ELSEVIER
ONCOCYTIC ADRENOCORTICAL CARCINOMA
KAZUSHI TANAKA, YOSHIFUMI KUMANO, NAOKI KANOMATA, MASASHI TAKEDA, ISAO HARA, MASATO FUJISAWA, GAKU KAWABATA, AND SADAO KAMIDONO
ABSTRACT
Oncocytic adrenocortical carcinoma is rare. To our knowledge, only 6 cases have been previously reported. We describe an additional case of oncocytic adrenocortical carcinoma. A 54-year-old man presented with a right subcostal mass. Computed tomography demonstrated a massive tumor in the right abdomen. Because renal or adrenal cancer was suspected, right adrenalectomy and nephrectomy were performed. Histologic examination revealed an oncocytic adrenocortical carcinoma. Five months postoperatively, multiple metas- tases had developed and were treated with surgical resection, chemotherapy, vascular embolization, and radiotherapy. At last follow-up, the patient was alive with pulmonary and adrenal metastases and undergoing treatment with mitotane. UROLOGY 64: 376.e5-376.e6, 2004. @ 2004 Elsevier Inc.
O ☐ ncocytic tumors are known to occur in the salivary duct, kidney, and other sites.1 Adre- nal oncocytic tumors are rare.2-5 In general, onco- cytic neoplasms may be divided into oncocytoma, oncocytic neoplasm of uncertain malignancy, and oncocytic carcinoma. To date, only 6 cases of on- cocytic adrenocortical carcinoma have been re- ported.2-5 We describe another such case, with multiple metastases, but also relatively responsive to treatment.
CASE REPORT
A 54-year-old man presented with a right sub- costal mass. Computed tomography (CT) demon- strated a massive tumor in the right abdomen. The blood and urine evaluations were unremarkable. Hormonal assays in the urine showed excessive 17-ketosteroids, 17-hydroxycorticosteroids, and cortisol, but no physical signs of endocrinopathy were appreciated. CT showed a massive tumor in the right abdomen (Fig. 1). On magnetic resonance imaging, the mass showed heterogeneous signal intensity and was located near the superior pole of
-APR-1948
4-MAY-2002
F-SP
3: 22: 56.71
139.5
MA 50
PI 3
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V 120
A 260
I 0.75
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the right kidney. Renal or adrenal cancer was sus- pected, and right adrenalectomy and nephrectomy were performed.
Histologic examination revealed an oncocytic carcinoma (Fig. 2). The neoplastic oncocytes were mainly arranged in a diffuse pattern. Large irregu- lar zones of necrosis were noted. The neoplastic cells were large and round to polygonal and had abundant granular and eosinophilic cytoplasm. Their nuclei were round and had prominent nucle- oli. Vascular invasion was evident, and mitotic ac- tivity was increased at 14 mitoses per 50 high- power fields. Immunohistochemically, the tumor
From the Division of Urology, Department of Organs Therapeu- tics, Kobe University Graduate School of Medicine; Department of Pathology, Kobe University Hospital, Kobe; and Department of Urology, Kawasaki Medical School, Kurashiki, Japan
Address for correspondence: Kazushi Tanaka, M.D., Division of Urology, Department of Organs Therapeutics, Kobe University Graduate School of Medicine, Kusunokicho 7-5-1, Chuo-ku, Kobe 650-0017, Japan
20 um
cells stained for synaptophysin, vimentin, alpha- inhibin, and Melan A. Cytokeratin was expressed focally.
Five months postoperatively, the patient devel- oped left chest pain. CT demonstrated a mass in the left sixth rib and also lung metastases. Aspira- tion biopsy of the bone lesion was performed. Pathologic examination of the specimen showed metastatic oncocytic adrenocortical carcinoma. Abdominal CT disclosed a liver metastasis and left adrenal metastasis. The patient was treated with mitotane, systemic chemotherapy (etoposide, doxorubicin, and cisplatin), and radiotherapy di- rected to the left sixth rib metastasis, which showed a partial response. The liver metastasis was embolized, resulting in a nearly complete re- sponse. The left rib and left lung metastases were resected. At last follow-up, 18 months after sur- gery, the patient was undergoing treatment with mitotane (4.5 g/day) and was alive with lung and left adrenal metastases, which were decreasing in size.
COMMENT
Six cases of oncocytic adrenocortical carcinoma have been reported to date, all nonfunctional in
terms of endocrine symptoms. By definition, onco- cytic neoplasms consist of oncocytes, whose light microscopic hallmark is abundant granular eosin- ophilic cytoplasm. Our case had these charac- teristics and features indicating malignancy. We, therefore, diagnosed oncocytic adrenocortical car- cinoma. Histologic malignancy in oncocytic adre- nocortical carcinoma has been a matter of contro- versy. Our case satisfied six of the criteria of Weiss,6,7 and the resected specimens of the left rib and lung also showed oncocytic adrenocortical carcinoma.
Among the previously reported cases, 2 of the 6 patients had distant metastasis, which our patient also developed. Radiotherapy, chemotherapy with cytotoxic anticancer agents and mitotane, and em- bolization were given. Mitotane appeared to be ef- fective in our patient, but the persistent small lung and left adrenal metastases mandated close follow- up. The treatment of oncocytic adrenocortical car- cinoma is complete surgical resection. In our case, chemotherapy, radiotherapy, and transcatheter embolization were effective adjuncts.
REFERENCES
1. Chang A, and Harawi SJ: Oncocytes, oncocytosis and oncocytic tumors. Pathol Ann 27: 263-304, 1992.
2. Kurek R, Knobloch RV, Feek U, et al: Local recurrence of oncocytic adrenocortical carcinoma with ovary metastasis. J Urol 166: 985-985, 2001.
3. El-Naggar AK, Evans DB, and Mackay B: Oncocytic ad- renocortical carcinoma. Ultrastruct Pathol 15: 549-556, 1991.
4. Krishnamurthy S, Ordonez NG, Shelton TO, et al: Fine-needle aspiration cytology of a case of oncocytic adrenocortical carcinoma. Diagn Cytopathol 22: 299-303, 2000.
5. Hoang MP, Ayala AG, and Saavedra JA: Oncocytic adre- nocortical carcinoma: a morphologic, immunohistochemical and ultrastructural study of four cases. Mod Pathol 15: 973- 978, 2002.
6. Weiss LM: Comparative histologic study of 43 metasta- sizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 8: 163-169, 1984.
7. Weiss LM, Mederios LJ, and Vickery AL: Pathologic fea- tures of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 13: 202-206, 1989.