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Ovary – Non-Epithelial Carcinoma

1. Diagnosis & Staging

1) Classification Criteria (non-epithelial tumours)

Sex Cord Stromal Tumours

Granulosa-Stromal Cell Tumours

  1. Granulosa cell tumour
    1. juvenile
    2. adult
  2. Tumours in the thecoma-fibroma group
    1. thecoma
      1. typical
      2. luteinized (a rare tumour has the features of luteinized thecoma and additionally, crystals of Reinke in the steroid cell component. This tumour has been called "stromal Leydig cell tumour".
    2. fibroma
    3. cellular fibroma
    4. fibrosarcoma
    5. stromal tumour with minor sex cord elements
    6. sclerosing stromal tumour
    7. (stromal luteoma) see Steroid (Lipid) Cell Tumours, stromal luteoma
    8. unclassified (fibrothecoma)
    9. others (This category includes the rare signet ring cell stromal tumour and the myxoma).

Sertoli-Leydig Cell Tumours; Androblastomas

  1. Well differentiated
    1. Sertoli cell tumour (tubular androblastoma)
    2. Sertoli-Leydig cell tumour
    3. (Leydig cell tumour) - see Steroid (Lipid) Cell Tumours, Leydig cell tumour
  2. Of intermediate differentiation
    1. variant - with heterologous elements (specify type)
  3. Poorly differentiated (sarcomatoid)
    1. variant - with heterologous elements (specify type)
  4. Retiform
    1. variant - with heterologous elements (specify type)

Sex Cord Tumour with Annular Tubules

Gynandroblastoma

Unclassified

Steroid (Lipid Cell Tumours)

  1. Stromal luteoma
  2. Leydig cell tumour (hilus cell tumour). Some Leydig cell tumours do not arise from hilus cells but within the ovarian stroma. These tumours have been called "Leydig cell tumours, non-hilar type".

Germ Cell Tumours

  1. Dysgerminoma
    1.  Variant - with syncytiotrophoblast cells
  2. Yolk Sac Tumour (Endodermal Sinus Tumour)
    1.  Variants
      1.  Polyvesicular vitelline tumour
      2. Hepatoid
      3. Glandular (some glandular yolk sac tumours resemble endometrioid adenocarcinoma and have been called "endometrioid-like".
  3. Embryonal Carcinoma
  4. Polyembryoma
  5. Choriocarcinoma
  6. Teratomas
    1.  Immature
    2. Mature
      1.  solid
      2. cystic (dermoid cyst)
      3. with secondary tumour (specify type)
      4. retiform (homunculus)
    3. Monodermal and highly specialized
      1.  struma ovarii
        • variant - with secondary tumour (specify type)
      2.  carcinoid
        • insular
        • trabecular
      3. struma carcinoid
      4. mucinous carcinoid
      5. neuroectodermal tumours (specify type)
      6. sebaceous tumours
      7. others
  7. Mixed (specify types)

Gonadoblastoma

  1. Variant - with dysgerminoma or other term cell tumour

Germ Cell - Sex Cord-Stromal Tumour

  1. Variant - with dysgerminoma or other term cell tumour

Tumours of Rete Ovarii

  1. Adenomatoid tumour
  2. Mesothelioma

Mesothelial Tumours

  1. Adenomatoid tumour
  2. Mesothelioma

Tumours of Uncertain Origin

  1. Small cell carcinoma (This tumour is of two types - one that is usually associated with paraendocrine hypercalcemia and has been called "small cell carcinoma, hypercalcemic type", and the other that has neuroendocrine features and has been designated "small cell carcinoma, pulmonary type". Probably most of the latter type belong in the surface epithelial-stromal category.)
  2. Tumour of probable Wolffian origin
  3. Hepatoid carcinoma
  4. Oncocytoma

Soft Tissue Tumours not Specific to Ovary

Malignant Lymphomas and Leukemias

Unclassified Tumours

Secondary (Metastatic) Tumours


2) Diagnostic Pathology

Gross Description

Gross description of oophorectomy specimens should include:

  1. Size of the ovary (tumour)
  2. Unilateral or bilateral involvement
  3. Surface appearance
  4. Cut surface appearance (solid, cystic, necrotic, hemorrhagic)
  5. Sampling of the lesion should include one block per cubic centimeter of tumour

Microscopic Description

Microscopic description should include the following features:

  1. Histologic type (if more than one pattern is seen, secondary pattern should be described)
  2. The histologic grade of tumour (I-III)
  3. If mucinous borderline tumour is diagnosed, it should be stated if it is of
    1. Mullerian type (endocervical)
    2. Intestinal type
  4. The presence or absence of lymphatic and/or venous invasion
  5. The presence or absence of serosal involvement
  6. The presence or absence of omental involvement
  7. Cytology of peritoneal fluid

*DNA ploidy for all borderline lesions (representative block should be selected for flow cytometry studies).

3) Staging (Ovary-Non-Epithelial Carcinoma)

Staging Diagram

Click image for larger version



2. Management

Revised: Mar. 2000

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 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

3. Follow-up

Updated 15 June 2007

Patients should be reminded that it is their responsibility to keep their recommended follow-up appointments. The objectives of the follow-up visits are as follows: 

  1. To determine the patient's immediate response to the treatment employed
  2. Early recognition and prompt management of treatment related complications
  3. Early detection of persistent or recurrent disease&
  4. Collection of meaningful data regarding the efficacy of existing treatment policies and their complications so that any appropriate modifications can be instituted

These objectives are best met by having the initial follow-up examination performed by the Agency medical staff. When appropriate, arrangements will be made for follow-up by the referring physician.

Year 1every 3 months
Year 2-3every 4 months
Years 4-5every 6 months
Years 5+annually

Note: follow-up consists of general exam, pelvic exam and pelvic-rectal exam. Annual Pap smear screening is not required in women who have undergone a hysterectomy as part of their treatment.  Women who have not had a hysterectomy should have cervical screening as per BCCA recommendations​.
Other investigations, such as tumor markers, are not required other than for the satisfaction of research protocol, and for specific indications when they arise.

SOURCE: Ovary – Non-Epithelial Carcinoma ( )
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