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BMC Urology Jul 2023Myelolipoma is a benign neoplasm of the adrenal cortex, composed of fat and hematopoietic cells. Although myelolipoma is benign, differentiation from adrenocortical...
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.
Topics: Male; Humans; Aged; Myelolipoma; Retroperitoneal Space; Adrenal Gland Neoplasms; Adrenalectomy; Laparoscopy
PubMed: 37420221
DOI: 10.1186/s12894-023-01288-5 -
IJU Case Reports Jul 2023Prader-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.
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.
PubMed: 37405031
DOI: 10.1002/iju5.12595 -
Medicina 2023Myelolipoma is a benign non-functional tumor. Most of them are asymptomatic and discovered incidentally, either through imaging studies or at autopsy. While it most...
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.
Topics: Female; Humans; Aged; Mediastinum; Myelolipoma; Thoracic Surgery, Video-Assisted; Radionuclide Imaging; Adrenal Gland Neoplasms
PubMed: 37379542
DOI: No ID Found -
Journal of Surgical Case Reports Jun 2023Adrenal myelolipoma is a rare benign neoplasm composed of mature adipose tissue and myeloid tissue with a variable amount of hematopoietic elements. Most patients are...
Adrenal myelolipoma is a rare benign neoplasm composed of mature adipose tissue and myeloid tissue with a variable amount of hematopoietic elements. Most patients are asymptomatic although some present with pain or even endocrine dysfunction. The rising use of CT and MRI scans has led to an increase of the detection of adrenal myelolipomas in recent years. The indications for surgery are symptomatic patients and lesions bigger than 5 cm or suspicious for malignancy. A case of a 50-year-old woman is presented here who was referred for surgical resection of a large nonfunctioning right adrenal mass. The neoplasm was resected through a midline laparotomy. Histopathology revealed a lesion consisting predominantly of fatty issue containing all types of hematopoietic stem cells and confirmed the diagnosis of myelolipoma.
PubMed: 37293332
DOI: 10.1093/jscr/rjad326 -
International Journal of Surgery Case... Jun 2023Once mostly discovered on autopsy, adrenal myelolipomas are now increasingly diagnosed due to the frequent use of modern imaging methods. However, bilaterality remains...
INTRODUCTION
Once mostly discovered on autopsy, adrenal myelolipomas are now increasingly diagnosed due to the frequent use of modern imaging methods. However, bilaterality remains quite rare. We present the case of a 31 years old female patient treated in our department for a bilateral adrenal myelolipoma which revealed an unknown peripheral adrenal insufficiency.
CASE PRESENTATION
We describe the case of a 31-year-old woman in apparent good health with no medical history who was explored for recurrent right lumbar pain by a computed tomography scanner which showed a large right adrenal mass and a smaller lesion in the left adrenal gland. Preoperative biology revealed an unknown peripheral adrenal insufficiency. Right open sub-costal adrenalectomy was performed, Histological examination confirmed the diagnosis of bilateral adrenal myelolipomas and radiological surveillance was planned for the left tumor.
DISCUSSION
Adrenal myelolipoma (AML) is a rare, benign and typically non-functional tumor of the adrenal gland, usually unilateral and asymptomatic, incidentally detected on CT. Commonly diagnosed between the fifth and seventh decades of life. It can affect both sexes our patient is a 31-year-old female and presented with bilateral AML. Unlike previous reported cases, our patient has a previously unknown peripheral adrenal insufficiency, which could be incriminated in the development of his bilateral adrenal myelolipomas. The optimal management depends on both clinical presentation and tumor characteristics.
CONCLUSION
Adrenal myelolipoma is a rare tumor. Endocrinological investigation should be performed to detect and treat endocrine disorders. The therapeutic attitude depends on tumor size complications and clinical complaints.
METHODS
This is a case report from our urology department, and has been reported in line with the SCARE criteria.
PubMed: 37230061
DOI: 10.1016/j.ijscr.2023.108330 -
Veterinary Sciences Mar 2023The histopathological diagnosis of canine splenic mass lesions is crucial for prognostication. However, thus far, no study has been conducted on the histopathology of...
The histopathological diagnosis of canine splenic mass lesions is crucial for prognostication. However, thus far, no study has been conducted on the histopathology of canine splenic mass lesions in Republic of Korea. Herein, the prevalence of splenic diseases was analyzed in 137 canine splenic mass lesions via histopathological diagnosis, and the microscopic pattern associated with each disorder was described. Immunohistochemistry was performed for CD31, CD3, PAX5, Iba1, and C-kit for a more accurate diagnosis of splenic tumors. The proportion of non-neoplastic disorders, including nodular hyperplasia (48.2%, n = 66) and hematoma (24.1%, n = 33), was 72.3%. Splenic tumors, including splenic hemangiosarcoma (10.2%, n = 14), splenic lymphoma (nodular and diffuse types, 8.0%, n = 11), splenic stromal sarcoma (7.3%, n = 10), myelolipoma (1.5%, n = 2), and mast cell tumors (0.7%, n = 1), accounted for 27.7% of cases. The results of this study will aid veterinary clinicians in communication with pet owners about prognoses, recommendations for splenectomy, and subsequent histopathological diagnoses. This study will facilitate further investigations with more detailed comparisons of splenic mass lesions between small- and large-breed dogs.
PubMed: 37104402
DOI: 10.3390/vetsci10040247 -
International Journal of Surgery Case... May 2023Myelolipoma is a rare benign neoplasm composed of mature elements of fatty and hematopoietic tissues. In addition to its low incidence, myelolipoma in kidney transplant...
INTRODUCTION AND IMPORTANCE
Myelolipoma is a rare benign neoplasm composed of mature elements of fatty and hematopoietic tissues. In addition to its low incidence, myelolipoma in kidney transplant patients is much rarer, with only a few cases of this entity reported in the literature.
CASE PRESENTATION
A 37-year-old diabetic patient was admitted to the hospital due to severe left abdominal pain and elevated creatinine levels of 4.4 mg/dl. The patient has a history of two kidney transplantations. The patient was investigated with different modalities of imaging, which revealed a mass adjacent to the renal graft that was suspicious for malignancy. Histopathological assessment of the resected lesion showed findings consistent with myelolipoma.
CLINICAL DISCUSSION
Although it's uncommon, myelolipoma should be considered in the differential diagnosis of any renal or extra-renal lesion in kidney transplant patients, including those with a history of graft rejection as in our patient.
CONCLUSION
In light of the rarity of the lesions, we affirm the significance of the clinicians' reporting such cases that they encounter in order to better understand the disease's biological behavior.
PubMed: 37043901
DOI: 10.1016/j.ijscr.2023.108134 -
Frontiers in Endocrinology 202321-hydroxylase deficiency (21OHD) is the most common cause of congenital adrenal hyperplasia (CAH). However, patients with 21OHD manifest various phenotypes due to a...
INTRODUCTION
21-hydroxylase deficiency (21OHD) is the most common cause of congenital adrenal hyperplasia (CAH). However, patients with 21OHD manifest various phenotypes due to a wide-spectrum residual enzyme activity of different CYP21A2 mutations.
METHODS
A total of 15 individuals from three unrelated families were included in this study. Target Capture-Based Deep Sequencing and Restriction Fragment Length Polymorphism was conducted on peripheral blood DNA of the three probands to identify potential mutations/deletions in CYP21A2; Sanger sequencing was conducted with the DNA from the family members of the probands.
RESULTS
Dramatically different phenotypes were seen in the three probands of CAH with different compound heterozygous mutations in CYP21A2. Proband 1 manifested simple virilizing with mutations of 30-kb deletion/c.[188A>T;518T>A], the latter is a novel double mutants classified as SV associated mutation. Although both probands carry the same compound mutations [293-13C>G]:[518T>A], gonadal dysfunction and giant bilateral adrenal myelolipoma were diagnosed for proband 2 and proband 3, respectively.
CONCLUSION
Both gender and mutations contribute to the phenotypes, and patients with the same compound mutations and gender could present with different phenotypes. Genetic analysis could help the etiologic diagnosis, especially for atypical 21OHD patients.
Topics: Humans; Adrenal Hyperplasia, Congenital; Steroid 21-Hydroxylase; Genotype; Genetic Association Studies
PubMed: 36992809
DOI: 10.3389/fendo.2023.1095719 -
Cureus Feb 2023Non-classic congenital adrenal hyperplasia (CAH) usually presents later in life with signs of androgen excess but may also be diagnosed after the detection of an...
Non-classic congenital adrenal hyperplasia (CAH) usually presents later in life with signs of androgen excess but may also be diagnosed after the detection of an incidental adrenal myelolipoma. This is a patient with previously undiagnosed CAH who presented to the emergency department with chest discomfort and palpitations. A computed tomography (CT) scan of the chest done to rule out pulmonary embolism showed bilateral large adrenal myelolipomas. She also had evidence of marked hirsutism on examination, which prompted further workup, and her laboratory data was in keeping with CAH. Further management was unable to be pursued due to the patient's poor compliance, and she was subsequently lost to follow-up. Chronic exposure of the adrenal glands to high adrenocorticotropic hormone (ACTH) levels increases the risk of developing myelolipomas. CAH needs to be considered as a diagnosis in the evaluation of incidental adrenal myelolipomas.
PubMed: 36938181
DOI: 10.7759/cureus.35017 -
Cureus Jan 2023Adrenal myelolipomas are benign adrenocortical tumors composed of adipose tissue mixed with hematopoietic precursor cells. An association of myelolipoma with adrenal...
Adrenal myelolipomas are benign adrenocortical tumors composed of adipose tissue mixed with hematopoietic precursor cells. An association of myelolipoma with adrenal cortical adenoma is rare and the pathogenesis of these tumors remains unclear. Here we present a case of an incidentally discovered adrenal tumor with radiologic characteristics of a myelolipoma who underwent adrenalectomy due to biochemical suspicion for pheochromocytoma. The final pathology, however, revealed a myelolipoma with a co-existing adrenal cortical adenoma without evidence of pheochromocytoma. Genetic analysis revealed the presence of a hitherto unreported heterozygous variant, c.329C>A (p.Ala110Asp), of the armadillo repeat-containing protein 5 (ARMC5) gene which when inactivated is commonly associated with bilateral adrenal nodularity.
PubMed: 36874660
DOI: 10.7759/cureus.34454