Rare gynecological cancers

The IUCT-Oncopole is a regional referral center within the national network of rare gynecological cancer centers (Rare Malignant Gynecological Tumors Network – accredited by the National Cancer Institute) for both treatment and the obligatory confirmatory diagnosis by a specialist anatomical pathologist. Every case is discussed at a multidisciplinary review meeting by the team of specialists (medical oncologists, radiation therapists, surgeons, radiologists, nuclear medicine physicians, anatomical pathologists) involved in treating gynecological cancers.

Rare ovarian tumors

Rare ovarian tumors, which account for approximately 20% of ovarian tumors, often affect young women and are generally diagnosed early. They include germinal tumors (dysgerminoma, endodermal sinus tumors, embryonal carcinoma, teratoma), tumors of the sex cords (granulosa cell tumor, Sertoli-Leydig cell tumors), clear-cell adenocarcinomas, mucinous adenocarcinomas, borderline tumors, small-cell carcinomas, carcinosarcomas and low-grade serous carcinomas.

Borderline ovarian tumors are noninvasive tumors of uncertain malignant potential. They are easier to cure than ovarian cancers and are usually treated by surgery. In young patients, it is sometimes possible to offer surgery that preserves the ovary and the uterus and thereby maintain fertility. Chemotherapy may be necessary if the disease has spread to the abdomen (a small percentage of patients).

National Cancer Institute guidelines stipulate that rare ovarian tumors must be treated at a referral center.

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Gynecological melanomas

Staging tests involve an ultrasound scan of the pelvic lymph nodes, an MRI or CT scan of the abdomen and pelvis, and a brain scan

 

Vaginal melanomas

Vaginal melanoma is a rare form of mucosal melanoma that accounts for less than 5% of vaginal cancers and less than 1% of all melanomas. Risk factors are not well known. Treatment depends on the location and extent of the disease. It may be necessary to remove just part of the vagina (colpectomy), but some cases may require more extensive surgery to remove the uterus and other neighboring organs. Lymph node dissection may also be required. Depending on the characteristics of the disease, surgery may be combined with radiotherapy and/or immunotherapy.

 

Vulvar melanomas

Treatment involves surgery of a large area around the lesion, with or without lymph node dissection, generally to look for a sentinel node.

 

Gynecological sarcomas

Uterine sarcoma is a rare form of cancer that accounts for less than 3% of malign gynecological tumors and less than 10% of uterine cancers. MRI scans of the pelvis and CT scans of the chest and abdomen are used to determine the extent of the disease. If the disease is restricted to the uterus, treatment will consist of surgery to remove the uterus and ovaries, possibly followed by radiotherapy. Chemotherapy may also be provided if the disease has spread beyond the uterus.

This treatment is provided in close collaboration with a multidisciplinary group of sarcoma specialists within the INCa-accredited NetSARC and RReps networks.

 

Gestational trophoblastic disease (moles)

Treatment for this rare disease, which begins in the placenta, is provided in collaboration with the national referral center for trophoblastic diseases.

The medical team may ask if you would be prepared to take part in a clinical trial. As well as giving patients the opportunity to benefit from a new form of treatment, clinical trials may be used to improve understanding of how cancer arises and develops, or to evaluate patients’ quality of life and see how it can be improved.