CASE REPORT
L. S. Kasat · S. S. Borwankar . A. Naregal M. Jain
Complete spontaneous regression of a functioning adrenocortical carcinoma in an infant
Accepted: 8 November 1999
Abstract A 9-month-old female presented with Cush- ing’s syndrome. Investigations suggested the diagnosis and revealed a functioning left adrenal carcinoma without metastasis. However, when the abdomen was explored 30 days later after controlling the hypertension only normal adrenals were encountered, suggesting complete spontaneous regression. This is the fourth case of its kind. The case along with a review of this rare phenomenon is presented.
Keywords Adrenocortical neoplasm · Regression . Spontaneous · Carcinoma
Introduction
Adrenocortical carcinoma (ACC) represents only 0.2% of all cancers. Although the peak incidence is in the 5th decade, paediatric patients with this cancer represent 6% of all paediatric adrenal tumours [4]. Presentation in the newborn or early infancy period of ACC with Cushing’s syndrome is very uncommon, and to complete sponta- neous regression is extremely rare.
Case report
A 9-month-old female had a history of rapidly-increasing weight and obesity since 4-5 months of age. The birth weight was 3.5 kg, which increased to 10 kg at 6 months and 13 kg at 9 months of age. She was the only child of parents married for 10 years. On exam- ination, she had cushingoid facies with generalised obesity but no striae, hyperpigmentation, or ecchymosis. She was hypertensive
L. S. Kasat ([)1 . S. S. Borwankar . A. Naregal . M. Jain Department of Pediatric Surgery, K.E.M. Hospital, Parel, Mumbai-400 012, India
Corresponding address: “c/o Dr. Girish M. Patwardhan, B-9, Swananda Housing Co. Op. Society, 4th Floor, Gokhale Road, Naupada, Thana, Mumbai-400 602, India
(blood pressure 130/96 mmHg). Abdominal examination revealed no palpable mass.
Basal serum cortisol levels were done twice and were elevated (42 µg/dl at 8 a.m., 52 µg/dl at 10 p.m., normal 8.25 µg/dl) with loss of normal diurnal variation. Fasting adrenocorticotropic hor- mone (ACTH) values were low at 36 pg/ml (normal <60 pg/ml). The 24 h urine free cortisol was 156 µg/day (normally >100 µg/ day in Cushing’s syndrome). The urinary level of 17-hydroxypro- gesterone was also high: 400 ng/dl (normal ≤70 ng/dl). Both low- and high-dose dexamethasone suppression tests failed to suppress cortisol levels. The serum electrolytes, proteins, liver and renal function tests, blood sugar, and blood gases were normal. Com- puterised tomography (CT) of the abdomen showed a left adrenal mass (Fig. 1) without any calcifications. The other adrenal, both kidneys, and the liver were normal. CT-guided aspiration cytology of the tumour suggested a carcinoma. DNA quantitation was not done due to its nonavailability. A chest radiograph was normal. CT of the brain revealed no metastasis. An I13 iodomethyl-1-19-nor- cholesterol (NP-59) scan was not done due to its nonavailability.
The child was operated upon via a generous transverse- abdominal incision under perioperative hydrocortisone cover after controlling the blood pressure for 30 days (she went home for 10 days in between for personal reasons against our advice). Surpris- ingly, the left adrenal was absolutely normal in size, shape, and texture. The left kidney, right adrenal, and liver were also normal. A total bilateral adrenalectomy was done in view of the Cushing’s syndrome. The patient developed a burst abdomen on the 15th postoperative day, which was successfully repaired. The blood pressure returned to normal in 2 months’ time and her weight de- creased by 1.9 kg. The pathologist reported normal adrenals. The child is well with replacement therapy.
Discussion
Cushing’s syndrome results mainly from adrenal cortical hyperplasia secondary to increased ACTH secretion in the pituitary or to an ectopic ACTH-secreting neoplasm. Adrenal tumours are uncommon causes of Cushing’s syndrome, and one-third of such tumours prove to be carcinomas [1]. However, in infants a functioning adrenocortical tumor as a cause of Cushing’s syndrome is relatively frequent.
Spontaneous regression of adrenocortical neoplasms in newborns and infants is extremely rare, with only three cases reported to date [2-4]. Of these there was spontaneous regression of a pulmonary metastasis in
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one case [3] and spontaneous regression of skin and cerebral metastases 2 months after removal of an ACC in a 1-day-old newborn in the second [4]. The latter child was noted to have a right hemihypertrophy at 2 months of age. Complete spontaneous regression of a tumour per se was reported only in one case of pigmented nodular adrenocortical disease [2]. Other disorders known to be associated with such cancers include
Beckwith-Wiedemann syndrome, multiple endocrine neoplasias type I syndrome, medulloblastoma, astrocy- toma, cutaneous abnormalities, urinary-tract anomalies, haemangiomas, and hemihypertrophy [1].
The cause of spontaneous regression is not known [4]. Stimulation of antibodies by an autoimmune mechanism is a possibility. This is similar to stimulation of the thyroid by thyroid-stimulating hormone immunoglobu- lin in Graves’ disease, where spontaneous remission is known. A similar mechanism may be involved here. The possibility of a special category of self-regressing pae- diatric ACC akin to the IV-S group of neuroblastoma is also under speculation [4]. We might have detected the true complete spontaneous regression in our case if we had at least done ultrasonography prior to the laparo- tomy. This may be a lesson to be learnt and followed strictly in the future.
References
1. Chandler RM, Kay R (1989) Adrenocortical carcinoma in children. Urol Clin North Am 16: 469-474
2. Garcia-Mayor RV, et al (1997) Complete remission of primary pigmented adrenocortical disease. J Clin Endocrinol Metab 82: 3517-3518
3. Gohjik, et al (1995) Spontaneous regression of pulmonary metastasis from non functioning adrenocortical, carcinoma after the removal of primary lesion: a case report. J Urol 154: 1854-1855
4. Saracco S, Abromowsky C, Taylor S, et al (1988) Spontaneously regressing adrenocortical carcinoma in a newborn. A case report with DNA ploidy analysis. Cancer 62: 507-511