Davari Paran, Lee Cortney Youens, Lee James Te-An
AACE Clin Case Rep. 2020 Dec 20;6(2):e54-e58. doi: 10.4158/ACCR-2019-0402. eCollection 2020 Mar-Apr.
Adrenal myelolipoma (AM) is a benign tumor composed of mature fat cells and hemopoietic elements. Most AMs are incidental findings on imaging and clinically asymptomatic. The purpose of this case report is to describe a rare case of AM and explore its clinical manifestations, imaging features, and treatment.
In this study, we report a case of a rapidly growing right AM in a patient with uncontrolled hemoglobin sickle cell disease. A 38-year-old male presented to our institution's endocrine surgery clinic for evaluation of an enlarging right adrenal mass. This mass was incidentally found during an abdominal ultrasound performed for transaminitis and thrombocytopenia. Patient was asymptomatic without any abdominal discomfort, back pain, nausea, or vomiting.
Patient was lost to follow up until 2018. Follow-up computed tomography scan in 2018 showed the right adrenal mass measuring 12.3 cm in greatest dimension with significant macroscopic fat. Given the imaging features, AM was the presumed diagnosis. However, with a medical history of uncontrolled sickle cell disease, extra-medullary hematopoiesis and rapidly growing liposarcoma could not be ruled out. Surgical excision was performed due to size and significant tumor growth. Diagnosis was confirmed with histopathology and revealed myelolipoma.
Image characteristics can be helpful in diagnosis of AM; however, the appearance of this lesion on computed tomography can be similar to other adrenal gland pathologies such as liposarcoma and mass-forming extramedullary hematopoiesis. Percutaneous needle biopsy may be indicated if the diagnosis remains unclear.
肾上腺髓脂肪瘤(AM)是一种由成熟脂肪细胞和造血成分组成的良性肿瘤。大多数肾上腺髓脂肪瘤是在影像学检查中偶然发现的,临床上无症状。本病例报告的目的是描述一例罕见的肾上腺髓脂肪瘤病例,并探讨其临床表现、影像学特征及治疗方法。
在本研究中,我们报告一例血红蛋白病未得到控制的镰状细胞病患者右肾上腺髓脂肪瘤迅速生长的病例。一名38岁男性因右侧肾上腺肿物增大就诊于我院内分泌外科门诊。该肿物是在因转氨酶升高和血小板减少而进行的腹部超声检查中偶然发现的。患者无症状,无任何腹部不适、背痛、恶心或呕吐。
该患者直到2018年才进行随访。2018年的随访计算机断层扫描显示,右侧肾上腺肿物最大径为12.3 cm,有大量肉眼可见的脂肪。根据影像学特征,推测诊断为肾上腺髓脂肪瘤。然而,鉴于患者有未得到控制的镰状细胞病病史,不能排除髓外造血和快速生长的脂肪肉瘤。由于肿物大小及显著生长,遂行手术切除。组织病理学检查确诊为髓脂肪瘤。
影像学特征有助于肾上腺髓脂肪瘤的诊断;然而,该病变在计算机断层扫描上的表现可能与其他肾上腺疾病相似,如脂肪肉瘤和形成肿块的髓外造血。如果诊断仍不明确,可能需要进行经皮穿刺活检。