Clinical Brief
Selective Testosterone Secreting Adrenocortical Carcinoma in an Infant
S.B. Bavdekar, R.R. Kasla1, Ramesh C. Parmar and G.S. Hathi1
Department of Pediatrics, Seth G.S. Medical College; K.E.M. Hospital and 1Dr. R.N. Cooper Hospital, Mumbai
Abstract. Adrenocortical carcinoma in children is a rare tumor of adrenal gland. An infant presented with signs of virilization due to selective testosterone hypersecretion. Diagnosis was established with the help of the computerized tomographic scan and histopathological examination. Following adrenalectomy patient made uneventful recovery and six months later does not have any clinical or laboratory evidence of recurrence or metastasis. [Indian J Pediatr 2001; 68 (1) : 95-97]
Key words : Adrenal cortex; Carcinoma; Testosterone
Adrenocortical carcinoma (ACC) is a rare childhood malignancy accounting for only 0.3% of all childhood cancers.1 It carries a high fatality rate. Endocrinological manifestations are the usual presenting features. Due to its rarity, the diagnostic probability of ACC is not considered even when an infant presents with acute virilization. A case of ACC is reported to highlight the clinical manifestations and a brief review of diagnostic and therapeutic modalities is provided.
Selective secretion of testosterone by an adrenal adenoma is well - known.2,3 However, adrenocortical carcinoma secreting testosterone alone is comparatively rare and has been reported mainly in the adults. The youngest pediatric patient reported has been over 12 years of age.4 This article reports a case of an infant with adrenocortical carcinoma secreting testosterone alone.
CASE REPORT
A 7,850 gm weighing 11-month-old girl presented with complaints of abnormal growth of hair over labia majora from the age of 6 months. The hair growth was prominent enough for the parents to shave the area regularly. The baby nor her mother was exposed to any androgenic drugs. On examination, the baby had normal anthropometry and was normotensive. There was a growth of thick, stage 2 pubic hair with the length of around 1.5 cm. There was no clitoral hypertrophy or posterior fusion of labial folds. There was no evidence of
axillary hair, breast enlargement or features of Cushing’s syndrome. No abdominal lump could be palpated. The biochemical profile of the patient is summarized in the Table . Ultrasonography revealed a well defined solid mass in the right suprarenal region with the evidence of calcific foci. The uterus, ovaries and other abdominal viscera were normal. Abdominal tomograph confirmed the presence of a rounded soft tissue tumor measuring 4.4 × 4.6 cm. in the right suprarenal region. The tumor indented the superior portion of the right kidney and was contiguous with the under surface of the liver but was not infiltrating it. It showed non-homogeneous enhancement after intravenous contrast.
Right adrenalectomy was performed. The tumor weighing 50 gm was largely encapsulated and showed a pale creamy cut surface. Microscopically it consisted of predominantly large cells with abundant acidophilic cytoplasm. These cells had hyperchromatic, pleomorphic and bizarre nuclei. Smaller cells with clear cytoplasm were also seen. Cell mitoses were more than 5 per high power field (Fig 1). Capsular infiltration, tumour embolus in some blood vessels and occasional foci of necrosis were also noted, too.
The patient’s biochemical parameters were repeated on the 10th post-operative day (Table 1). The patient made uneventful recovery and after 6 months does not have any clinical or laboratory evidence of recurrence or metastases. Regular follow-up has been advised.
DISCUSSION
ACC is a very rare tumor in children with an estimated incidence of 0.5-2 cases per million per year.5 Most
Reprint requests : S.B. Bavdekar, A2-9, Worli Seaside CHS, K.A.G. Khan Road, Worli, Mumbai-400018. E-mail : sandisha@bom7.vsnl.net.in.
childhood cases present with virilization with or without features of Cushing syndrome.6,7,8 Feminization and hyperaldosteronism have been noted less frequently. Virilization presents itself with facial and pubic hair, hirsutism, penile or clitoral enlargement, acne, deepening of voice and rapid growth. Axillary hair was not noticed in our case and seems to be an uncommon finding7. A number of congenital abnormalities like hemihypertrophy, aniridia, double ureter, ectopic kidneys, crossed-fused kidneys and undescended testes9,10 have been associated with childhood ACC.
Endocrinological function is assessed by estimation of urinary 17-ketosteroids (17 KS) and 17 hydroxy corticosteroids (17- OHCS) and by determination of serum levels of dehydroepiandrosterone (DHEA), cortisol, testosterone and aldosterone. Most cases with virilization have raised levels of DHEA. Elevated testosterone levels are usually attributed to peripheral
conversion of adrenal androgens. In our case, the carcinoma was selectively secreting testosterone.
Ultrasonography is usually the first radiological investigation undertaken. The CT scan is considered as an important method for assessing primary and metastatic disease at diagnosis and follow-up and may obviate the need for adrenal venography.8 Petrus et al11 opine that MRI is more accurate in detecting the organ of origin and extent of adrenal lesion and hence should be the radiological investigation of choice. Differentiation of ACC from benign adenoma is important and can be confirmed only by histopathological examination in a patient presenting with virilization.
Adrenal tumors selectively secreting testosterone are extremely rare.12-15 Ours is probably the youngest case of ACC selectively secreting testosterone. Many of the patients with adrenal tumor have been subjected to oophorectomy as excess testosterone has been wrongly sourced to ovaries.11-15
Staging of ACC is done by using classification provided by MacFarlane and Sullivan.16 Our case could be classified as stage I as there was no regional or metastatic extension and the tumor was less than 5 cm. in diameter. At such an early stage surgery with complete tumor resection is considered curative.5 Adjuvant chemotherapy is used for metastatic or recurrent disease.17,18 but paucity of cases makes its evaluation difficult. The role of radiation is controversial.19
ACC in adults differs from that in children. In adults, the proportion of non-functional tumors is higher and Cushing’s syndrome is the commonest presentation when the tumor is secreting hormones. In addition, the ACC in adults is resistant to radiotherapy.
Tumor size of more than 200 cm2 is the single-most poor prognostic feature.5 Surgery is the only definitive therapeutic modality in children, which can be curative only if ACC is diagnosed early and treated promptly. The children, however, have to be monitored for occurrence of other primary tumors like astrocytoma, osteogenic sarcoma, amelanotic melanoma10 and renal cell carcinoma.20 They require careful follow-up for
| Investigation | Normal levels | Pre-operative levels | Post operative levels |
|---|---|---|---|
| Urinary 17-Ketosteroids (mg/24 hr) | <1 | 0.8 | - |
| Serum cortisol (early morning, mcg/dl) | 5-25 | 10.8 | 9.2 |
| Serum FSH (mIU/ml) | 0-3 | 0.28 | - |
| Serum LH (mIU/ml) | 0-5 | 0.71 | - |
| Serum dehydroepiandrosterone sulfate (mcg/dl) | 40-200 | 30.0 | 30.0 |
| Serum testosterone (ng/ml) | 0.15-0.95 | 2.80 | ND |
| Serum estradiol (pg/ml) | 1-20 | 20.0 | - |
ND = Not detectable
Selective Testosterone Secreting ACC
reappearance of symptoms and signs, as recurrence can occur, too.
Acknowledgement
The authors thank The Dean, Seth G. S. Medical College and K. E. M. Hospital, Parel, Mumbai for permitting them to publish the case report.
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