|Year : 2020 | Volume
| Issue : 1 | Page : 82-84
Adrenal myelolipoma: A rare entity
Shubhangi Tayade, Akshay R Bondge, Archana Buch, Harsh Kumar
Department of Pathology, Dr. D. Y. Patil Medical College Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
|Date of Submission||18-Apr-2019|
|Date of Decision||09-Jul-2019|
|Date of Acceptance||03-Sep-2019|
|Date of Web Publication||16-Dec-2019|
Akshay R Bondge
802, E1, Ganga Skies Housing Society, Vallabh Nagar, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
Adrenal myelolipoma is an uncommon benign tumor of the adrenal gland, usually asymptomatic, unilateral, and nonsecreting. Adrenal myelolipoma is usually an incidental finding. It is composed of variable mixture of mature adipose tissue and trilineage hematopoietic elements. We report a case of 26-year-old female patient with complaints of headache, acne, hoarseness of voice, facial hair growth, poor breast growth, and enlarged clitoris. On investigating, serum testosterone, serum cortisol, serum androstenedione, and serum adrenocorticotropic hormone levels were found increased. The patient was operated for left adrenalectomy. On histopathology, it showed predominantly adipose tissue and areas of hemorrhage with foci of hematopoietic elements, comprising lymphocytes, plasma cells, megakaryocytes, as well as myeloid and erythroid cells. The case is presented to highlight this rare large functional tumor in a young adult female.
Keywords: Adrenal gland, benign neoplasm, myelolipoma
|How to cite this article:|
Tayade S, Bondge AR, Buch A, Kumar H. Adrenal myelolipoma: A rare entity. Med J DY Patil Vidyapeeth 2020;13:82-4
| Introduction|| |
The term “myelolipoma” was coined by Dr. Oberling in 1926, long after its description in 1905 by Dr. Gierke. Adrenal myelolipoma is an uncommon tumor of the adrenal gland that is benign, usually asymptomatic, unilateral, and nonsecreting. There is no gender predilection, and the peak age range at diagnosis is between 40 and 79 years. Adrenal myelolipoma is usually incidental finding either at autopsy or as a result of radiological investigation performed for other indications. It is composed of variable mixture of mature adipose tissue and trilineage hematopoietic elements. The pathogenesis is unclear and histopathological examination gives confirm diagnosis. Here, we describe a large secreting myelolipoma in a very young age patient having raised adrenal hormones.
| Case Report|| |
A 26-year-old female patient presented with headache and hypertension for the last 10 years which was treated pharmacologically. She also had complaints of acne, hoarseness of voice, facial hair growth, poor breast growth, and enlarged clitoris for 10 years. On examination, she showed hirsutism, acne, deep voice, and noncushingoid features. She had poorly developed breast and clitoromegaly. The patient was advised routine hematological and hormonal investigations and magnetic resonance imaging (MRI) abdomen. Hemogram and biochemical values are within normal range. T3, T4, and thyroid stimulating hormone levels are within normal limits. Karyotyping study showed 46XX normal female karyotype. On investigation, serum cortisol levels was 44.72 μg/dl (normal range: 6.2–19.4 μg/dl); serum testosterone level was 121.46 ng/dl (normal range:14–76 ng/dl); serum androstenedione level was 9.87 ng/ml (normal range: 0.3–3.3 ng/ml) and serum ACTH was 980 pg/dl (normal range: 0–46 pg/dl). The patient's MRI suggest a welldefined heterogeneous intraperitoneal mass lesion in the left suprarenal region measuring 72 mm × 70 mm × 45 mm invading superior pole of left kidney, suggestive of adrenal gland neoplasm ?adrenal cortical carcinoma [Figure 1]a. Computed tomography of the brain showed normal pituitary gland.
|Figure 1: (a) Magnetic resonance imaging abdomen showing a mass in the left suprarenal lesion measuring 72 mm × 70 mm × 48 mm. (b) Gross appearance of the tumor, a large, rounded, and encapsulated mass with smooth external surface|
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In light of these findings and after a thorough preoperative workup, the patient was operated for left adrenalectomy and the specimen was sent for histopathological examination. Gross examination of the specimen revealed a large, rounded, and encapsulated mass with smooth external surface measuring 8.5 cm × 8.0 cm × 4.6 cm [Figure 1]b. Cut surface of the tumor is brownish yellow, solid, soft to firm. Histopathology examination showed predominantly adipose tissue and areas of hemorrhage with foci of hematopoietic elements, comprising lymphocytes, plasma cells, megakaryocytes, as well as myeloid and erythroid cells [Figure 2]a and [Figure 2]b. The final histopathology was reported as myelolipoma of the adrenal gland.
|Figure 2: (a) Adrenal gland with mature adipocytes and hematopoietic cells (H and E, ×100). (b) Hematopoietic elements prominently showing megakaryocytes (arrow) (H and E, ×1000)|
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The patient had uneventful postoperative course and was discharged on the postoperative day 7. Three months after surgery, hormonal levels were within normal range.
| Discussion|| |
Adrenal myelolipomas are called “incidentalomas” because these tumors are frequently discovered incidentally. Its incidence in old autopsy series is about 0.08%–0.25%. The most common site is adrenal gland but can also occur in retroperitoneum around kidney and presacral space or in liver. There is no gender predilection, and the peak age range at diagnosis is between 40 and 79 years.
These tumors are usually asymptomatic but can become symptomatic because of its bulky effect, and the most consistent complaint is abdominal pain due to rupture and retroperitoneal hemorrhage. Hypertension and hematuria may be other symptoms reported by the patient. The most widely accepted theory is adrenocortical cell metaplasia response to stimuli, such as necrosis, inflammation, infection, stress, or excess ACTH secretion over a long period of time. The myelolipomatous tissue can replace either the tumorous or hyperplastic adrenocortical cells. Routinely, an endocrinological study is performed to find a congenital or secondary endocrinal disorder or a secreting neoplasm.
In our case, tumor is discovered incidentally in a relatively young patient with secretary activity. Presence of fat and hematopoietic elements such as myeloid, erythroid, and megakaryocytic series on microscopic picture along with radiological correlation clinched the diagnosis of myelolipoma.
The differential diagnosis should include renal angiomyolipoma, retroperitoneal lipoma, and liposarcoma.
Asymptomatic myelolipomas are treated conservatively. Surgical intervention is required in symptomatic tumors and in tumors bigger than 7 cm 4. Laparoscopic adrenalectomy is now preferred with good outcome.
| Conclusion|| |
Myelolipoma is a rare tumor and rarer in young age. Earlier recognition is important as they have the risk of spontaneous retroperitoneal hemorrhage. Thorough preoperative hormonal study is mandatory to look for endocrine abnormalities. Radiological findings help in early suspicion of this asymptomatic tumor although histopathological examination remains the gold standard for definitive diagnosis of adrenal myelolipoma.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]