Abstract
Objective
To analyse ovarian Sertoli-Leydig cell tumours (SLCTs) for potential prognostic markers
and their use for treatment stratification.
Patients
Forty-four patients were included. Patients were prospectively reported to the German
MAKEI (Maligne Keimzelltumoren) studies (n = 23), French TGM protocols (n = 10), Italian Rare Tumour Project (TREP) registry (n = 6), and the Polish Pediatric Rare Tumour Study group (n = 5). Tumours were classified according to World Health Organisation (WHO) and staged
according to International Federation of Gynecological Oncology (FIGO).
Results
Median age was 13.9 (0.5–17.4) years. All patients underwent resection by tumour enucleation
(n = 8), ovariectomy (n = 17), adenectomy isolated (n = 18) or with hysterectomy (n = 1). FIGO-stage: Ia 24 pts., Ic 17 pts., II/III 3 pts. One patient had bilateral tumours. Four patients (stage Ia: 3, stage Ic: 1) developed
a metachronous contralateral tumour. Otherwise, all stage Ia patients remained in
complete remission. Among 20 patients with incomplete resection or tumour spread (stage
Ic–III), eight relapsed, and five patients died. Eleven patients were initially treated
with two to six cycles of cisplatin-based chemotherapy. Of these, seven patients are in continuous
remission. Poor histological differentiation was associated with higher relapse rate
(5/13) compared to intermediate (3/18) and high differentiation (0/4). Tumours with
retiform pattern or heterologous elements showed a high relapse rate, too (5/11).
After a median follow-up of 62 months, event-free survival is 0.70 ± 0.07, relapse-free survival 0.81 ± 0.06 and overall survival 0.87 ± 0.05.
Conclusions
Prognosis of SLCTs is determined by stage and histopathologic differentiation. Complete
resection with careful avoidance of spillage is a prerequisite of cure. The impact
of chemotherapy in incompletely resected and advanced stage tumours remains to be
evaluated.
Keywords
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Article info
Publication history
Published online: December 13, 2014
Accepted:
November 15,
2014
Received in revised form:
September 26,
2014
Received:
June 1,
2014
Identification
Copyright
© 2014 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.
