title: A nationwide study of granulosa cell tumors in pre- and postpubertal girls: missed diagnosis of endocrine manifestations worsens prognosis.
abstract: BACKGROUND
  There are very few data on the natural history of ovarian granulosa cell tumors (OGCT) in children. The aim of this study was to determine whether early recognition and diagnosis of the initial endocrine signs could improve the outcome of these tumors.
  
  
  METHODS
  In a nationwide study from 1990 to 2004, we analyzed the clinical, biological and pathologic data from 40 pre- and postpubertal girls presenting an OGCT.
  
  
  RESULTS
  1. Among the prepubertal girls (n = 29), 17 OGCTs were diagnosed on the basis of precocious pseudopuberty. None of the 17 girls had a peritoneal spread of the tumor (100% FIGO stage Ia). Diagnosis based on a tumoral or acute abdomen (12 cases) was associated with frequent intraperitoneal ruptures of the tumor (50%) and a risk of relapse (2 cases). Of the eight girls who had had a misdiagnosed precocious pseudopuberty, five had a pre- or perioperative tumoral rupture. 2. Among the postpubertal girls (n = 11), endocrine manifestations such as secondary amenorrhea or virilization had been underevaluated in three of them and the diagnosis was established from a tumoral abdomen. This clinical presentation was associated with frequent ruptures of the mass in the peritoneum (80%) and a higher risk of recurrence (30%). 3. A delayed diagnosis of OGCT despite previous endocrine signs (11 cases; 8 pre- and 3 postpubertal) was associated with a high risk of pre- or peri-operative peritoneal tumor spreading (70% FIGO stage Ic or IIc, p <0.05). The mean delay for diagnosis ranged from 3 to 11 months.
  
  
  CONCLUSIONS
  This study highlights the critical role of early diagnosis of OGCT in pre- and postpubertal girls, particularly at the first seemingly banal signs of endocrine disorder. Peritoneal spread of the tumor may thereby be prevented, which improves the prognosis.
authors.affiliation: Hôpital Arnaud de Villeneuve, CHU Montpellier, France.
authors.forename: N
authors.initials: N
authors.lastname: Kalfa
authors.forename: C
authors.initials: C
authors.lastname: Patte
authors.forename: D
authors.initials: D
authors.lastname: Orbach
authors.forename: C
authors.initials: C
authors.lastname: Lecointre
authors.forename: C
authors.initials: C
authors.lastname: Pienkowski
authors.forename: F
authors.initials: F
authors.lastname: Philippe
authors.forename: E
authors.initials: E
authors.lastname: Thibault
authors.forename: D
authors.initials: D
authors.lastname: Plantaz
authors.forename: R
authors.initials: R
authors.lastname: Brauner
authors.forename: H
authors.initials: H
authors.lastname: Rubie
authors.forename: A M
authors.initials: AM
authors.lastname: Guedj
authors.forename: A
authors.initials: A
authors.lastname: Ecochard
authors.forename: F
authors.initials: F
authors.lastname: Paris
authors.forename: C
authors.initials: C
authors.lastname: Jeandel
authors.forename: P
authors.initials: P
authors.lastname: Baldet
authors.forename: C
authors.initials: C
authors.lastname: Sultan
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D000328:Adult; D000568:Amenorrhea / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D005260:Female; D006106:Granulosa Cell Tumor / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007223:Infant; D010051:Ovarian Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D010534:Peritoneal Neoplasms / Q000473:pathology*; D011379:Prognosis; D011627:Puberty; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D012421:Rupture