Acta Paediatr 81: 1061-4. 1992
CASE REPORT
Cystic adult granulosa cell tumor causing precocious pseudopuberty in a six-year-old girl 0 Arisaka, T Matsumoto’, A Hosaka, N Shimura, Y Nakayama, H Fujita, Y Yamashiro and K Yabuta Department of Pediatrics and ?he Second Division, Department of Pathology’, Juntendo Universi?y School of Medicine, Tokyo, Japan
Arisaka 0, Matsumoto T, Hosaka A, Shimura N, Nakayama Y, Fujita H, Yamashiro Y, Yabuta K. Cystic adult granulosa cell tumor causing precocious pseudopuberty in a six-year-old girl. Acta Pzdiatr 1992;81:10614. Stockholm. ISSN 0803-5253 A very rare Occurrence of adult granulosa cell tumor of the ovary (not of the juvenile type) causing precocious pseudopuberty in a six-year-old girl is described.An additional feature of interest was that the tumor appeared entirely cystic. To our knowledge, this condition in such a young premenarchal patient has not been reported previously. 0 Adult granulosa cell tumor, cyst, precociouspseudopuberty, prepubertal girl 0 Arisaka, Department of Pediatrics, Juntendo University School of Medicine. 2-1-1 Hongo Bunkyo-ku, Tokyo 113. Japan
Neoplastic ovarian tumors share 1-2% of all causes of precocious pseudopuberty in girls ( I , 2). The most common forms of ovarian tumors presenting as pseudopuberty are the granulosa cell tumors (GCT) although the tumors account for only 3% of all ovarian tumors ( I ) . In addition, the clinical and pathological features of GCT that occur frequently in young girls (so-called juvenile GCT) differ from those of the forms encountered in older women (adult GCT) (3). Therefore, adult GCT as a cause of precocious pseudopuberty in young girls are extremely rare (3-6). We report a six-year-old girl presenting with signs of isosexual precocious puberty due to a large ovarian tumor. Grossly, the tumor resembled a multilocular cystadenoma on ultrasound examination, but the microscopic features of the resected specimen were definitely consistent with those of adult GCT, and not juvenile GCT. To our knowledge, this type of cystic adult GCT in such a young prepubertal patient has not been previously reported.
Case report A girl aged 6 years and 11 months was referred for evaluation of breast budding and vaginal bleeding. Breast growth had occurred during the preceding six months and intermittent vaginal bleeding had occurred three months prior to this visit. The patient had not complained of abdominal pain. Physical examination revealed a height of 116.8 cm (1 SD above the mean height for age) and a weight of 25 kg (1 SD above mean weight). It appeared that her growth velocity had
accelerated (from 6 cm/year to 10 cm/year) over the previous six months. Her breast development was Tanner stage 111, and no pubic or axillary hair was present. The external genitalia showed pubertal development of the labia major. In the lower part of the abdomen, a firm non-tender mass, about 10 cm in diameter, was palpable. Pelvic ultrasound examination showed a huge multilocular cyst occupying the pelvic cavity; there were no solid areas (Fig. I). The uterus was visible, but appeared enlarged for age. Pelvic computed tomography revealed findings similar to those of ultrasonography. Plasma sex hormone values under diurnal basal conditions were estradiol 1853 pmol/l (prepubertal < 40 pmol/l), progesterone 3.5 nmol/l (prepubertal < 1.5 nmol/l), testosterone 0.26 nmol/l (prepubertal female < 0.40 nmol/l) and insulin-like growth factor I 3.88 U/ ml (prepubertal < 1.5 U/ml). Urinary total estrogen excretion was 91 pg/day (prepubertal female < 1 pg/ day). A standard bolus luteinizing hormone-releasing hormone test (2 pg/kg) revealed entirely suppressed levels of luteinizing hormone and follicle stimulating hormone (
CASE REPORT
Cystic adult granulosa cell tumor causing precocious pseudopuberty in a six-year-old girl 0 Arisaka, T Matsumoto’, A Hosaka, N Shimura, Y Nakayama, H Fujita, Y Yamashiro and K Yabuta Department of Pediatrics and ?he Second Division, Department of Pathology’, Juntendo Universi?y School of Medicine, Tokyo, Japan
Arisaka 0, Matsumoto T, Hosaka A, Shimura N, Nakayama Y, Fujita H, Yamashiro Y, Yabuta K. Cystic adult granulosa cell tumor causing precocious pseudopuberty in a six-year-old girl. Acta Pzdiatr 1992;81:10614. Stockholm. ISSN 0803-5253 A very rare Occurrence of adult granulosa cell tumor of the ovary (not of the juvenile type) causing precocious pseudopuberty in a six-year-old girl is described.An additional feature of interest was that the tumor appeared entirely cystic. To our knowledge, this condition in such a young premenarchal patient has not been reported previously. 0 Adult granulosa cell tumor, cyst, precociouspseudopuberty, prepubertal girl 0 Arisaka, Department of Pediatrics, Juntendo University School of Medicine. 2-1-1 Hongo Bunkyo-ku, Tokyo 113. Japan
Neoplastic ovarian tumors share 1-2% of all causes of precocious pseudopuberty in girls ( I , 2). The most common forms of ovarian tumors presenting as pseudopuberty are the granulosa cell tumors (GCT) although the tumors account for only 3% of all ovarian tumors ( I ) . In addition, the clinical and pathological features of GCT that occur frequently in young girls (so-called juvenile GCT) differ from those of the forms encountered in older women (adult GCT) (3). Therefore, adult GCT as a cause of precocious pseudopuberty in young girls are extremely rare (3-6). We report a six-year-old girl presenting with signs of isosexual precocious puberty due to a large ovarian tumor. Grossly, the tumor resembled a multilocular cystadenoma on ultrasound examination, but the microscopic features of the resected specimen were definitely consistent with those of adult GCT, and not juvenile GCT. To our knowledge, this type of cystic adult GCT in such a young prepubertal patient has not been previously reported.
Case report A girl aged 6 years and 11 months was referred for evaluation of breast budding and vaginal bleeding. Breast growth had occurred during the preceding six months and intermittent vaginal bleeding had occurred three months prior to this visit. The patient had not complained of abdominal pain. Physical examination revealed a height of 116.8 cm (1 SD above the mean height for age) and a weight of 25 kg (1 SD above mean weight). It appeared that her growth velocity had
accelerated (from 6 cm/year to 10 cm/year) over the previous six months. Her breast development was Tanner stage 111, and no pubic or axillary hair was present. The external genitalia showed pubertal development of the labia major. In the lower part of the abdomen, a firm non-tender mass, about 10 cm in diameter, was palpable. Pelvic ultrasound examination showed a huge multilocular cyst occupying the pelvic cavity; there were no solid areas (Fig. I). The uterus was visible, but appeared enlarged for age. Pelvic computed tomography revealed findings similar to those of ultrasonography. Plasma sex hormone values under diurnal basal conditions were estradiol 1853 pmol/l (prepubertal < 40 pmol/l), progesterone 3.5 nmol/l (prepubertal < 1.5 nmol/l), testosterone 0.26 nmol/l (prepubertal female < 0.40 nmol/l) and insulin-like growth factor I 3.88 U/ ml (prepubertal < 1.5 U/ml). Urinary total estrogen excretion was 91 pg/day (prepubertal female < 1 pg/ day). A standard bolus luteinizing hormone-releasing hormone test (2 pg/kg) revealed entirely suppressed levels of luteinizing hormone and follicle stimulating hormone (
