Dysgerminoma

lbollen

My daughter was scheduled to have a large fibroid removed but was in alot of pain so I called the doctor to up her surgery date. When he went in he found that it was a very rare malignant tumor 15 cm called dysgerminoma. She also had to have one ovary and tube removed, however she did not have any chemo/radiation or anything but markers were placed and they have been doing blood work every 2 months and then 6 months. However I question why blood work because when they thought it was a fibroid her blood work never showed anything. She has had many UTIs since and I'm wondering if anyone knows of any blood work that would detect this? Very nervous, yes she has been 3 years cancer free, or at least we think so. 

BubMunkeyBles

What tumor markers did they

What tumor markers did they order when they thought it was a fibroid? Pure dysgerminoma isn't always associated with elevated tumor markers. It was hard to tell with my first case what was elevated because I was pregnant and what may have been a tumor marker. I was monitored with ultrasound the first few years and then blood work also. I did do EP chemo though. If you're worried definitely push for an ultrasound with your doctor though, mine have thankfully always been really receptive to any worries. 

NoTimeForCancer

Ladies, and for any men who

Ladies, and for any men who may visit here concerned for their loved ones, if you have ever been found to have cancer in any gynecologic areas PLEASE work with a gynecologic oncologist.  They specialize in 'below the belt' cancers that are truly unique to women.  

Many of us have blood work to monitor our CA125 but that is NOT always a good indication that something is going on.  I would add that you have to be your own advocate for care.  

LorettaMarshall

Ibollen~Referenced article seems 2 answer many of your questions

Dear Concerned Parent:

 Like most of us here no doubt, I had never heard of this type of Ovarian Cancer, but now I know more because of your letter.  You have a right to be concerned about just having blood check after blood check.  Are you sure that it is the only follow-up necessary for your daughter?  This is quite a lengthy article with several parts to it.  In order to understand it more fully, it will have to be read in its entirety.  I’ve copied some of the parts, but not the whole.   However, I find that quoting enough for you to know that this is information that will be useful in your situation, and educational for the rest of us OC patients

 Here’s hoping you find out all you need to know, and this is a good place to start to learn more about dysgerminoma.  I’ve scanned many sections of this article, and it seems to cover all the questions you are wondering about.  I would be careful to read ALL of it.  I would make a note of things this article suggests, and compare it with the treatment, or lack of it, that your daughter is receiving.  I'm the kind of person that would print this out, highlight in yellow the parts I have questions about, and take it with me to the next doctor's appointment.    Ovarian cancer is no respecter of age it appears. 

 Loretta – (age 78) Peritoneal Carcinomatosis/Ovarian Cancer Stage IV since Nov. of 2012, still coping by the grace of God.

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 1.      http://emedicine.medscape.com/article/253701-overview

“Ovarian Dysgerminomas

Updated: Apr 15, 2015 - …

 The 3 major types of ovarian tumors are epithelial, sex cord, and germ cell.

Epithelial cell tumors represent the majority of all ovarian neoplasms (82%).

Conversely, germ cell tumors (GCTs) are rare, comprising approximately 20% of all ovarian tumors, both benign and malignant.

 Approximately 3-5% of ovarian GCTs are malignant.

 The most commonly occurring GCT is the dysgerminoma, which accounts for approximately 2% of all ovarian cancers.

 Although rare, dysgerminomas are important irrespective of incidence because they most commonly affect women of reproductive age (ie, < 30 y).

 In fact, dysgerminomas make up two thirds of all malignant ovarian neoplasms in women younger than 20 years.

 Moreover, once diagnosed, dysgerminomas respond well to therapy, potentially sparing patients from infertility and early mortality.

_________________________________________

 2.      http://emedicine.medscape.com/article/253701-overview#a5

Pathophysiology

 “Typically, germ cells are encapsulated at birth within the primordial follicle. If they somehow escape encapsulation, cell death usually occurs.

 If the germ cells survive, rapid growth ensues, owing to the lack of normal contact inhibition, hence germ cell tumor (GCT) formation.

 All dysgerminomas are considered malignant, but only one third of dysgerminomas behave aggressively.

The exact etiology of dysgerminomas has not been determined, although recent molecular studies have implicated loss of function with potential tumor suppressor gene TRC8/RNF139 as a possible etiology. ^[1]

 Additionally, 5% of all dysgerminomas occur in dysgenetic gonads and may be associated with gonadoblastomas.

 Genetic disorders of the ovary are associated with karyotypic abnormalities and are discussed in Dysgerminomas in patients with karyotypic abnormalities in Complications…”

 Age

Although most ovarian cancers occur during the menopausal and perimenopausal years (ie, 50-59 y), dysgerminomas tend to occur frequently in the pediatric population.

 Dysgerminomas are most commonly observed in younger women. Seventy-five percent of dysgerminomas occur in patients in the third and fourth decades of life, with the mean age being 22 years…”

 SECTIONS

• Ovarian Dysgerminomas
• Overview

 Background

• Presentation
• DDx
• Workup
• Treatment
• Medication
• Follow-up
• References

3.      http://emedicine.medscape.com/article/253701-clinical

4.      http://emedicine.medscape.com/article/253701-workup

“Ovarian Dysgerminomas Workup

Laboratory Studies

Tests for pregnancy and sexually transmitted diseases

One should always initially obtain a pregnancy test. This test should be mandatory in any woman of reproductive age, even at the extremes of reproductive age, who presents with abdominopelvic symptoms.

Because dysgerminoma tumors affect women of a reproductive and sexually active age, cultures for gonorrhea and chlamydia and a wet mount are recommended at the time of speculum examination, especially if patients experience abdominopelvic pain and/or fevers. In this way, sexually transmissible diseases may be detected and treated before surgery.

Once a pelvic mass is detected, especially in younger female patients, the standard workup for suspected germ cell tumors (GCTs) requires lactate dehydrogenase (LDH), alpha-fetoprotein (AFP), and beta-human chorionic gonadotropin (beta-hCG) levels.

If any levels are elevated, they may assist in diagnosis and/ or follow-up of women diagnosed with malignant ovarian GCTs.

Other laboratory studies

Dysgerminomas have most commonly been associated with elevations in LDH, although it is not elevated in all cases. Occasionally, dysgerminomas may become infiltrated with syncytiotrophoblastic giant cells, which produce beta-hCG. Elevations in AFP are even less common. However, preoperative evaluation of all of these markers is suggested in patients with suspected ovarian GCTs/dysgerminomas.

 Additionally, serum inhibin levels can be useful in this age group.

 Although inhibin B seems to be more sensitive and has a greater elevation in GCTs, inhibin A can also be elevated with inhibin B or, more rarely, without elevation of inhibin B, as sex cord stromal tumors are also in the differential for women in this age group who present with pelvic masses.

 Therefore, useful tumor markers for the workup of dysgerminomas include the following: 

• Beta-hCG
• AFP
• LDH
• Inhibin A and B
• Cancer antigen 125 (CA-125) - For epithelial tumors…”

 

5.     http://emedicine.medscape.com/article/253701-treatment

 Medical Care

A preponderance (75-80%) of dysgerminomas present as stage I cancers and, therefore, can be treated by surgical resection alone with a unilateral salpingo-oophorectomy and staging. This is preferred when attempting to preserve fertility; however, diligent follow-up care, with serial pelvic examinations and tumor markers (ie, beta-human chorionic gonadotropin [beta-hCG], alpha-fetoprotein [AFP], lactate dehydrogenase [LDH]) is mandatory if resection is the only treatment modality.

 Adjuvant therapy should be reserved for women with stage Ib-IV ovarian dysgerminomas.

 Platinum-based chemotherapy has become the standard of care for these patients and is generally well tolerated. Radiation therapy has also been administered to patients with stage I-III tumors, with excellent response rates overall. However, this has been mostly abandoned due to high success rates with platinum-based chemotherapy, as well as avoiding long-term complications from radiation, including sterility and early menopause.

Stage Ia dysgerminomas

Typically, the authors do not recommend any adjuvant chemotherapy for stage Ia dysgerminomas.

 Although 10-15% of stage Ia tumors may recur, essentially all of them are salvaged with chemotherapy.

Patients with completely resected stage Ib and Ic tumors should receive 3 cycles of BEP (bleomycin, etoposide, platinum), and those with completely resected stage II-IV tumors should receive 4 cycles of BEP.

 Patients with bulky residual disease may require additional cycles. However, care should be taken to watch for pulmonary toxicity with bleomycin and the potential for secondary leukemias with high cumulative doses of etoposide; these precautions should be discussed with the patient.

Fertility after chemotherapy

In studies of young women who received platinum-based chemotherapy for ovarian germ cell tumors (GCTs), 62 (87%) of 71 resumed regular menstrual function and 24 of these women delivered 37 children after chemotherapy. ^[3] In a study by Weinberg et al, ^[4] 40 women with malignant ovarian GCTs were followed for reproductive outcomes. Twenty-two women underwent fertility-sparing surgery and 16 of these received chemotherapy. The majority of patients (14 of 16) received BEP. Follow-up was available for 14 of the 16 patients. All 14 returned to normal menstrual function. Eight of the 10 women who attempted pregnancy had 11 pregnancies with 14 live births.

Adjuvant chemotherapy regimens

The 4 regimens for chemotherapy are as follows: (1) BEP, which is the preferred regimen; (2) methotrexate, actinomycin D, and chlorambucil (MAC); (3) cisplatin, vincristine, and bleomycin (PVB); and (4) vincristine, actinomycin D, and cyclophosphamide (VAC). Although the efficacy has been analyzed for each protocol, the BEP protocol has been favored in recent years owing to high cure rates with a favorable toxicity profile.

 Important to note is that the authors attempt to keep patients on schedule despite bone marrow toxicity that may develop. Given the high cure rates and relatively low rates of neutropenic fever, it is best to continue treatment as scheduled even if blood counts are lower than what is typically considered acceptable to begin a new cycle. Consideration for dose reduction and addition of growth factors should be made on a case-by-case basis and should be used after neutropenic fever.

BEP protocol (generally preferred)

The protocol is as follows ^[5] :

• Bleomycin - Maximum 30 U IV per week for 9 weeks; dose at 20 U/m ²
• Etoposide (ie, VP-16) - 100 mg/m ² on days 1-5 q3wk for 3 courses; reduced 20% for granulocytic fever or previous radiotherapy
• Cisplatin - 20 mg/m ² on days 1-5 q3wk for 3 courses

 In 2004, the Gynecologic Oncology Group (GOG) published their experience using carboplatin and etoposide in ovarian dysgerminomas. ^[6] They followed 39 eligible patients with completely resected stage Ib-III disease for a median follow-up of 7.8 years. There were no disease recurrences, although one patient had an intercurrent death from lung cancer. Although not considered the standard, this regimen may be considered in patients with preexisting renal or neurologic disease.

 This regimen is carboplatin t 400 mg/m² on day 1 and etoposide at 120 mg/m² on days 1-3 q4wk for 3 cycles.

Antiemetics

Antiemetics that may be used in chemotherapy are as follows:

• Chlorpromazine - 25-50 mg PO/IM/PR q4h
• Odansetron 4-8 mg IV/PO prior to chemotherapy
• Lorazepam 1-2 mg PO/IV q6h
• Dexamethasone 8 mg IV prior to cisplatin and 4 mg q4h for 2 doses

Radiation therapy

Primary therapy with radiation is reserved for patients who cannot tolerate chemotherapy or surgical resection.

 Radiation is used to treat periaortic and pelvic lymph node metastases. Shielding the remaining ovary in an attempt to preserve fertility is not uncommon. Oophoropexy may be used to mechanically hold the remaining ovary away from the radiation field.

 Radiation therapy may be used for any dysgerminomas staged Ib-III. The field of exposure extends from T11 to L5, with shielding of the contralateral ovary and the femur head.

 The use of radiation in stage Ia cancers is considered precautionary. Most patients present with stage I disease and usually can be treated with simple resection (eg, unilateral salpingo-oophorectomy).

 Some authors have advocated radiation therapy for stage IA tumors larger than 10 cm. However, owing to the high sensitivity for radiation and platinum-based chemotherapy, the authors do not recommend treating stage Ia tumors with any adjuvant therapy.

De Palo, ^[7] Freed, and Lawson developed the 3 major radiation therapy protocols. These protocols differ mainly in their treatment of the abdomen for node-positive disease and in prophylactic treatment of the mediastinum…”

 

_____________________Referenced article should be read in its entirety_________

BubMunkeyBles

I agree with seeing a GYN/ONC

I agree with seeing a GYN/ONC. Both of my GYN/ONC have had to look up treatment for this type of cancer as dysgerminoma only makes up about 2% of all ovarian cancers. I got it in 2009 and just had a recurrence 8 years later (pretty much unheard of, especially after doing chemo). I'm starting BEP chemo in the next couple weeks (did EP with carboplatin and etoposide first time). Your GYN/ONC should be able to answer any concerns and if they can't they have lots of resources to ask. It's thankfully almost always a very treatable cancer but the surveillance period is super important and after having a reoccurence so late I'm so glad my primary doctor listened to my concerns I was having.