Results
Overall, 350 children were identified from 18 centers. Table 1 describes
the patient and tumor characteristics. The median age at tumor diagnosis
was 11 years (range, 0-17). Data on OMT affecting another family member,
most frequently at the first-degree level (80%), was retrieved in 10
cases (3%). None involved three consecutive generations. Genetic
investigations were conducted in seven cases, but no anomalies were
identified. Past medical history revealed a known germinal genetic
anomaly in two children without other OMTs in the family: STIM1pathogenic variant in exon 7 (one case) and familial SNX10pathogenic variant (one case).
The most frequent revealing symptom was abdominal pain in 61% of cases,
40% being related to adnexal torsion. Large palpable masses (27%),
urinary disorders (6%) and transit disorders (22%) were the other
symptoms, and these were explained by the tumor volume and local
compression. Also, menstrual cycle disorders were noted (4%). Data on
pubertal status were available for 51% of females, and 59% of them had
achieved puberty with onset menarche. An early puberty’s context was
observed in 12 cases (3%). These patients presented a median age of
nine years at OMT diagnosis. For seven of them, the early puberty was
considered unrelated to the OMT. OMT was incidentally diagnosed in 25%
of cases. Data on serum tumor markers (AFP and HCG) were available in
330 cases, being negative in 325 cases (98%). A low increase in AFP was
noted in five cases (1.5%). Eighteen patients (5%) presented
synchronous bilateral OMT at diagnosis.
Surgical procedures were carried out with a median delay of 10.5 days
(range, 0-1504) after diagnosis, 72% of them being planned and 28%
performed as an emergency. Adnexal torsions (n= 87) represented 76% of
emergency surgeries and were managed by laparoscopic detorsion
associated with tumor resection in one- or two-step procedures. TO was
performed in 57% of adnexal torsion cases. Tables 1 and 2 describe the
initial surgical management characteristics of the whole cohort.
Laparotomy was performed in 83% of cases, was performed as a single
procedure in 53% of cases, was associated with initial exploratory
laparoscopy in 32% of cases. Fifteen percent of the cohort underwent
exclusive laparoscopy. OSS was performed in 56% of unilateral OMTs and
in all bilateral OMTs. Considering the whole cohort, OSS was performed
in 59% of cases (n=208), and TO was performed in the other cases
(n=142). TO was performed by laparotomy associated or not to laparoscopy
in 87% of cases and by laparoscopy in 13% (Table 2).
Preoperative rupture was suspected and confirmed during surgery in eight
cases. Perioperative tumor rupture occurred in 23 cases (7%). If
perioperative rupture was more frequent when laparoscopy was performed
(11.3%), this was not significant (p=0.09) (Table 2).
All tumors underwent macroscopic complete resection independently of the
surgical approach. No peritoneal tumor spreading was observed on
peritoneal cytologic examination of the collected fluids, which was
performed systematically in all cases. Pathologic examination of the
specimen revealed minor associated immature malignant components in
seven cases, all of which exhibited moderate serum AFP elevation.