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LETTER TO THE EDITOR
Year : 2022 | Volume
: 59
| Issue : 2 | Page : 297-299
Oncocytic variant of adrenocortical carcinoma: A rare entity
Siddhi G Sinai Khandeparkar, Maithili M Kulkarni, Smita G Solanke, Prajakta A Shinde
Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Pune, Maharashtra, India
Correspondence Address:
Siddhi G Sinai Khandeparkar
Department of Pathology, Smt. Kashibai Navale Medical College and General Hospital, Pune, Maharashtra
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/ijc.IJC_278_21
How to cite this article:Dear Editor,
A 45-year female presented to the surgery outpatient department with complaints of abdominal mass and fever on and off for 1 month. In addition, she complained of a headache for 2 months which aggravated in the winter season. Physical examination revealed blood pressure of 216/110 mmHg, pulse rate of 102/min and oxygen saturation of 92%. On abdominal examination, a lump was felt in the left lumbar region. Respiratory examination showed bilateral wheezing and basal crepitations. She gave the history of being hypertensive for 15 years. Clinical diagnosis of pheochromocytoma was suggested. Computed tomography (CT) of the abdomen and pelvis showed a large well-defined heterogeneously enhancing soft tissue density lesion replacing the left adrenal gland. It measured 16.2 × 15.4 × 13.9 cm. Multiple non-enhancing areas suggestive of necrosis and few foci of calcification were noted. Multiple prominent veins were seen along its periphery which drained into the left renal vein. Fat planes with adjacent structures were well maintained. Inferiorly, the mass was seen abutting and displacing the left kidney. However, the left kidney showed normal renal excretion. The right adrenal gland showed homogeneous enhancement with no focal lesion. Radiological diagnosis suggestive of neoplastic etiology favoring pheochromocytoma was offered. Urinary vanillylmandelic acid (VMA) level was 3.54 mg/24 hours (normal value <13.6 mg/24 hours by ion exchange chromatography followed by photometry). Other systemic examination and routine laboratory findings were noncontributory. Her coronavirus disease 2019 (COVID-19) test by reverse transcription polymerase chain reaction was negative. Cortisol levels were not done in this case. Intraoperatively, 20 × 15 × 10 cm left adrenal tumor was seen with dense adhesions to spleen, pancreas, and mesentery. Dilated left adrenal vein was noted.
Gross examination showed a single, globular, yellowish-brown encapsulated tumor mass measuring 19 × 18 × 7.5 cm with prominent blood vessels on the external surface [Figure 1]. The Weight of the tumor was 1700 g. Cut section showed a variegated friable appearance with the presence of solid cystic areas, foci of necrosis, and hemorrhage. Tumor areas showed negative dichromate reaction. Microscopic examination showed an encapsulated tumor. The tumor cells were arranged in sheets and nests with many of them individually interspersed. Individual tumor cells were large with eccentrically placed highly pleomorphic, vesicular nuclei and one to two prominent nucleoli. All the tumor cells showed abundant eosinophilic cytoplasm giving an oncocytic appearance. Intranuclear and intracytoplasmic inclusions were visualized. There were seen binucleate and multinucleate giant cells. Extensive areas of necrosis and hemorrhage were noted. No mitotic figure was found after an extensive search. Surrounding area of normal adrenal cortical tissue was seen. Lymphovascular and capsular invasion was identified. Histopathological diagnosis of Oncocytic variant of Adrenocortical Carcinoma (OAC) was given. Close differentials of pleomorphic rhabdomysarocma, pheochromocytoma, and chromophobe renal cell carcinoma (RCC) were carefully ruled out through clinicoradiological and meticulous histopathological examination.
Figure 1: (a) Gross photograph of adrenal gland showing cut surface with grayish white and focal yellow areas (a inset), external surface showing globular encapsulated yellowish specimen, and photomicrograph showing (b) (with b inset) lobules of oncocytic tumor cells separated by fibrous strands, (c) (with c inset) tumor cells showing binucleation, nuclear, and cytoplasmic inclusions, and rhabdoid appearance, (d) tumor showing areas of necrosis, (e) normal adrenal gland, tumor cells showing (f) capsular and vascular invasion, (g) melan A positivity, and (h) desmin negativityImmunohistochemical studies (IHC) performed showed melan A positivity and desmin negativity. Diagnosis of OAC was reinforced. Positron emission tomography (PET) scan of the patient could not be done due to financial constraints.
Postoperatively, the patient developed breathlessness. CT of the chest showed moderate left pleural effusion. The patient succumbed to pneumonia before receiving adjuvant therapy.
Adrenocortical neoplasms are the most frequent abnormalities of the adrenal cortex. They are found in about 1% of the general population, increasing with age to 6% in the elderly.[1] Oncocytic adrenocortical neoplasms (OAN) represent a rare group of tumors with approximately 147 cases reported in the literature so far. OAC represents a rare subgroup of OAN with approximately 36 cases documented in the literature.[2] Here, we intend to document a rare case of OAN in a 45-year female with IHC study.
Adrenocortical tumors are usually solitary lesions and their vast majority occur in adults without sex predilection.[3] Bisceglia et al. have proposed categories for OAN as pure oncocytic tumor, if a tumor is exclusively or almost entirely composed (greater than 90%) of oncocytic cells, mixed oncocytic tumor, when a clear cell component is also present (ranging from 10 to 50%), and ordinary adrenocortical tumor with focal oncocytic changes, if the oncocytic component is not a predominant one (less than 50% of the tumor mass). It is critical to extensively sample an OAN as one should discriminate a pure oncocytic tumor either from an ordinary adrenocortical tumor with focal oncocytic changes or its conventional counterpart of the compact cell type in order not to incorrectly apply the Weiss system and accordingly inadequately estimate its biological behavior. Also, an oncocytic tumor can only be labeled as pure after quantifying each individual component (oncocytic and clear) since pure and mixed oncocytic tumors do not seem to share similar clinical outcome.[4] The tumor was composed of entirely oncocytic cells in the present case.
Bisceglia et al. developed criteria for the classification of OAN. These were based on parameters of the Weiss system and constituted the Lin–Weiss–Bisceglia system. This included major criteria (a mitotic rate of more than five mitoses per 50 high-power fields, any atypical mitoses or venous invasion), minor criteria [large size (>10 cm and/or >200 g), necrosis, capsular invasion or sinusoidal invasion], and definitional criteria (predominantly cells with eosinophilic-granular cytoplasm, high nuclear grade, and diffuse architectural pattern). The latter are not used as they are present in all subgroups of pure oncocytic neoplasms and are not determinants of biological behavior. According to the proposed working rules, the presence of any one of the major criteria indicates malignancy (OAC), and the presence of one to four minor criteria is indicative of uncertain potential (borderline oncocytic neoplasm of uncertain malignant potential), while the absence of all major and minor criteria indicates benign behavior (adrenocortical oncocytoma).[4] The presence of features such as purely oncocytic tumor, vascular and capsular invasion, size of 19 cm, weight of 1700 g, and necrosis helped in arriving at the diagnosis of OAC in the present case.
Clinicopathological differential diagnosis considered in this case, included pleomorphic rhabdomyosarcoma, pheochromocytoma, and chromophobe RCC. Pheochromocytoma was ruled out as urinary VMA levels were within normal limits and gross specimen showed negative dichromate reaction.[5],[6] The presence of normal adrenal tissue on microscopic examination together with radiology and intraoperative finding of adrenal mass and normal corresponding kidney ruled out the possibility of chromophobe RCC.[2] IHC studies showing the absence of desmin reaction excluded the diagnosis of rhabdomyosarcoma.[7] OAC shows a similar expression of IHC markers such as vimentin, Melan A, calretinin, and synaptophysin, similar to conventional adrenocortical carcinomas.[3]
The mainstay therapeutic approach in OAC is wide surgical excision. In OAC radiotherapy, mitotane and/or chemotherapy is given individually post bulky cytomassive excision, depending on disease staging and predominant symptoms.[3] Our patient could not afford PET scan; hence, evidence of spread of the disease could not be established. Furthermore, she developed pneumonia postoperatively and succumbed to it without receiving any further adjuvant therapy.
Our experience with present case puts on record this rare entity and need for a triad of clinical, radiological, and meticulous immunohistopathological analysis for arriving at an accurate diagnosis and ruling out the differentials.
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
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Conflicts of interest
There are no conflicts of interest.
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