|Year : 2021 | Volume
| Issue : 2 | Page : 96-98
A case report of rare, giant adrenal myelolipoma
Puneet Kumar, Katyayani Kumari Choubey, Ajay K Khanna, Satyendra K Tiwary
Department of General Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
|Date of Submission||26-May-2020|
|Date of Decision||29-Jun-2019|
|Date of Acceptance||09-Aug-2020|
|Date of Web Publication||12-Sep-2020|
Dr. Satyendra K Tiwary
Department of General Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Giant adrenal myelolipoma is a rare entity. It becomes evident due to symptoms of dragging sensation and discomfort or pain. We report hereby a rare case report giant adrenal myelolipoma. A 51-year-old female presented with pain in the right upper abdomen, dull aching, non radiating, no aggravating, or relieving factor present. It was associated with a lump in the right upper abdomen. Computed tomography of the abdomen suggested a 20 cm × 18 cm well encapsulated, hypodense mass, arising from right suprarenal gland inferior to the right lobe of the liver, pushing right kidney inferiorly, and inferior vena cava medially. It showed predominantly fat component and a small area of soft-tissue attenuation with maintained adjacent fat planes, suggestive of right adrenal myelolipoma. After preoperative stabilization, excision of right adrenal myelolipoma was done under general anesthesia and epidural anesthesia.
Keywords: Adrenal, myelolipoma, adrenalectomy
|How to cite this article:|
Kumar P, Choubey KK, Khanna AK, Tiwary SK. A case report of rare, giant adrenal myelolipoma. J Med Sci 2021;41:96-8
| Introduction|| |
Myelolipoma is a rare, benign tumor arising from hemopoietic and adipose tissues. Most of them are small in size and are diagnosed incidentally, more because of the availability of various radiological modalities. Giant adrenal myelolipoma is a rare entity and becomes evident due to symptoms of dragging sensation and discomfort or pain.
| Case Report|| |
A 51-year-old female presented with pain in the right upper abdomen, dull aching, nonradiating, no aggravating, or relieving factor present. The pain was associated with a lump in the right upper abdomen. There was no history of nausea, vomiting, fever, jaundice, hematemesis, or melena. On physical examination, a vague lump was evident in the right lumbar region, nontender, hard in consistency, immobile with a smooth surface, and irregular border. Computed tomography (CT) of the abdomen suggested a 20 cm × 18 cm well encapsulated, hypodense mass [Figure 1] arising from the right suprarenal gland inferior to the right lobe of the liver pushing right kidney inferiorly and inferior vena cava medially. It showed predominantly fat components and a small area of soft-tissue attenuation with maintained adjacent fat planes, suggestive of right adrenal myelolipoma. Routine parameters and hematological investigations were within the normal range, including complete blood count, liver function test, renal function test , and blood sugar. Her serum level of cortisol was 11.2 ng/dl (10.4–35.0), and androgen was 195 pg/dl (35.7–240.0). The serum level of ACTH was also in the normal range at 58.70 pg/ml (7.2–63.3), confirming no hormonal activity of the tumor.
|Figure 1: Contrast-enhanced computed tomography of large adrenal myelolipoma of the right adrenal gland|
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After preoperative stabilization, excision of right adrenal myelolipoma was done under general anesthesia and epidural anesthesia. Intraoperative findings corresponded to CT findings. The right adrenal vein was ligated, and the right adrenalectomy was done [Figure 2]. No lymphadenopathy was evident.
In the postoperative period, the patient was stable. Drain output was 50 ml on the postoperative day 1 (POD 1), which decreased over time and was removed on POD 3. The patient was orally allowed after drain removal, which she tolerated well. The patient was discharged in satisfactory condition on POD 5. In the follow-up period, there were no complaints. Histopathological examination (×200) with hematoxylin and eosin staining revealed proliferation of mature and variable-sized adipocytes [Figure 3] along with the presence of component tissues, including, lipid vacuoles, and hematological elements, including myeloid, erythroid, and megakaryocytic cells confirming the diagnosis of adrenal myelolipoma.
|Figure 3: Microphotograph (×200) with hematoxylin and eosin (H and E,) staining showing fat cells and myeloid cells confirming myelolipoma|
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| Discussion|| |
Myelolipomas predominantly involve the adrenal gland, but extra-adrenal sites, including the presacral area, stomach, liver, lymph nodes, mediastinum, and cranium, have also been recorded. Adrenal myelolipoma was first introduced by Gierke in 1905, and the name myelolipoma was coined by Oberling in 1929. It consists mainly of lipoid and hematopoietic elements, and reticulum is present only in the fatty areas.
Myelolipoma is a neoplastic lesion of monoclonal origin and is usually nonfunctional. No gender predilection is noted. Unilateral adrenal myelolipoma is more frequently observed on the right side, and bilateral disease is very rare. The etiology is not clear but it has been variously hypothesized that it is the resultant of metaplasia of adrenocortical cells precipitated by chronic stress, infection, or necrosis. Acute hemorrhage is the most significant complication, especially with large myelolipomas. It can manifest with local pain in the back, epigastrium, or flanks, accompanied by nausea, vomiting, hypotension, and anemia. Only 10% of adrenal myelolipomas are associated with Cushing's syndrome, Conn's syndrome, or congenital adrenal hyperplasia.
It is important to distinguish adrenal myelolipomas from extramedullary hematopoietic tumors, which are first, more often multiple than solitary, second, are frequently associated with splenomegaly and hepatomegaly and third, are secondary to severe anemia (e.g., thalassemia, hereditary spherocytosis), various myeloproliferative diseases, myelosclerosis, and skeletal disorders.
Adrenal myelolipomas are generally clinically silent, mostly diagnosed as incidental findings on imaging. Usually, contrast-enhanced CT (CECT) of the abdomen is considered the primary imaging modality of choice for diagnosis and further workup plan for treatment. In >90% cases, CECT is a diagnostic investigation with fair accuracy. It shows a heterogeneous hypoattenuating mass with an enhancing rim of soft tissue in the adrenal fossa.
Hyper attenuation may be a salient feature of hemorrhage or necrosis. Magnetic resonance imaging (MRI) usually shows high intensity on the T1-weighted image because of the adrenal tissue, and intermediate to high intensity on the T2-weighted image, due to mixed myeloid and fat tissue. This appearance may be confused with adrenal metastasis or primary adrenal carcinoma. The diagnosis may be confirmed by fat suppressing the MRI technic, which shows loss of signal intensity in the fat component. Adrenal myelolipoma may also mimic angiomyolipoma of the kidney arising from the upper pole, but the reconstruction image of the multislice CT scan easily differentiates the origin.,
Treatment of an asymptomatic small adrenal myelolipoma (<5 cm) is usually conservative, with monitoring at 6–12 months intervals with ultrasound or CT scan. Surgical removal should be considered for symptomatic myelolipomas, although they are generally asymptomatic. When the tumor size is >10 cm, the operation is recommended because of the potential risk of hemorrhage, rupture, and malignant change., Reports on laparoscopic excision of large adrenal myelolipomas are few, but Yamashita reports that assisted with advanced preoperative diagnostic imaging techniques, laparoscopic adrenalectomy is a feasible, safe, and effective treatment for adrenal myelolipoma.
In conclusion, dragging discomfort and pain in the abdomen due to organ enlargement were the only symptoms associated with the patient. Hormonal evaluation with normal serum cortisol, serum androgen, and serum ACTH confirmed the nature of the tumor without endocrine disorder and no hormonal activity even after gigantic size attainment ruling out the possibility of pheochromocytoma and Conn's Syndrome. Large-sized adrenal mass endocrine activity confirmed by hormonal assay and successful surgical excision were integral parts in this case evaluation, treatment, and follow-up.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]