)
- Balasingam Balagobi
- Department of Surgery, Faculty of Medicine, University of Jaffna, Sri Lanka.
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10
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Duarte Regalado CS, Guzmán Mejía JI, Gutiérrez Uvalle GE, Vargas Rodríguez AE, González Ledo J. A Case Report and Literature Review of Adrenal Myelolipoma. Cureus 2023; 15:e43240. [PMID: 37692624 PMCID: PMC10491497 DOI: 10.7759/cureus.43240] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/26/2023] [Indexed: 09/12/2023] Open
Abstract
Adrenal myelolipoma is considered a benign neoplasm that accounts for 6% to 16% of adrenal incidentalomas, and it is the second most common incidental adrenal tumor after adrenal adenomas. They are usually asymptomatic; however, in the presence of symptoms, significant growth, or complications, open surgical resection is indicated. We present the case of a 46-year-old woman with obesity and diabetes who experienced five years of left hemiabdominal pain, which was unsuccessfully treated symptomatically. A computed tomography scan revealed findings suggestive of pancreatic lipoma and a suggestive image of left adrenal myelolipoma. Resection of the tumor was performed using an anterior midline approach, and histopathological examination confirmed left adrenal myelolipoma. The presented case represents the typical presentation of these tumors in a patient in the fifth decade of life with obesity, diabetes, and nonspecific abdominal pain possibly related to the size of the lesion found. Surgical intervention was indicated due to the presence of symptoms, lesion size, contiguity with abdominal organs, and the absence of a precise diagnosis. An anterior midline approach was chosen, and histopathological examination provided a definitive diagnosis. Adrenal myelolipoma is a rare entity that is often asymptomatic and incidentally diagnosed through imaging studies. However, they should be resected when symptomatic to prevent complications. Open surgical resection is the preferred approach.
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11
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Ahmed DH, Bapir R, Radha BMM, Aghaways I, Rashid RJ, Ismaeil DA, Ghalib Hawramy OH, Gharib DT, Kaka Ali HH, Kakamad FH, Abdalla BA, Abdullah AM. Laparoscopic removal of a giant adrenal myelolipoma: A case report with review of the literature. INTERNATIONAL JOURNAL OF SURGERY OPEN 2023; 57:100658. [DOI: 10.1016/j.ijso.2023.100658] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
Abstract
Abstract
Introduction
Adrenal myelolipomas (AMLs) are non-functioning, benign adrenal tumors with an unknown etiology. This study aims to report a case of giant AML managed successfully by laparoscopic surgery.
Case presentation
A 43-year-old man presented with mild, dull, and non-radiating left loin pain. Physical examination and hormonal assays were normal. Imaging revealed a retroperitoneal mass (13 × 12 × 8.5cm) containing gross fat. Left laparoscopic adrenalectomy was done under general anesthesia. The postoperative period was uneventful. The patient was followed up for a duration of six months, and no recurrence was detected.
Discussion
AMLs are lipomatous tumor-like growths equally seen in both genders and are commonly incidental findings. Symptomatic AMLs are characterized by their large size, leading to the manifestation of pressure-related symptoms or abnormalities in hormonal levels. Most AMLs are associated with chronic diseases like diabetes mellitus and hypertension. The tumor is commonly diagnosed through a biochemical and imaging workup. Asymptomatic AMLs are managed conservatively, while in the presence of symptoms, surgery is usually considered. However, the open approach is regarded as the standard option, but minimally invasive approaches can be carried out due to their feasibility.
Conclusion
AMLs are rare, benign, non-functional tumors of the adrenal gland. Laparoscopy may be a safe and feasible management modality for these tumors.
Highlights
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12
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Inoue T, Todaka M, Nakazono Y, Fukata Y, Shin T. A case of adrenal myelolipoma complicated with Prader-Willi syndrome. IJU Case Rep 2023; 6:235-238. [PMID: 37405031 PMCID: PMC10315240 DOI: 10.1002/iju5.12595] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2022] [Accepted: 04/22/2023] [Indexed: 07/06/2023] Open
Abstract
Introduction Prader-Willi syndrome is a congenital disorder that occurs in one in 10 000-30 000 children and is characterized by obesity, short stature, and intellectual disability. Case presentation A 24-year-old male patient with Prader-Willi syndrome presented with an enlarged adrenal tumor. Computed tomography detected a well-defined mass. Magnetic resonance imaging revealed an increased signal intensity predominantly in fatty areas, suggesting adrenal myelolipoma. Laparoscopic left adrenalectomy was performed. Postoperatively, the patient developed mild pulmonary atelectasis, myelolipoma was confirmed by histopathology, and there was no recurrence at approximately 2 years postoperatively. Conclusion This is the first report of Prader-Willi syndrome complicated with adrenal myelolipoma, which was removed laparoscopically.
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Affiliation(s)
- Toru Inoue
- Department of Urology, Faculty of MedicineOita UniversityYufu‐CityOitaJapan
- Department of Urology, Beppu Medical CenterNational Hospital OrganizationBeppuOitaJapan
| | - Masahiro Todaka
- Department of Urology, Beppu Medical CenterNational Hospital OrganizationBeppuOitaJapan
| | - Yuichi Nakazono
- Department of PathologyBeppu Medical Center, National Hospital OrganizationBeppuOitaJapan
| | - Yoko Fukata
- Department of Diabetes and EndocrinologyBeppu Medical Center, National Hospital OrganizationBeppuOitaJapan
| | - Toshitaka Shin
- Department of Urology, Faculty of MedicineOita UniversityYufu‐CityOitaJapan
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13
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Katsoulis IE, Dafnis AN, Sourouppi C, Katsaounis D, Boti E, Arnogiannaki N. A large adrenal myelolipoma: case report and review of the literature. J Surg Case Rep 2023; 2023:rjad326. [PMID: 37293332 PMCID: PMC10247334 DOI: 10.1093/jscr/rjad326] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2023] [Accepted: 05/20/2023] [Indexed: 06/10/2023] Open
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.
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Affiliation(s)
- Iraklis E Katsoulis
- Correspondence address. Agios Savvas Oncology Hospital,171 Alexandra's Avenue, 11522 Athens, Greece. Tel: +306944747226; E-mail:
| | - Andreas N Dafnis
- Department of Surgical Oncology, Agios Savvas Oncology Hospital, Athens, Greece
| | | | - Dionysis Katsaounis
- Department of Surgical Oncology, Agios Savvas Oncology Hospital, Athens, Greece
| | - E Boti
- Pathology Department, Agios Savvas Oncology Hospital, Athens, Greece
| | - Niki Arnogiannaki
- Pathology Department, Agios Savvas Oncology Hospital, Athens, Greece
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14
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Madani MA, Cherchir F, Bibi M, Zehani A, Chaker K, Nouira Y. Bilateral adrenal myelolipoma revealing an adrenal insufficiency: A case report. Int J Surg Case Rep 2023; 107:108330. [PMID: 37230061 DOI: 10.1016/j.ijscr.2023.108330] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2023] [Revised: 05/11/2023] [Accepted: 05/12/2023] [Indexed: 05/27/2023] Open
Abstract
INTRODUCTION Once mostly discovered on autopsy, adrenal myelolipomas are now increasingly diagnosed due to the frequent use of modern imaging methods. However, bilaterality remains quite rare. We present the case of a 31 years old female patient treated in our department for a bilateral adrenal myelolipoma which revealed an unknown peripheral adrenal insufficiency. CASE PRESENTATION We describe the case of a 31-year-old woman in apparent good health with no medical history who was explored for recurrent right lumbar pain by a computed tomography scanner which showed a large right adrenal mass and a smaller lesion in the left adrenal gland. Preoperative biology revealed an unknown peripheral adrenal insufficiency. Right open sub-costal adrenalectomy was performed, Histological examination confirmed the diagnosis of bilateral adrenal myelolipomas and radiological surveillance was planned for the left tumor. DISCUSSION Adrenal myelolipoma (AML) is a rare, benign and typically non-functional tumor of the adrenal gland, usually unilateral and asymptomatic, incidentally detected on CT. Commonly diagnosed between the fifth and seventh decades of life. It can affect both sexes our patient is a 31-year-old female and presented with bilateral AML. Unlike previous reported cases, our patient has a previously unknown peripheral adrenal insufficiency, which could be incriminated in the development of his bilateral adrenal myelolipomas. The optimal management depends on both clinical presentation and tumor characteristics. CONCLUSION Adrenal myelolipoma is a rare tumor. Endocrinological investigation should be performed to detect and treat endocrine disorders. The therapeutic attitude depends on tumor size complications and clinical complaints. METHODS This is a case report from our urology department, and has been reported in line with the SCARE criteria.
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Affiliation(s)
- Mohamed Anouar Madani
- Department of Urology, LA RABTA Hospital, University of TUNIS EL MANAR, Tunis, Tunisia.
| | - Faten Cherchir
- Department of Endocrinology, LA RABTA Hospital, University of TUNIS EL MANAR, Tunis, Tunisia
| | - Mokhtar Bibi
- Department of Urology, LA RABTA Hospital, University of TUNIS EL MANAR, Tunis, Tunisia
| | - Alia Zehani
- Department of Anatomic Pathology, LA RABTA Hospital, University of TUNIS EL MANAR, Tunis, Tunisia
| | - Kais Chaker
- Department of Urology, LA RABTA Hospital, University of TUNIS EL MANAR, Tunis, Tunisia.
| | - Yassine Nouira
- Department of Urology, LA RABTA Hospital, University of TUNIS EL MANAR, Tunis, Tunisia
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15
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Xu C, Kasajima A, Novotny A, Friess H. Meta-analysis of reported presacral myelolipomas, including a report of a new case. J Med Case Rep 2023; 17:31. [PMID: 36721209 PMCID: PMC9890845 DOI: 10.1186/s13256-022-03746-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/17/2022] [Accepted: 12/27/2022] [Indexed: 02/02/2023] Open
Abstract
BACKGROUND Presacral myelolipomas form a rare disease and are often found incidentally in imaging diagnostics. CASE PRESENTATION In this study, we report the case of a 71-year-old caucasian female with an incidental finding of a retroperitoneal tumor on magnetic resonance imaging scan. This report aimed at presenting the clinical course of this patient with emphasis on analysis of pathological, clinical, and epidemiological features in a meta-analysis of reported cases. CONCLUSION Presacral myelolipomas are rare and its etiology remains unclear. Surgical resection is indicated in symptomatic lesions and lesions > 4 cm. More clinical and pathological research on this rare entity is warranted.
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Affiliation(s)
- Congde Xu
- grid.6936.a0000000123222966Klinikum rechts der Isar, Klinik und Poliklinik für Chirurgie, TU München, Ismaninger Straße 22, 81675 Munich, Deutschland
| | - Atsuko Kasajima
- grid.6936.a0000000123222966Klinikum rechts der Isar, Institut für Allgemeine Pathologie und Pathologische Anatomie, TU München, Ismaninger Straße 22, 81675 Munich, Deutschland
| | - Alexander Novotny
- grid.6936.a0000000123222966Klinikum rechts der Isar, Klinik und Poliklinik für Chirurgie, TU München, Ismaninger Straße 22, 81675 Munich, Deutschland
| | - Helmut Friess
- grid.6936.a0000000123222966Klinikum rechts der Isar, Klinik und Poliklinik für Chirurgie, TU München, Ismaninger Straße 22, 81675 Munich, Deutschland
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16
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Feng Q, Li H, Chen X, Feng X, Li J. Case report: Adrenal myelolipoma resected by laparoscopic surgery. Front Oncol 2022; 12:1058211. [PMID: 36544699 PMCID: PMC9760819 DOI: 10.3389/fonc.2022.1058211] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2022] [Accepted: 11/16/2022] [Indexed: 12/08/2022] Open
Abstract
INTRODUCTION Adrenal myelolipomas are benign tumors composed mainly of lipomatous elements with myeloid cells. With the development of medical imaging technology, the detection rate has gradually increased. We report a case of adrenal myelolipoma successfully excised through the laparoscope and reviewed existing literature in recent ten years to summarize the feasibility of the laparoscopic approach for this tumor. CASE PRESENTATION Herein, we described a case of adrenal myelolipoma resected by laparoscope in a 63-year-old male patient. He did not have any other symptoms except the incidental finding of a left adrenal mass. An abdominal CT examination revealed a mixed-density lesion containing some amount of adipose tissue. In conjunction with the patient's willingness, we performed a laparoscopic operation to remove the lump. The definite diagnosis was confirmed as an adrenal myelolipoma according to the pathology. The patient recovered well postoperatively and without signs of recurrence at a 5-month follow-up. CONCLUSION Adrenal myelolipoma is commonly benign, asymptomatic, and hormonal inactivity. A surgical strategy is suggested for high-complication-risk patients. The laparoscopic approach is safe and effective with an obvious advantage over open procedures.
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Affiliation(s)
- Qingbo Feng
- Department of Liver Surgery and Liver Transplantation Centre, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Hancong Li
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Xinyang Chen
- West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Xuping Feng
- Department of Liver Surgery and Liver Transplantation Centre, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Jiaxin Li
- Department of Liver Surgery and Liver Transplantation Centre, West China Hospital, Sichuan University, Chengdu, Sichuan, China
- Department of General Surgery, Dafang County People’s Hospital, Bijie, Guizhou, China
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17
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Paice B, Oyebola T, Ball K, Alexander S, Sherwood B. A case report on a rare presentation of adrenal myelolipoma with low-impact traumatic haemorrhage and the challenges of conservative management. J Surg Case Rep 2022; 2022:rjac554. [DOI: 10.1093/jscr/rjac554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2022] [Accepted: 11/12/2022] [Indexed: 12/13/2022] Open
Abstract
Abstract
Adrenal myelolipomas are rare, hormonally silent, adipose and myeloid-containing lesions that are mostly asymptomatic. If they do present it is usually with mass-related flank pain or spontaneous haemorrhage. A 55-year-old female presented with right flank pain after a fall from a static pushbike. Computer tomography identified a large adrenal lesion with surrounding acute retroperitoneal haemorrhage. A conservative approach to treatment was decided on as the patient remained haemodynamically stable. The patient developed a pulmonary embolism during the time of conservative management and therefore had to be anticoagulated with close monitoring. Outpatient surveillance imaging was reassuring, hormonal screening was negative and biopsy confirmed myelolipoma. We report a rare presentation of adrenal myelolipoma with the sequelae of haemorrhage from low-impact trauma and the challenges of conservative management.
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Affiliation(s)
- Bronte Paice
- Nottingham University Hospitals Department of Urology, , Nottingham , UK
| | - Taiwo Oyebola
- University Hospitals of Derby and Burton Department of Urology, , Derby , UK
| | - Kathryn Ball
- University Hospitals of Derby and Burton Department of Urology, , Derby , UK
| | - Shaun Alexander
- United Lincolnshire Hospitals Foundation Training, , Lincoln , UK
| | - Benedict Sherwood
- Nottingham University Hospitals Department of Urology, , Nottingham , UK
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18
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Cao J, Huang X, Cui N, Wang X, You C, Ni X, Gao X, Wang J, Liu T. Surgical Management and Outcome of Extra-adrenal Myelolipomas at Unusual Locations: A Report of 11 Cases in a Single Center. J Bone Oncol 2022; 35:100438. [PMID: 35721369 PMCID: PMC9198452 DOI: 10.1016/j.jbo.2022.100438] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2022] [Revised: 06/04/2022] [Accepted: 06/04/2022] [Indexed: 11/28/2022] Open
Abstract
The first case series with eleven consecutive patients diagnosed with EAMs in the spinal or intraosseous regions. EAMs involving the thoracic vertebral body or the humerus have not previously been reported in the English literature. The surgical outcome and prognosis of EAMs are excellent and surgery can serve as the method of radical treatment. Purpose Extra-adrenal myelolipomas (EAMs) are rare benign tumors composed of both mature adipose and hematopoietic tissues with unclear etiology. There have been only sporadic case reports about the clinical characteristics and management of EAMs. Here we present our experience and practice in the clinical diagnosis and treatment of 11 consecutive patients with EAMs. Method We retrospectively reviewed 11 consecutive patients, who received surgeries in our department and were confirmed as having EAMs by postoperative histopathology from April 2016 to December 2021. Clinical information and follow-up data of all patients were collected and analyzed afterwards. Results Of the 11 EAM patients (7 male and 4 female) with a mean age of 47.6 years, 3 were asymptomatic and 8 were symptomatic with a mean symptom duration of 6.07 months. EAMs were found in the thoracic spine in 4 cases, paravertebral mediastinal regions in 3 cases, ilium in 2 cases, humerus in 1 case, and rib in 1 case. All patients were initially misdiagnosed as other tumors by radiologists. All 11 patients received gross total excision or curettage with a mean intraoperative blood loss of 781.82 ± 1143.3 ml and a mean operation duration of 180.91 ± 98.41 min. Patients’ Frankel scores and Karnofsky Performance Status score were improved or at least preserved postoperatively. No significant complications occurred postoperatively. All the 11 patients survived, and no local recurrence or distant metastasis occurred during the mean follow-up period of 42.0 months. Conclusion The surgical outcome and prognosis of EAMs are excellent and surgery can serve as the method of radical treatment.
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Affiliation(s)
- Jiashi Cao
- Department of Orthopedics, No. 455 Hospital of the Chinese People's Liberation Army, The Navy Medical University, No. 338 Huaihai West Road, Shanghai 200052, China
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
| | - Xing Huang
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
| | - Na Cui
- Department of Pathology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
| | - Xiang Wang
- Department of Radiology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
| | - Chaoqun You
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
- Clinical Medical College of Weifang Medical University, No.7166 Baotong West Street, Weifang, Shandong 261000, China
| | - Xiangzhi Ni
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
| | - Xin Gao
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
- Corresponding authors.
| | - Jing Wang
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
- Corresponding authors.
| | - Tielong Liu
- Department of Orthopaedic Oncology, Changzheng Hospital of the Navy Medical University, No. 415 Fengyang Road, Shanghai 200003, China
- Corresponding authors.
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19
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Giant symptomatic adrenal myelolipoma: A case report. Ann Med Surg (Lond) 2022; 75:103333. [PMID: 35198182 PMCID: PMC8844840 DOI: 10.1016/j.amsu.2022.103333] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/28/2021] [Revised: 01/27/2022] [Accepted: 02/01/2022] [Indexed: 11/23/2022] Open
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20
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Calissendorff J, Juhlin CC, Sundin A, Bancos I, Falhammar H. Adrenal myelolipomas. Lancet Diabetes Endocrinol 2021; 9:767-775. [PMID: 34450092 PMCID: PMC8851410 DOI: 10.1016/s2213-8587(21)00178-9] [Citation(s) in RCA: 59] [Impact Index Per Article: 14.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/12/2021] [Revised: 06/22/2021] [Accepted: 06/22/2021] [Indexed: 12/24/2022]
Abstract
Adrenal myelolipomas are benign, lipomatous tumours with elements of myeloid cells, most of which present as adrenal incidentalomas and comprise 3·3-6·5% of all adrenal masses. Adrenal myelolipomas are usually unilateral (in 95% of cases), variable in size, most often found during midlife, and affect both sexes almost equally. On imaging, adrenal myelolipomas show pathognomonic imaging features consistent with the presence of macroscopic fat. Large adrenal myelolipomas can cause symptoms of mass effect, and can occasionally be complicated by haemorrhage. In the event of a concomitant adrenal cortical adenoma or hyperplasia, adrenal hormone excess might be detected in patients with adrenal myelolipoma. Patients with congenital adrenal hyperplasia exhibit a higher prevalence of adrenal myelolipomas than other patient groups, and are at risk of developing large and bilateral lesions. This Review discusses the pathogenesis, clinical presentation, and management of adrenal myelolipomas.
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Affiliation(s)
- Jan Calissendorff
- Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
| | - Carl Christofer Juhlin
- Department of Pathology and Cytology, Karolinska University Hospital, Stockholm, Sweden; Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
| | - Anders Sundin
- Department of Surgical Sciences, Radiology and Molecular Imaging, Uppsala University, Uppsala, Sweden
| | - Irina Bancos
- Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA
| | - Henrik Falhammar
- Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
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21
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Zulia YS, Gopireddy D, Kumar S, Singareddy A, Lall C. A Rare Case of Hemorrhagic Giant Adrenal Myelolipoma: Radiographic and Pathologic Correlation. Cureus 2021; 13:e17353. [PMID: 34567893 PMCID: PMC8452227 DOI: 10.7759/cureus.17353] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/20/2021] [Indexed: 11/16/2022] Open
Abstract
Myelolipomas are rare benign tumors made up of adipose and hematopoietic tissue that commonly occur in the adrenal glands unilaterally. Spontaneous hemorrhage occurs in < 5% of these tumors, and often present as large masses. A 50-year-old male presented with right flank pain that had been growing increasingly worse over a two-week period. Contrast-enhanced Computed Tomography (CT) revealed a large suprarenal 15-cm mass exerting mass effect on the kidney and liver along with possible hemorrhage. T1 fat saturated and T2 non-fat saturated magnetic resonance imaging (MRI) confirmed the diagnosis of a myelolipoma with hemorrhage. The patient was treated with surgical resection of the mass and the follow-up pathology report confirmed a giant hemorrhagic adrenal myelolipoma. Spontaneous hemorrhage of a large myelolipoma measuring 15 cm is a rare entity and the correct imaging needs to be done in order to carry out the appropriate treatment.
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Affiliation(s)
- Yanni S Zulia
- Radiology, Lake Erie College of Osteopathic Medicine, Bradenton, USA
| | - Dheeraj Gopireddy
- Radiology, University of Florida College of Medicine, Jacksonville, USA
| | - Sindhu Kumar
- Radiology, University of Florida College of Medicine, Jacksonville, USA
| | | | - Chandana Lall
- Abdominal Imaging, University of Florida College of Medicine, Jacksonville, USA
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22
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Lin L, Gong L, Cheng L, Liu Z, Shen S, Zhu Y, Zhou L. Adrenal Myelolipoma: 369 Cases From a High-Volume Center. Front Cardiovasc Med 2021; 8:663346. [PMID: 34568440 PMCID: PMC8462508 DOI: 10.3389/fcvm.2021.663346] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2021] [Accepted: 07/30/2021] [Indexed: 02/05/2023] Open
Abstract
Background: Adrenal myelolipoma (AML) is a nonfunctional benign neoplasm from the adrenal cortex, composed of mature fat and hematopoietic tissue. Usually, patients have no symptoms. However, some patients with hypertension and blood pressure normalize after AML surgery, indicating some connections between AML and hypertension. Materials and Methods: This was a retrospective cohort study of 369 patients diagnosed with AML from September 2008 to December 2018 collected in the Urology Department of West China Hospital, Chengdu, Sichuan, China. We collected clinical records of patients before surgery. Postoperative follow-up was also carried out for those with hypertension and whether patients needed to take antihypertensive drugs and postoperative blood pressure were recorded. We aim to explore the characteristics of both patients with AML having hypertension and having remission of hypertension in 1 year after surgery. Results: There were 369 patients with AML included in the study, 156 men and 213 women, aged 49.86 ± 11.61 years old. Among them, 121 (32.8%) patients presented with hypertension. Body mass index was significantly higher in the hypertension group than that in the nonhypertension group, even after adjusting other variables (26.26 ± 3.43 vs. 24.28 ± 3.38 kg/m2, P < 0.001 for both univariate and multivariate analyses). Sixty patients were followed up for 1–9 years, with a median follow-up of 52 months. The duration of hypertension in the remission group was shorter than that in the non-remission group (P = 0.020), and the tumor lateralization was significantly different between the two groups (P = 0.005). Conclusions: Nearly one-third of patients with AML suffered from hypertension in our study, and there existed some potential links between AML and hypertension. To be more specific, AML-related hypertension was more likely to result from obesity and renal compression by perirenal fat than from endocrine disorders or blood vessels compression. Patients with AML and with more than 3 years of hypertension might have less possibility to recover.
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Affiliation(s)
- Lede Lin
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
| | - Lina Gong
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
| | - Liang Cheng
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
| | - Zhihong Liu
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
| | - Sikui Shen
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
| | - Yuchun Zhu
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
| | - Liang Zhou
- Department of Urology, West China Hospital, Sichuan University, Chengdu, China
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23
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Anbardar MH, Soleimani N, Nikeghbalian S, Mohebbi M. Adrenocortical adenoma with myelolipomatous metaplasia: a potential diagnostic pitfall: a case report and review of the literature. J Med Case Rep 2021; 15:333. [PMID: 34217375 PMCID: PMC8255017 DOI: 10.1186/s13256-021-02937-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2021] [Accepted: 05/31/2021] [Indexed: 11/10/2022] Open
Abstract
Background Adrenal incidentalomas are often found during investigation for another tumor or unrelated problems. Except for adrenal myelolipoma (second most common primary adrenal incidentaloma following adrenocortical adenomas), adrenal lipomatous tumors are uncommon generally and are often described as case reports in the literature. Since the amount of fat is variable, without the help of advanced imaging techniques, some adrenal lipomatous tumors may be misdiagnosed before pathologic examination. Herein, we report a case of adrenal adenoma with myelolipomatous metaplasia that was excised as a periceliac mass in the setting of recurrent pancreatic cyst. Case report A 45-year-old Iranian woman with hypertension and end-stage renal disease presented with recurrence of a pancreatic cyst (previous pathologic report was mucinous cyst adenoma). During exploratory laparotomy, the mentioned pancreatic cyst was tightly attached to the stomach and jejunum. There was also a periceliac round rubbery lesion (firstly diagnosed by endoscopic ultrasound) that was excised for ruling out malignancy. Histologic examination of the periceliac mass was found to be adrenocortical adenoma with foci of myelolipomatous metaplasia. The pancreatic cyst histology was just a pseudocyst. Conclusion Our case highlights the significance of complete evaluation of incidental findings before surgical intervention, even in the setting of another primary tumor. Myelolipoma and myelolipomatous change (metaplasia) are two different entities. Although very similar as to pathogenesis, there are still some differences.
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Affiliation(s)
- Mohammad Hossein Anbardar
- Department of Pathology, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran.,Department of Pathology, Shiraz Transplant Center, Abu Ali Sina Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Neda Soleimani
- Department of Pathology, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran. .,Department of Pathology, Shiraz Transplant Center, Abu Ali Sina Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.
| | - Saman Nikeghbalian
- Department of hepatopancreatobiliary and organ transplant surgery, Shiraz Transplant Center, Abu Ali Sina Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.,Department of Surgery, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Maryam Mohebbi
- Department of Pathology, Shiraz Medical School, Shiraz University of Medical Sciences, Shiraz, Iran
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24
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Sakai T, Aiba H, Nakagawa M, Hattori H, Murakami H, Kimura H. Myelolipoma mimicking osteosarcoma in the distal femur. Int J Surg Case Rep 2021; 83:105997. [PMID: 34051447 PMCID: PMC8176310 DOI: 10.1016/j.ijscr.2021.105997] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/03/2021] [Revised: 05/11/2021] [Accepted: 05/16/2021] [Indexed: 12/25/2022] Open
Abstract
Introduction and importance Periosteal reactions indicate malignant bone tumors, including osteosarcoma; establishing an accurate diagnosis is key to determining the most appropriate treatment strategy. We describe a rare case of myelolipoma in the distal femur metaphysis with massive extraskeletal lesions and periosteal reactions. Case presentation A 25-year-old woman was referred to our hospital to treat a gradually expanding mass around her knee that grew to the size of a baby's head. She had a history of hydrocephalus caused by congenital cytomegalovirus infection and was bedridden for life. Radiography showed a prominent osteoblastic rim and osteolytic lesion with a moth-eaten appearance. Osteosarcoma was suspected due to excessive extraskeletal invasion and periosteal reactions. T1- and T2-weighted magnetic resonance images showed a high-signal-intensity homologous lesion. Biopsy specimens contained adipose and hematopoietic tissues. A myelolipoma was diagnosed. Due to her fragility, surgical intervention was suspended. Two years after diagnosis, the tumor size did not change. Clinical discussion Myelolipomas are benign tumors that typically arise from the adrenal gland and rarely develop in the extremities. This type of tumor typically does not cause any tumor-related symptoms or endocrine disturbances and has been reported as a type of incidentaloma. To effectively manage myelolipoma patients, differential diagnosis of tumors mimicking malignant bone tumors is important. Conclusion We successfully managed a destructive ectopic myelolipoma in the distal femoral metaphysis, with massive extraskeletal lesions and periosteal reactions. Clinicians should appropriately differentiate myelolipoma from tumors mimicking malignant bone tumors.
Report discusses a rare case of ectopic extra-adrenal myelolipoma in the femur. The extraskeletal invasion and periosteal reactions resemble malignant bone tumors. Clinicians should properly differentiate it from mimics of malignant bone tumors.
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Affiliation(s)
- Takao Sakai
- Department of Orthopaedic Surgery, Nagoya City University, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan
| | - Hisaki Aiba
- Department of Orthopaedic Surgery, Nagoya City University, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan.
| | - Motoo Nakagawa
- Department of Radiology, Nagoya City University, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan
| | - Hideo Hattori
- Department of Pathology and Molecular Diagnostics, Nagoya City University, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan
| | - Hideki Murakami
- Department of Orthopaedic Surgery, Nagoya City University, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan
| | - Hiroaki Kimura
- Department of Orthopaedic Surgery, Nagoya City University, 1, Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi 467-8601, Japan
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25
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Li KY, Wei AL, Li A. Primary hepatic myelolipoma: A case report and review of the literature. World J Clin Cases 2020; 8:4615-4623. [PMID: 33083426 PMCID: PMC7559654 DOI: 10.12998/wjcc.v8.i19.4615] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/29/2020] [Revised: 06/01/2020] [Accepted: 08/25/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Primary hepatic myelolipoma is a rare hepatic mesenchymal tumor mixed by adipose tissue and bone marrow, and there is a lack of general guidelines related to its epidemiology and clinical management. CASE SUMMARY A 26-year-old woman was admitted to our department complaining of a newly found tumor on her left lobe of the liver. The tumor was painless and not associated with any systemic or localized compressive symptoms. Serological tests disclosed a slight increase of gamma-glutamyl transpeptidase (70.0 U/L) and total bilirubin (19.2 μmol/L). Ultrasonography showed a mass about 5.0 cm × 5.0 cm in size that was located in the left lobe of the liver and displayed hyperechoic and well-encapsulated characteristics. Surgical resection was applied, and the following histopathological examination observed a variable proportion of myeloid tissues scattering throughout mature fibrotic adipose tissues, in which myeloid, erythroid, and megakaryocytic cells can be found in magnified view. The follow-up did not show any changes 6 mo after surgery. CONCLUSION This case highlights an extremely rare hepatic mesenchymal tumor, the primary hepatic myelolipoma, and discloses the common characteristics behind this disease and gives clinical recommendations.
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Affiliation(s)
- Ke-Yu Li
- Department of Hepato-Bilio-Pancreatic Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Ai-Lin Wei
- Department of Hepato-Bilio-Pancreatic Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
| | - Ang Li
- Department of Hepato-Bilio-Pancreatic Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
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Introini C, Campodonico F, Ennas M, Di Domenico A, Foppiani L. Non-secreting adrenal myelolipoma in a middle-aged male patient manifesting with sudden onset of severe lower back pain. Arch Ital Urol Androl 2020; 92. [DOI: 10.4081/aiua.2020.3.205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2020] [Accepted: 03/15/2020] [Indexed: 11/23/2022] Open
Abstract
Adrenal myelolipoma (AML) is a rare benign tumor, usually non-functioning and asymptomatic until it reaches large size. AML is mostly detected incidentally by imaging and is composed of adipose tissue and hematopoietic elements. Only symptomatic tumor needs surgical excision. We report the case of a large non-functioning adrenal tumor discovered by means of combined imaging techniques in a middle-aged male patient who complained the sudden onset of severe lower back pain; successful laparoscopic removal was performed, and AML was diagnosed at histopathology.
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Hamidi O, Raman R, Lazik N, Iniguez-Ariza N, McKenzie TJ, Lyden ML, Thompson GB, Dy BM, Young WF, Bancos I. Clinical course of adrenal myelolipoma: A long-term longitudinal follow-up study. Clin Endocrinol (Oxf) 2020; 93:11-18. [PMID: 32275787 PMCID: PMC7292791 DOI: 10.1111/cen.14188] [Citation(s) in RCA: 40] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2020] [Revised: 03/23/2020] [Accepted: 03/30/2020] [Indexed: 12/31/2022]
Abstract
OBJECTIVE We aimed to describe clinical course of myelolipoma and to identify predictors of tumour growth and need for surgery. DESIGN A retrospective study. PATIENTS Consecutive patients with myelolipoma. RESULTS A total of 321 myelolipomas (median size, 2.3 cm) were diagnosed in 305 patients at median age of 63 years (range, 25-87). Median follow-up was 54 months. Most myelolipomas were incidentally detected (86%), whereas 9% were discovered during cancer staging and 5% during workup of mass effect symptoms. Thirty-seven (12%) patients underwent adrenalectomy. Compared to myelolipomas <6 cm, tumours ≥6 cm were more likely to be bilateral (21% vs 3%, P < .0001), cause mass effect symptoms (32% vs 0%, P < .0001), have haemorrhagic changes (14% vs 1%, P < .0001) and undergo adrenalectomy (52% vs 5%, P < .0001). Among patients with ≥6 months of imaging follow-up, median size change was 0 mm (-10, 115) and median growth rate was 0 mm/y (-6, 14). Compared to <1 cm growth, ≥1 cm growth correlated with larger initial size (3.6 vs 2.3 cm, P = .02), haemorrhagic changes (12% vs 2%, P = .007) and adrenalectomy (35% vs 8%, P < .0001). CONCLUSIONS Most myelolipomas are incidentally discovered on cross-sectional imaging. Myelolipomas ≥6 are more likely to cause mass effect symptoms, have haemorrhagic changes and undergo resection. Tumour growth ≥1 cm is associated with larger myelolipoma and haemorrhagic changes. Adrenalectomy should be considered in symptomatic patients with large tumours and when there is evidence of haemorrhage or tumour growth.
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Affiliation(s)
- Oksana Hamidi
- Division of Endocrinology and Metabolism, UT Southwestern Medical Center, Dallas, TX, USA
- Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, MN, US
| | - Ram Raman
- Department of Medicine, Charles University, Prague, Czech Republic
| | - Natalia Lazik
- Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA
| | - Nicole Iniguez-Ariza
- Department of Endocrinology and Metabolism, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | | | | | | | - Benzon M. Dy
- Department of Surgery, Mayo Clinic, Rochester, MN, USA
| | - William F. Young
- Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, MN, US
| | - Irina Bancos
- Division of Endocrinology, Diabetes, and Nutrition, Mayo Clinic, Rochester, MN, US
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Kabeel K, Marjara J, Bhat R, Gaballah AH, Abdelaziz A, Bhat AP. Spontaneous hemorrhage of an adrenal myelolipoma treated with transarterial embolization: A case report. Radiol Case Rep 2020; 15:961-965. [PMID: 32419895 PMCID: PMC7214766 DOI: 10.1016/j.radcr.2020.04.034] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2020] [Revised: 04/14/2020] [Accepted: 04/15/2020] [Indexed: 01/14/2023] Open
Abstract
Adrenal myelolipoma is a benign tumor of the adrenal cortex composed predominantly of fat and hematopoietic tissue. These lesions are usually asymptomatic, and most often incidentally detected on imaging. Uncommonly, they present with retroperitoneal hemorrhage, and these have been traditionally treated with emergent surgery. Although, transarterial embolization has been effectively and safely used in patients presenting with active hemorrhage from acute traumatic and nontraumatic causes, literature specifically pertaining to adrenal artery embolization is scant, perhaps due to smaller size and variability of adrenal arteries. With recent advances in endovascular techniques and imaging, there are emerging case reports and series of adrenal artery embolization in acute and nonacute settings. We report a case of spontaneous hemorrhage within an adrenal myelolipoma in a 43-year-old male patient, successfully treated with transarterial embolization, thereby avoiding major surgery. Our report adds to the growing body of literature pertaining to adrenal artery embolization.
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Affiliation(s)
- Khalid Kabeel
- Department of Radiology, Body Imaging Section, University of Missouri, Columbia, MO 65212, USA
| | - Jasraj Marjara
- University of Missouri-Columbia School of Medicine, Columbia, MO 65212, USA
| | - Roopa Bhat
- Department of Radiology, Body Imaging Section, University of Missouri, Columbia, MO 65212, USA
| | - Ayman H. Gaballah
- Department of Radiology, Body Imaging Section, University of Missouri, Columbia, MO 65212, USA
| | - Amr Abdelaziz
- Department of Radiology, Body Imaging Section, University of Missouri, Columbia, MO 65212, USA
| | - Ambarish P. Bhat
- Department of Radiology, Section of Vascular and Interventional Radiology, University of Missouri, One Hospital Drive, Columbia, MO 65212, USA
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Mhammedi WA, Ouslim H, Ouraghi A, Irzi M, Elhoumaidi A, Elhoumaidi A, Chennoufi M, Mokhtari M, Elmouden A, Barki A. Adrenal myelolipoma: from tumorigenesis to management. Pan Afr Med J 2019; 34:180. [PMID: 32153720 PMCID: PMC7046110 DOI: 10.11604/pamj.2019.34.180.20891] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/04/2019] [Accepted: 11/21/2019] [Indexed: 12/12/2022] Open
Abstract
Adrenal myelolipoma (MLS) is a rare, benign and non-functional neoplasm, composed of adipose tissue and myeloid. We report a rare case of adrenal myelolipoma of a 20-year-old female revealed with chronic abdominal pain. Computed tomography (CT) scan of the abdomen guided diagnosis and surgical resection was performed given symptomatic and bulky mass. Histological examination confirmed the diagnosis. At 18 months after the surgery, the patient had no evidence of recurrence. The diagnosis of MLS is radiological. Therapeutic abstention is the rule for a small, asymptomatic tumor. The surgical removal is indicated when it is bulky (exceeds 7cm), symptomatic or hormonal activity.
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Affiliation(s)
| | - Hicham Ouslim
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Abdelghani Ouraghi
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Mohammed Irzi
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Amine Elhoumaidi
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Amine Elhoumaidi
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Mehdi Chennoufi
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Mohammed Mokhtari
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Anouar Elmouden
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
| | - Ali Barki
- Urology Department, Mohammed the Sixth University Hospital, Oujda, Morocco
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Adapa S, Naramala S, Gayam V, Gavini F, Dhingra H, Hazard FKG, Aeddula NR, Konala VM. Adrenal Incidentaloma: Challenges in Diagnosing Adrenal Myelolipoma. J Investig Med High Impact Case Rep 2019; 7:2324709619870311. [PMID: 31434506 PMCID: PMC6709432 DOI: 10.1177/2324709619870311] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022] Open
Abstract
Adrenal myelolipomas (AMLs) are rare benign adrenal tumors, containing adipose
and hematopoietic tissue, a result of reticuloendothelial cell metaplasia.
Incidence on autopsy has been reported from 0.08% to 0.4%. AMLs are generally
considered nonsecretory. The functional aspect of adrenal incidentaloma should
be evaluated. In this article, we report a case of a 40-year-old male, who
presented with uncontrolled hypertension and renal failure, with imaging
revealing an adrenal incidentaloma. He was started on dialysis for acute fluid
overload, and workup for pheochromocytoma revealed an elevated serum
norepinephrine level of 1181 pg/mL. Free metanephrine and normetanephrine levels
were low when checked pre- and post-dialysis. Complete resection of the
encapsulated right adrenal mass was performed. Pathology of the adrenal tumor
demonstrates an 11.5 × 9.5 × 7.5 cm well-circumscribed, partially encapsulated
proliferation of mature adipose tissue with admixed hemopoietic elements
consistent with myelolipoma weighing 29.3 g. This case highlights the inclusion
of a full metabolic workup for all adrenal incidentalomas, including AML.
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Affiliation(s)
| | | | - Vijay Gayam
- 3 Interfaith Medical Center, New York, NY, USA
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Larose S, Bondaz L, Mermejo LM, Latour M, Prosmanne O, Bourdeau I, Lacroix A. Coexistence of Myelolipoma and Primary Bilateral Macronodular Adrenal Hyperplasia With GIP-Dependent Cushing's Syndrome. Front Endocrinol (Lausanne) 2019; 10:618. [PMID: 31572300 PMCID: PMC6749096 DOI: 10.3389/fendo.2019.00618] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/07/2019] [Accepted: 08/27/2019] [Indexed: 12/14/2022] Open
Abstract
Introduction: Adrenal myelolipomas are usually isolated benign adrenal lesions, but can be adjacent to steroid-secreting adrenocortical tumors. We studied the aberrant regulation of cortisol secretion in a 61 year-old woman with combined bilateral myelolipomas and primary bilateral macronodular adrenal hyperplasia (BMAH) causing Cushing's syndrome. Materials and Methods: Cortisol response was measured during in vivo tests that transiently modulated the levels of ligands for potential aberrant receptors, including GIP. Response to medical therapies decreasing GIP was monitored. Expression of ACTH and of GIP receptors were examined in resected adrenal tissues by immunohistochemistry and reverse transcription polymerase chain reaction (RT-PCR). Results: In vivo, cortisol increased in response to mixed meals (+353%), oral 75 g glucose (+71%), GIP infusion (+416%), and hLH IV (+243%). Suppression of GIP by pasireotide improved cortisol secretion but produced hyperglycemia. The left adrenal was predominantly composed of myelolipoma and strands of BMAH, while the right was mainly composed of BMAH with some foci of myelolipoma on pathology. No ACTH was detectable by immunohistochemistry in BMAH or myelolipomas tissue. Ectopic GIP receptor was confirmed by RT-PCR and immunohistochemistry in BMAH tissues but not in the myelolipomas. No germline mutations were identified in the ARMC5 gene of the patient's leucocyte DNA. Conclusion: This is the first report of interspersed myelolipoma and BMAH with GIP-dependent Cushing's syndrome. In contrast with the BMAH tissues, myelolipoma tissue did not express specific GIP receptors. The potential mechanisms responsible for the interspersed growth of those two lesions remain to be identified.
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Affiliation(s)
- Stéphanie Larose
- Division of Endocrinology, Department of Medicine, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
| | - Louis Bondaz
- Division of Endocrinology, Department of Medicine, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
| | - Livia M. Mermejo
- Division of Endocrinology, Department of Medicine, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
| | - Mathieu Latour
- Department of Pathology, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
| | - Odile Prosmanne
- Department of Radiology, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
| | - Isabelle Bourdeau
- Division of Endocrinology, Department of Medicine, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
| | - André Lacroix
- Division of Endocrinology, Department of Medicine, Centre Hospitalier de L'Université de Montréal (CHUM), Montreal, QC, Canada
- *Correspondence: André Lacroix
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Bourdeau I, El Ghorayeb N, Gagnon N, Lacroix A. MANAGEMENT OF ENDOCRINE DISEASE: Differential diagnosis, investigation and therapy of bilateral adrenal incidentalomas. Eur J Endocrinol 2018; 179:R57-R67. [PMID: 29748231 DOI: 10.1530/eje-18-0296] [Citation(s) in RCA: 53] [Impact Index Per Article: 7.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2018] [Accepted: 05/10/2018] [Indexed: 01/11/2023]
Abstract
The investigation and management of unilateral adrenal incidentalomas have been extensively considered in the last decades. While bilateral adrenal incidentalomas represent about 15% of adrenal incidentalomas (AIs), they have been less frequently discussed. The differential diagnosis of bilateral incidentalomas includes metastasis, primary bilateral macronodular adrenal hyperplasia and bilateral cortical adenomas. Less frequent etiologies are bilateral pheochromocytomas, congenital adrenal hyperplasia (CAH), Cushing's disease or ectopic ACTH secretion with secondary bilateral adrenal hyperplasia, primary malignancies, myelolipomas, infections or hemorrhage. The investigation of bilateral incidentalomas includes the same hormonal evaluation to exclude excess hormone secretion as recommended in unilateral AI, but diagnosis of CAH and adrenal insufficiency should also be excluded. This review is focused on the differential diagnosis, investigation and treatment of bilateral AIs.
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Affiliation(s)
- Isabelle Bourdeau
- Division of Endocrinology, Department of Medicine, Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), Université de Montréal, Montréal, Canada
| | - Nada El Ghorayeb
- Division of Endocrinology, Department of Medicine, Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), Université de Montréal, Montréal, Canada
| | - Nadia Gagnon
- Division of Endocrinology, Department of Medicine, Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), Université de Montréal, Montréal, Canada
| | - André Lacroix
- Division of Endocrinology, Department of Medicine, Centre de recherche du Centre hospitalier de l'Université de Montréal (CRCHUM), Université de Montréal, Montréal, Canada
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Gadelkareem R, Reda A, Khalil M, Farouk M, Abdelraouf A, Mohammed N. Laparoscopic excision of a giant adrenal myelolipoma and review of the literature. AFRICAN JOURNAL OF UROLOGY 2018; 24:108-113. [DOI: 10.1016/j.afju.2018.02.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
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Giant bilateral adrenal myelolipoma:Case presentation and a brief literature review. Urol Case Rep 2018; 18:67-69. [PMID: 29785374 PMCID: PMC5958930 DOI: 10.1016/j.eucr.2018.03.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/03/2018] [Revised: 03/07/2018] [Accepted: 03/09/2018] [Indexed: 11/22/2022] Open
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[Benign mesenchymal tumors of the abdomen]. Radiologe 2018; 58:25-35. [PMID: 29318350 DOI: 10.1007/s00117-017-0338-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
BACKGROUND Benign mesenchymal tumors of the abdomen are-except for hemangiomas-rare neoplasms that are associated with potentially life-threatening complications. Thus, awareness of the most important entities and knowledge of their imaging characteristics is fundamental for the radiologist. OBJECTIVES An overview of the most frequent benign abdominal soft tissue tumors and their common imaging features is given. Furthermore, other facts concerning the individual entities that are relevant to radiologic practice are outlined. MATERIALS AND METHODS A literature search and evaluation of the institutional image database were performed. RESULTS Hemangiomas, desmoid tumors, lipomas and lipoma variants are presented. Typical characteristics and radiological signs (e. g., iris diaphragm sign, band sign, India ink artifact) are elucidated and illustrated by image examples. CONCLUSION Despite the lack of distinctive imaging features, there are a few radiological characteristics suggestive for each of the entities that in many cases allow for correct diagnosis. In cases of doubt, biopsy or surgery is necessary to distinguish them from malignancies.
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[FIVE CASES OF ADRENAL MYELOLIPOMA WITH SURGICAL TREATMENT; A CLINICAL REVIEW]. Nihon Hinyokika Gakkai Zasshi 2018; 109:178-183. [PMID: 31631080 DOI: 10.5980/jpnjurol.109.178] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
(Purpose) We report five cases of adrenal myelolipoma with surgical treatment, and analyze the patients' background and clinical courses. (Patients and methods) From 2004 to 2017, five patients diagnosed adrenal myelolipoma were underwent surgical treatment at our hospital. We investigate the patients' background and clinical courses retrospectively. (Results) Median age was 53 years old. Four of them were male and one was female. The tumor was located on the right side in four cases and the left side in one case. All cases were incidentally found by abdominal ultrasound or computer tomography (CT) during a medical check or image examination for other disease. Whereas all cases were asymptomatic, they have past history either hypertension, diabetes or obesity. The tumor size at the time of diagnosis was from 28 mm to 80 mm (median 58 mm). All tumors were nonfunctioning, and diagnosed by CT scan preoperatively. The median tumor size at the time of operation was 66 mm. (Conclusion) We report five cases of adrenal myelolipoma treated surgically. The opportunity of encountering this disease has been increasing as the recent improvement of diagnostic imaging such as CT, MRI, and etc. However, there is no widely-accepted treatment algorithm. We should manage them carefully, because spontaneous rupture of adrenal myelolipoma has been reported in some cases.
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Abstract
INTRODUCTION Adrenal myelolipoma is an invariably benign neoplasm of the adrenal gland that is the second most common primary adrenal incidentaloma following adrenocortical adenomas. It is composed of elements of adipose tissue and extramedullary hematopoiesis. Hypotheses on stem cells and hormonal factors have been formulated regarding its pathogenesis that is still obscure. Despite its benign behavior, adrenal myelolipoma is clinically relevant as it might cause significant difficulties in the differential diagnosis of adrenal tumors. METHODS We have reviewed 420 cases reported between 1957 and 2017 on adrenal myelolipoma retrieved from PubMed and Scopus databases and also 20 of our case series to provide a comprehensive analysis of their pathology, epidemiological and clinical features. RESULTS AND CONCLUSIONS The average age for its diagnosis was 51 years, and no gender difference was observed. The average size of tumors was 10.2 cm. Congenital adrenal hyperplasia was associated to 10% of all cases analyzed, while other adrenal hypersecretory disorders (cortisol, aldosterone) were found in 7.5% of cases. Computed tomography and magnetic resonance imaging can be reliably used for its differential diagnosis. If the diagnosis of an adrenal myelolipoma is unambiguous, and no associated symptoms or hormonal activity are established, surgical intervention is usually not necessary.
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Affiliation(s)
- Ábel Decmann
- 2nd Department of Medicine, Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary
| | - Pál Perge
- 2nd Department of Medicine, Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary
| | - Miklós Tóth
- 2nd Department of Medicine, Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary
| | - Peter Igaz
- 2nd Department of Medicine, Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary.
- MTA-SE Molecular Medicine Research Group, Hungarian Academy of Sciences and Semmelweis University, Szentkirályi str. 46., Budapest, 1088, Hungary.
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Feng X, Kline G. Massive adrenal incidentalomas and late diagnosis of congenital adrenal hyperplasia in prostate cancer. Endocrinol Diabetes Metab Case Rep 2017; 2017:EDM170108. [PMID: 29118987 PMCID: PMC5670323 DOI: 10.1530/edm-17-0108] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2017] [Accepted: 10/03/2017] [Indexed: 11/08/2022] Open
Abstract
In a 61-year-old Caucasian male with prostate cancer, leuprolide and bicalutamide failed to suppress the androgens. He presented to endocrinology with persistently normal testosterone and incidental massive (up to 18 cm) bilateral adrenal myelolipomas on CT scan. Blood test did not reveal metanephrine excess. The patient was noted to have short stature (151 cm) and primary infertility. Elementary school photographs demonstrated precocious puberty. Physical examination revealed palpable abdominal (adrenal) masses. Abiraterone and glucocorticoid treatment was commenced with excellent suppression of testosterone. Genetic testing revealed a mutation in CYP21A2 confirming 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH). Association of large myelolipomas with CAH has been reported in the literature. Our case highlights the importance of considering CAH in patients with non-suppressed testosterone despite androgen deprivation therapy. Large myelolipomas should raise the suspicion of congenital adrenal hyperplasia. Learning points Adrenal myelolipomas are rare benign lesions that are more common in patients with longstanding untreated congenital adrenal hyperplasia thought to be due to ACTH stimulation.Consider undiagnosed congenital adrenal hyperplasia in patients with adrenal myelolipoma.Glucocorticoid replacement may be an efficacious treatment for patients with prostate cancer and CAH. Abiraterone therapy has a risk of adrenal crisis if glucocorticoids are not replaced.
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Affiliation(s)
- Xin Feng
- Division of Endocrinology, Department of Medicine, University of Calgary, Calgary, Alberta,Canada
| | - Gregory Kline
- Division of Endocrinology, Department of Medicine, University of Calgary, Calgary, Alberta,Canada
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Campbell MJ, Obasi M, Wu B, Corwin MT, Fananapazir G. The radiographically diagnosed adrenal myelolipoma: what do we really know? Endocrine 2017; 58:289-294. [PMID: 28866749 DOI: 10.1007/s12020-017-1410-6] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2017] [Accepted: 08/24/2017] [Indexed: 12/19/2022]
Abstract
PURPOSE Adrenal myelolipomas are uncommon, benign tumors of the adrenal glands that are not routinely evaluated for autonomous hormone secretion or followed by repeat imaging, but may lead to retroperitoneal hemorrhage. Little is known about the natural history these tumors with the majority of previous publications being confined to case reports and small case series. METHODS We reviewed the computed tomography (CT) reports of 62,279 patients evaluated at a tertiary referral center between 2002 and 2015 for the presence of an adrenal myelolipoma >1 cm in size. We then reviewed the CT imaging and patient charts to determine tumor size, growth, evidence of retroperitoneal hemorrhage, and endocrine dysfunction. RESULTS We identified 150 (0.24%) patients with 155 radiographically diagnosed adrenal myelolipomas found on a CT scan during our study period. The median tumor size at discovery was 2.1 cm. Twelve (7.7%) tumors were >6 cm. Sixty-nine (46%) patients had a follow-up CT with a mean time interval between scans of 3.9 years. Eleven tumors (16%) grew over our follow-up period with a median growth rate of 0.16 cm/year. No patient developed a retroperitoneal hemorrhage. Twenty (13%) patients underwent an evaluation for endocrine dysfunction. Three patients had evidence of hypercortisolism and one patient had primary aldosteronism. CONCLUSION The majority of radiographically diagnosed adrenal myelolipomas found on abdominal CT imaging are small and slow growing. Retroperitoneal hemorrhage is uncommon, but the rate of associated endocrine dysfunction may be underestimated.
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Affiliation(s)
- Michael J Campbell
- Departments of Surgery and Radiology, University of California, Davis Medical Center, Sacramento, CA, USA.
| | - Mary Obasi
- Departments of Surgery and Radiology, University of California, Davis Medical Center, Sacramento, CA, USA
| | - Bingling Wu
- Departments of Surgery and Radiology, University of California, Davis Medical Center, Sacramento, CA, USA
| | - Michael T Corwin
- Departments of Surgery and Radiology, University of California, Davis Medical Center, Sacramento, CA, USA
| | - Ghaneh Fananapazir
- Departments of Surgery and Radiology, University of California, Davis Medical Center, Sacramento, CA, USA
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Abstract
The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours. Amongst them, schwannoma and haematological tumours are newly documented. The major updates in adrenal cortical lesions are noted in the genetics of the cortical carcinoma and cortical adenoma based on the data from The Cancer Genome Atlas (TCGA). Also, a system for differentiation of oncocytoma from oncocytic cortical carcinoma is adopted. Tumours of the adrenal medulla and extra-adrenal paraganglia comprise pheochromocytoma, paraganglioma (head and neck paraganglioma and sympathetic paraganglioma), neuroblastic tumours (neuroblastoma, nodular ganglioneuroblastoma, intermixed ganglioneuroblastoma, and ganglioneuroma), composite pheochromocytoma, and composite paraganglioma. In this group, neuroblastic tumours are newly included in the classification. The clinical features, histology, associated pathologies, genetics, and predictive factors of pheochromocytoma and paraganglioma are the main changes introduced in this chapter of WHO classification of endocrine tumours. The term "metastatic pheochromocytoma/paraganglioma" is used to replace "malignant pheochromocytoma/paraganglioma." Also, composite pheochromocytoma and composite paraganglioma are now documented in separate sections instead of one. Overall, the new classification incorporated new data on pathology, clinical behaviour, and genetics of the adrenal tumours that are important for current management of patients with these tumours.
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Affiliation(s)
- Alfred King-Yin Lam
- Cancer Molecular Pathology, School of Medicine and Menzies Health Institute Queensland, Griffith University, Gold Coast, Q4222, Australia.
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Taffurelli G, Ricci C, Casadei R, Selva S, Minni F. Open adrenalectomy in the era of laparoscopic surgery: a review. Updates Surg 2017; 69:135-143. [DOI: 10.1007/s13304-017-0440-1] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2016] [Accepted: 04/01/2017] [Indexed: 12/27/2022]
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Lam AKY. Lipomatous tumours in adrenal gland: WHO updates and clinical implications. Endocr Relat Cancer 2017; 24:R65-R79. [PMID: 28143811 DOI: 10.1530/erc-16-0564] [Citation(s) in RCA: 41] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2017] [Accepted: 01/31/2017] [Indexed: 12/11/2022]
Abstract
Adrenal lipomatous tumour is a group of adrenal tumours with a significant component of adipose tissue. According to the current World Health Organization (WHO) classification of tumours of endocrine organs, adrenal myelolipoma is the only entity amongst the group of tumours being described. In the literature, other more recently documented adrenal lipomatous tumours included 24 lipomas, 32 teratomas and 16 angiomyolipomas. Rare fatty tumours of the adrenal gland comprised liposarcoma, hibernoma, adrenocortical tumours with fat component and rare adrenal tumours with fat component. Myelolipoma comprises approximately 3% of primary adrenal tumour. It is noted more commonly in females and in the right adrenal gland. Approximately 40 bilateral myelolipomas were reported. The tumour is most frequently recorded in patients between fifth and seventh decades of life. Adrenal lipomas are often seen in males and in the right adrenal gland. They were commonly noted in patients in the sixth decade of life. The diagnosis could only be possible on examination of the surgically removed specimen. Adrenal teratomas were more common in females and with a bimodal age distribution. Slightly over 60% of the patients with adrenal teratoma are symptomatic. Adrenal angiomyolipomas were often symptomatic, more common in females and in the fifth decades of life. To conclude, adrenal lipomatous tumour is uncommon. They are often benign and non-functional. It is important to recognize the features of this group of lipomatous tumours in the adrenal gland as they are being detected on increasing incidence as a result of the wide-spread use of modern imaging modalities.
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Affiliation(s)
- Alfred King-Yin Lam
- Cancer Molecular PathologyMenzies Health Institute Queensland and School of Medicine, Griffith University, Gold Coast, Australia
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