Survival After Pulmonary Lobectomy for Metastatic Adrenocortical Carcinoma
Lawrence W. O’Neal, M.D., and Thomas H. Burford, M.D.
L” ong-term survival after removal of adrenal cortical carcinoma is rare. Death usually occurs within two or three years after diag- nosis [2, 3]. The survivors are chiefly patients who had removal of localized encapsulated tumors which were given a histological diag- nosis of carcinoma. Surgical treatment of recurrent and metastatic adrenal cortical cancers has not often been possible. Some good results have been obtained following resection of pulmonary metastases from renal carcinoma, colon cancer, and uterine cancer [4]. Experience with the case reported below suggests that occasionally aggressive surgical treatment in selected cases of pulmonary metastases from adrenal corti- cal cancer may be rewarded with success.
F. D.’s symptoms began in 1950, when she was 25 years old, with amenorrhea. Thereafter she noted increase in facial hair. In 1952 she was admitted on the service of Dr. Alvin E. Vitt at St. Mary’s Hospital, St. Louis, Mo. Physical exam- ination showed ruddy face, acne, increase in facial hair, and enlarged clitoris. Blood pressure was 164/98 mm. Hg. Intravenous pyelography showed a large tumor above the right kidney. Urinary 17-ketosteroid excretion was 356 mg. per 24 hours. The 10 cm. x 14 cm. tumor was removed via a flank incision. Patho- logical examination showed tumor composed of polygonal cells with abundant eosinophilic cytoplasm and moderate pleomorphism. Vascular invasion was not seen, but the capsule appeared to be invaded in several areas. A diagnosis of adrenal tumor, possibly malignant was made.
Menses were reestablished two months postoperatively, and four months postoperatively she became pregnant. Spontaneous abortion occurred at four months. The hirsutism became less marked and normal menses resumed. In 1956 she became depressed and electro-convulsive therapy was given without benefit. In the spring of 1957 menses ceased and increased hair growth began again. She was soon shaving daily. She was admitted to Barnes Hospital on November 12, 1957.
Examination at that time showed ruddy face, increased hair on chin, chest and abdomen. The clitoris was enlarged. Hemoglobin was 14.7 gm. per 100 ml .; fasting sugar, glucose tolerance, and plasma electrolytes were normal. Urinary 17-ketosteroid excretion was 198.3 mg. per 24 hours, and urinary 17-hydroxy- corticosteroid was 11.9 mg. per 24 hours. Intravenous pyelography showed only postoperative deformity of the right kidney. Chest films showed two metastatic lesions in the lower lobe of the left lung (Fig. 1).
From the Departments of Surgery and Thoracic Surgery, Washington University School of Medicine and Barnes Hospital, St. Louis, Mo.
Accepted for publication Feb. 23, 1966.
On November 19, 1957, left thoracotomy revealed two yellow tumor masses in the left lower lobe. One of these was 2 cm. in diameter and deep in the lung parenchyma. The lower one was 8 cm. in diameter and was attached to the cen- tral tendon of the diaphragm. Left lower lobectomy was done en bloc with an 8 cm. circular segment of the diaphragm. The diaphragmatic incision was en- larged, and exploration of the abdomen revealed no recurrence in the right suprarenal area or metastases in the liver or elsewhere. The left adrenal gland was exposed and appeared moderately atrophic. Her postoperative course was benign. One week following operation, urinary 17-ketosteroid excretion was 6 mg. per 24 hours.
Pathological examination showed two metastatic lesions in the resected lung tissue. The metastatic lesions were similar to the primary tumor except that there was more pleomorphism and more numerous mitoses. Direct invasion of pleura and diaphragm was noted (Figs. 2, 3).
In May, 1958, she reentered Barnes Hospital because of spontaneous left pneumothorax, which was successfully treated with catheter thoracostomy. A urinary 17-ketosteroid determination was 7.6 mg. per 24 hours. Hirsutism was less, and menses were regular.
In April, 1959, she was admitted with profuse vaginal bleeding and miscar- ried a five-month fetus. In April, 1960, urinary 17-ketosteroid excretion was 5.8 mg. per 24 hours, and chest films showed no metastases. Since then she has re- mained well and has had two successful pregnancies. A baby boy, born June 27, 1961, was and has remained normal in all respects. A baby girl, born January 14, 1965, required exchange transfusion (Rh incompatibility) but was and has re- mained endocrinologically normal.
The patient was seen again on November 5, 1965. She felt and appeared entirely well. Menses were regular. Examination showed only minimal facial hirsutism. The excess hair of chest and abdomen had disappeared. The clitoris was normal. Chest films showed only postoperative changes in the left chest. Urinary 17-ketosteroid excretion was 13.6 mg. per 24 hours.
Ordinarily in cases with recurrent or metastatic functioning adre- nal cortical carcinoma, only palliation of the endocrine effects with o-p’-DDD can be achieved [1]. Roentgen therapy to the tumor is usually ineffective. Hypophysectomy or pituitary roentgen therapy is unavail- ing. An attempt to palliate the virilism in this patient by removal of what we believed at first to be the greater part of her persisting func- tioning tumor resulted, unexpectedly, in long survival without recur- rence.
The presence of pulmonary metastases is not a contraindication to pulmonary resection. A relatively long interval between removal of the primary tumor and evidence of pulmonary metastases (in this case five
O’NEAL AND BURFORD
years) is correlated with better results [4]. Single metastases are more favorable than multiple. Of course, control of the primary tumor should be achieved before pulmonary resection.
SUMMARY
Pulmonary lobectomy done in a patient with metastatic virilizing adrenal cortical carcinoma was successful in abolishing virilism, re- establishing fertility, and restoring good health for eight years.
REFERENCES
1. Danowski, T. S., Sarver, M. E., Moses, C., and Bonessi, V. V. O-p’ DDD therapy in Cushing’s syndrome and in obesity with cushingoid changes. Amer. J. Med. 37:235, 1964.
2. Heinbecker, P., O’Neal, L. W., and Ackerman, L. V. Functioning and non- functioning adrenal cortical tumors. Surg. Gynec. Obstet. 105:21, 1957.
3. Rapaport, E., Goldberg, M. B., Gorman, G. S., and Hinman, F., Jr. Mortal- ity in surgically treated adrenocortical tumors: II. Review of cases reported for the 20-year period 1930-1949, inclusive. Postgrad. Med. 11:325, 1952.
4. Wilkins, E. W., Jr., Burke, J. F., and Head, J. M. The surgical management of metastatic neoplasms in the lung. J. Thorac. Cardiov. Surg. 42:298, 1961.