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Year : 2023  |  Volume : 66  |  Issue : 3  |  Page : 627-631
The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review

1 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, India
2 Department of Urology, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, India
3 Department of Surgical Oncology, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, India

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Date of Submission26-May-2021
Date of Decision12-Nov-2021
Date of Acceptance14-Nov-2021
Date of Web Publication07-Jun-2022


Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. Extensive rhabdoid morphology in ACC has been described recently in very few cases. The proportion of rhabdoid morphology and the role of SMARCB1/ INI1 expression in these tumor cells to diagnose the specific variant is not described in the literature. We reviewed the clinicopathological features of nine cases of adrenocortical neoplasm. Out of which, three cases of ACC showed predominant rhabdoid morphology. Large discohesive cells with abundant cytoplasm containing eosinophilic inclusions, eccentric vesicular nucleus, and prominent nucleoli. INI1 immunostain was retained in all cases. We reported the rhabdoid variant of ACC, a novel entity, and its diagnostic approach from their histological mimickers. Identifying more cases of this entity will help to clearly understand the pathogenesis, biologic behaviour, and any specific molecular alterations in the future.

Keywords: Adrenocortical carcinoma, INII1, rhabdoid variant, SMARCB1

How to cite this article:
Ayyanar P, Sable MN, Adhya AK, Das MK, Kar M, Mishra P. The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review. Indian J Pathol Microbiol 2023;66:627-31

How to cite this URL:
Ayyanar P, Sable MN, Adhya AK, Das MK, Kar M, Mishra P. The rhabdoid variant of adrenocortical carcinoma-Report of three cases and literature review. Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Sep 27];66:627-31. Available from:

   Introduction Top

Adrenocortical carcinoma (ACC) is a primary tumor arising from the adrenal cortex. It has three variants (oncocytic, myxoid, and sarcomatoid).[1] The Rhabdoid variant/rhabdoid features in adrenocortical neoplasms is an uncommon and recently described entity.

A malignant rhabdoid tumor (MRT) was initially reported in the pediatric kidney. Histopathological features include discohesive tumor cells arranged in sheets, abundant cytoplasm containing eosinophilic inclusions, eccentrically placed round nucleus with a prominent nucleolus. Extra-renal MRT includes atypical teratoid/rhabdoid tumor [AT/RT], tumors located in the soft tissue, lung, esophagus, etc. Poorly and undifferentiated carcinoma will show rhabdoid morphology. However, the cut-off of rhabdoid morphology to consider it as a specific variant has not been mentioned in the literature.[2]

SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1)/INI1 (Integrase interactor 1) deficient tumors are characterized by the inactivation of the SMARCB1 gene by mutations. Although this group of malignant tumors tends to show rhabdoid morphology, INI1 inactivation has also been reported in epithelioid sarcoma, renal medullary carcinoma, epithelioid malignant peripheral nerve sheath tumor, and sinonasal basaloid carcinoma.[3] The role of INI1 expression by molecular or immunohistochemistry (IHC) method in malignant rhabdoid tumors and tumors with rhabdoid morphology is not clearly documented. Rhabdoid morphology/differentiation indicates clinically aggressive behaviour and needs proper management and follow-up.

We report three cases of a rhabdoid variant of ACC, and a diagnostic approach. Only five cases of adrenocortical neoplasms have been reported to date.[4],[5],[6]

   Case Details Top

Case 1

A 54-year-old female patient presented with anorexia, abdominal pain for 20 days, and incidentally detected right suprarenal mass. Right-sided adrenalectomy was done. Grossly a single encapsulated solid gray-brown mass weighing about 917 grams and measuring 14 cm in greatest dimension. Microscopic examination revealed two morphological types of tumor cells arranged in solid sheets and nests [Figure 1]a and [Figure 1]b. One group of morphology is arranged in discohesive cells in sheets; these were large cells with abundant cytoplasm containing eosinophilic hyaline inclusion, eccentrically placed round to oval vesicular nucleus and prominent nucleoli [Figure 1]c. This morphology was seen in 70% of the total tumor area. The other tumor morphology was sheets of large cells with abundant cytoplasm containing vacuolar and granular appearance resembling “spider cells” and also showed eosinophilic hyaline inclusions, central and eccentrically placed vesicular nucleus, and large intranuclear inclusion like nucleoli, which were PAS negative [Figure 1]b. Multinucleation, mitotic activity-8--10/50 high power field (HPF), necrosis, capsular and sinusoidal invasion were noted. There was no atypical mitosis.
Figure 1: (a) The histomorphology of case 1- ACC, rhabdoid variant showing diffuse sheets of two types of morphology (Hematoxylin and Eosin, 100x); (b) The other morphology of tumor cells containing abundant cytoplasm with peripheral vacuolization as well as eosinophilic cytoplasmic inclusion, a moderate degree of nuclear pleomorphism, eccentrically placed vesicular nucleus and large prominent inclusion like nucleoli (arrow) (Hematoxylin and Eosin, 200x); Inset showed large intranuclear inclusions (arrow) were PAS negative (Periodic Acid Schiff, 400x); (c) sheets of discohesive tumor cells (rhabdoid morphology) with abundant eosinophilic cytoplasm and a moderate degree of cellular pleomorphism, large intranuclear cytoplasmic inclusions (Hematoxylin and Eosin, 200x, 400x); (d) retained INI1 expression in tumor cells (immunostain, 200x); (e) The histomorphology of case 2- ACC, sheets of tumor cells showing rhabdoid morphology (Hematoxylin and Eosin, 100x); (f) discohesive tumor cells with high mitotic activity, atypical mitotic figure (arrow), a moderate degree of cellular pleomorphism (Hematoxylin and Eosin, 400x); (g) Along with these tumor cells and few spider cells (arrow) (Hematoxylin and Eosin, 200x); 1h) retained INI1 expression in tumor cells (immunostain, 400x)

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Case 2

A 14-year-old female patient presented abdominal distention involving the left flank region for four months and had a left thigh mass. The thigh mass was diagnosed as rhabdomyosarcoma, and she had a left adrenal mass. She underwent excision of the left adrenal mass. Gross examination showed a single encapsulated gray-brown solid mass weighing about 220 grams and measuring 9.5 cm in greatest dimension. Microscopic examination showed similar morphology as described in case 1 [Figure 1]e, [Figure 1]f and [Figure 1]g. This morphology was comprised 50% of the total tumor area. The other morphology of tumor was nests of cohestive cells, mitotic activity-12-15/50HPF, atypical mitotic figures [Figure 1]f, necrosis, capsular, and lymphovascular invasion.

Case 3

A 38-year-old male patient presented with incidentally detected left adrenal mass and had hypertension. Excision of the mass showed a gray-brown solid mass weighing about 808 grams and measuring 14.5 cm in the greatest dimension. Microscopic examination showed an encapsulated tumor arranged predominantly in solid sheets and lobules with similar morphology as described in case 1 & 2 [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. This morphology was seen in 65% of the total tumor area. The mitotic count was 20/50HPF. Atypical mitotic figures, necrosis, capsular invasion were absent. No lymphovascular invasion. Focal areas of alveolar pattern containing haemorrhage [Figure 2]e and sarcomatoid pattern [Figure 2]f were also noted.
Figure 2: (a) The histomorphology of case 3 -ACC, rhabdoid variant showing tumor with peripheral normal adrenal parenchyma (arrow) (Hematoxylin and Eosin, 20x); (b& c) sheets of rhabdoid cells with a moderate degree of cellular pleomorphism (Hematoxylin and Eosin, 200x); (d) tumor cells with eosinophilic cytoplasmic inclusions (Hematoxylin and Eosin, 20x); (e) alveolar pattern of rhabdoid tumor cells (Hematoxylin and Eosin, 200x); (f) focal sarcomatoid pattern (Hematoxylin and Eosin, 200x); (g) retained INI1 expression in tumor cells (immunostain, 400x)

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IHC for INI1: Immunostain for INI1 was performed in all three cases, and it was retained [Figure 1]d and [Figure 1]h, [Figure 2]g. We reported as ACC, rhabdoid variant.


The first case was lost follow-up; the second and third cases were alive without recurrence after 24 months and five months after the diagnosis.

   Discussion Top

We reviewed the clinicopathological features of reported cases, shown in [Table 1].[4],[5],[6] Weissferdt A et al.[6] have mentioned the rhabdoid areas in 70–90% of the tumor mass. We had noticed rhabdoid morphology comprising 70%, 50%, and 65% of the tumor mass in three cases, respectively. We compared the other features of reported cases, the similarities noted in age, sex, and functional clinical presentation. Tumor size and weight are significantly large in our cases compared to the previous cases.
Table 1: Clinicopathological review of adrenocortical tumors with rhabdoid features/variant

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It is important to identify this entity from the histologic mimickers, which include MRT, the oncocytic variant of ACC, rhabdoid renal cell carcinoma, and rhabdomyosarcoma. MRT shows classical morphology, cytoplasmic inclusions, and loss of INI1 expression.[2] This finding will help to differentiate it from the malignant rhabdoid tumor of the kidney in the pediatric population. Oncocytic tumors will show abundant granular eosinophilic cytoplasm with a centrally placed nucleus containing mitochondria. Immunostain for antimitochondrial antibody indicates oncocytic differentiation.[7] We did not have the antimitochondrial antibody. Hence it was not performed. Application of Weiss score for determining malignant in the rhabdoid variant is yet to be studied as only very few cases have been reported. Renal cell carcinoma will show conventional morphology and positive immunostaining for PAX8 and CD10.

Rhabdoid morphology of carcinoma (of different sites) was compared with INI1 immunoexpression in a few studies. Kinoshita F et al.[8] noted that SMARCB1 expression was intact in 96% cases of renal cell carcinoma with rhabdoid features. Retained INI1 expression was reported in three undifferentiated and three dedifferentiated endometrial carcinomas with rhabdoid morphology.[9] Undifferentiated malignant tumors with rhabdoid phenotype showed retained INI1.[10] Most of these rhabdoid morphology tumors showed loss of SMARCA4. Although rhabdoid morphology is commonly associated with INI1 deficiency, it does not always show loss of INI1. The rhabdoid morphology extends its pathological alteration to other core subunits of SWI/SNF deficient family tumors, especially SMARCA4.

We conclude that rhabdoid variant of ACC is a recently described entity. Immunostain for INI1 will be retained in these tumors. A proper clinicopathological and immunohistochemical features approach towards a correct diagnosis.

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.

   References Top

DeLellis RA, Lloyd RV, Heitz PU, Eng C, editors. Pathology and Genetics of Tumours of Endocrine Organs (World Health Organization Classification of Tumours). Lyon, France: IARC Press; 2004.  Back to cited text no. 1
Zimmermann A. Malignant rhabdoid tumors and tumors with rhabdoid features. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Cham: Springer; 2017.  Back to cited text no. 2
Kohashi K, Oda Y. Oncogenic roles of SMARCB1/INI1 and its deficient tumors Cancer Sci 2017;108:547-52.  Back to cited text no. 3
Dundr P, Povýsil C, Zelinka T, Tvrdík D, Ciprová V, Novák K. Adrenocortical adenoma with rhabdoid features. Pathol Res Pract 2006;202;177-81.  Back to cited text no. 4
Lou E, Goodwin J, Howell DN, Hicks J, Caram LB. A G-CSFsecreting adrenal carcinoma with rhabdoid differentiation causing leukocytosis. Nat Rev Urol 2009;6;392-7.  Back to cited text no. 5
Weissferdt A, Phan A, Suster S, Moran CA. Primary rhabdoid adrenocortical carcinoma: A clinicopathological and immunohistochemical study of three cases. Histopathology 2013;62:771-7.  Back to cited text no. 6
Wong DD, Spagnolo DV, Bisceglia M, Havlat M, McCallum D, Platten MA. Oncocytic adrenocortical neoplasms—A clinicopathologic study of 13 new cases emphasizing the importance of their recognition. Hum Pathol 2011;42:489-99.  Back to cited text no. 7
Kinoshita F, Kohashi K, Sugimoto M, Takamatsu D, Kiyozawa D, Eto M, et al. The SWI/SNF chromatin-remodeling complex status in renal cell carcinomas with sarcomatoid or rhabdoid features. Virchows Arch 2020;477:651-60.  Back to cited text no. 8
Ramalingam P, Croce S, McCluggage WG. Loss of expression of SMARCA4 (BRG1), SMARCA2 (BRM) and SMARCB1 (INI1) in undifferentiated carcinoma of the endometrium is not uncommon and is not always associated with rhabdoid morphology. Histopathology 2017;70:359-66.  Back to cited text no. 9
Mei L, Alikhan M, Mujacic I, Parilla M, Antic T. Genomic alterations in undifferentiated malignant tumors with rhabdoid phenotype and loss of BRG1 immunoexpression identified by fine needle aspirates. Acta Cytol 2019;63:438-44.  Back to cited text no. 10

Correspondence Address:
Pritinanda Mishra
Department of Pathology and Lab Medicine, All India Institute of Medical Sciences (AIIMS), Bhubaneswar - 751 019, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_515_21

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