METASTATIC ADRENOCORTICAL CARCINOMA TREATED BY REPEATED RESECTION: A CASE REPORT OF LONG-TERM SURVIVAL OVER 18 YEARS
Kimitaka Sakamoto,1* Asami Ariyoshi,’ and Masatoshi Okazaki2
Departments of ‘Urology and 2Radiology, School of Medicine, Fukuoka University, Fukuoka, Japan
A case of recurrent and metastatic nonfunctioning adrenocortical carcinoma in a 40-year-old woman is reported. The patient received 4 laparotomies and 1 thoracotomy for recurrent and metastatic disease after removal of the primary adrenal tumor. She has been alive for over 18 years following multiple surgery for diseases after the first adrenalectomy. In selected patients with recurrence and/ or metastasis, repeated surgical resection offers the possibility of a cure or extended palliation. Int J Urol 1995;2:50-52
Key words: adrenocortical carcinoma, metastasis, surgical treatment
INTRODUCTION
Adrenocortical carcinomas, especially those that are nonfunctional and presenting as large advanced masses, are associated with a short life expectancy. The role of surgery in the management of patients with this tumor has been limited to resection of the primary lesion. While remission of the disease can occur following surgery, recurrence and eventual death from metastatic disease is the rule. However, long-term survival has been reported in selected patients with repeated resection of recurrence or metastasis following initial adrenalectomy. In this paper we emphasize that an aggressive surgical approach to recurrent or metastatic disease should be adopted for prolonged survival.
CASE REPORT
A 40-year-old Japanese female was referred to the Fukuoka University Hospital for a large mass in the left flank in December 1976. Extensive workup re- vealed a huge tumor probably arising from the left adrenal gland. However, there were neither stigmata nor hormonal abnormalities associated with adrenal hyperfunction. In December 1976, she underwent left adrenalectomy involving a thoracoabdominal ap- proach. The gross tumor appeared to have been com- pletely removed but lymph nodes were not excised during this operation. The tumor was 900 g in weight and the cut surface was yellow-whitish in color and lobulated in appearance (Fig. 1: A). The tumor cells consisted mainly of clear cells and with some granular cells arranged in a cord-like or nodular pattern
Received Aug. 4, 1994; accepted for publication in revised form Dec. 12, 1994. * Requests for reprints: Department of Urology, School of Medicine, Fukuoka University, Nanakuma, Jonan-ku, Fukuoka 814-80, Japan.
A
N. T 40 .F 58275
B
(Fig. 1: B). Because of local invasion without distant metastasis, the tumor was pathologically classified as stage III adrenocortical carcinoma. Postopera- tively the patient received 60 Gy of telecobalt radiation therapy to the adrenal fossa as adjuvant therapy.
After a 47 month period free from any untoward events, she visited us for a follow-up examination.
A
B
C
D
E
Abdominal computed tomography (CT) showed a mass in the left adrenal fossa, highly suggestive of recurrent disease. She underwent reoperation in November 1980. A tumor, weighing 50 g, was suc- cessfully resected (Fig. 2: B).
In June 1983, a routine CT scan again revealed the presence of 2 isolated masses: one at the upper pole of the left kidney and the other behind the pancreas. Removal of the tumors combined with left nephrectomy was performed at that time (Fig. 2: C).
Seventy months later, the development of new he- patic nodules and a left chest wall tumor was noticed on abdominal CT scan, hepatic arteriography and chest X-ray tomography performed in April 1989. She underwent a laparothoracotomy for both enucleation of 4 hepatic nodules, 4 to 20 mm in diameter, and resection of the chest wall tumor along with the invaded pulmonary tissue in June 1989 (Fig. 2: D).
The patient began to have abdominal pain associ- ated with bloody stools in April 1992. Examination of the gastrointestinal tract revealed a tumor within the transverse colon. Partial resection of the transverse colon was performed in June 1992 (Fig. 2:E). The histological findings of all recurrent and metastatic tumors were essentially identical to those of the primary adrenocortical carcinoma.
Irrespective of the above-mentioned multiple sur- gery she was very well and led a fully active life in August 1994.
DISCUSSION
Complete resection of the primary lesion is the only effective and potentially curative treatment for adrenocortical carcinoma. However, even in patients who undergo radical adrenalectomy, recurrent and/ or metastatic disease is common.’ The treatment of patients presenting with recurrent or metastatic dis- ease after removal of the primary adrenocortical carci- noma has been predominantly chemotherapy, with agents such as o,p’-DDD and cisplatin, and occasion- ally radiotherapy. In previous reports, however, chemotherapy and radiotherapy for recurrent and metastatic tumors have had a limited effect on sur- vival.2 Reports from some institutions have suggested that reoperation is the only effective treatment for recurrence and metastasis.1,3 Pommier and Brennan1 stated that the mean survival time of 56 months for 45 patients treated with reoperation for disease was clearly superior to that of 19 months for 19 patients treated medically. In a review of the literature, there have been two isolated cases reported of survival over 10 years after initial adrenalectomy in patients with repeated resections for disease.4-5 Our case was also treated successfully by repeated resections with excel- lent long-term survival.
We emphasize that complete surgical resection for recurrent and metastatic disease remains the only mode of therapy offering the possibility of prolonged survival.
ACKNOWLEDGMENTS
Grateful acknowledgment is made to Drs. H. Igimi, T. Shirakusa and R. Kumashiro for their able collabora- tion, and also to Drs. H. Hashimoto, F. Tanaka, K. Minoda, S. Kanegae and K. Yoshida for their generous assistance.
REFERENCES
1. Pommier RF, Brennan MF. An eleven-year experience with adrenocortical carcinoma. Surgery 1992;112:963- 971
2. Bodie B, Novick AC, Pontes JE, Straffon RA, Montie JE, Babiak T, Sheeler L, Schumacher P. The Cleveland Clinic experience with adrenal cortical carcinoma. J Urol 1989;141:257-260
3. Icard P, Louvel A, Chapuis Y. Survival rates and prognos- tic factors in adrenocortical carcinoma. World J Surg 1992;16:753-758
4. Appelqvist P, Koikkalainen K, Tala P, Kuisman A. Surgi- cal treatment of pulmonary metastases. Int Surg 1979; 64:13-16
5. Potter DA, Strott CA, Javadpour N, Roth JA. Prolonged survival following six pulmonary resections for meta- static adrenal cortical carcinoma: A case report. J Surg Oncol 1984;25:273-277