Etiology and clinical characteristics of 30 children with tumor-associated precocity

Abstract

Objective To analyze the etiology and clinical characteristics of children with tumor-associated precocity. Methods Thirty children with tumor-associated precocity hospitalized in Department of Surgery of Beijing Children's Hospital Affiliated to Capital Medical University from Jan. 2006 to Mar. 2012 were selected as research subjects. The causes, clinical characteristics and treatment situation of the patients were retrospectively studied. Results The group of patients included 14 boys and 16 girls, with average age of (3.74±2.44)years. Twenty-two patients(73.3%) were younger than 5 years old, and their etiological distributions listed as follows: 8 cases were hypothalamic hamartoma(HH), 2 cases were hypothalamic germinoma, 1 case was arachnoid cyst, 7 cases were adrenocortical tumor(in which 1 case was adenoma and 6 cases were adenocacinoma respectively), 5 cases were ovarian cyst, 2 cases were ovarian tumor (in which 1 case was endodermal sinus tumor and 1 case was sex cord-stromal tumor respectively), 2 cases were MuCune-Albright syndrome, and 1 case was mediastinal teratoma, 1 case was penis primitive neuroectoderm tumor, and 1 case was Leydig cell proliferation accompanied with neoplasma. Eleven patients(36.7%) suffered central precocious puberty, with HH(n=8) being the most common causes. Four patients with HH presented with gelastic epilepsy. Precocious puberty caused by HH patients could be safely controlled by gonadotropin-releasing hormone agonists. Nineteen patients(63.3%) suffered peripheral precocious puberty, with adrenocortical tumor being the most common cause for the boys and ovarian cyst being the most common cause for the girls. Besides that, the onset symptom of a patient with adenocacinoma was facial acne accompanied with hypertrichiasis and another patient with ovarian tumor had intermittent abdominal pain, and the onset symptoms of all the boys were external genital development and those of the girls were mammary development or colporrhagia, respectively. Conclusions Tumors are one of the most causes of precocious puberty in children. During the process of diagnosis and treatment of precocious puberty, imaging examinations on pituitary, gonad and adrenal gland should be paid great attention to and seldom occurred tumors should also be consi-dered. For ovarian cyst patients with precocious puberty attention shall be paid attention to the differentiation from MuCune-Albright syndrome. Key words: Precocious puberty; Hypothalamic hamartoma; Adrenocortical tumor; Ovarian cyst; MuCune-Albright syndrome; Teratoma