Chiari I Malformation With Concomitant Nonfunctioning Pituitary and Adrenal Tumors.

Chiari 1 malformation (CM1) is a rare finding that has been described with growth hormone (GH)-secreting pituitary adenomas and with an endothelial PAS domain protein 1 gain-of-function mutation syndrome. We describe the first reported case of a patient diagnosed with CM1 and nonfunctioning pituitary and adrenal incidentalomas. Our case describes a 45-year-old female who was found to have cerebellar tonsillar ectopia consistent with CM1, a pituitary tumor, and bilateral adrenal incidentalomas. She was diagnosed after presenting with 2 weeks of upper extremity weakness and paresthesia. A comprehensive endocrine workup including insulin like growth factor (IGF-1) was normal. She underwent posterior fossa decompression without complication. Pituitary adenectomy was not pursued as there was no evidence of compression of the chiasm or the surrounding structures. In previous case reports it has been proposed that GH-secreting adenomas contribute to CM1 by causing hypertrophy of soft tissue structures in the skull base, overcrowding the posterior fossa. Given that our patient had normal IGF-1 levels, there could be a different underlying mechanism that contributed to the concomitant occurrence of CM1 with the pituitary and adrenal tumors.

Splenic Myelolipomas in the Domestic Cat-To Operate or Not to Operate?

Myelolipoma is a benign, typically inert neoplasm of uncertain aetiology that is rarely reported in cats. Although commonly asymptomatic, in some cases, myelolipomas can cause abdominal discomfort or present with rupture and haemorrhage. Here, we describe a rare case of a splenic myelolipoma in a Domestic Long Hair cat in which, after extensive diagnostic investigations, clinical signs of hyporexia and weight loss were attributable to the presence of an intra-abdominal mass. The patient was treated by splenectomy and unexpectedly died during the post-operative period. Although splenectomy appears to be a sensible intervention in symptomatic patients, the optimal management of splenic myelolipomas in cats remains unknown. The risk-benefit ratio of surgical management needs to be carefully considered, and therapeutic intervention should be tailored individually to each patient.

Stealthy Encroachment: Unraveling an Encounter With Renal Myelolipoma.

Myelolipoma of the kidney is an exceedingly unusual benign tumor of hematological components mixed with mature adipose tissue. We present a case of a 59-year-old male who presented with left flank pain and was found to have an atrophic left kidney on imaging studies. A computed tomography (CT) scan revealed a small and shrunken left kidney with an extrarenal pelvis. A diethylenetriamine pentaacetate (DTPA) scan results showed a total glomerular filtration rate (GFR) of 45.6 ml/min with a non-functional left kidney. The patient underwent a left nephrectomy, and a histopathological examination confirmed the diagnosis and highlighted the distinctive morphological features of this rare entity. Postoperatively, the patient experienced a complete resolution of symptoms. This case underscores the importance of considering myelolipoma in the differential diagnosis of renal masses and highlights the successful management of symptomatic cases through surgical intervention. Awareness of this rare tumor is crucial for accurate diagnosis and appropriate management. Further studies are needed to elucidate the natural history and optimal treatment strategies for renal myelolipomas.

A Rare Case of Giant Bilateral Adrenal Myelolipomas in a Patient With Classical Congenital Hyperplasia.

Congenital adrenal hyperplasia (CAH) is caused by genetic defects in the enzymes involved in cortisol biosynthesis in the adrenal gland and, in more than 90% of cases, due to a deficiency in the 21-hydroxylase enzyme. Classical CAH due to 21-hydroxylase deficiency is a severe form of the disease that presents with cortisol deficiency and is further categorized into salt-wasting or simple-virilizing types. Appropriate steroid replacement has been shown to effectively treat patients with classical CAH and prevent complications. Individuals who receive inadequate treatment or fail to comply with their prescribed steroid hormone regimen are susceptible to the development of adrenal myelolipomas. Myelolipomas are benign tumors composed of both adipose and hematopoietic tissues. While documented cases of adrenal myelolipomas exist in medical literature, instances of large bilateral myelolipomas remain exceedingly rare. This case report highlights a 40-year-old female patient with a known history of classical congenital adrenal hyperplasia who presented with unusually large bilateral adrenal myelolipomas. A diagnostic CT scan of the abdomen and pelvis revealed a 13.4 x 10.8 cm myelolipoma on the left adrenal gland and a 10 x 8.6 cm myelolipoma on the right adrenal gland. Prior to her presentation, the patient experienced recurrent nausea and vomiting, along with left upper quadrant pain, over five months. Hormonal assessments indicated significantly elevated serum androgen levels, suggesting inadequate management of her CAH. In this report, we present a rare case of symptomatic bilateral large adrenal myelolipomas, underscoring the significance of adhering to treatment regimens, diagnostic assessments, and management for adrenal myelolipomas in individuals diagnosed with CAH.

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.

Concurrent adrenal and extra-adrenal myelolipoma: A case report.

INTRODUCTION: Myelolipoma, a benign tumor characterized by mature fat cells and hematopoietic cells, is predominantly found in the adrenal glands, accounting for 6-16 % of all adrenal tumors. These tumors are often asymptomatic and discovered incidentally during imaging. We present a rare case of concurrent adrenal and extra-adrenal myelolipomas, contributing to the limited research in this area. CASE PRESENTATION: A 65-year-old female with a history of Steven-Johnson syndrome presented with epigastric pain, initially diagnosed with emphysematous cholecystitis. Imaging revealed unexpected lesions near the left kidney. During surgery for presumed cholecystitis, significant hemorrhaging occurred following an attempted biopsy of the left adrenal lesion. This complication necessitated a complete adrenalectomy. Pathological examination confirmed the presence of myelolipomas in the left adrenal gland, para-aortic, and left para-iliac regions. DISCUSSION: The simultaneous occurrence of adrenal and extra-adrenal myelolipomas is exceptionally rare, posing diagnostic and management challenges. This case highlights the complexity of managing patients with multiple comorbidities and the critical importance of differentiating myelolipomas from other fat-containing retroperitoneal masses. The incidental discovery of these tumors and their potential for significant intraoperative complications, as seen in our case, underscores the need for careful surgical planning and thorough preoperative assessment. CONCLUSION: This case emphasizes the diagnostic challenges and management complexities in patients with incidental findings of myelolipoma, particularly when accompanied by significant medical histories. The occurrence of unexpected intraoperative complications highlights the importance of cautious decision-making in surgical interventions. This report provides valuable insights into the unpredictable nature of medical practice and the management of rare pathologies.

Hemorrhagic giant adrenal myelolipoma discovered incidentally: a case report.

Adrenal myelolipomas are rare, benign, nonfunctional tumors composed of mature adipose tissue and hematopoietic elements. Hemorrhage within an adrenal myelolipoma is an uncommon occurrence, and when it happens, it can present with various clinical manifestations. Here, we report a case of a hemorrhagic giant adrenal myelolipoma in a 45-year-old female that was discovered incidentally. We discuss the clinical presentation, radiological findings, surgical intervention, and postoperative outcomes in this case report.

Bilateral Adrenal Myelolipoma and Breast Cancer in a Patient With Congenital Adrenal Hyperplasia.

Adrenal myelolipoma is a rare, benign tumor of the adrenal gland, typically non-functional, asymptomatic and unilateral. With the increased use of radiological imaging, it has been discovered more frequently as incidental mass. It is common to occur concurrently with hormonal dysfunction conditions like congenital adrenal hyperplasia. However, there are few previous reported cases of malignancy concomitant with adrenal myelolipoma. We present a case of a 33-year-old patient diagnosed with congenital adrenal hyperplasia since birth. She was diagnosed with giant bilateral adrenal myelolipoma incidentally during the investigation done for staging her breast cancer. To the best of our knowledge, this is the second reported case of breast cancer concomitant with adrenal myelolipoma. Although this entity is very rare, physicians should be familiar with such rare adrenal masses and their associations in order to manage them appropriately.

Unusual discovery: Incidental diagnosis of adrenal myelolipoma, initially confused with liposarcoma, following bee sting bite, resolving chronic right hypochondrial discomfort - A case report.

INTRODUCTION AND IMPORTANCE: Adrenal myelolipomas are uncommon benign tumors of the adrenal gland. It has various other sites of origin. CASE PRESENTATION: A 51-year-old male with elevated serum creatinine and an incidentally discovered adrenal mass underwent right adrenalectomy for an adrenal myelolipoma, a benign tumor with fat and haematopoietic components after bee string bite. As the patient was experiencing right hypochondrial pain surgery was planned. After surgery, he developed a surgical site infection, which was treated with antibiotics, and his wound healed well. Excision biopsy confirmed the non-malignant nature of the tumor. CLINICAL DISCUSSION: Adrenal myelolipomas are often unilateral, non-functional, and diagnosed using imaging (CT/MRI). While radiological findings can be specific, inconclusive cases may require biopsy for confirmation. Surgical intervention is reserved for hormonally active, symptomatic, or rapidly growing myelolipomas, particularly if they exceed 5 cm. CONCLUSION: Adrenal myelolipomas are common in adrenal glands, typically diagnosed in midlife with no gender bias. Imaging, especially CT, is effective in detecting fat components. In cases of diagnostic uncertainty, fine needle biopsy is crucial for confirmation, and surgery is considered when tumors are hormonally active, grow substantially, or cause symptoms, highlighting personalized care based on patient and imaging data.

Bilateral giant adrenal myelolipoma associated with congenital adrenal hyperplasia / Mielolipoma adrenal gigante bilateral associado a hiperplasia adrenal congênita

Adrenal myelolipomas are rare benign tumors, often non-functioning, located in the adrenal cortex, consisting mainly of mature adipose tissue and hematopoietic tissue. Although uncommon, the number of reported cases has increased due to the greater use of diagnostic imaging techniques. This tumor is usually unilateral and found as an adrenal incidentaloma, although there is a predominance of bilaterality in patients with congenital adrenal hyperplasia (CAH). In this study, we report the case of a 33-year-old male patient with CAH due to 21-hydroxylase deficiency, in non-regular use of the control medication, with bilateral giant adrenal myelolipoma and subsequent evolution of bilateral testicular adrenal rest tumors. He underwent bilateral adrenalectomy by video laparoscopy. The anatomopathological analysis, which confirmed myelolipomas' diagnosis, revealed the right adrenal with 430 g and 12.5 x 9.3 cm and the left with 257 g and 11.5 x 10.4 cm. This tumor may be accompanied by adrenocortical adenoma and carcinoma, ganglioneuroma, pheochromocytoma, Addison's disease, Cushing's syndrome, or CAH. Among the hypotheses of its pathogenesis, we highlight an association between the development of adrenal myelolipoma and chronic hormonal stimulation by the adrenocorticotrophic hormone (ACTH), especially in CAH. The non-regular treatment of CAH with glucocorticoids may have contributed to the chronic and elevated secretion of ACTH and, consequently, to the development of bilateral giant adrenal myelolipoma (AU).

Mielolipomas adrenais são tumores benignos raros, com frequência não-funcionantes, localizados no córtex da adrenal, constituídos, principalmente, por tecido adiposo maduro e tecido hematopoético. Apesar de incomum, o número de casos relatados tem aumentado devido ao maior uso de técnicas diagnósticas de imagens. Esse tumor é geralmente unilateral e encontrado como um incidentaloma adrenal, embora haja predominância de bilateralidade em casos de portadores de hiperplasia adrenal congênita (HAC). Neste estudo, relatamos o caso de um paciente do sexo masculino, de 33 anos, portador de HAC por deficiência de 21-hidroxilase, em uso não-regular da medicação de controle, com mielolipoma adrenal gigante bilateral e posterior evolução de tumor bilateral testicular de restos de adrenais. Ele foi submetido à adrenalectomia bilateral por videolaparoscopia. A análise anátomo-patológica, que confirmou o diagnóstico de mielolipomas, revelou adrenal direita com 430 g e 12,5 x 9,3 cm, e esquerda com 257 g e 11,5 x 10,4 cm. Esse tumor pode vir acompanhado de adenoma e carcinoma adrenocortical, glanglioneuroma, feocromocitoma, doença de Addison, Síndrome de Cushing ou HAC. Dentre as hipóteses de sua patogênese, des-tacamos uma associação entre o desenvolvimento do mielolipoma adrenal e a estimulação hormonal crônica pelo hormônio adrenocorticotrófico (ACTH), especialmente na HAC. O tratamento não-regular da HAC com glicocorticoides pode ter contribuído para a secreção crônica e elevada de ACTH e, consequentemente, para o desenvolvimento do mielolipoma adrenal gigante bilateral (AU).

Coexistence of Bilateral Giant Adrenal Myelolipomas and Congenital Adrenal Hyperplasia: A Case Report.

Adrenal myelolipomas (AMs) are rare and benign neoplasms, consisting of adipose and mature hematopoietic tissue. They are commonly discovered incidentally with increased use of radiologic imaging. A small number of giant bilateral adrenal masses are reported, particularly in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 36-year-old male with a history of CAH on steroids since childhood, self-discontinued shortly after diagnosis, presenting mainly with abdominal distension and pain besides infertility. Imaging revealed giant bilateral adrenal masses. Subsequently, he underwent bilateral adrenalectomy, and the surgical pathology report revealed myelolipomas measuring 39×17×8 cm on the left and weighing 4050 grams and 28×16×5 cm on the right and weighing 1702 grams. AMs are found to coexist with many other conditions such as Cushing's syndrome, pheochromocytoma, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as a stimulator leading to myelolipomas. This case report highlights the proper history taking and biochemical evaluation for early detection and intervention to avoid catastrophic consequences.

Bilateral adrenal giant medullary lipoma combined with disorders of sex development: a rare case report and literature review.

Bilateral adrenal myelolipoma is rare in clinics and patients with disorders of sex development (DSDs). One case was reported in our center. A 45-year-old patient was admitted to the hospital after discovering a left abdominal mass for more than a year and worsening abdominal pain for 18 days. An imaging examination showed bilateral adrenal masses. Physical examination showed clitoris hypertrophy with patelliform changes, thick and dense pubic hair, normal development of bilateral labia majora without labia minora, and urethral opening. After the relevant preoperative examinations, bilateral adrenal mass resection was performed under general anesthesia. The postoperative pathology confirmed adrenal myelolipoma. The incision healed well without recurrence over 10 years after the operation. Her enlarged clitoris decreased in size. This case report has a detailed diagnosis and treatment process and sufficient examination results. It can provide a reference for diagnosing and treating patients with bilateral adrenal myelolipoma and DSD and reduce the risk of misdiagnosis and mistreatment.

A Case Report and Literature Review of Adrenal Myelolipoma.

Adrenal myelolipoma is considered a benign neoplasm that accounts for 6% to 16% of adrenal incidentalomas, and it is the second most common incidental adrenal tumor after adrenal adenomas. They are usually asymptomatic; however, in the presence of symptoms, significant growth, or complications, open surgical resection is indicated. We present the case of a 46-year-old woman with obesity and diabetes who experienced five years of left hemiabdominal pain, which was unsuccessfully treated symptomatically. A computed tomography scan revealed findings suggestive of pancreatic lipoma and a suggestive image of left adrenal myelolipoma. Resection of the tumor was performed using an anterior midline approach, and histopathological examination confirmed left adrenal myelolipoma. The presented case represents the typical presentation of these tumors in a patient in the fifth decade of life with obesity, diabetes, and nonspecific abdominal pain possibly related to the size of the lesion found. Surgical intervention was indicated due to the presence of symptoms, lesion size, contiguity with abdominal organs, and the absence of a precise diagnosis. An anterior midline approach was chosen, and histopathological examination provided a definitive diagnosis. Adrenal myelolipoma is a rare entity that is often asymptomatic and incidentally diagnosed through imaging studies. However, they should be resected when symptomatic to prevent complications. Open surgical resection is the preferred approach.

A case of perirenal extra-adrenal myelolipoma mimicking liposarcoma.

Myelolipoma is a benign tumor composed of mature adipose tissue and normal hematopoietic components. It usually occurs in the adrenal glands but rarely in the extra-adrenal region. However, it is difficult to differentiate extra-adrenal myelolipoma from well-differentiated liposarcoma on the basis of the radiological findings. We report the case of a 66-year-old male with perirenal and extra-adrenal myelolipoma who underwent radical tumor resection with nephrectomy after a preoperative diagnosis of liposarcoma. Intraoperative assessment by the surgeon and intraoperative pathological evaluation are important considering the divergent prognoses of myelolipoma and liposarcoma.

Bronchoscopic treatment of multiple bronchial myelolipomas: a case report and literature review.

BACKGROUND: Extra-adrenal myelolipoma is an unusual entity, and endobronchial myelolipoma is rarer, which is often ignored by clinicians, delaying the disease and affecting the prognosis. CASE PRESENTATION: A 71-year-old man with a history of chronic obstructive pulmonary disease (COPD) and type 2 diabetes mellitus, with recurrent fever, cough, and expectoration for more than 2 weeks experienced relief in cough, phlegm reduction, and glycemic control with anti-inflammatory treatment. Further examination revealed that new growths obstructing all lobar bronchi impaired flexible bronchoscope entry. In order to relieve the patient's symptoms, under general anesthesia, we performed liquid nitrogen cryobiopsy at multiple bronchial openings, and then used argon plasma coagulation (APC) to achieve hemostasis. The pathological diagnosis was bronchial myelolipoma. The largest volume of the resected tissue was a mass measuring 0.6 cm × 0.4 cm × 0.3 cm at the bronchial opening of the upper lobe of the left lung. The patient's condition was stable and the symptoms were partially relieved after surgery. No recurrence was observed during the 12-month follow-up, although the long-term treatment efficacy is unknown. CONCLUSION: Pathological biopsy is key to the diagnosis of endobronchial myelolipoma, and the development of the endobronchial myelolipomas may have been associated with long-term poor control of steroid levels in this patient.

Mielolipoma primario mediastinal / Primary mediastinal myelolipoma

Resumen 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 es tudios 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 mag nética son efectivos en el diagnóstico del mielolipoma primario mediastinal, la evaluación histopatológica es esencial para el diagnóstico definitivo.

Abstract 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 tomo graphy and magnetic resonance imaging are effective in diagnosing mediastinal myelolipoma, histopathological examination is essential for the definitive diagnosis.

Simultaneous adrenal and retroperitoneal myelolipoma resected by laparoscopic surgery: a challenging case.

BACKGROUND: Myelolipoma is a benign neoplasm of the adrenal cortex, composed of fat and hematopoietic cells. Although myelolipoma is benign, differentiation from adrenocortical cancer may be difficult. The presence of adrenal and extra-adrenal myelolipomas simultaneously is sporadic, making it a challenging case, especially when the preoperative diagnosis is ambiguous. CASE PRESENTATION: A 65-year-old man was referred to our clinic due to a mass in the adrenal fossa. In the abdominopelvic computed tomography (CT), a well-circumscribed fat-containing 78 × 61 × 65 mm bi-lobulated mass was reported in the left adrenal fossa. The first differential diagnosis was myelolipoma. The patient was then referred to our clinic for a mass excision. He was asymptomatic and was scheduled to undergo laparoscopic-assisted adrenalectomy. After adrenalectomy and mass dissection, surprisingly, another mass was detected in the retroperitoneal area. The second mass was also dissected. The final diagnosis was myelolipoma for both masses. The patient has been symptom-free for nine months after the operation. CONCLUSION: Simultaneous adrenal and extra-adrenal myelolipoma should be considered as one of the differential diagnoses. However, because this situation is extremely rare, the probability of malignancy should be highly regarded, and we suggest an obsessive approach when approaching this condition. It is essential to manage these cases on a case-by-case basis and tailor the management concerning intraoperative biopsy, the intraoperative appearance of tumors, and the location of extra-adrenal masses.

Indium chloride bone marrow scintigraphy for hepatic myelolipoma: A case report.

BACKGROUND: As hepatic myelolipoma is rarely encountered, its radiological diagnosis using ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) is challenging. Hepatic myelolipoma is similar to fat-contained hepatic lesions seen in hepatocellular carcinoma and angiomyolipoma. Therefore, further development of techniques to diagnose hepatic myelolipoma is warranted. CASE SUMMARY: A 44-year-old obese man was found to have a hepatic lesion during his medical checkup. The lesion was 50 mm × 57 mm in size and was detected in segment 8 (S8) of the liver by US. The patient was diagnosed with hepatic lesion 20 years ago, but it was left unresolved. The patient had no symptoms, liver dysfunction, hepatitis virus antibody, or tumor marker elevation. Plain CT showed a well-defined lesion in S8 of the liver. The central and peripheral areas of the lesion primarily exhibited fat density and hypodensity, respectively. MRI revealed a capsule-like structure. Biopsy was performed to address the probability of hepatocellular carcinoma. The lesion was pathologically confirmed as a myelolipoma. Bone marrow scintigraphy performed using 111InCl3 revealed accumulation of the radiopharmaceutical in the soft tissue component, except in the fat-dominant part of the tumor, as well as in the surrounding liver parenchyma due to the presence of reticuloendothelial cells in the liver. CONCLUSION: This is the first report on the diagnosis of hepatic myelolipoma using 111InCl3 scintigraphy. The effectiveness of bone marrow scintigraphy for diagnosing hepatic myelolipoma might be limited. As radiopharmaceuticals accumulate in both hematopoietic and reticuloendothelial cells, the accumulation of radiopharmaceuticals in the lesion is obscure.

A case of adrenal myelolipoma complicated with Prader-Willi syndrome.

Introduction: Prader-Willi syndrome is a congenital disorder that occurs in one in 10 000-30 000 children and is characterized by obesity, short stature, and intellectual disability. Case presentation: A 24-year-old male patient with Prader-Willi syndrome presented with an enlarged adrenal tumor. Computed tomography detected a well-defined mass. Magnetic resonance imaging revealed an increased signal intensity predominantly in fatty areas, suggesting adrenal myelolipoma. Laparoscopic left adrenalectomy was performed. Postoperatively, the patient developed mild pulmonary atelectasis, myelolipoma was confirmed by histopathology, and there was no recurrence at approximately 2 years postoperatively. Conclusion: This is the first report of Prader-Willi syndrome complicated with adrenal myelolipoma, which was removed laparoscopically.