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Gynecological Cancer – 10 Uncommon Histologies

Gynecological Cancer  – 10

(All the articles published in past are available at www.shyamhemoncclinic.com/blog/)
Question: Thank you Chiragbhai for salient points about advance stages of Uterine Cancer. 1. Stage 3 and 4 require postoperative chemotherapy. 2. Stage 4 with resectable disease limited to pelvis should be operated. All stage 4 disease is not incurable. There is no role for only Debulking surgery however. 3. Sarcoma and other uncommon histologies are more aggressive and need postoperative chemotherapy even in early stages. What is the next cancer we are going to discuss?
Answer: Today we will discuss some of the uncommon histologies, as they are important in terms of very different management plan, and generally very good results.
1. Borderline epithelial tumor of ovary: These are generally seen in younger women, frequently in early stages. These look like typical ovary cancer, but do not have typical invasion on pathology examination, which is hallmark of “malignant potential”. They are very slow growing with excellent 5 year survival, and hence fertility sparing surgery is possible i.e. removing only one ovary and fallopian tube, and leaving other ovary fallopian tube and uterus intact, thus allowing child bearing. Of course with close monitoring. Post operative treatment is generally not required. Role of chemotherapy is controversial at best. On pathology evaluation, some patients have occasional peritoneal implants with focus of invasion. Some experts recommend postoperative chemotherapy for these patients, however benefit is not clear.
2. GTD (gestational trophoblastic disease): It is extremely important to consider this tumor in women of child bearing age. Since even in stage 4, even with significant metastatic disease, large majority can still be cured. But only if it is suspected, proper diagnosis and treatment will be feasible. We had a young woman, about age 25, with brain metastases. Her family doctors had written her off saying nothing is possible now, as most people think for metastatic disease, especially when patient has limited resources. Family just took a chance of last opinion. We suspected this diagnosis, a simple blood test beta HCG was done, which was extremely high. Diagnosis was confirmed and with appropriate treatment, she is cured, now several years since therapy.
Chemotherapy is also fairly simple, inexpensive and includes old well known drugs like methotrexate, cyclophoshphamide, vincristine etc.
There is a significant variation in the grade of these tumors however, from fairly benign like hydatidiform mole to gestational choriocarcinoma. Later grows exceedingly fast, and can be rapidly fatal if not treated. If treated, however has excellent outcome, that too with comparatively much less chemotherapy. Very low malignant potential tumors can be treated with only surgery. Those with malignant potential but low risk by certain criteria can be treated even with a single agent methotrexate, and high risk need combination chemotherapy.
These tumors arise from placental tissue, hence seen only in women of child bearing age.
3. Germ cell tumors of Ovary: Generally seen in young women, and essentially treated like testicular germ cell tumors. Monitoring is also done similarly using AFP, and beta HCG levels. About half are dysgerminoma. These are often limited to one ovary, and low grade. Fertility sparing surgery is possible in such cases. Higher stages, and most other histologies require chemotherapy, most commonly BEP combination (bleomycin, etoposide, cisplatin).
4. Sex cord stromal tumors of ovary: These arise from stroma. Commonest among these is Granulosa cell tumor, arising mainly after age 40, frequently early stage, operable, and with good prognosis. Less common is Sertoli-Leydig tumors, arising mainly before age 40, again early stage at diagnosis, operable, and with good prognosis. Later tumors produce androgen, hence may lead to virilization (male pattern hair growth, voice change etc).
Que: These are quite interesting, and very intriguing too. Borderline Tumor – first time I have heard something like this – neither benign nor malignant.
What about cancer of Vulva, an uncommon tumor?
Ans: Yes. An uncommon cancer, mostly squamous cell carcinoma, frequently diagnosed in early stage. Same as cervix ca, it is also associated to some extent with HPV infection, smoking, immunocompromised status. Treatment is generally surgery. In locally advance cases, chemoradiation is an option.
September 9th 2017.

Dr. Chirag A. Shah; M.D. Oncology/Hematology (USA), 079 26754001. Diplomate American Board of Oncology and Hematology. Ahmedabad. drchiragashah@gmail.com

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