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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., Competing Interests: Declarations. Ethical approval: Ethical approval was provided for this study from the Tallaght Hospital Research Committee. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s).)

Objective: Isolated adrenal myelolipoma (IAM) is an uncommon, nonfunctioning tumour of the adrenal gland, primarily composed of adipose tissue and hematopoietic trilinear cells. The etiopathogenesis and clinical relevance of this neoplasm remain poorly understood. However, similar myelolipomatous alterations can also occur within adrenocortical adenomas. This report presents our findings on IAM and adrenocortical adenomas with a myelolipomatous component (AMC), focusing on comparing these cases' clinical, demographic, and pathological characteristics., Methods: Data from twenty patients were retrospectively analyzed, including twelve diagnosed with IAM and eight with AMC, all of whom underwent adrenalectomy. In the IAM cohort, surgical intervention was primarily indicated based on tumour size and/or related symptoms, while in AMC cases, the indication was often driven by hormonal activity., Results: Patients with IAM had an average age of 52.5 years, with a predominance of female patients (75%). A significant proportion of this group was obese, with concomitant hypertension (HT) and/or type 2 diabetes mellitus (DM). The tumours were predominantly right-sided, with a median size of 69.0 ± 40.0 mm. Notably, 58% of patients with IAM presented with flank or abdominal pain attributed to the mass effect. None of the IAM cases exhibited hormonal activity. Conversely, the AMC group had a younger average age of 46 years, with a high prevalence also in female patients (63%). The tumours were generally smaller, with a median size of 40.0 ± 16.0 mm, and were mostly left-sided. All patients in this group had a history of HT and/or DM, with six exhibiting hormonally active tumours, which manifested as various clinical syndromes, including Cushing syndrome, pheochromocytoma, and Conn syndrome., Conclusions: While IAM and AMC share several common features, they also demonstrate distinct differences. The presence of endocrinological syndromes was more frequent in AMC cases, whereas IAM cases rarely showed hormonal activity. The most pronounced difference between the two groups was the tumour size at diagnosis, which contributed to the varying clinical presentations upon hospital admission. Furthermore, the high prevalence of obesity, HT, and DM in both groups suggests that these comorbidities may play a role in the development of myelolipomatous patterns observed in these tumours., Competing Interests: The authors declare no conflict of interest., (© 2024 The Author(s).)

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., Competing Interests: Disclosure I.B. reports consulting/advisory board/data safety monitoring board fees to institution from HRA Pharma, Recordati, Corcept, Sparrow, Xeris, NovoNordisk, AstraZeneca, Crinetics, Neurocrine, Diurnal, Adrenas, Spruce, and Adrenas and funding for investigator-initiated research from HRA Pharma and Recordati all unrelated to the current paper. A.V. reports consulting fees from Corcept, Mineralys, and HRA Pharma, all unrelated to the current work. A.H. reports research funding from Corcept, AstraZeneca, and Spruce Biosciences consulting and advisory board fees from Corcept, all unrelated to the current paper. O.H. reports advisory board participation with Corcept Therapeutics, Neurocrine Biosciences, Recordati Rare Diseases, Xeris Pharma, Amryt Pharma, and Lantheus. The remaining authors have no conflicts of interest to disclose., (Copyright © 2024 AACE. All rights reserved.)

Background: Myelolipoma is an uncommon benign tumor composed of mature adipose tissue and hematopoietic elements. These tumors generally affect the adrenal glands, with anomalous presentations being rare and with few cases described in the literature. Most myelolipomas are asymptomatic and discovered incidentally, either through imaging tests or at autopsies. However, depending on the location and size of the lesion, myelolipomas can cause symptoms of mass effect. This article aims to report a very rare presentation of a symptomatic primary myelolipoma affecting the ribs., Case Presentation: A 21-year-old white female patient presented with a complaint of burning chest pain over 3 months, with gradual worsening in intensity, accompanied by a progressively growing bulge in the right thoracic wall. The patient underwent thoracotomy of the fifth and sixth ribs with complete excision of the lesion with a safety margin. Thoracic wall reconstruction was performed using a polypropylene mesh. The patient had a good postoperative course and was discharged on postoperative day 3. Histopathological examination revealed a histological image consistent with myelolipoma., Conclusions: This report underscores the importance of considering a myelolipoma diagnosis for tumor masses in the ribs., (© 2024. The Author(s).)

Mediastinal myelolipoma is a rare condition and has no obvious symptoms. In the past 20 years, some clinical cases have been documented. However, the literature has not systematically summarized its imaging features. The aim of this paper is to present a case of right posterior mediastinal myelolipoma and to review and summarize its imaging features. Twenty-six articles were included in our study, which included a total of 26 patients and 33 lesions; 90.9% of the lesions were located in the mediastinum at the level from the 8th thoracic vertebral body to the thoracic 12th vertebral body. Among the cases with unilateral mediastinum, 68.4% of the cases were located in the right posterior mediastinum. Bilateral lesions accounted for almost one-fourth of all lesions. After contrast medium was injected, 93.9% of the lesions had mild to moderate enhancement; 84.8% of the lesions contained fat density; and 75.8%, 69.7%, 87.9%, and 75.8% of the lesions showed clear boundary, regular shape, heterogeneity and were encapsulated, respectively. Only 12.1% of the lesions contained calcification. An inhomogeneous mass in the right posterior mediastinum near the spine, including fat density, is the predominant imaging marker of most mediastinal myelolipomas., (© 2024. The Author(s).)

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.

Background: Adrenocortical adenoma (ADA) and myelolipoma are two common benign neoplasms of the adrenal cortex that have been reported to occur together., Case Report: A 14-year-old girl presented with the features of ACTH-independent endogenous Cushing syndrome. Abdominal CECT revealed a left adrenal 2.3 × 1.8 × 1.5 cm arterially enhancing nodular lesion with central hypodensity. Histologically, this was an ADA with oncocytic change and myelolipomatous differentiation/metaplasia., Discussion/conclusion: ADA with myelolipomatous differentiation/metaplasia can occur in the pediatric age group.

Myelolipoma is a benign non-functional tumor. Most of them are asymptomatic and discovered incidentally, either through imaging studies or at autopsy. While it most commonly occurs in the adrenal gland, it has also been reported at extra-adrenal sites. We present the case of a 65-year-old woman with a primary mediastinal myelolipoma. Computer tomographic scan of the thorax showed an ovoid tumor with well-defined borders of 6.5 × 4.2 cm, located in the posterior mediastinum. A transthoracic biopsy of the lesion was made, and the microscopic observation revealed hematopoietic cells and mature adipose tissue. Although computed tomography and magnetic resonance imaging are effective in diagnosing mediastinal myelolipoma, histopathological examination is essential for the definitive diagnosis.

A 1-year-old male Persian cat was presented for castration. Liver incarcerated in a peritoneopericardial diaphragmatic hernia (PPDH) was diagnosed through pre-anesthetic tests. Multiple homogeneous hyperechoic nodules in the hepatic parenchyma were identified using ultrasound. The nodules showed decreased attenuation compared with normal hepatic parenchyma, and the herniated hepatic parenchyma showed increased arterial and decreased portal enhancement on computed tomography. From the histopathology, we diagnosed hydropic degeneration with portal fibrosis and myelolipoma. This report presents diagnostic imaging features of hepatic myelolipoma incarcerated in a PPDH in a cat. When perfusion of the hepatic parenchyma is altered, surgical treatment should be considered., Competing Interests: The authors declare no conflicts of interest., (© 2022 The Korean Society of Veterinary Science.)

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%)., Clinical Case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively., Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications., (© 2022 Revista Medica del Instituto Mexicano del Seguro Social.)

Objectives: Adrenal adenoma with myelolipomatous degeneration (AMD) is a rarely reported and often overlooked entity. The aim of this study is to improve understanding of these lesions by characterizing the imaging findings with pathologic and clinical correlation., Methods: In the largest series to date, we report 11 nodules in 11 patients confirmed with a pathologic diagnosis of AMD. The available cross-sectional imaging and histopathologic features were reviewed by two radiologists and two pathologists, respectively. Clinical and laboratory data for each patient were obtained from the electronic medical records, when available., Results: All 11 patients had a CT prior to resection or biopsy of the adrenal nodule, with five having received an adrenal mass protocol study. An MRI was available in three patients. The median size of the nodules on imaging was 4.5 cm (range 2.8-8.7) and all but one had macroscopic fat. The largest focus of macroscopic fat had a median size of 0.7 cm (range 0.2-1.6) and on average was 14.4% the size of the tumor, using greatest dimensions. Four (36.4%) patients had a diagnosis of Cushing syndrome prior to nodule resection., Conclusions: Not all adrenal nodules with macroscopic fat on imaging are pure myelolipomas. An AMD should be considered, especially if the foci of fat are small and other features of an adenoma are present. Some may also be associated with Cushing syndrome., Advances in Knowledge: Myelolipomatous degeneration within an adrenal adenoma has only rarely been previously reported with very few reports emphasizing the imaging features. There may be an association with cortisol hypersecretion and improved recognition of this entity could lead to changes in clinical management.

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., Competing Interests: Declaration of Competing interest The authors state that there are no conflicts of interest with respect to the research, authorship, and/or publication of this article., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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., Competing Interests: Declaration of interests IB is supported by the National Institute of Diabetes and Digestive and Kidney Diseases of the National Institutes of Health (NIH) USA under award K23DK121888. The views expressed are those of the authors and not necessarily those of the National Institutes of Health. IB reports consulting with Strongbridge, HRA Pharma, Corcept, CinCor, and Sparrow Pharmaceutics, and serves on the data safety board for Adrenas Therapeutics. HF has consulted for Neurocrine Biosciences, Diurnal, Roche Diagnostics International, and Adrenas Therapeutics. The other authors declare no competing interests., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

A 38-yearr-old man presented with erectile dysfunction and infertility. On examination, he was hypertensive and detected to have a left flank mass. Blood investigations were unremarkable except raised serum noradrenaline levels. Imaging revealed multiple well-defined fat-containing hypodense lesions in left suprarenal area with largest one measuring 14×16 cm, suggestive of left adrenal myelolipoma. Diagnostic dilemma was posed due to discordance between clinical, biochemical and imaging findings. Left adrenal mass resection was planned keeping the possibility of pheochromocytoma. However, histopathology revealed it to be adrenal myelolipoma. Hypertension was resolved in the postoperative period and serum noradrenaline levels were normalised. Final diagnosis of a secretary adrenal myelolipoma was made, which is an extremely rare entity., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Mediastinal myelolipoma is extremely rare. It is a benign nonfunctioning tumor composed of hematopoietic tissue and mature fatty tissue. Although computed tomography and magnetic resonance imaging are effective in diagnosing mediastinal myelolipoma, a definitive diagnosis is difficult to establish for rare extra-adrenal myelolipomas by imaging alone. Such tumors are often misdiagnosed as malignant retropleural liposarcoma, denoting a poor prognosis. Case presentation: We herein describe a 72-year-old man with multiple bilateral paravertebral mediastinal myelolipomas and discuss the imaging findings and differential diagnoses. We used a computed tomography-guided core biopsy to attain a preoperative diagnosis. Using this technique, we avoided an unnecessary surgical procedure for the patient's asymptomatic and relatively small lesions., Conclusions: Instead of biopsy by lesion excision, we advocate conducting a precisely targeted, minimally invasive computed tomography-guided core biopsy to obtain a definitive preoperative diagnosis and thus avoid unnecessary surgery for mediastinal myelolipoma, a benign nonfunctioning tumor.

Macroscopic fat inside an adrenal lesion on CT is pathognomonic for myelolipoma, a benign neoplasm. Without macroscopic fat, it can be difficult to discern the nature of an indeterminate adrenal mass on imaging. One possible diagnosis for an indeterminate lesion without macroscopic fat is an adrenal oncocytic neoplasm (AON). Despite its typical benign presentation cases of malignant AON have been reported. Patients with malignant AON are often not surgical candidates and survival for unresected disease is 20%-35%. We present the third report of an AON of uncertain malignant potential (AONUMP) associated with a macroscopic fatty component with the largest reported size and longest duration of follow-up in the literature. This report details diagnostic and therapeutic challenges posed by AONUMP. This case highlights how pathognomonic radiographical findings associated with benign myelolipoma might mask rare, potentially malignant, lipomatous tumours., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Adrenal incidentalomas are tumors located in the adrenal glands and found on imaging done for purposes not related to adrenal disease. In other cases adrenal mases can be radiologically found when an adrenal hormone secreting tumor is suspected, such as a pheochromocytoma or Cushing's diseases. Adrenal incidentalomas may be classified as functional or non-functional based on whether they produce hormones, such as aldosterone, cortisol, and androgens, or catecholamines. Studies indicate that around 8% of adrenal incidentalomas are adrenal gland myelolipomas (AGMs). AGMs are non-malignant masses that can cause the compression of vital organs and vessels if said masses become large enough. In patients with congenital adrenal hyperplasia (CAH), adrenocorticotropic hormone (ACTH) levels tend to be elevated due to the lack of adrenal-hormone production. Patients with CAHs are treated with steroids that suppress ACTH levels and prevent adrenal gland hyperplasia. Around 10% of AGMs are found in untreated CAHs. Our patient was a 36-year-old male who was on steroids due to CAH and intermittent abdominal pain; a CT scan revealed a large left adrenal mass that was displacing organs towards the right. Pathological analysis revealed an AGM exceeding 30 x 23.6 x 16.7 cm. This AGM is one of the largest ever to be reported in the literature.

Introduction: The indication for choosing the minimally invasive approach for large adrenal mass remains controversial. This study is to assess perioperative outcomes after minimally invasive adrenalectomy (MIA) versus open adrenalectomy (OA) for adrenal mass ≥ 6 cm., Materials and Methods: A cohort of 173 patients underwent adrenalectomy for adrenal mass ≥ 6 cm in our urology center between May 2005 and April 2018 was included. MIA was performed in 96 patients, whereas 77 patients underwent OA. We performed a retrospective propensity score-matched study to compare MIA versus OA., Results: After propensity score-matched, 58 matched pairs of patients identified from each group. There were no significant differences between the groups in postoperative morbidity (p = 0.146), operative time (p = 0.163), intraoperative hypertension (p = 0.248) and drainage time (p = 0.188). Estimated blood loss, the rate of blood transfusions postoperative hospital stay was less in MIA group (p < 0.0001; p = 0.007; p < 0.0001). Total expense was significantly more in the MIA group [49379.28 (38496.69, 68008.69) CNY vs 39951.48 (30666.33, 50292.03) CNY, p = 0.001]., Conclusions: MIA results in equivalent perioperative outcomes compared with OA and is an effective and safe surgical method for patients with an adrenal mass more than 6 cm in diameter., Competing Interests: Declaration of competing interest No conflicts of interests., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

The aim of this study was to reveal clear epidemiologic and clinical characteristics of incidentally discovered adrenal masses, termed adrenal incidentalomas (AIs), and to establish appropriate managemental and therapeutic regimens in Japan. This study had been originally carried out as a project of a research proposed on behalf of the Japanese Ministry of Health, Labour and Welfare, from 1999 to 2004. This nationwide multicenter study on AIs included 3,672 cases with clinically diagnosed AIs, involving 1,874 males and 1,738 females, with mean age 58.1 ± 13.0 years (mean ± SD). In the present study, we focused on the investigation of the real prevalence of various adrenal disorders with AI. The mean nodule size of AI based on computed tomography was 3.0 ± 2.0 cm. Compared to non-functioning adenomas (NFAs), tumor diameters were significantly larger in adrenocortical carcinomas (ACCs), pheochromocytomas, cortisol-producing adenomas (CPAs), myelolipomas, metastatic tumors, cysts, and ganglioneuromas (p < 0.01). Endocrinological evaluations demonstrated that 50.8% of total AIs were non-functioning adenomas, while 10.5%, including 3.6% with subclinical Cushing's syndrome, were reported as CPAs, 8.5% as pheochromocytomas, and 5.1% as aldosterone-producing adenomas. ACCs were accounted for 1.4% (50 cases) among our series of AIs. In conclusion, while almost 50 % of AIs are non-functional adenomas, we must be particularly careful as AIs include pheochromocytomas or adrenal carcinomas, because they may be asymptomatic. To our knowledge, this is the first and the largest investigation of AI, thus providing basic information for the establishment of clinical guidelines for the management of AI.

Rationale: Congenital adrenal hyperplasia (CAH) is caused by various enzyme deficiencies, among which 21-hydroxylase (21-OH) deficiency accounts for more than 90% of cases. Neonatal screening became mandatory only a few decades ago. Many patients who were born before this went undiagnosed and some of the severely virilized females were raised as men., Patient Concerns: A 58-year old man with a history of excisional surgery in the external genitalia when he was a toddler presented with three days of dysuria and low abdominal pain., Diagnosis: The patient's laboratory results showed leukocytosis and elevated C-reactive protein (CRP); thus, the physicians decided to perform a computed tomography (CT) scan. The CT demonstrated pelvic inflammatory disease (PID), left adrenal gland myelolipoma, and a mesenteric mass. Meanwhile, we suspected CAH based on the clinical history and assessed the patient's hormone levels. Seventeen-hydroxyprogesterone (17-OH-PG) was markedly elevated and the patient was diagnosed with classic simple virilizing CAH., Interventions: Intravenous antibiotics were administered, and positron emission tomography-CT (PET-CT) was performed to evaluate any metastases., Outcomes: After 2 weeks of antibiotic treatment, CRP decreased to 0.12 mg/dL and PID was resolved. The patient opted for resection of the female genitalia along with the mesenteric and adrenal gland tumors in the near future, and was safely discharged., Lessons: The adrenal gland myelolipoma was thought to have developed as a result of a longstanding exposure to adrenocorticotropic hormone. There are controversies regarding the management of female genitalia in CAH patients who identify themselves as men. In this case, the physician and patient decided to remove the female genitalia because the surgery for the mesenteric mass was inevitable and there was a possibility of recurrent PID. To our knowledge, this is the first article to report primary mesenteric tumor in a CAH patient to date. In conclusion, patients who were born before neonatal screening for CAH became the mainstay, who are suspected to have CAH from their history, and present with abdominal pain must be diagnosed by performing an imaging study, testing levels of serum 17-OH-PG, and screening for female genitalia and adrenal gland myelolipoma.

Introduction: Myelolipomas are rare, benign neoplasms usually arising from the retroperitoneum. They represent an unusual diagnostic challenge due to their vague GI symptoms. We present a case of an 81-year-old patient complaining of severe dyspepsia. An abdominal CT scan and a fine needle biopsy lead to a diagnosis of giant retroperitoneal myelolipoma. A complete surgical resection was performed; no evidence of recurrent tumor was noted after 10 months., Areas Covered: Giant myelolipomas are very rare lesions. Clinical diagnosis of myelolipomas can be problematic due to their indefinite symptoms. CT scan and fine needle biopsy can be useful to reach a diagnosis, although they cannot be used to exclude malignancy in giant lesions. Surgery is the principal treatment if the tumor is symptomatic or > 7 cm. Follow-up is not mandatory due to the lack of any example of recurrence described in literature., Expert Commentary: Despite the size of the neoplasms, since most of the complaints are vague, patients with this diagnosis should be considered among patients with unexplained gastrointestinal symptoms. Since malignancy cannot be excluded based on preoperative and intraoperative biopsy, an aggressive surgical approach is essential.

Background: Myelolipoma is a rare benign tumor composed of mature adipose and hematopoietic tissues. Most myelolipomas are found in the adrenal glands, whereas intrathoracic myelolipoma is extremely rare. In particular, bronchial myelolipoma without the involvement of lung parenchyma has never been reported., Case Presentation: A previously healthy 38-year-old male developed dyspnea and a productive cough. Computed tomography revealed an endobronchial mass at the right bronchus intermedius and subsequent atelectasis of the right middle and lower lobes. Flexible bronchoscopy found a total obstruction of the right bronchus intermedius due to an endobronchial tumor. Using a rigid bronchoscope, the endobronchial tumor was resected and the base of the tumor was additionally ablated with a diode laser to prevent recurrence. The removed endobronchial tumor was a 13 mm × 20 mm-sized oval-shaped mass and was pathologically diagnosed as bronchial myelolipoma. Chest radiographs, obtained on the day following the procedure, showed an improvement of atelectasis, and accompanying symptoms were immediately improved. Follow-up bronchoscopy performed after 12 months evidenced no recurrence of the bronchial myelolipoma., Conclusions: We used bronchoscopic intervention in patients with solitary bronchial myelolipoma and there was no evidence of recurrence.

In this article, we describe a case of conventional epithelioid hemangioendothelioma (EHE) arising within an extra-adrenal myelolipoma. This composite neoplasm arose in the mediastinum of a 51-year-old female. The tumor was composed of a large myelolipoma that contained nodules of EHE consisting of CD31-positive epithelioid endothelial cells that grew in solid cords and were enmeshed in a basophilic hyalinized stroma. Both EHE and myelolipoma are characterized genetically by alterations of WWTR1. We demonstrated the expression of CAMTA-1 chimeric protein by immunohistochemistry both in the neoplastic endothelial cells of EHE and some of the endothelial cells lining the blood vessels in the myelolipoma. To the best of our knowledge, this is the first report of a malignant vascular neoplasm arising in association with myelolipoma.

Myelolipoma is an uncommon benign mesenchymal tumor consisting of mature adipocytes and hemopoietic elements that is mostly found in adrenal glands. Bilateral posterior mediastinal primary myelolipoma manifests extremely rarely, and only 11 such occurrences have been previously described. With no definite symptoms, pathologic evaluation is essential for diagnosis. This report presents a case of a 63-year-old man with a diagnosis of bilateral posterior mediastinal primary myelolipoma on the basis of radiologic findings who underwent surgical resection by video-assisted thoracic surgery. The aims of this report are to show the diagnosis of an unusual mediastinal lesion and improve understanding of this disease., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: To report the presence and evaluate the frequency of plasma cell neoplasms within adrenal myelolipomas., Methods: Adrenal myelolipomas within our institution were reviewed for the presence of hematologic neoplasia, and a review of the literature was performed., Results: Two (9%) of 23 adrenal myelolipomas were involved by plasma cell myeloma. The patients were 71 and 81 years old, one woman and one man, with tumors measuring 7 cm and 8.5 cm, respectively. Both tumors contained large aggregates of dysplastic plasma cells occupying at least one ×10 field and demonstrated light chain restriction. Neither had an established diagnosis of plasma cell neoplasm previously. After receiving therapy, one patient exhibited a stable clinical course 1 year after diagnosis while the other died of disease 3 years later., Conclusions: We report the first two cases of adrenal myelolipoma involved by plasma cell myeloma, a rare and subtle finding that has significant clinical implications.

Introduction: Adrenal myelolipoma (AML) is the second most common and invariably benign primary adrenal neoplasm. Due to the variable proportion of fat and hematopoietic elements and its often large size, it can cause differential diagnostic problems. Several reports confirmed the utility of miRNAs in the diagnosis of tumors, but miRNA expression in AML has not yet been investigated., Materials and Methods: Next-generation sequencing (NGS) was performed on 30 formalin-fixed, paraffin-embedded (FFPE) archived tissue samples [10 each of AML, adrenocortical adenoma (ACA), and adrenocortical carcinoma (ACC)]. Validation was performed by real-time quantitative reverse transcription polymerase chain reaction on a cohort containing 41 further FFPE samples (15 AML, 14 ACA, and 12 ACC samples). Circulating miRNA counterparts of significantly differentially expressed tissue miRNAs were studied in 33 plasma samples (11 each of ACA, ACC, and AML)., Results: By NGS, 256 significantly differentially expressed miRNAs were discovered, and 8 of these were chosen for validation. Significant overexpression of hsa-miR-451a, hsa-miR-486-5p, hsa-miR-363-3p, and hsa-miR-150-5p was confirmed in AML relative to ACA and ACC. hsa-miR-184, hsa-miR-483-5p, and hsa-miR-183-5p were significantly overexpressed in ACC relative to ACA but not to AML. Circulating hsa-miR-451a and hsa-miR-363-3p were significantly overexpressed in AML, whereas circulating hsa-miR-483-5p and hsa-miR-483-3p were only significantly overexpressed in ACC vs ACA., Conclusions: We have found significantly differentially expressed miRNAs in AML and adrenocortical tumors. Circulating hsa-miR-451a might be a promising minimally invasive biomarker of AML. The lack of significantly different expression of hsa-miR-483-3p and hsa-miR-483-5p between AML and ACC might limit their applicability as diagnostic miRNA markers for ACC.

Rationale: Extra-adrenal myelolipoma in the presacral area often raises a concern for liposarcoma because they have similar radiologic features., Patient Concerns: A 70-year-old woman with multiple abdominal surgeries in the past presented with persistent lower abdominal pain and anemia. A presacral mass, found on a pelvic magnetic resonance image (MRI), was suspicious of liposarcoma, as it is the most common fat containing mass in the presacral area. It is often difficult to make a diagnosis of myelolipoma just based on the radiological studies, which necessitates a histopathologic examination of the resected mass for a definitive diagnosis., Diagnoses: Myelolipoma is an encapsulated, benign tumor containing mature adipocytes and hematologic cells. Most of the patients with myelolipoma remain asymptomatic but continued growth of tumor without local invasion can lead to compression of nearby structures, causing persistent pain., Interventions: Asymptomatic patients do not require intervention but surgical resection is indicated for persistent pain or hemorrhage inside the tumor. Our patient had 2 indications for surgery - persistent pain as well as a provisional diagnosis of liposarcoma. After the surgical resection, pelvic pain was resolved, and a diagnosis of myelolipoma was made based on histopathologic examination., Outcomes: Patient was reassured that it was myelolipoma, a benign tumor, not requiring subsequent surveillance for recurrence., Lessons: Despite advancement in imaging techniques, and knowledge of the radiological features of myelolipoma, it still remains as a challenge for clinicians to make the distinction between liposarcoma and myelolipoma only based on diagnostic radiology. Although myelolipoma is a benign tumor, if patient suffers from persistent pain due to local mass effect, surgical resection is required.

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.

Adrenal myelolipoma is a rare benign non secreting tumor. It is often unexpectedly detected. Pathophysiologically, it is adrenal cortex cell metaplasia into reticuloendothelial cells, resulting from infection, chronic stress or adrenal gland degeneration. The mean age at diagnosis is late forties. Histologically, the tumor is composed of mature fat tissue associated with normal haematopoietic tissue. Tumor echogenicity depends on the predominance of the greasy or myeloid component. Its diagnosis is based on CT scan that identifies the fat percentage. However, these radiological aspects may lead to diagnostic confusion with kidney angiomyolipoma, lipoma and liposarcoma, hence the key role of MRI. Surgical resection of myelolipoma is usually performed and is indicated when tumor is voluminous, compressive or at risk of hemorrhage. We here report the case of AL A, 75 years old, hospitalized for adrenal mass revealed by right back pain radiating to the right hypochondrium, without signs of endocrine hypersecretion. Clinical examination showed sensitivity of the right lumbar fossa. Pheochromocytoma and corticosurrenaloma assessment was without abnormalities, including 24-hours urinary methoxyl derivatives and dexamethasone suppression test. The patient underwent coelioscopy; anatomo-pathological examination showed adrenal myelolipoma.

A 30-year-old immunocompetent female presented with right flank pain since 3 years. MRI revealed a large well-defined T1 and T2 hypointense mildly enhancing lesion in the right anterior pararenal space displacing the right kidney and encasing the right ureter with T2 hyperintense wall thickening of the left renal pelvis and ureter. A provisional diagnosis of solitary fibrous tumour was kept. Bilateral double J stenting was done for hydronephrosis. Surgical debulking of the lesion was done with biopsy from the left periureteral wall thickening and was found to be myelolipoma on histopathological examination. This case is a novel variety of myelolipoma which is lipid poor, extra-adrenal and in bilateral perirenal and periureteric location., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Hepatic myelolipoma is a rare entity with only 17 cases described in the literature. A 73mm right liver mass was fortuitously discovered in a 55-year-old man. The biopsy showed normal hepatic tissue adjacent to a normal medular like hematopoïetic tissue, showing trilieage hematopoieses, including myeloid cells, erythroid cells and megakaryocytic cells. The diagnosis of hepatic myelolipoma was proposed. This benign tumor was initially described in adrenal gland, which is the most common topography. No malignancy or bleeding complication has been described in its hepatical location. The diagnosis is histological due to non-specific radiological presentation; it allows to avoid a surgical treatment in relation to its excellent prognosis. The etiology is not well established; but some hypotheses are discussed: adrenal or medullar heterotopia, bone marrow embolism, myeloïd metaplasia., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Case Report: A 71-year-old male with acute exacerbation of chronic bronchitis was treated in summer of 2015. The CT scan has revealed a mass on the right side of 11th thoracic vertebra in the adipose tissue with a sharp edge towards the lung and containing a small amount of contrast agent. The radiologist recommended histological sampling of the mass. The tumor was removed by Video-Assisted Thoracic Surgery (VATS) in August of 2015. The patient was discharged on the fifth postoperative day without complication. Myelolipoma was diagnosed by histological examination. Recurrence was not detected during the one-year-follow-up period., Discussion: Myelolipoma is a benign tumor consisting of mature lobulated adipose tissue and hemopoetic bone marrow. It arises mainly from the adrenal gland. Surgical resection is recommended due to the potential of progressive enlargement. Although the extraadrenal myelolipoma is an uncommon entity, in case of mediastinal, encapsulated, slow-growing tumor, myelolipoma should be considered as a differential diagnosis.

Extra-adrenal myelolipoma happens in adrenal glands, and the thoracic location is extremely unusual. This is the first study involving 36 of patients with thoracic myelolipoma of English literature by investigating the clinical data, pathologic findings, radiological manifestation, and treatment strategy of all patients. Imageologic diagnosis including computed tomography, magnetic resonance imaging, and positron emission tomography/computed tomography scans is useful to identify the feature of extra-adrenal myelolipoma. Pathologic analysis is an effective method to clarify the diagnosis. In view of the potential progressive enlargement of the lesion, most myelolipomas are removed by surgery, and this operation has frequently been accomplished by using video-assisted thoracic surgery., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Myelolipoma is a rare tumor composed of fat and bone marrow components, most of which are located in the adrenal gland. Myelolipoma in the liver is extremely rare. To date, only 10 cases have been reported in the English-language medical literature. In one of these cases, the hepatic myelolipoma was found within a hepatocellular carcinoma (HCC). In the present study, we report the first case of the synchronous occurrence of hepatic myelolipoma and HCCs in different liver sections of one patient, a 26-year-old female who was admitted to our hospital because of a 4-d history of upper abdominal pain. The unenhanced computed tomography (CT) images showed a well-defined low-density mass with adipose components in the right liver lobe, 4.2 cm × 4.1 cm in size. Two inhomogeneous low-density masses were found in the left liver lobe, 8.6 cm × 7.7 cm and 2.6 cm × 2.6 cm in size. The masses in both the right and left liver lobes were heterogeneously enhanced in the contrast-enhanced CT images. Based on the results of the imaging examination, the mass in the right liver lobe was preliminarily considered to be a hamartoma, and the two masses in the left liver were preliminarily considered to be HCCs. We performed a right hepatectomy, a left hepatic lobectomy, and a cholecystectomy. Microscopic and immunohistochemical results revealed that the tumor in the right liver lobe was a hepatic myelolipoma, and that the two tumors in the left liver lobe were HCCs., Competing Interests: Conflict-of-interest statement: No commercial or associative interest in any form has been received or will be received from a commercial party related directly or indirectly to the subject of this paper.

Objective: Myelolipoma is a benign tumor composed of fatty and hematopoietic elements. Presacral myelolipoma is rare, with fewer than 40 cases reported in the English literature. It is important to make an accurate diagnosis because there are malignant diseases included in the differential diagnosis of a presacral fatty mass, particularly liposarcoma., Conclusion: The combination of a well-encapsulated fat-containing presacral mass, lack of coinciding hematologic disease, and uptake on sulfur colloid scintigraphy support the diagnosis of a presacral myelolipoma.

We have noted an increasing number of cases of enlarged adrenal glands where the underlying diagnosis was masked by a diffusely hemorrhagic process. We identified from our database 59 cases (32 consults, 27 routine) of adrenal glands with diffuse (>25%) hemorrhage received between 2000 and 2014. Fifty-three adrenalectomies and 6 biopsies were identified. The diagnoses after central review were 41 adrenocortical adenomas, 1 nodular adrenocortical hyperplasia with associated myelolipoma, 1 benign adrenocortical cyst, and 10 nonneoplastic adrenal glands with hemorrhage. A definitive diagnosis for the 6 biopsies was precluded by the sample size. The adrenocortical adenomas (size, 1-13 cm; 25%-95% hemorrhage) showed clear cell change in the neoplastic area (10%-80% of the tumor), 19 showed focal calcification (1 with ossification), 11 showed areas of papillary endothelial hyperplasia, 10 showed scattered lymphoplasmacytic inflammation, 6 showed benign cortical tissue extending beyond the adrenal capsule into soft tissue, 1 showed necrosis in the form of ghost cells, 2 showed lipomatous change, and 6 were associated with incidental benign lesions (1 cortical cyst, 1 schwannoma, and 4 myelolipomas). Twenty-four of the adrenocortical adenomas were consults where the referring pathologist had trouble classifying the lesion. Of the 10 nonneoplastic adrenals (4.5-22 cm; 40%-80% hemorrhage), 2 were consults. In summary, pathologists have difficulties recognizing adrenocortical adenomas in the setting of a massively enlarged and hemorrhagic adrenal gland. Although there is a correlation between adrenocortical malignancy and size, hemorrhage into nonmalignant adrenal glands can result in markedly enlarged adrenals., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The incidence of many fungal and parasitic lesions are on the rise over the last few decades. In this study, we have analyzed the spectrum of non-malignant lesions in adrenal gland diagnosed on fine-needle aspiration biopsy (FNAB) in a tertiary health care center., Methods: FNAB of the adrenal gland was done under CT (4 cases) and ultrasound guidance (11 cases). In six cases, the lesions were present in both the adrenal glands. Special stains were done whenever needed. No complications were encountered in any of the FNAB procedure. Detailed cytomorphological study was done along with clinical history., Results: There were total of 15 cases comprising of 5 tuberculosis, 5 Histoplasma, 2 acute inflammation, 2 granulomatous inflammation, and 1 myelolipoma. Acid fast bacilli positive on Ziehl Neelsen stain were labeled as mycobacterial infection possibly tuberculosis. The capsule of Histoplasma was bright pink on Periodic Acid Schiff stain., Conclusions: FNAB is an easy, reliable, and minimally invasive method to diagnose and categorize the various benign non-neoplastic diseases of adrenal gland. Diagn. Cytopathol. 2016;44:472-476. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Myelolipomas are benign tumors, consisting of hematopoietic cells and mature adipose tissue, which mainly occur within the adrenal gland. Extra-adrenal myelolipomas are rare, and fewer than 60 cases have been reported in the literature. Here, we report a case of intrasplenic myelolipoma in a 42-year-old man with more than 1 month of abdominal pain. Computed tomography scanning revealed a giant, heterogeneous, well-demarcated mass in the spleen. Splenectomy was performed, and an intrasplenic giant mass was completely excised. The diagnosis of myelolipoma was made based on morphological examination. To the best of our knowledge, this is the third reported case of myelolipoma in the human spleen., (© The Author(s) 2015.)