Evidence of the Role of Inflammation and the Hormonal Environment in the Pathogenesis of Adrenal Myelolipomas in Congenital Adrenal Hyperplasia.

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.

[Primary mediastinal myelolipoma]. / Mielolipoma primario mediastinal.

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.

El mielolipoma es un tumor benigno no funcional, la mayoría de ellos son asintomáticos y descubiertos de forma incidental a través de estudios de imagen o en estudios de autopsia. Aun cuando la mayoría de los casos se presenta en la glándula suprarrenal, también se han informado en sitios extra-adrenales. Presentamos el caso de una mujer de 65 años de edad con un mielolipoma primario mediastinal. La tomografía computarizada de tórax mostró un tumor ovoide de bordes bien definidos de 6.5 × 4.2 cm, localizado en el mediastino posterior. Se realizó biopsia transtorácica de la lesión y el estudio microscópico reveló elementos hematopoyéticos y tejido adiposo maduro. Aun cuando los estudios de imagen como la tomografía computarizada y la resonancia magnética son efectivos en el diagnóstico del mielolipoma primario mediastinal, la evaluación histopatológica es esencial para el diagnóstico definitivo.

A Two-in-One Tumor in the Adrenal: A Functional Adrenocortical Adenoma with Myelolipomatous Differentiation.

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.

[Adrenal myelolipoma, review of the literature in Mexico apropos of two cases]. / Mielolipoma Suprarrenal, revisión de literatura en México a propósito de dos casos.

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.

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

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

Diagnostic imaging features of hepatic myelolipoma incarcerated in a peritoneopericardial diaphragmatic hernia in a cat.

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.

Imaging characteristics of pathologically proven adrenal adenomas with myelolipomatous degeneration: correlation with clinical and pathologic features.

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 myelolipomas.

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.

Primary adrenal diffuse large B cell lymphoma arising within an adrenal myelolipoma: A case report and review of the literature.

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.

Giant secretory multifocal adrenal myelolipoma: a diagnostic dilemma.

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.

An unusual diagnosis of paravertebral lesions: mediastinal myelolipoma.

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.

A Rare Case of Congenital Adrenal Hyperplasia with Giant Adrenal Myelolipoma.

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.

Adrenocortical neoplasm of uncertain malignant potential with a myelolipomatous component.

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.

Minimally invasive adrenalectomy results in equivalent perioperative outcomes versus open adrenalectomy for adrenal mass larger than 6 cm: A retrospective propensity score-matched study.

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.

Congenital adrenal hyperplasia presenting as pelvic inflammatory disease in a phenotypic male: A case report.

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.

A nationwide survey of adrenal incidentalomas in Japan: the first report of clinical and epidemiological features.

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.