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Mochizuki Y, Araki K, Mitsunari K, Iwata T, Matsuo T, Hakariya T, Ohba K, Imamura R. Spontaneous Rupture of Adrenal Myelolipoma: A Case Report With Review of the Literature. IJU Case Rep 2025; 8:253-256. [PMID: 40336729 PMCID: PMC12055212 DOI: 10.1002/iju5.70018] [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: 11/23/2024] [Revised: 02/26/2025] [Accepted: 03/14/2025] [Indexed: 05/09/2025] Open
Abstract
Introduction Adrenal myelolipoma is often asymptomatic and is commonly diagnosed using imaging modalities. Herein, we report a case of adrenal myelolipoma detected through spontaneous rupture and review similar cases from the literature. Case Presentation A 27-year-old man was admitted to our hospital with sudden right flank pain. Abdominal computed tomography revealed right retroperitoneal hemorrhage due to a spontaneous rupture of a retroperitoneal tumor. He underwent emergency transcatheter arterial embolization because of his severe symptoms and poor general condition, which included abdominal pain and progressive anemia. We performed laparoscopic resection of the tumor with the right adrenal gland 3 months after the emergency embolization. Consequently, a definitive diagnosis of adrenal myelolipoma was confirmed. Conclusion Adrenal myelolipoma, although scarcely reported, can rupture spontaneously and lead to severe consequences. Treatment options may include percutaneous arterial embolization and subsequent surgery.
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Affiliation(s)
- Yasushi Mochizuki
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Kyohei Araki
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Kensuke Mitsunari
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Takahisa Iwata
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Tomohiro Matsuo
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Tomoaki Hakariya
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Kojiro Ohba
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
| | - Ryoichi Imamura
- Department of UrologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
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Schyrr F, Alonso-Calleja A, Vijaykumar A, Sordet-Dessimoz J, Gebhard S, Sarkis R, Bataclan C, Ferreira Lopes S, Oggier A, de Leval L, Nombela-Arrieta C, Naveiras O. Inducible CXCL12/CXCR4-dependent extramedullary hematopoietic niches in the adrenal gland. Blood 2024; 144:964-976. [PMID: 38728427 DOI: 10.1182/blood.2023020875] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2023] [Revised: 04/24/2024] [Accepted: 04/24/2024] [Indexed: 05/12/2024] Open
Abstract
ABSTRACT Adult hematopoietic stem and progenitor cells (HSPCs) reside in the bone marrow (BM) hematopoietic niche, which regulates HSPC quiescence, self-renewal, and commitment in a demand-adapted manner. Although the complex BM niche is responsible for adult hematopoiesis, evidence exists for simpler, albeit functional and more accessible, extramedullary hematopoietic niches. Inspired by the anecdotal description of retroperitoneal hematopoietic masses occurring at higher frequency upon hormonal dysregulation within the adrenal gland, we hypothesized that the adult adrenal gland could be induced into a hematopoietic-supportive environment in a systematic manner, thus revealing mechanisms underlying de novo niche formation in the adult. Here, we show that upon splenectomy and hormonal stimulation, the adult adrenal gland of mice can be induced to recruit and host functional HSPCs, capable of serial transplantation, and that this phenomenon is associated with de novo formation of platelet-derived growth factor receptor α/leptin receptor (PDGFRα+/LEPR+/-)-expressing stromal nodules. We further show in CXCL12-green fluorescent protein reporter mice that adrenal glands contain a stromal population reminiscent of the CXCL12-abundant reticular cells, which compose the BM HSPC niche. Mechanistically, HSPC homing to hormonally induced adrenal glands was found dependent on the CXCR4-CXCL12 axis. Mirroring our findings in mice, we found reticular CXCL12+ cells coexpressing master niche regulator FOXC1 in primary samples from human adrenal myelolipomas, a benign tumor composed of adipose and hematopoietic tissue. Our findings reignite long-standing questions regarding hormonal regulation of hematopoiesis and provide a novel model to facilitate the study of adult-specific inducible hematopoietic niches, which may pave the way to therapeutic applications.
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Affiliation(s)
- Frédérica Schyrr
- Laboratory of Regenerative Hematopoiesis, Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland
- Laboratory of Regenerative Hematopoiesis, Swiss Institute for Experimental Cancer Research and Institute of Bioengineering, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
| | - Alejandro Alonso-Calleja
- Laboratory of Regenerative Hematopoiesis, Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland
- Laboratory of Regenerative Hematopoiesis, Swiss Institute for Experimental Cancer Research and Institute of Bioengineering, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
| | - Anjali Vijaykumar
- Department of Medical Oncology and Hematology, Comprehensive Cancer Center, University of Zürich and University Hospital Zürich, Zürich, Switzerland
| | - Jessica Sordet-Dessimoz
- Histology Core Facility, School of Life Sciences, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
| | - Sandra Gebhard
- Centre vaudois anorexie boulimie, Espace CHUV Service de psychiatrie de liaison, Département de psychiatrie, Lausanne University Hospital, Lausanne, Switzerland
| | - Rita Sarkis
- Laboratory of Regenerative Hematopoiesis, Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland
- Laboratory of Regenerative Hematopoiesis, Swiss Institute for Experimental Cancer Research and Institute of Bioengineering, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
| | - Charles Bataclan
- Laboratory of Regenerative Hematopoiesis, Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland
| | - Silvia Ferreira Lopes
- Laboratory of Regenerative Hematopoiesis, Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland
| | - Aurélien Oggier
- Laboratory of Regenerative Hematopoiesis, Swiss Institute for Experimental Cancer Research and Institute of Bioengineering, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
| | - Laurence de Leval
- Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
| | - César Nombela-Arrieta
- Department of Medical Oncology and Hematology, Comprehensive Cancer Center, University of Zürich and University Hospital Zürich, Zürich, Switzerland
| | - Olaia Naveiras
- Laboratory of Regenerative Hematopoiesis, Department of Biomedical Sciences, University of Lausanne, Lausanne, Switzerland
- Laboratory of Regenerative Hematopoiesis, Swiss Institute for Experimental Cancer Research and Institute of Bioengineering, École Polytechnique Fédérale de Lausanne, Lausanne, Switzerland
- Hematology Service, Departments of Oncology and Laboratory Medicine, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
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Kennedy DJ, Ferreira HMT, Dobromylskyj MJ, Hobi S, Almendros A, Bęczkowski PM. Splenic Myelolipomas in the Domestic Cat-To Operate or Not to Operate? Animals (Basel) 2024; 14:1700. [PMID: 38891746 PMCID: PMC11171139 DOI: 10.3390/ani14111700] [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: 04/30/2024] [Revised: 05/30/2024] [Accepted: 06/04/2024] [Indexed: 06/21/2024] Open
Abstract
Myelolipoma is a benign, typically inert neoplasm of uncertain aetiology that is rarely reported in cats. Although commonly asymptomatic, in some cases, myelolipomas can cause abdominal discomfort or present with rupture and haemorrhage. Here, we describe a rare case of a splenic myelolipoma in a Domestic Long Hair cat in which, after extensive diagnostic investigations, clinical signs of hyporexia and weight loss were attributable to the presence of an intra-abdominal mass. The patient was treated by splenectomy and unexpectedly died during the post-operative period. Although splenectomy appears to be a sensible intervention in symptomatic patients, the optimal management of splenic myelolipomas in cats remains unknown. The risk-benefit ratio of surgical management needs to be carefully considered, and therapeutic intervention should be tailored individually to each patient.
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Affiliation(s)
- Diana J. Kennedy
- Beaumont Veterinary Hospital, 172 Oxford Rd., Kidlington OX5 1EA, UK;
| | | | | | - Stefan Hobi
- Department of Veterinary Clinical Sciences, Jockey Club College of Veterinary Medicine and Life Sciences, City University of Hong Kong, Kowloon Tong, Hong Kong SAR, China; (S.H.); (A.A.)
| | - Angel Almendros
- Department of Veterinary Clinical Sciences, Jockey Club College of Veterinary Medicine and Life Sciences, City University of Hong Kong, Kowloon Tong, Hong Kong SAR, China; (S.H.); (A.A.)
| | - Paweł M. Bęczkowski
- Department of Veterinary Clinical Sciences, Jockey Club College of Veterinary Medicine and Life Sciences, City University of Hong Kong, Kowloon Tong, Hong Kong SAR, China; (S.H.); (A.A.)
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Shen X, Yao Q, Qi X, Ma L. Malicious Tumor? Pathological Fracture of the Femur in Children Caused by Myelolipoma: A Case Report and Review of Literatures. Case Rep Oncol Med 2024; 2024:5838618. [PMID: 38778879 PMCID: PMC11111296 DOI: 10.1155/2024/5838618] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2023] [Revised: 03/03/2024] [Accepted: 04/17/2024] [Indexed: 05/25/2024] Open
Abstract
Myelolipoma is a kind of benign lipoma containing myeloid cells. It is a rare type of tumor that typically presents as an occasional adrenal tumor, generally manifesting as a nonfunctional adrenal mass. Although it can occur in extra-adrenal tissues, its occurrence in bone tissue is extremely rare. Most cases are discovered accidentally during physical examinations of adults, and there are currently no reports of cases with pathological fractures as the main symptoms. We present a case of a 15-year-old teenager who developed a pathological fracture caused by femoral myelolipoma. The diagnosis of the specific type of bone tumor of the patient was determined through pathology and imaging. To treat the condition, we utilized a technique known as the "soft drill" to fully access the tumor space, remove the bone septum, and scrape away the diseased tissue. The fracture was then stabilized using a hybrid external fixation. After a 2-year follow-up period, there was no recurrence of the bone tumor. This case is the first case of intraosseous myelolipoma that occurred in a minor with the initial symptom of pathological fracture, filling the gap in our existing body of knowledge and providing a reference for the treatment of this type of intraosseous myelolipoma.
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Affiliation(s)
- Xiaoyu Shen
- Department of Orthopaedic Surgery, Hebei Medical University Third Hospital, Shijiazhuang 050035, China
| | - Qiang Yao
- Internal Medicine, The First Hospital of Hebei Medical University, Shijiazhuang 050000, China
| | - Xiangbei Qi
- Department of Orthopaedic Surgery, Hebei Medical University Third Hospital, Shijiazhuang 050035, China
| | - Lijie Ma
- Department of Orthopaedic Surgery, Hebei Medical University Third Hospital, Shijiazhuang 050035, China
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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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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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Afzal A, Asadbeigi SN, Kamal M, Hassell L, Fung KM. Intramedullary/intraosseous myelolipoma in a patient with pathologic fracture. SURGICAL AND EXPERIMENTAL PATHOLOGY 2022. [DOI: 10.1186/s42047-021-00102-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Intramedullary lipomatous tumors are rare and mostly reported in the metaphysis of the femur, tibia, and fibula. Myelolipomas are very rare tumors composed of adipose tissue and hematopoietic cells commonly reported in adrenal gland. We report the first reported case of intraosseous myelolipoma with a pathologic fracture in a young woman.
Case presentation
Patient is a young woman who carried a diagnosis of systemic lupus erythematosus, antiphospholipid syndrome, and hyperparathyroidism. Radiologic studies were done after the patient reported right lower leg pain lasting for a month. Radiologic examination showed a large osteolytic lesions with pathologic fracture affecting the medial aspect of the proximal tibia with extensive soft tissue calcification. The initial clinical and radiographic suspicion was brown tumor secondary to the hyperparathyroidism. Curettage of the lesion yielded large fragments of mature adipose tissue punctuated by a variable amount of mature hematopoietic cells. The histopathologic features were suggestive of myelolipoma. The overall radiographic and histopathologic features supported a diagnosis of myelolipoma. The diagnosis of intraosseous myelolipoma can be challenging in small samples such as core biopsies.
Conclusions
The differential diagnoses of intraosseous myelolipoma include normal bone marrow, intramedullary hematopoiesis, and other benign lesions. The gold standard diagnosis is histopathologic examination. However, clinical and radiographic features have important roles in the diagnosis of this rare lesion. Due to the rarity of this tumor and lack of formal guidelines for management, the case-to-case basis treatment is recommended.
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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: 60] [Impact Index Per Article: 15.0] [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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Gug D, Park HY, Lee BL, Kim KH, Lee JE, Park JK. A Case of Adrenal Myelolipoma Associated with Hereditary Spherocytosis. CLINICAL PEDIATRIC HEMATOLOGY-ONCOLOGY 2021. [DOI: 10.15264/cpho.2021.28.2.98] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Affiliation(s)
- Dahui Gug
- Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Ha Young Park
- Department of Pathology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Bo Lyun Lee
- Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Kwang Hoon Kim
- Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Jeong Eun Lee
- Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
| | - Ji Kyoung Park
- Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
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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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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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12
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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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Zhang HZ, Wang SY. Primary adrenal diffuse large B cell lymphoma arising within an adrenal myelolipoma: A case report and review of the literature. Curr Probl Cancer 2021; 45:100724. [PMID: 33744015 DOI: 10.1016/j.currproblcancer.2021.100724] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2020] [Revised: 01/23/2021] [Accepted: 02/09/2021] [Indexed: 10/22/2022]
Abstract
Adrenal myelolipoma (AML) is a benign tumor that consists of mature adipose tissue and bone marrow elements. We report a case of a 57-year-old woman who presented with complaint of lower abdominal discomfort. Computed tomography scan of abdomen and pelvis revealed a mass in the left adrenal measuring 2.0 cm which was radiologically considered to be AML. Pathological evaluation of the lesion showed foci of lymphoid aggregate in a background of AML that were confirmed to be diffuse large B cell lymphoma by immunohistochemistry and gene rearrangement. To our knowledge, this collision tumor has not been reported previously. The clinical, radiological, pathological features, and treatment are discussed.
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Affiliation(s)
- Hui-Zhi Zhang
- Department of Pathology, Ningbo Diagnostic Pathology Center, Ningbo, Zhejiang, China.
| | - Su-Ying Wang
- Department of Pathology, Ningbo Diagnostic Pathology Center, Ningbo, Zhejiang, China
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14
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Un incidentalome inhabituel. Ann Pathol 2020; 40:488-490. [DOI: 10.1016/j.annpat.2020.04.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2016] [Accepted: 04/28/2020] [Indexed: 11/17/2022]
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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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16
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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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17
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Gomez M, Mehta R. A rare case of adrenal collision tumor: Myelolipoma and schwannoma in the adrenal gland. SAGE Open Med Case Rep 2020; 8:2050313X20921074. [PMID: 32477562 PMCID: PMC7234350 DOI: 10.1177/2050313x20921074] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2019] [Accepted: 03/27/2020] [Indexed: 11/16/2022] Open
Abstract
Adrenal collision tumors refer to coexistence of two adjacent, but histologically distinct, neoplasms involving the adrenal gland without histologic admixture at the interface. Myelolipoma is a rare but benign neoplasm. As its name implies, displays both mature adipose tissue and hematopoietic elements. These are usually unilateral and asymptomatic. Schwannomas, tumors derived from the peripheral nerve sheath, are also uncommon lesions in the adrenal gland. Here, we present a rare case of a 65-year-old male with clinical history of abdominal aortic aneurysm who was found to have a 13-cm “incidentaloma” by interventional radiologist during his aortic endograft control. Giving the size of the mass, team decided to excise it, and histopathological examination was performed. While most incidentally discovered adrenal tumors are benign, surgical excision is recommended in large lesions to exclude malignancy, avoid hemorrhage, and/or acute adrenal insufficiency. Given the rarity of such entity, its clinical course and prognosis remains unclear.
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Affiliation(s)
- Mariangela Gomez
- Department of Pathology, SUNY Upstate Medical University, Syracuse, NY, USA
| | - Rohin Mehta
- Department of Pathology, SUNY Upstate Medical University, Syracuse, NY, USA
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18
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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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19
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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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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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21
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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: 42] [Impact Index Per Article: 5.3] [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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22
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Aguilera N, Auerbach A. Extra-adrenal myelolipoma presenting in the spleen: A report of two cases. HUMAN PATHOLOGY: CASE REPORTS 2016. [DOI: 10.1016/j.ehpc.2016.01.003] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022] Open
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Abstract
Adrenal myelolipomas (AMLs) are rare, benign neoplasms of the adrenal gland with varied clinical presentations. The rarity of these tumors precludes any case-controlled or randomized study into their management. The available literature is limited to case reports and short series from referral centers. This review is an effort to put the available literature into perspective such that clinical decision making can be done with some clarity. The PubMed and Cochrane databases were searched with key words Adrenal Myelolipoma, Adrenal Incidentaloma (AI) and Adrenal Collision Tumor (ACT). From over 1300 search results, 547 relevant publications dating from 1954 to 2014 were reviewed. Details of about 1231 AMLs in the indexed literature were analyzed. Increasing usage of imaging studies has significantly increased the discovery of AMLs. Although AMLs are benign tumors, those measuring larger than 6 cm are prone to rupture and hemorrhage. Thorough endocrine work-up may benefit a selected group of patients, especially those who are hypertensive, diabetic/pre-diabetic, young patients (<50 years) and those with bilateral AML. Regular observation is needed for AML patients who are being treated non-operatively, as many of them may require surgery during follow-up. Although the AACE/AAES guidelines for AI (2009) exclude AML from mandatory metabolic work-up for a newly discovered AI, we feel that a significant number of patients with AML would benefit from metabolic work-up. In the literature, endocrine dysfunction in AML is 7% as compared with 11% in AI. Endocrine dysfunction in AML is probably underdiagnosed.
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Affiliation(s)
- Vasanth G Shenoy
- Department of Urology, Dr. B.R. Ambedkar Medical College, Bangalore, Karnataka, India
| | - Anuroop Thota
- Department of Urology, Dr. B.R. Ambedkar Medical College, Bangalore, Karnataka, India
| | - Ravi Shankar
- Department of Urology, Dr. B.R. Ambedkar Medical College, Bangalore, Karnataka, India
| | - Mallikarjun G Desai
- Department of Urology, Dr. B.R. Ambedkar Medical College, Bangalore, Karnataka, India
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Giant bilateral adrenal myelolipoma with congenital adrenal hyperplasia. Case Rep Surg 2014; 2014:728198. [PMID: 25140269 PMCID: PMC4124659 DOI: 10.1155/2014/728198] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2014] [Revised: 05/29/2014] [Accepted: 06/03/2014] [Indexed: 11/18/2022] Open
Abstract
Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing's syndrome, Addison's disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.
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Ramirez M, Misra S. Adrenal myelolipoma: To operate or not? A case report and review of the literature. Int J Surg Case Rep 2014; 5:494-6. [PMID: 24995663 PMCID: PMC4147662 DOI: 10.1016/j.ijscr.2014.04.001] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2014] [Revised: 03/21/2014] [Accepted: 04/01/2014] [Indexed: 12/13/2022] Open
Abstract
INTRODUCTION Myelolipoma is a rare, benign neoplasm that predominantly occurs in the adrenal gland and is composed of mature adipose tissue and scattered islands of hematopoietic elements. Although usually small and asymptomatic, there are some cases of adrenal myelolipoma that cause symptoms such as chronic pain. Elective surgery can prevent a more severe symptom presentation and life threatening progression while also allowing accurate diagnosis in patients with tumors larger than 6cm. PRESENTATION OF CASE This report presents an unusual case of a 28-year-old female who suffered with chronic pain from a growing left-sided adrenal myelolipoma. Without the financial means for additional testing along with many symptoms warranting a high suspicion for malignancy, this patient decided to have the mass surgically excised, which was both diagnostic and curative. DISCUSSION For this specific patient, surgery was the most cost effective option, as well as both diagnostic and curative. Surgery can also prevent complications such as spontaneous rupture and hemorrhage of the mass, which can lead to subsequent cardiovascular shock. CONCLUSION Management of adrenal myelolipoma should be considered on an individual basis. Although it is a benign tumor, surgery plays an important role for symptomatic cases and those lesions that cannot be distinguished reliably from malignancy.
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Affiliation(s)
- Mary Ramirez
- Texas Tech University Health Sciences Center, 1400 South Coulter Street, Amarillo, TX 79106-1786, United States.
| | - Subhasis Misra
- Texas Tech University Health Sciences Center, 1400 South Coulter Street, Amarillo, TX 79106-1786, United States.
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