Large Adrenocortical Carcinoma Presenting as an Adenoma with Precocious Puberty
Aliza Mittal · Mukul Aggarwal · Pradeep Debata
Received: 12 June 2011 / Accepted: 30 November 2011 /Published online: 16 December 2011
C Dr. K C Chaudhuri Foundation 2011
Sir, We present a case of peripheral precocious puberty due to a large adrenal mass, with hemorrhagic and necrotic areas, despite long history and no metastasis, making the distinc- tion between carcinoma and adenoma very difficult.
A 3.5-y-boy presented with progressive enlargement of penis, appearance of pubic hair and hoarseness of voice for 2 y. There was no history of focal neurological deficits, testicular swelling or chronic drug intake. There was no family history of precocious puberty. The child had a mus- cular look, penile length and testicular volume were 8.5 cm and 2.3 cc respectively. Child belonged to Sexual Maturity Rating stage III. He weighed 23 kg, height was 107.5 cm and arm span of 103.7 cm. The blood pressure recorded was 90/60 mmHg which was normal for his age. A 5 cm round- ed, firm, non tender lump in left lumbar region was felt.
The bone age was 13-15 y. CECT abdomen revealed a large well defined heterogeneously enhancing necrotic mass involving left adrenal with specs of calcifications, closely abutting spleen and left kidney, however the fat planes were maintained. No evidence of metastatis was found on MRI brain and CT chest. Per-operatively a 13×10 cm tumor was found in left suprarenal area with intact capsule and hemor- rhagic areas (Fig. 1), without any evidence of invasion.
A. Mittal · M. Aggarwal · P. Debata Department of Pediatrics, VMMC and Safdarjung Hospital, New Delhi, India
A. Mittal Room No 57, Second Floor, Resident Doctors Hostel, Safdarjung Hospital, New Delhi 110029, India e-mail: alizamittal@gmail.com
Considering the Weiss criteria, weight, size and presence of necrosis and hemorrhage (Fig. 2), diagnosis of adreno- cortical carcinoma was considered.
The hormonal profile revealed raised 17 hydroxy proges- terone (5.14 ng/ml), testosterone (30 ng/dl), DHEAS (543 µg/dl) and cortisol (104 µg/ml) levels which came down to serum cortisol level of 48 µg/dl, serum testosterone of 10 ng/dl and serum DHEAS level of 152 ug/dl which were within normal limits 3 mo after surgery.
Adrenocortical carcinomas are very rare cause of gonado- tropin independent precocious puberty with an incidence of 1- 2 per million populations annually. It is usually unilateral and seen in females [1]. It has a bimodal age distribution [2].
Clinically, virilization is most common and only 10% present with cushingoid features and palpable mass. In 20- 40% cases, no history of hormonal overproduction is found. Red flag signs for adrenal carcinoma are large size (>4 cm), intra-lesional calcification, hemorrhage, necrosis, tumor
invasion into major vessels, distant metastasis, secretions of more than one class of corticosteroids, associated hyperten- sion and hypokalemia [3].
In a study by Ribiero et al. it was suggested that an interval of greater than or equal to 6 mo between first symptoms and diagnosis, tumor weight greater than 100 g,
tumor size greater than 200 cm3, and high levels of urinary 17-ketosteroids (17-KS) and 17-hydroxycorticosteroids (17- OH) were associated with an unfavorable outcome [4].
However, it is said that clinical, laboratory, or pathologic features are not reliable predictors for recurrence and dis- crimination of malignancy in these tumors [5].
In our case despite being such a large mass, it was peculiar as it was encapsulated with no evidence of regional invasion or metastasis, slowly progressing and presented like a benign lesion. Such a large adrenal malignant mass of this nature has not been described in the pediatric litera- ture to the best of our knowledge.
References
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2. Allolio B, Fassnacht M. Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab. 2005;91:2027-37.
3. Oncology. [Online]. 2007 [cited 2007 Jan 14];Available from : URL: http://www.oncology-articles.blogspot.com/2007/01/adrenal- carcinoma.html
4. Ribeiro RC, Sandrini Neto RS, Schell MJ, et al. Adrenocortical carcinoma in children: a study of 40 cases. J Clin Oncol. 1990;8:67-74.
5. Ciftci AO, Senocak ME, Tanyel FC, Büyükpamukçu N. Adreno- cortical tumors in children. J Pediatr Surg. 2001;36:549-54.