In view of the rarity and expectation of cure all patients with germ cell tumours should be referred to BCCA for assessment.
Sex Cord Tumours
See 2.1 (Diagnosis & Staging) for histological subtypes.
Management of these uncommon cancers is primarily surgical, but treatment must often be individualized. Discussion with BCCA staff is recommended.
Germ Cell Tumours
These rare malignancies occur predominantly in children and young women. Preoperative assessment is as for epithelial ovarian cancer with the exceptions that a barium enema may not be necessary. Pre-operative tumour markers are extremely important.
In the young patient surgery should be conservative. Most germ cell tumours are unilateral (with the exception of dysgerminoma). Even in the presence of extra-ovarian disease the uterus and contralateral ovary should be preserved as these tumours are curable with chemotherapy. Preservation of fertility of these young patients is important. (A thorough assessment of the abdominal cavity should be carried out with documentation of all sites of extra-ovarian disease.)
Caution: If frozen section is equivocal about the diagnosis, surgery must be conservative preserving fertility until a precise diagnosis is known.
Consultation with or referral to a division member is recommended before starting therapy to discuss the regimen and trial duration.
Non-dysgerminomatous Germ Cell Tumours
For purposes of treatment, patients with immature teratomas, endodermal sinus tumours, embryonal carcinoma and choriocarcinoma of the ovary (non-dysgerminoma) are treated in an identical fashion with chemotherapy.
All patients who have no visible residual disease after surgery and staging investigations (including CBC, liver functions, creatinine clearance, chest X-ray and whole lung tomograms, CT scan of brain, abdomen and pelvis) are treated with adjuvant chemotherapy. The treatment comprises cisplatin-based combination.
Repeat laparotomy may be required in patients with marker negative elements in the tumour. There must be a complete staging laparotomy. Please contact a gynecologic oncologist in this regard.
Patients with adverse factors e.g., bulky residual or extra abdominal metastases at presentation or those with failed primary treatment are offered high dose intensity multi drug treatment. Treatment is with curative intent with a high dose intensity cisplatin based regimen, also including etoposide, vincristine and prophylactic antimicrobials.
The prognosis is excellent for cure and maintenance of fertility.
Dysgerminomas
Dysgerminoma has a very high rate of cure. Given appropriate chemotherapy the prognosis is excellent. Surgery should be considered diagnostic as opposed to therapeutic. In women in the child-bearing years, who desire fertility, preservation of the uterus and one ovary is mandatory. Unilateral salpingo-oophorectomy is considered adequate except in the case of dysgenetic gonads. As this tumour has a propensity to spread via the lymphatic route careful assessment of the pelvic and para-aortic nodes should be carried out. Since the individual gynecologist may see only one or two patients with this tumour in their practice career, telephone consultation with the BCCA Gyne Oncology Group should be used liberally. As a general rule, in the young patient the initial surgery for germ cell malignancy should be conservative. TAH/BSO should not be used as primary management of this disease.
Chemotherapy is the cornerstone of treatment. However, these tumours are also very radiosensitive and therefore radiotherapy may be used with curative intent if chemotherapy fails or where fertility is not an issue.
Since the individual surgeon may not have extensive experience of this uncommon tumour, if necessary at the time of laparotomy, members of the Gynecologic Oncology Group may be contacted for consultation by phone. We welcome intra-operative consultations by phone.
Chemotherapy Protocols
Granulosa Cell Tumours
The natural history of this disease is often indolent, but is remarkably variable. Prognosis is decidedly worse if residual tumour remains after laparotomy and if the tumour shows DNA aneuploidy. A BCCA pathology consultation to determine ploidy is recommended in all cases. Those with completely resected DNA diploid tumours have an excellent prognosis and can be spared adjuvant therapy. For those with residual or aneuploid tumours, referral is recommended.
Estrogen Replacement Therapy - Site Specific Information
The relationship between estrogen and ovarian cancer is obscure. It is known that there are both estrogen and progesterone receptors present in many epithelial ovarian tumours. Unfortunately, the effects of exogenous hormones on these receptors are not well known. There is no evidence that estrogen has either the potential to promote recurrence or to decrease the time to recurrence.
In the absence of scientific evidence to the contrary, it is not felt necessary to withhold estrogen replacement therapy from any symptomatic woman with ovarian malignancy regardless of risk category.
Recommendation:
In absence of a uterus, estrogen alone
With uterus in situ, continuous estrogen plus progesterone
Revised: Mar. 2000