Case Reports
SPONTANEOUS REGRESSION OF PULMONARY METASTASIS FROM NONFUNCTIONING ADRENOCORTICAL CARCINOMA AFTER REMOVAL OF THE PRIMARY LESION: A CASE REPORT
KAZUO GOHJI,* TOMOHIKO KIZAKI AND AKIO FUJII
From the Departments of Urology and Pathology, Hyogo Medical Center for Adults, Akashi, Hyogo, Japan
KEY WORDS: carcinoma; adrenal cortex; lung neoplasms; neoplasm metastasis; neoplasm regression, spontaneous
We report a rare case of the spontaneous regression of pulmonary metastasis from adrenocortical carcinoma after resection of the primary tumor.
CASE REPORT
A 46-year-old man presented for general fatigue. Abdomi- nal computerized tomography (CT) obtained by his physician showed a tumor in the left adrenal gland but no other lesions. Physical examination demonstrated no abnormality. Labora- tory evaluations revealed the elevation of serum carcinoem- bryonic antigen (80 ng./ml., normal less than 5) and slight anemia but no pulmonary infection. CT of the chest showed an irregular 8.9 × 6.9 cm. mass in the upper lobe of the left lung (fig. 1, A). Levels of urinary 17-ketosteroids, 17-hydroxy- cortico-sterone, vanillylmandelic acid, serum cortisol, and serum and urinary catecholamine and aldosterone were within normal limits.
Transtracheal biopsy of the lung tumor revealed that the tumor had metastasized from adrenocortical carcinoma with- out inflammation. Endoscopy of the gastrointestinal tract, and bone and gallium scintigraphy did not show any abnor- mal findings suggesting metastatic tumor except in the lung and left adrenal. Recently complete resection of metastatic and recurrent tumor combined with adjuvant mitotane (0, p’-DDD) therapy has been recommended,1 and the meta- static lesion in our case seemed to be resectable. Therefore, surgical resection of the primary and metastatic tumors was performed (fig. 2, A).
Initially the patient underwent en bloc resection of the tumor, which was well encapsulated. The left kidney, to which the tumor was adhering, was also resected but the tumor had not invaded beyond the capsule. Microscopic ex- amination of the resected tumor revealed solid clusters of viable tumor cells with pleomorphic nuclei. The diagnosis was clinically and biochemically nonfunctional adrenocorti- cal carcinoma (fig. 2, B).
CT of the chest demonstrated partial regression of the lung tumor (fig. 1, B). The tumor gradually decreased to 10% of its size at initial presentation 2 months after re- moval of the left adrenal tumor and left kidney. Segmental resection of the upper lobe of the left lung was then per- formed. No swollen regional lymph nodes were noted at either surgery. Major parts of the lung tumor showed necrosis on microscopy. Some areas consisted of large ple-
omorphic cells with abundant eosinophilic granular cyto- plasm and considerable nuclear pleomorphism (fig. 2, C). Immunohistochemical study showed that the lung and ad- renal tumors negatively stained with antibody against ep- ithelial basement membrane antigen.
After removal of the adrenal and lung tumors serum car- cinoembryonic antigen became normal. The patient refused adjuvant mitotane therapy. He remains with no evidence of disease and normal serum carcinoembryonic antigen levels 38 months after the initial surgery.
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DISCUSSION
Adrenocortical carcinoma is rare, and high grade or ad- vanced tumor is associated with a poor prognosis. All re- ported patients with distant metastases died of disease within 2 years after diagnosis.2 The most effective treatment for early stage disease is surgical resection.2 Usually patients with distant metastases are treated with mitotane, which has also been used for inoperable disease. However, the ob- jective response rate by World Health Organization criteria was 23% with a relatively lower rate in patients with a large tumor burden.3 Recently others have reported that the sur- vival rate of patients with metastatic or recurrent tumor who underwent total tumor resection combined with adjuvant mitotane therapy was better than that of those who under- went subtotal tumor resection or no surgery.1 Therefore, complete tumor resection appears to be the best treatment method even in advanced disease.1
Although it is possible that the lung tumor was primary and the adrenal tumor was the metastasis in our case, im- munohistochemical study indicated that the adrenal tumor was primary and the lung tumor was the metastasis. It is also possible that the lung lesion was obstructing a bronchus with secondary infection but no laboratory data or clinical features indicated pulmonary infection, and tumor biopsy did not show inflammation. Our patient was not given any med- ication, such as steroids or mitotane. These findings indicate that regression of the lung tumor, which was not induced by secondary infection, occurred spontaneously. To our knowl- edge we report the first case of spontaneous regression of pulmonary metastasis after removal of a nonfunctional ad- renocortical carcinoma.
CONCLUSIONS
Our case suggests that the treatment of adrenocortical cancer should recognize the rare possibility of spontaneous regression of the metastatic lesion after removal of the pri- mary tumor. Spontaneous regression allowed complete resec- tion of the metastatic lesion.
REFERENCES
1. Haak, H. R., Hermans, J., van de Velde, C. J., Lentjes, E. G., Goslings, B. M., Fleuren, G. J. and Krans, H. M .: Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Brit. J. Cancer, 69: 947, 1994.
2. Sullivan, M., Boileau, M. and Hodges, C. V .: Adrenal cortical carcinoma. J. Urol., 120: 660, 1978.
3. Van Slooten, H., Moolenaar, A. J., van Seters, A. P. and Smeenk, D .: The treatment of adrenocortical carcinoma with o, p’-DDD: prognostic implications of serum level monitoring. Eur. J. Can- cer Clin. Oncol., 20: 47, 1984.