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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).)

BACKGROUND 21-hydroxylase deficiency, an essential enzyme for glucocorticoid and mineralocorticoid synthesis, is the cause of congenital adrenal hyperplasia (CAH) in more than 95% of cases. It is an autosomal recessive disorder encoded by the CYP21A2 gene, categorized into classical forms, which encompass the salt-wasting (SW) and simple virilizing (SV) forms, as well as the nonclassical form (NC). The aim of medical treatment is to replace missing glucocorticoids and, if necessary, mineralocorticoids, while also reducing elevated adrenal androgens. CASE REPORT We present the case of a 42-year-old woman with CAH who discontinued therapy during adolescence and was admitted to hospital with fatigue, nausea, and severe abdominal pain. A CT scan showed an extreme enlargement of the adrenal glands. Laboratory tests revealed elevated levels of 17-hydroxyprogesterone and other adrenal androgens, along with normal plasma metanephrine levels. Decreased morning cortisol levels suggested partial adrenal insufficiency requiring glucocorticoid replacement therapy. Due to the development of several serious complications and clinical deterioration, the multidisciplinary team recommended bilateral removal of masses measuring 300×250×200 mm on the right side and 250×200×200 mm on the left side. Histological and immunochemical examination confirmed the presence of giant myelolipomas with adrenal cortex hyperplasia. CONCLUSIONS Adrenal tumors, particularly myelolipomas, have a higher prevalence in patients with CAH. Our case report provides further evidence of the suspected link between non-compliant CAH therapy and the development of myelolipomas, along with promotion of their pronounced growth.

Abstract: A 57-year-old male had abdominal pain and distension for 6-7 months with a palpable swelling in the right lumbar region. Contrast-enhanced computed tomography abdomen showed a large heterogeneous lesion with fat density measuring 22 cm ´ 16.5 cm in the right suprarenal region. Laparotomy was done which showed an encapsulated mass measuring 21 cm ´ 14 cm ´ 5 cm. Cut section revealed yellowish areas admixed with hemorrhage and large areas of necrosis. Microscopy revealed adrenal myelolipoma. The patient was found to have sickling positive. High-performance liquid chromatography showed double heterozygous for HbS and beta-thalassemia trait. The association of giant adrenal myelolipoma with double heterozygous for HbS and beta-thalassemia trait is rare, and as clinically it simulates retroperitoneal sarcoma, awareness of this rare entity is critical for its accurate diagnosis and proper management., (Copyright © 2023 Copyright: © 2023 Journal of Cancer Research and Therapeutics.)

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.

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.)

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.)

Myelolipomas are benign tumours typically occurring in the adrenal glands, made up of fat and trilineage haematopoeitic cells resembling bone marrow. Their aetiology is not well understood; however, they have a clear association with elevated serum adrenocorticotropic hormone (ACTH). Extra-adrenal myelolipomas are rare, and to our knowledge there are no previously reported cases of multiple enlarging hepatic and retroperitoneal myelolipomas in the setting of Cushing disease. We present the case of a patient with an ACTH-producing pituitary adenoma who developed multiple enlarging fat containing lesions in the liver and retroperitoneum, which were histologically proven multifocal myelolipomas., Competing Interests: Competing interests: DMR discloses personal fees for written work from Elsevier, which is unrelated to the current body of work., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

We present a case of androgen and glucocorticoid secreting adrenocortical carcinoma with concomitant myelolipoma. A giant adrenal tumor which was initially nonfunctional was reassessed four years later due to the patient's refusal to treat. The patient was a 48-year-old woman with hypertension and acne lesions on the face. Laboratory findings were consistent with glucocorticoid and androgen hypersecretion. Computed tomography revealed a heterogeneously contrasting mass of 145x118x100 mm with lobular contour and soft tissue areas. The patient underwent left laparoscopic transperitoneal adrenalectomy with three port technique. There were no complications in the perioperative period. The resected specimen weighed 850 grams. Pathological findings showed a combination of myelolipoma-adrenal cortical cancer. In the postoperative period, hypertension improved and the hormone panel was normalized. Postoperative computed tomography and PET-CT showed no residual mass and metastasis. Although imaging is compatible with benign masses such as myelolipoma, coexistence of ACC-myelolipoma should be kept in mind and functional evaluation should be performed.

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.

A 17-year-old, female cockatiel ( Nymphicus hollandicus ) was presented for a 1-week history of respiratory distress. The clinical signs were consistent with tracheal obstruction; therefore, an air sac cannula was placed to reduce respiratory compromise. Radiographic images of the patient revealed a round, intraluminal, soft tissue opacity in the trachea. The obstruction could be visualized via transtracheal illumination, but tracheoscopy was not feasible because of the size of the patient. Attempts to manually extirpate the obstruction were unsuccessful, and the trachea subsequently avulsed in the midcervical region. Tracheal resection and anastomosis was performed, and the obstruction was removed with the associated tracheal ring. The patient recovered uneventfully and was doing well at follow-up evaluations at 9 days, 24 days, and 10 months after the surgical procedure. The histologic diagnosis was ossifying myelolipoma arising from bone marrow within the tracheal ring.

Objective: We aimed to describe clinical course of myelolipoma and to identify predictors of tumour growth and need for surgery., Design: A retrospective study., Patients: Consecutive patients with myelolipoma., Results: A total of 321 myelolipomas (median size, 2.3 cm) were diagnosed in 305 patients at median age of 63 years (range, 25-87). Median follow-up was 54 months. Most myelolipomas were incidentally detected (86%), whereas 9% were discovered during cancer staging and 5% during workup of mass effect symptoms. Thirty-seven (12%) patients underwent adrenalectomy. Compared to myelolipomas <6 cm, tumours ≥6 cm were more likely to be bilateral (21% vs 3%, P < .0001), cause mass effect symptoms (32% vs 0%, P < .0001), have haemorrhagic changes (14% vs 1%, P < .0001) and undergo adrenalectomy (52% vs 5%, P < .0001). Among patients with ≥6 months of imaging follow-up, median size change was 0 mm (-10, 115) and median growth rate was 0 mm/y (-6, 14). Compared to <1 cm growth, ≥1 cm growth correlated with larger initial size (3.6 vs 2.3 cm, P = .02), haemorrhagic changes (12% vs 2%, P = .007) and adrenalectomy (35% vs 8%, P < .0001)., Conclusions: Most myelolipomas are incidentally discovered on cross-sectional imaging. Myelolipomas ≥6 are more likely to cause mass effect symptoms, have haemorrhagic changes and undergo resection. Tumour growth ≥1 cm is associated with larger myelolipoma and haemorrhagic changes. Adrenalectomy should be considered in symptomatic patients with large tumours and when there is evidence of haemorrhage or tumour growth., (© 2020 John Wiley & Sons Ltd.)

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.

As a rare and normally-benign and functionless tumor, primary adrenal myelolipoma comprises adipose tissue and myeloid cells, and its diagnosis is usually difficult owing to its asymptomatic nature. Imaging techniques can detect these masses in over 90% of the cases. CT scan is the most sensitive imaging technique, which can display the tissue nature of this tumor with a high resolution. The present case report involves a 45-year-old woman with bilateral adrenal myelolipoma diagnosed with imaging methods.

Adrenal myelolipomas represent a benign neoplasm with known associations with many chronic diseases, 21-hydroxylase deficiency and cancer. However, the aetiology of adrenal myelolipomas remains unknown. Here, we present a case of a patient with image-proven bilateral adrenal haemorrhages caused by trauma with the subsequent development of bilateral adrenal myelolipomas several years later. Resection and pathological analysis of left adrenal gland confirmed the presence of multiple adrenal myelolipomas. Our case strongly suggests that trauma was the inciting event that led to the formation of these lesions., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

Adrenal myelolipomas (AMLs) are rare benign adrenal tumors, containing adipose and hematopoietic tissue, a result of reticuloendothelial cell metaplasia. Incidence on autopsy has been reported from 0.08% to 0.4%. AMLs are generally considered nonsecretory. The functional aspect of adrenal incidentaloma should be evaluated. In this article, we report a case of a 40-year-old male, who presented with uncontrolled hypertension and renal failure, with imaging revealing an adrenal incidentaloma. He was started on dialysis for acute fluid overload, and workup for pheochromocytoma revealed an elevated serum norepinephrine level of 1181 pg/mL. Free metanephrine and normetanephrine levels were low when checked pre- and post-dialysis. Complete resection of the encapsulated right adrenal mass was performed. Pathology of the adrenal tumor demonstrates an 11.5 × 9.5 × 7.5 cm well-circumscribed, partially encapsulated proliferation of mature adipose tissue with admixed hemopoietic elements consistent with myelolipoma weighing 29.3 g. This case highlights the inclusion of a full metabolic workup for all adrenal incidentalomas, including AML.

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.

A 66-year old woman had a left breast mass. Biopsy showed invasive ductal carcinoma. A PET/CT scan demonstrated hypermetabolism in the left breast and atypical heterogeneously increased uptake throughout the skeleton as well as a minimally FDG-avid right adrenal myelolipoma. PET/CT 4 months later after 6 cycles of neoadjuvant chemotherapy demonstrated increased size and FDG avidity of this adrenal mass concerning for metastasis and uniformly increased skeletal FDG avidity. Biopsy demonstrated adrenal myelolipoma. The growth and increased FDG avidity of the adrenal myelolipoma were due to the action of colony-stimulating factors on the tumor's hematopoietic component.

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.

Rationale: Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive., Patient Concerns: During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Abdominal computed tomography revealed a well-defined, heterogeneously enhanced bilateral adrenal mass, suggesting a myelolipoma., Diagnoses: The hormonal evaluation revealed adrenocorticotropic hormone (ACTH) dependent Cushing syndrome., Interventions: The patient underwent left adrenalectomy, and transsphenoidal resection of a pituitary mass. The final diagnosis was adrenal myelolipoma associated with Cushing disease., Outcomes: Growth of right adrenal myelolipoma was detected during the 7-year follow-up. There were enhancing pituitary lesions in repeat magnetic resonance imaging of the sellar region, which implies persistent or recurrent pituitary adenoma. This case reinforces relationship between Cushing disease and adrenal myelolipoma., Lessons: To the best of our knowledge, this is the first reported pathologically confirmed bilateral adrenal myelolipoma associated with Cushing disease. This report supports the idea that ACTH is associated with the development of adrenal myelolipoma., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

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.

Teratoma is a germ-cell tumour that is common in para-axial regions. Retroperitoneal teratoma is rare and comprise only about 1% of all teratomas. We present a 21-year-old female who presented with a lump in the abdomen. After radiological investigations, she was diagnosed as case of adrenal myelolipoma which on excision turned out histologically to be retroperitoneal teratoma. Our case highlights the limitation of radiological investigations in preoperative diagnosis of teratoma., 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.)

Primary adrenal myelolipomas, relatively rare benign tumours of the adrenal cortex are typically unilateral, hormonally inactive and asymptomatic, hence often diagnosed as 'adrenal incidentaloma'. Bilateral adrenal myelolipomas, in particular, may be associated with underlying endocrinopathies associated with elevated circulating adrenocorticotropic hormone (ACTH) concentration. Subclinical cortisol hypersecretion, irrespective of its ACTH dependency, does not manifest typical clinical phenotype of hypercortisolemia, and thus termed subclinical Cushing's syndrome. In this article, hormonal evaluation in a middle-aged woman with diabetes, hypertension and incidentally discovered unilateral adrenal myelolipoma revealed underlying subclinical Cushing's disease. Abdominal CT revealed another tiny focus in the contralateral adrenal gland, probably representing incipient myelolipoma., 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.)

Objective: To describe the imaging characteristics of extra-adrenal myelolipoma and mass-forming extramedullary hematopoiesis (EMH) occurring in the presacral region., Materials and Methods: Retrospective review between 1980 and 2015 revealed 11 patients with biopsy-proven presacral extra-adrenal myelolipoma and 11 patients with presacral EMH and radiologic imaging studies., Results: All cases of both myelolipoma and EMH directly abutted the anterior sacrum and were centered on the midline. Myelolipomas were round or bilobed (8/11), or were multilobulated in appearance (3/11). On CT, margins could be smooth or ill defined. On MRI, all masses were well-marginated with evidence of a "capsule". All myelolipomas showed evidence of macroscopic fat on both CT and MRI with scattered hazy and nodular soft tissue components. On MRI, all cases showed areas of high T1 signal intermixed with areas of intermediate T1 signal. On T2, all cases showed areas of low and high T2 signal. Enhancement was usually minimal or mild. Most EMH cases had a well-defined round, bilobed or macro-lobulated presacral mass (9/11); 2/11 cases were ill-defined. On CT, when a dominant fatty component was present, the soft tissue components had hazy and nodular features, similar to myelolipoma. On MRI, the masses demonstrated intermediate T1 signal, isointense to skeletal muscle with varying amounts of hyperintense T1 signal. On T2-weighted images, 3 of 4 cases showed areas of mildly hyperintense T2 signal with varying amounts of dark signal. All patients with clinically or biopsy-proven EMH had a hematologic or myeloid disorder., Conclusion: Presacral myelolipoma and presacral EMH are two benign fat-containing masses that can present as discrete, often encapsulated, lipomatous masses with varying amounts of non-lipomatous soft tissue components and should be considered in the differential diagnosis of a well-defined fat-containing presacral mass., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Adrenal myelolipoma is an uncommon, benign, and hormonally non-functioning tumor that is composed of mature adipose tissue and normal hematopoietic tissue. Most cases to date are asymptomatic or have epigastric pain. Acute hemorrhage is the most dramatic manifestation of adrenal myelolipoma; though, it is a rare entity. Hemorrhagic shock due to adrenal myelolipoma, to our knowledge, was much less mentioned so far. Persistent bleeding and uncontrollable hypotension are considered to be absolute indications for immediate surgical operation., Case Presentation: Herein we presented a 32-year-old male patient with initial symptoms of nausea, vomiting, and epigastric pain progressing to altered consciousness and hypotension during ER course. Hemorrhagic shock due to a giant adrenal myelolipoma, R’t was diagnosed. Emergent exploratory laparotomy was executed, and en bloc excision of tumor was done., Conclusion: Adrenal myelolipoma might be diagnosed as a adjunction to other main causes of illness; furthermore, adrenal myelolipoma could be asymptomatic in lifetime. In our case, however, manifesting as hemorrhage shock was challenging to diagnose step by step; instead, maintaining vital organs perfusion and identifying bleeding sources were to be done. Management of myelolipoma should be done on a case-to-case basis.

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., (© 2017 Society for Endocrinology.)

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.)

Myelolipomas represent 3-7% fromthe primary tumors of the adrenal gland. Most often they are incidental findings. In most cases are asymptomatic, rarely they present symptoms (pain, abdominal discomfort, hematuria or signs of internal hemorrhage). Histologically benign, this variety of tumor requires only local excision, in symptomatic forms. Their dimensions are generally up to 4-5 cm, so the laparoscopic approach seems the most appropriate. We present the case of a 65 years old patient, electively operated for a right adrenal tumor formation.Alaparoscopic right adrenalectomy was performed using an anterior transabdominal approach. No ligatures, clips or sutures were used. The intervention was accomplished with the Ligasure Maryland forceps and the Force Triad platform (Covidien Medtronic-USA). The postoperative evolution was favorable and the pathological examination highlighted an adrenal myelolipoma with intratumoral hemorrhage., (Celsius.)

Background: Myelolipoma is a rare benign tumor composed of mature adipose tissue and normal hematopoietic tissue. Although surgical resection has been recommended due to the potential of progressive enlargement, the natural history of mediastinal myelolipoma has not yet been described. Herein we report a surgically resected mediastinal myelolipoma showing gradual enlargement over a period of 9 years., Case Presentation: A 70-year-old woman presented with a posterior mediastinal mass shadow detected by computed tomography (CT) examination. She had a medical history of sigmoidectomy for colon cancer 13 years previously. A CT scan showed a smooth, well-demarcated 2.8 × 2.1-cm paravertebral mass shadow, composed of a fat density area and a soft tissue density area, which showed gradual enlargement from a 1.6 × 1.0-cm nodule 9 years previously. This was not accompanied by chronic anemia or hematologic disease including thalassemia, and no abnormal accumulation was observed on bone marrow scintigraphy or fluoro-2-deoxyglucose positron emission tomography. With a clinical diagnosis of mediastinal myelolipoma, surgical resection was performed, and pathological examination confirmed the diagnosis., Conclusions: We experienced a rare case with mediastinal myelolipoma showing gradual enlargement, with a tumor doubling time of 1,212 days.

The paper describes a case of von Hippel--Lindau-related pancreatic neuroendocrine tumor and adrenal myelolipoma in a 44-year-old woman. The pancreatic tumor and a left retroperitoneal mass were removed in the women in July 2014 and May 2015. Histological examination of the pancreatic tumor revealed that the latter consisted of clear cells forming tubular and tubercular structures showing the expression of chromogranin A, synaptophysin, and cytokeratins 18 and 19 and a negative response to CD10 and RCC. The adrenal medullary mass presented as clear-cell alveolar structures with inclusions of adipose tissue mixed with erythroid, myeloid, and lymphoid cells. The clear-cell component of the adrenal gland expressed neuroendocrine markers with a negative response to cytokeratins, CD10, and RCC. Molecular genetic examination yielded a signal corresponding to two copies of the VHL gene. No deletions or amplifications of the gene were detected. Cases of von Hippel--Lindau disease concurrent with adrenal pheochromocytoma and myelolipoma and simultaneous pancreatic involvement were not found in the literature.

Extra-adrenal myelolipomas are rare, benign tumors composed of adipose tissue and hematopoietic cells. Almost all myelolipomas occur within the adrenal gland. Only 50 cases of myelolipomas were described in literature and none of these were associated with gastric mesenchymal neoplasia. A 72-year-old male patient presented to a family medicine outpatient clinic with dyspnea and urinary urgency. His abdominal sonography revealed a 9-cm intra-abdominal mass. An incidental finding was 2 separate masses 1 cm each on the serosal surface of the stomach. The pathology specimen of the retroperitoneal mass revealed myelolipoma histopathology while gastric masses were reported as spindle cell mesenchymal neoplasias. The association of gastric spindle cell tumor and myelolipoma was not reported before in medical literature. Extra-adrenal myelolipomas are rare lesions, but should be considered in the differential diagnosis of fat containing retroperitoneal masses that are well circumscribed.