J Pathol Transl Med.  2018 Nov;52(6):416-419. 10.4132/jptm.2018.07.20.

An Intrarenal Adrenocortical Carcinoma Arising in an Adrenal Rest

• Lee JH
• Choi YD
• Cho NH

• KMID: 2427525
• DOI: http://doi.org/10.4132/jptm.2018.07.20

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.

Keyword

Adrenocortical carcinoma; Adrenal rest tumor; Carcinoma; Renal cell

MeSH Terms

Actins
Adrenal Rest Tumor
Adrenocortical Carcinoma*
Back Pain
Carbonic Anhydrases
Carcinoma, Renal Cell
Chromogranin A
Desmin
Humans
Integrases
Keratin-7
Lymphoma
MART-1 Antigen
Melanoma
Middle Aged
Mitosis
Mucin-1
Muscle, Smooth
Neoplasm Metastasis
Nephrectomy
Phosphotransferases
Prognosis
Recurrence
Synaptophysin
Transcription Factors
Vimentin
Actins
Carbonic Anhydrases
Chromogranin A
Desmin
Integrases
Keratin-7
MART-1 Antigen
Mucin-1
Phosphotransferases
Synaptophysin
Transcription Factors
Vimentin

Figure

• 

  Fig. 1. Imaging and gross findings of adrenocortical carcinoma. (A) Abdominopelvic computed tomography reveals a 13 cm heterogeneous mass in the right kidney. (B) Bisected kidney specimen showing a lobulated hard mass with necrosis and hemorrhage.

• 

  Fig. 2. Histologic findings of adrenocortical carcinoma. (A) Sheets and nests of hypercellular tumor cells with thick fibrous septation. (B) Pleomorphic cells with frequent mitoses. (C) Areas of adrenal cortical-like tissue.

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  Fig. 3. Immunohistochemical staining results of adrenocortical carcinoma. The tumor cells were positive for inhibin-α (A), vimentin (B), synaptophysin (C), and melan A (D) and negative for epithelial membrane antigen (E), paired box 8 (F), and pan-cytokeratin (G).

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