Surgery
- Since most tumors present as localized, stage FIGO Ia tumors,
fertility sparing surgery, i.e. tumor resection by oophorectomy or
adnexectomy will constitute the only therapy of these tumors [Level
IV, Grade A].8, 22, 23, 25
- Tumor resection with oophorectomy or adnexectomy should be the first
surgical care [Level III; grade A]. Prior biopsy is discouraged
[Level II, grade E].
- Median laparotomy constitutes the standard surgical approach in adults
[Level II, Grade A], but in children also a sub-umbilical
transverse incision or a Pfannenstil laparotomy can be accepted
(depending on the size of the tumor and the initial tumor spread),
which both allow for a good tumor exposure and a better cosmetic
result [Level III, Grade B]. In case of small tumors, laparoscopic
resection may be performed by experienced surgeons [Level IV, grade
C]. However, oncologic criteria must be respected also with
minimally invasive procedures [Level II, grade A]. Tumor rupture,
puncture or any other violation of the tumor capsule have to be
avoided stringently [Level IV, Grade E].26
- Staging includes cytological evaluation of ascites and/or peritoneal
washings in absence of ascites, ideally before tumor mobilisation
[Level IV, Grade B].25
- Staging includes inspection and palpation of the contralateral ovary,
inspection of the peritoneal cavity, focussing on the pelvis, pouch of
Douglas and diaphragmatic cupola, with biopsy of any suspicious
lesions, inspection and biopsy of any suspicious lymph nodes [Level
IV, Grade B].25
- Tumors confined to the ovary should be resected via ovariectomy; in
cases of pelvic adhesion/infiltration, ipsilateral adnexectomy has to
be performed [Level IV, Grade B].25
- In cases of adhesions to the omentum, omentectomy is recommended;
routine omentectomy is not required, if unsuspicious [Level IV,
Grade B].
- In cases of bilateral tumors, ovary sparing tumor resection may be
considered as an individual approach by an experienced surgeon and
with appropriate equipment [Level V, Grade B].25
- Routine retroperitoneal lymph node dissection is not recommended, if
unsuspicious [Level IV, Grade B].
- Biopsy of an unsuspicious (in palpation and by ultrasound)
contralateral ovary is not required [Level IV, Grade B].
- There is no role for debulking surgery (apart from palliative
surgery). Hysterectomy as well as contralateral ovariectomy or other
mutilating surgery should never be performed as an upfront surgery.
Instead, inoperable tumors should be cautiously biopsied in order to
assure the pathological diagnosis, and upfront chemotherapy should be
initiated followed by delayed tumor resection [Level IV, Grade
A].25
Adjuvant Therapy including Chemotherapy (Table IV)
- Stage IA/IB tumors do not require any adjuvant chemotherapy, in
particular if histology shows good to intermediate differentiation; in
selected patients with specific histologic criteria e.g. sarcomatous
elements within SLCT, adjuvant chemotherapy can be discussed after
individual consultation [Level III, Grade E].1,
8, 22, 23
- In stage IC juvenile GrCTs, chemotherapy is certainly recommended in
case of pre-operative spontaneous tumor rupture (FIGO IC2) and/or
malignant ascites (FIGO IC3) [Level IV, Grade A]. The indication
for chemotherapy is disputable in stage IC1 juvenile GrCTs if
intraoperative tumor spread occurs and appropriate surgical management
(peritoneal washings) has been performed (FIGO Stage IC1) [Level IV,
Grade C].26
- Adjuvant chemotherapy is recommended in all stage IC SLCTs,
irrespective of the time of the tumor rupture [Level IV, Grade
A].18
- Adjuvant chemotherapy is recommended in all tumors with locoregional
spread or distant metastases (FIGO stage II, III, IV) [Level III,
Grade A].1, 8, 22, 23, 25
- In unresectable tumors, up-front chemotherapy may be considered
followed by delayed tumor resection [Level IV, Grade C].
- All other histologic subtypes of SCSTs (different from juvenile GrCTs
and SLCTs) rarely present beyond stage IA and rarely require adjuvant
chemotherapy [Level IV, Grade D].21
- Chemotherapy is chosen in analogy to GCT protocols and commonly
includes cisplatin-based regimen (e.g. bleomycin-etoposide-cisplatin
or etoposide-ifosfamide-cisplatin) [Level III, Grade A] (Table
V).1, 25
- In stage IC tumors, (three to) four cycles of chemotherapy and in
stage II, III, IV tumors four cycles of chemotherapy are recommended,
with second look surgery in case of initial macroscopic incomplete
resection or residual disease [Level IV, Grade B]. Some study
groups recommend a minimum of four cycles of chemotherapy, with
escalation to up to six cycles in metastatic tumors [Level IV, Grade
C].
- Radiotherapy is not routinely recommended [Level IV, Grade E]
- In case of insufficient response or tumor progression, therapy
intensification can be considered on an individual basis, after
discussion with national and international experts. Prognosis of these
patients is poor. Therapeutic options include addition of bevacizumab,
HIPEC (hyperthermic intraperitoneal chemotherapy with cytoreductive
surgery), regional deep hyperthermia in combination with platin-based
chemotherapy, high dose chemotherapy with autologous hematopoietic
stem cell transplantation, and radiotherapy [Level V, Grade
C].27, 28
Follow-up
- Regular follow-up is recommended, at least for the first five to ten
years [Level IV, Grade B].
- As up to 10% of patients may develop metachronous contralateral
tumors, long-term follow-up may be offered [Level IV, Grade
B].16, 18
- In adult GrCTs, longer follow-up is recommended, because these tumors
may recur after more than ten years [Level IV, Grade
B].12
- In DICER1 positive tumors, life long surveillance is
recommended, because DICER1 associated tumors may develop even
at older age [Level IV, Grade A].16
- Follow-up investigations include anamnesis, physical examination, and
measurement of serum tumor markers only if these have been elevated
perioperatively [Level IV, Grade A].
- In SLCTs with DICER1 pathogenic variant, thyroid function and
structure should be monitored with ultrasound and laboratory
investigations (association with multinodular goitre) at diagnosis and
then at least every two years [Level IV, Grade
B].3, 16
- Radiographic follow-up includes pelvic and abdominal ultrasound, in
three-monthly intervals during the first three years after diagnosis
and in increasing intervals thereafter [Level IV, Grade B].
- Abdominal MRI is recommended in case of equivocal findings and in
(adolescent) patients with poor visibility on ultrasound [Level IV,
Grade B].
- Routine chest X-ray is not recommended for follow-up [Level IV,
Grade E].