Case Report
Non-functioning adrenal cortical carcinoma presenting with metastasis to the tongue
Alberto Dominici1 Michelangelo Rizzo1 Fabrizio Travaglini1 Gabriella Nesi2 Alessandro Franchi2
1Department of Urology, and 2Department of Human Pathology and Oncology, University of Florence Medical School, Florence, Italy
Correspondence to:
Dr Alessandro Franchi Department of Human Pathology and Oncology, Viale G.B. Morgagni 85, 50134 Firenze, Italy
Tel .: +39 554 478102
Fax: +39 554 379868
e-mail: franchi@unifi.it
Accepted for publication August 5, 2002
Copyright @ Blackwell Munksgaard 2003 J Oral Pathol Med . ISSN 0904-2512
Printed in Denmark . All rights reserved
Abstract
We report a case of non-functioning adrenal cortical carcinoma (ACC) presenting with metastatic disease to the tongue, which is an extremely uncommon onset for this neoplasm. Histologically, the lesion had the appearance of an anaplastic neoplasm, and a panel of immunohistochemical markers including vimentin, MART-1, S100 protein, HMB-45, smooth muscle actin, common muscle actin, desmin, CD31, CD34, CD68, EMA and cytokera- tins, was helpful in excluding melanoma, as well as other mesenchymal and epithelial neoplasms.
Key words: adrenal cortical carcinoma; metastasis; tongue J Oral Pathol Med 2003: 32: 185-7
A 63-year-old patient presented with a one-month history of hemoptysis, followed by increasing pain to the right hemithorax and lumbar area. Intraoral examination revealed a necrotic and hemorrhagic mass involving the base of the tongue, the glosso- epiglottic fold and the epiglottis. Thoracic roentgenogram and computerized tomography showed enlarged left mediastinal lymph nodes. Bronchoscopy and total body scan were negative. Biopsy of the tongue revealed a malignant tumor, consisting of large anaplastic cells with abundant pale eosinophilic cytoplasm and with focal aspects of vacuolization (Fig. 1). Immunohisto- chemically, neoplastic cells were positive for vimentin and MART- 1 (Fig. 2), while they were negative for cytokeratins (CAM 5.2, AE1/AE3, cytokeratin 7), EMA, CD31, CD34, CD68, S100 pro- tein, HMB-45, common muscle actin, smooth muscle actin and desmin. Computerized tomography and magnetic resonance imaging (MRI) revealed an 8.4 × 8.9 × 11 cm mass of the right adrenal gland (Fig. 3) which was negative at 131I-iodocholesterol (NP-59) scintigraphy. There were no clinical or biochemical endocrinological abnormalities. A radical right adrenalectomy was performed. The histopathologic and immunohistochemical profile of the adrenal mass was similar to that of the tongue lesion, and a diagnosis of adrenal cortical carcinoma (ACC) was rendered. The patient died after two months of disease progression.
(a)
(b)
Comments
About half the patients affected with ACC have distant metastases at diagnosis, most commonly in the lungs, liver, peritoneum, lymph nodes and bones, and this is associated with significantly shorter survival (1, 2). Histologically, ACC may appear as an anaplastic carcinoma, and may cause diagnostic difficulties pre- senting with distant metastasis. In intraoral location, the differ- ential diagnosis includes primary tumors (anaplastic carcinoma and sarcomas) and metastatic carcinoma from lungs, kidneys, liver and stomach, as well as primary and metastatic melanoma (3). Although a pathognomonic immunohistochemical profile has not been described for ACC, even negative results may aid in the differential diagnosis, since adrenal cortical carcinoma is typically negative for cytokeratins and occasionally positive for vimentin, while in most cases renal cell carcinomas and other anaplastic carcinomas are positive for cytokeratins and EMA. Recently, it has been reported that MART-1 immunostaining, a marker initi- ally identified in melanoma cells, is useful in distinguishing adrenal cortical tumors, which are always positive, from meta-
H
Q14764
11.1 cm
4
(a)
(b)
static carcinomas and pheochromocytomas (4, 5). Accordingly, MART-1 immunoreactivity was detected in the present case, and we suggest that this immunohistochemical marker should be added to the panel employed in the differential diagnosis of anaplastic tumors of the tongue, along with other markers of melanocytic differentiation (S100 protein, HMB-45) which are of help in excluding the diagnosis of melanoma.
References
1. Nakata M, Yamashita T, Watanabe Y, Ono H, Kirimoto K. Nonfunctioning adrenocortical carcinoma revealed by metastasis to the base of the tongue. Gan No Rinsho 1986; 32: 1855-9.
2. Venkatesh S, Hickey RC, Sellin RV, Fernandez JF, Samaan NA. Adrenal cortical carcinoma. Cancer 1989; 64: 765-9.
3. Lucas RB. Pathology of Tumours of the Oral Tissues, 3rd edn. London: Churchill Livingstone, 1976.
4. Busam KJ, Iversen K, Coplan KA et al. Immunoreactivity for A103, an antibody to melan-A (Mart-1), in adrenocortical and other steroid tumors. Am J Surg Pathol 1998; 22: 57-63.
5. Loy TS, Phillips RW, Linder CL. A103 immunostaining in the diagnosis of adrenal cortical tumors. An immunohistochemical study of 316 cases. Arch Pathol Lab Med 2002; 126: 170-2.