An Intrarenal Adrenocortical Carcinoma Arising in an Adrenal Rest

Abstract

We describe a case of a 61-year-old Korean man who was diagnosed with renal cell carcinoma that was discovered on abdominopelvic computed tomography obtained after the patient complained of back pain. A radical nephrectomy was performed, and the surgical specimen showed a relatively well-circumscribed and yellowish lobulated hard mass. Microscopically, the tumor showed sheets and nests of hypercellular pleomorphic cells with thick fibrous septation, frequent mitoses, and areas of adrenal cortical-like tissue. Immunohistochemical staining revealed that the tumor cells were positive for inhibin-α, vimentin, synaptophysin, and melan A. It also revealed that the tumor cells were negative for pan-cytokeratin, epithelial membrane antigen, paired box 8, α-methylacyl-coenzyme A racemase, CD10, cytokeratin 7, carbonic anhydrase 9, c-Kit, renal cell carcinoma, transcription factor E3, human melanoma black 45, desmin, smooth muscle actin, S-100, chromogranin A, CD34, anaplastic lymphoma kinase, and integrase interactor 1. Based on these histopathological and immunohistochemical findings, we diagnosed the tumor as intrarenal adrenocortical carcinoma arising in an adrenal rest. Several cases of intrarenal adrenocortical carcinoma have been reported, although they are very rare. Due to its poor prognosis and common recurrence or metastasis, clinicians and pathologists must be aware of this entity.

Ectopic adrenal tissue in the kidney: A systematic review

“…In patients with localization at the kidney, the lesion most commonly occurred in the upper pole, while only 3 patients, including our case, had a lesion located in the middle third of the kidney (11,31).…”

“…Seven cases showed endocrinological disorders such as Cushing syndrome, primary hyperaldosteronism or were present in the context of congenital anomalies such as Beckwith-Wiedemann syndrome (9,16,20,23,24,26,30,33,35). Abdominal pain may be due to an ureteropelvic obstruction due to the mass effect of the neoplasm, as in the case presented by Goren et al (14) and Lee et al (31).…”

“…More frequently these adrenal changes have a benign behavior and can be classified as functioning or nonfunctioning adenomas. However, in some cases, they may experience neoplastic degeneration as published by Yokoyama et al and Lee et al (25,31). Godin et al in 2014 published the first case of adrenocortical heterotopic oncocytoma of the kidney.…”

Extra-adrenal, non-functional adrenocortical carcinoma presenting with acute abdomen: a case report

“…[ 7 ] 34 F 6.5 cm Retroperitoneum Retroperitoneal mass between right kidney and IVC resection Resection NED at 120 m Lee et al . [ 8 ] 61 M 13 cm Right intrarenal Adjuvant chemotherapy (VAP; vincristine, doxorubicin, and prednisolone) with mitotane NED at 3 m F female, IVC inferior vena cava, LR local recurrence, M male, m month, NED no evidence of disease, RT radiotherapy, s/p status post …”

“…Extra-adrenal non-functional ACC is an extremely rare tumor with only six cases having been reported to the best of our knowledge [ 3 – 8 ], and here we present the seventh case. We try to explain the different aspects of this kind of tumor, from pathology and diagnosis, to management and prognosis.…”