Adrenocortical Tumor In Children – Report of a Case

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Adrenocortical Tumor In Children – Report of a Case

Mansoor Mahmood, NadeemAkhtar, Saqib Ismail, SheemaLuqman and Yazeed Owiwii
Department of Pediatric Surgery, Children Hospital, Pakistan Institute of Medical Science, Islamabad

Abstract
Adrenocortical carcinoma in childhood is a rare, potentially fatal disease.Adrenocortical carcinoma has a bimodal age distribution with cases clustering in children under 6, and in adults 30–40years old. We are presenting a case of 2 years old girl who presented with symptoms of virilization. She was operated for the adrenal mass. A diagnosis of adrenocortical carcinoma was rendered.
Key words: Adrenocortical Carcinoma in Children, Virilization, Adrenal Tumors
Introduction
Adrenocortical neoplasms are rare in the pediatric population, accounting for less than 0.2% of all pediatric tumors
and 6% of all adrenal tumors in children.1 adrenocortical tumors occur more frequently in girls, with a male to female ratio of approximately 1:2 to 1:3.2Most adrenocortical tumors in children are hormonally active, it may lead to either a virilizing or a feminizing features. Virilization with or without hypercortisolisim is the most common presentation. 3These virilizing tumors may be more difficult to recognize in boys than in girls. Boys may present with precocious puberty, including penile enlargement, acne, and premature development of pubic, axillary, and facial hair. Girls may develop clitoral hypertrophy, hirsutism, and acne. The treatment of choice is adrenalectomy. Adrenocortical tumors are associated with several congenital anomalies, including hemihypertrophy; other tumors associated with
hemihypertrophy include nephroblastoma and
hepatoblastoma. Patients with Beckwith-Wiedemann syndrome (exomphalos, macroglossia, and gigantism) also have
a higher than expected incidence of adrenocortical carcinoma. 4 Most adrenocortical tumors, however, occur sporadically.5
Case Report
A 2 years old female patient presented with history of excessive hair growth on body, increased weight gain, and
polyphagia for 6 months duration. Her past medical or surgical history & family history were not significant. Finding
on examination

         

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