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CASE REPORT |
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| Year : 2021 | Volume
: 37
| Issue : 3 | Page : 277-280 |
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Adrenocortical oncocytoma associated with androgen excess: A rare cause of hirsutism
Prachi1, Hema Malini Aiyer1, Vikas Jain2
1 Department of Pathology, Dharamshila Narayana Superspeciality Hospital, New Delhi, India
2 Department of Urology, Dharamshila Narayana Superspeciality Hospital, New Delhi, India
| Date of Submission | 17-Dec-2020 |
| Date of Decision | 21-Feb-2021 |
| Date of Acceptance | 19-Mar-2021 |
| Date of Web Publication | 1-Jul-2021 |
Correspondence Address:
Prachi
Department of Pathology, Dharamshila Narayana Superspeciality Hospital, New Delhi
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/iju.IJU_636_20
 Abstract | | |
Adrenal oncocytomas are rare neoplasms that are usually benign and nonfunctional, and often detected incidentally. Very few cases have been reported of functioning adrenal oncocytomas. We report a rare case of adrenocortical oncocytoma in a 29-year-old female presenting with hirsutism and irregular menstrual history. The tumor was functional and was successfully managed by laparoscopic adrenalectomy. Detailed radiological, histological, and immunohistochemical workup was done to come to a definitive diagnosis of adrenal oncocytoma.
How to cite this article:
Prachi, Aiyer HM, Jain V. Adrenocortical oncocytoma associated with androgen excess: A rare cause of hirsutism. Indian J Urol 2021;37:277-80 |
| Introduction | |  |
Adrenocortical oncocytoma is an extremely rare tumor of the adrenal gland. It was first reported by Kakimoto[1] in 1986. Since then, around 120 cases of adrenocortical oncocytoma have been reported in the English language literature. There is no uniform consensus on diagnostic criteria and biological behavior.
Oncocytic neoplasms are benign in most cases, commonly arising in the kidneys or thyroid, parathyroid, salivary or pituitary glands. Rarely, they have been reported at other sites including the respiratory tract (as oncocytic neuroendocrine carcinoma), larynx, and choroid plexus. Oncocytomas in the adrenal are extremely rare and are usually non– functional and benign; around 20% of the adrenocortical oncocytomas demonstrate some elements of malignancy and 10%–20% of them appear to affect hormone production in the adrenal glands.[2]
The term “oncocyte,” first used by Hamperl[3] in 1950, describes a large, highly eosinophilic, granular cell associated with a Hurthle cell tumor of the thyroid gland. Adrenal cortical oncocytomas often present as large adrenal masses. Imaging alone cannot distinguish between benign and malignant tumors. Hence, a combination of clinical, radiological, and pathological findings is essential for definitive diagnosis.
It is well established that D4-androstenedione can be metabolized into testosterone by different 17b-HSD iso-forms. Occurrence of both 17b-HSD types 3 and 5 isoforms in the tumor at transcript and protein levels is consistent with testosterone synthesis. The oncocytoma expresses proteins synthesized in adrenal zona fasciculata, such as 17a-OH, 3b-HSD2, 21-OH, 11b-OH, and MC2R, as well as enzymes known to be produced in zona reticulata, including 17a-OH, CYB5, and 17b-HSD3/5, indicated an intermediate adrenocortical phenotype.[4] The rarity of the disease prompted this study, which discusses the clinical, radiological, histological, and immunohistochemical findings of an adrenal oncocytoma.
| Case Report | | |
A 29-year-old female patient presented with irregular menstrual bleeding and hirsutism for 3 years. The patient was on oral contraceptive pills for 4–5 months, and since then she was amenorrheic. A physical examination revealed increased hair over the chest and abdomen with no petechiae or abdominal striae (features suggestive of Cushing syndrome). An ultrasound showed a 7.8 cm × 7.8 cm well-defined heterogeneously hypoechoic solid vascular lesion in the right lobe of the liver which was further re-evaluated by contrast-enhanced computed tomography (CECT) with triphasic liver study, which revealed a well-defined 80 mm × 82 mm mildly enhancing soft-tissue density supra-renal mass indenting the liver and the right adrenal was not seen separate from the mass [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. On positron emission tomography-CT, a non-DOTANOC avid well defined rounded heterogeneously enhancing mass lesion in the right suprarenal location was observed. | Figure 1: (a and b) Contrast-enhanced computed tomography axial and (c and d) contrast-enhanced computed tomography coronal images show a well-defined mildly enhancing hypodense mass (white arrow) arising from the right adrenal gland displacing the right kidney inferiorly and indenting the medial surface of liver superiorly (e) Gross photograph of well-encapsulated adrenal mass. (f) Gross photograph of cut-section of the mass showing mahogany brown homogenous areas of foci of hemorrhage
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Baseline liver function test, renal profile, and serum electrolytes were normal . However, her TSH (7.02 μU/L, normal 0.25–5.0 μU/L), testosterone (725.5 ng/dl, normal 6–82 ng/dl) and DHEAS (12714 μg/dl, normal <340 μg/dl) levels were increased [Table 1].
A laparoscopic right adrenalectomy was performed using the lateral transperitoneal approach. The mass was completely encapsulated and easily dissected from the superior pole of the right kidney. The post-operative recovery was uneventful and on follow-up, the hormonal levels were within normal limits.
Pathology findings
A specimen of the right adrenalectomy weighed 280 gm and measured 8.5 cm × 7.5 cm × 5.5 cm with attached fat-measuring 2.5 cm × 1.5 cm × 1 cm. The capsule was intact. Cut surface showed, mahogany brown tumor replacing the entire adrenal parenchyma with areas of hemorrhage, but no necrosis [Figure 1]e and [Figure 1]f. Microscopic examination revealed an encapsulated tumor composed of sheets and nests of lipid poor large cells with abundant granular eosinophilic cytoplasm and foci of marked nuclear atypia with multinucleated cells. No necrosis was seen (mitotic Figures 0 to 1 per 50 hpf). No capsular or vascular invasion was seen. There was no Reinke crystalloid feature. According to the Lin-Weiss-Bisceglia criteria,[5] it was negative for malignancy [Figure 2]a. Immunohistochemically, the tumor cells were immunoreactive for CK (4+), calretinin (2+), melan-A (2+), synaptophysin (3+), and inhibin (4+) and were negative for chromogranin [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]e, [Figure 2]f. Based on the above morphologic and IHC findings, a final diagnosis of adrenocortical oncocytoma was made. Her postoperative period was uneventful. | Figure 2: Microphotograph of abundant granular eosinophilic cytoplasm and foci of marked nuclear atypia seen (H and E, ×400). (b) Immunoreactive score 4+ in neoplastic cells (IHC stain CK, ×400). (c) Immunoreactive score 3+ in neoplastic cells (IHC stain synaptophysin, ×400). (d) Immunoreactive score 2+ in neoplastic cells (IHC stain Melan A, ×400). (e) Immunoreactive score 4+ in neoplastic cells (IHC stain inhibin, ×400). (f) NonImmunoreactive score in neoplastic cells (IHC stain Chromogranin, ×400)
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| Discussion | | |
Adrenal masses are found in 1%–2% of abdominal CT scans and are detected incidentally. These adrenal “incidentalomas” can be cortical adenomas, cysts, myelolipomas, ganglioneuromas, pheochromocytomas, adrenocortical carcinomas, and adrenal metastases. The most common adrenal incidentalomas are non-functioning cortical adenomas. A biochemical evaluation aids in differentiating non-functional from functional adrenal masses. As both usually present as an incidental, large adrenal masses, CT and magnetic resonance imaging findings cannot be used to differentiate benign from malignant oncocytic neoplasms and only microscopic criteria are able to define malignancy and clinical behavior. Hence, laparoscopic adrenalectomy being the most common approach is the mainstay of therapy.
Adrenal oncocytic tumors are more common on the left side. Tumor size ranges from 3 to 15 cm. Oncocytomas are generally benign tumors. The Weiss system[5] distinguishes benign from malignant adrenocortical neoplasms. The Lin-Weiss-Bisceglia system for categorizing malignant oncocytic neoplasm are as follows (1) Major criteria (a mitotic rate of >5 mitoses per 50 high-power fields, any atypical mitoses or venous invasion), (2) Minor criteria (large size [>10 cm and/or >200 g], necrosis, capsular invasion, or sinusoidal invasion) and (3) definitional criteria (predominantly cells with eosinophilic-granular cytoplasm, high nuclear grade, and diffuse architectural pattern). The presence of any one of the major criteria indicates malignancy, the presence of one to four minor criteria is indicative of uncertain malignant potential, whereas the absence of all major and minor criteria indicates benign behavior.[5]
Grossly these tumors are rounded, well-circumscribed, and encapsulated. Cut surface shows mahogany brown color with areas of hemorrhage and necrosis. Histologically, these tumors contain cells arranged in solid, tubular, papillary, or trabecular patterns.[4] Cells are strongly eosinophilic and granular, due to the presence of numerous mitochondria.[4] The pathogenesis of these tumors is still not known. Some oncocytomas might be tumors of mitochondria at the subcellular level.
The present case was a right-sided well-defined supra-renal mass, on CECT, absence of fat attenuation (excluding myelolipoma). Large tumor size and heterogeneous echotexture on imaging raised the doubt of adrenocortical carcinoma. Investigations revealed the functional nature of the mass. Thus, a detailed microscopic evaluation was mandatory to come to a definitive diagnosis. In our case, a preoperative diagnosis was difficult. Immunohistochemistry revealed non-immunoreactivity for chromogranin A, thus ruling out pheochromocytomas.
| Conclusions | | |
Adrenal oncocytic neoplasm is one of the histological subtypes of incidentally detected adrenal masses. They are generally large, benign, non-functional adrenal tumors, with a prevalence in women and on the left side. However, the possibility of a functional adrenal oncocytoma should be considered. The assessment of their exact biologic behavior requires a detailed gross, microscopic, and comprehensive immunohistochemical evaluation with particular attention to the criteria that define malignancy.
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.
Financial support and sponsorship: Nil.
Conflicts of interest: There are no conflicts of interest.
| References | | |
| 1. | Kakimoto S, Yushita Y, Sanefuji T, Kondo A, Fujishima N, Kishikawa M, et al. Non-hormonal adrenocortical adenoma with oncocytoma-like appearances. Hinyokika Kiyo 1986;32:757-63.  |
| 2. | Bisceglia M, Ludovico O, Di Mattia A, Ben-Dor D, Sandbank J, Pasquinelli G, et al. Adrenocortical oncocytic tumors: Report of 10 cases and review of the literature. Int J Surg Pathol 2004;12:231-43. |
| 3. | Hamperl H. Onkocytes and the so called hurthle cell tumor. Arch Pathol 1950;49:563-70. |
| 4. | Tetsi Nomigni M, Ouzounian S, Benoit A, Vadrot J, Tissier F, Renouf S, et al. Steroidogenic enzyme profile in an androgen-secreting adrenocortical oncocytoma associated with hirsustism. Endocr Connect 2015;4:117-27. |
| 5. | Weiss LM, Medeiros LJ, Vickery AL Jr. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 1989;13:202-6. |
[Figure 1], [Figure 2]
[Table 1]
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