Ectopic Calcitonin in Adrenocortical Carcinoma: A New Tumor Marker
By Walter Pegoli, Jr, Anne Kolbe, Bonnie L. Beaver, Stuart A. Chalew, and J. Laurance Hill Baltimore, Maryland
· Ectopic calcitonin has never before been described in association with pediatric malignancy. This is the first report of ectopic calcitonin in a child with adrenocortical carcinoma. It may prove to be a useful tumor marker in this, as well as other childhood malignancies. ®1987 by Grune & Stratton, Inc.
INDEX WORDS: Ectopic calcitonin; adrenocortical carci- noma.
P RODUCTION of ectopic hormones by neoplastic tissues continues to arouse clinical and experi- mental interests.’ Ectopic calcitonin is one such hor- mone, being produced by primary malignancies other than medullary carcinoma of the thyroid. Since its discovery, ectopic calcitonin has been used as a tumor marker in adults2; however, its recognition in pediatric malignancy has not been documented. This is the first report of ectopic calcitonin in a child with adrenocorti- cal carcinoma.
CASE REPORT
A 17-month-old white male presented with a 24-hour history of right-sided abdominal pain and vomiting. His height (89 cm) and weight (14.5 kg) were above the 95th percentile for age. On physical
examination, a right upper quadrant abdominal mass, phallomegaly, and Tanner II pubic hair were noted. Radiographic evaluation showed a calcified right suprarenal mass, without evidence of metastasis (Fig 1). Laparotomy revealed a 9 x 15 x 20 cm hemorrhagic suprarenal mass that had ruptured into Gerota’s fascia, but without intraabdominal spread. Histologically, the tumor was of adrenocortical origin (Fig 2). Calcitonin immunoperoxidase stain was positive (Fig 3). Preoperative serum calcitonin elevation (247 pg/mL, N < 100 pg/mL) normalized “postsurgery” (60 pg/mL).
From the Department of Surgery, University of Maryland, Baltimore.
Presented at the 18th Annual Meeting of the American Pediatric Surgical Association, Hilton Head Island, South Carolina, May 6-9, 1987.
Address reprint requests to J. Laurance Hill, MD, University of Maryland Hospital, 22 S Greene St, Baltimore, MD 21201.
@ 1987 by Grune & Stratton, Inc. 0022-3468/87/2212-0023$03.00/0
| Preoperative | Postsurgery | 1 Year Postoperative | Normal | |
|---|---|---|---|---|
| DHEA (ng/dL) | 314.2 | 26 | 0 | 33 |
| DHEA-S (ng/dL) | 1066.9 | 75.9 | 9.7 | 10-60 |
| Androstenedione (ng/dl) | 337.1 | <20 | 23.5 | 55 |
| Testosterone | 45.6 | <13 | 0 | <50 |
| Calcitonin (pg/mL) | 247 | 60 | 9 | <100 |
The androgenic steroids behaved similarly (Table 1). Provocative pentagastrin stimulation was normal. One year postoperatively, the child has been asymptomatic with normal tumor markers.
DISCUSSION
Demonstration of ectopic hormone production requires fulfillment of certain diagnostic criteria. The
diagnosis rests on demonstration of the hormone in tumor tissue, together with elevated preoperative and normal postoperative serum levels.
Until 1971, hypercalcitoninemia was considered pathognomonic for medullary carcinoma of the thy- roid. At that time, Milhaud first described elevated calcitonin levels in a patient with pheochromocytoma.3 Several years later, it was discovered that distinction of hypercalcitoninemic states was possible using the pen- tagastrin stimulation test. Serum calcitonin rises sharply after intravenous infusion of pentagastrin in medullary carcinoma, while ectopically secreted calci- tonin is unsensitive.2
Ectopic calcitonin has been demonstrated in 40% of primary adult malignancies (ie, breast, kidney, and lung cancer).4 As a tumor marker, it can serve as an index of tumor volume and gauge the efficiency of antitumor therapy.
The ectopic production of calcitonin by childhood adrenocortical carcinoma has never been described. It may prove to be a useful tumor marker in adrenocorti- cal carcinoma. Its place in the management of other pediatric malignancies remains to be investigated.
ACKNOWLEDGMENT
The authors express their appreciation for the tireless assistance and editoral expertise of Carol L. Lilly.
REFERENCES
1. Mulder H, Hackeng WHL: Ectopic secretion of calcitonin. Acta Med Scan 204:253-256, 1978
2. Hennesy JF, Wells SA, Outjes DA, et al: A comparison of pentagastric injection and calcium infusion as provocative agents for the detection of medullary carcinoma of the thyroid. J Clin Endocri- nol Metab 39:487-495, 1974
3. Milhaud G, Calmettes C, Courtis G, et al: Thyrocalcitonin in a new chapter in human pathology. Isr J Med Sci 7:358-360, 1971
4. Mulder H: Ectopic secretion of calcitonin as a tumor marker. Anticancer Res 3:247-250, 1983