A nationwide study of granulosa cell tumors in pre- and postpubertal girls: Missed diagnosis of endocrine manifestations worsens prognosis

被引:0
作者
Kalfa, N
Patte, C
Orbach, D
Lecointre, C
Pienkowski, C
Philippe, F
Thibault, E
Plantaz, D
Brauner, R
Rubie, H
Guedj, AM
Ecochard, A
Paris, F
Jeandel, C
Baldet, P
Sultan, C [1 ]
机构
[1] CHU Montpellier, Unite Endocrinol Gynecol Pediat, Hop Arnaud Villeneuve, F-34295 Montpellier, France
[2] Inst Curie, Paris, France
[3] Inst Gustave Roussy, Dept Oncol, Villejuif, France
[4] Hop Charles Nicolle, Serv Endocrinol Pediat, Rouen, France
[5] CHU Toulouse, Hop Enfants, Serv Endocrinol Pediat, Toulouse, France
[6] CHU Grenoble, Serv Pediat, Grenoble, France
[7] Hop Necker Enfants Malad, Serv Endocrinol, Paris, France
[8] Hop St Joseph, Serv Endocrinol Pediat, F-75674 Paris, France
[9] CHU Toulouse, Hop Enfants, Serv Oncol, Toulouse, France
[10] CHU Nimes, Hop Caremeau, Serv Endocrinol, Nimes, France
[11] CHU Montpellier, Hop Lapeyronie, Serv Anatomopathol, Montpellier, France
关键词
ovarian granulosa cell tumors; children; diagnosis; endocrine signs; outcome;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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 post-pubertal 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. Conclusion: 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.
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页码:25 / 31
页数:7
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