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JCEM Case Reports Jan 2023The presence of macroscopic fat on computed tomography (CT) imaging has been traditionally regarded as an indication that an adrenal lesion is likely to be a benign...
The presence of macroscopic fat on computed tomography (CT) imaging has been traditionally regarded as an indication that an adrenal lesion is likely to be a benign myelolipoma, for which further investigation is not usually required. Two cases are described where an adrenal lesion was eventually found to be malignant on histology (adrenocortical carcinoma in the first case, undifferentiated sarcoma in the second case), despite the presence of macroscopic fat on CT. In both cases there were other clinical and radiological indicators of potential malignant pathology. These cases add to increasing awareness in the literature that malignant adrenal tumors may rarely contain macroscopic fat, emphasizing a need for clinical vigilance.
PubMed: 37908270
DOI: 10.1210/jcemcr/luac029 -
Polish Archives of Internal Medicine Dec 2023
Topics: Humans; Adrenal Hyperplasia, Congenital; Leiomyoma; Myelolipoma; Adrenal Gland Neoplasms
PubMed: 37874249
DOI: 10.20452/pamw.16587 -
Frontiers in Endocrinology 2023Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of multiple epithelial neuroendocrine tumors (NETs) and...
BACKGROUND
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of multiple epithelial neuroendocrine tumors (NETs) and non-NETs in various organs. encodes a 610-amino acid-long tumor suppressor protein, menin. The optimal treatment for multiple tumors, identification of the most critical tumors for patient prognosis, and menin immunohistochemistry findings remain controversial. Therefore, we aimed to elucidate these issues through a histological analysis of tumors and tumor-like lesions in a Japanese family, comprising a father and his two sons, who had MEN1 with Zollinger-Ellison syndrome (ZES).
PATIENTS AND METHODS
All family members had a germline alteration in exon 10, c.1714-1715 del TC of , and exhibited multiple synchronous and metachronous tumors. The patients had pulmonary NETs, hyperparathyroidism, hypergastrinemia, pituitary adenomas, pancreaticoduodenal NETs, adrenocortical adenoma with myelolipoma, nodular goiter of the thyroid, lipomas, and angiofibroma. Most tumors were resected and histologically examined. We compared their clinical courses and tumor histology, and conducted menin immunohistochemistry (IHC).
RESULTS
Two patients died of pulmonary NET G2. One patient who underwent pancreaticoduodenectomy was cured of ZES; however, the two other patients who did not undergo pancreaticoduodenectomy suffered persistent ZES despite treatment with octreotide. Menin IHC revealed varying NET intensities, ranging from positive to negative stains.
CONCLUSION
Pancreaticoduodenectomy is the most effective treatment for ZES. Long-term follow-up is essential for pulmonary NET G2 owing to the risk of distant metastasis and/or multiplicity. Moreover, the variability of menin IHC in MEN1-related tumors may indicate the pattern of tumor formation rather than the diagnostic utility of menin in MEN1.
Topics: Humans; East Asian People; Immunohistochemistry; Multiple Endocrine Neoplasia Type 1; Neuroendocrine Tumors; Transcription Factors; Zollinger-Ellison Syndrome
PubMed: 37867522
DOI: 10.3389/fendo.2023.1221514 -
Cureus Aug 2023Adrenal 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...
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.
PubMed: 37692624
DOI: 10.7759/cureus.43240 -
Frontiers in Oncology 2023Bilateral 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...
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.
PubMed: 37681022
DOI: 10.3389/fonc.2023.1210679 -
Urology Case Reports Sep 2023Myelolipoma is a benign tumor composed of mature adipose tissue and normal hematopoietic components. It usually occurs in the adrenal glands but rarely in the...
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.
PubMed: 37664534
DOI: 10.1016/j.eucr.2023.102523 -
BMC Pulmonary Medicine Aug 2023Extra-adrenal myelolipoma is an unusual entity, and endobronchial myelolipoma is rarer, which is often ignored by clinicians, delaying the disease and affecting the... (Review)
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.
Topics: Male; Humans; Aged; Myelolipoma; Cough; Diabetes Mellitus, Type 2; Bronchi; Lipoma
PubMed: 37653374
DOI: 10.1186/s12890-023-02608-z -
World Journal of Clinical Cases Jun 2023As hepatic myelolipoma is rarely encountered, its radiological diagnosis using ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) is...
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 InCl 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 InCl 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.
PubMed: 37449238
DOI: 10.12998/wjcc.v11.i18.4377