Chapter 093. Gynecologic Malignancies (Part 5)

Malignant germ cell tumors are usually large (median—16 cm). Bilateral disease is rare except in dysgerminoma (10–15% bilaterality). Abdominal or pelvic pain in young women is the usual presenting symptom. Serum human chorionic gonadotropin (β-hCG) and α fetoprotein levels are useful in the diagnosis and management of these patients. Before the advent of chemotherapy, extensive surgery was routine, but it has now been replaced by careful evaluation of extent of spread, followed by resection of bulky disease and preservation of one ovary, the uterus, and the cervix, if feasible. This allows many affected women to preserve fertility. After surgical...
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Chapter 093. Gynecologic Malignancies (Part 5) Chapter 093. Gynecologic Malignancies (Part 5) Malignant germ cell tumors are usually large (median—16 cm). Bilateraldisease is rare except in dysgerminoma (10–15% bilaterality). Abdominal orpelvic pain in young women is the usual presenting symptom. Serum humanchorionic gonadotropin (β-hCG) and α fetoprotein levels are useful in thediagnosis and management of these patients. Before the advent of chemotherapy,extensive surgery was routine, but it has now been replaced by careful evaluationof extent of spread, followed by resection of bulky disease and preservation of oneovary, the uterus, and the cervix, if feasible. This allows many affected women topreserve fertility. After surgical staging, 60–75% of women have stage I diseaseand 25–30% have stage III disease. Stages II and IV are infrequent. Most of the malignant germ cell tumors are managed with chemotherapyafter surgery. Regimens similar to those used in testicular cancer, such as BEP(bleomycin, etoposide, and cisplatin), with three or four courses given at 21-dayintervals, have produced 95% long-term survival in patients with disease stages I–III. This regimen is the treatment of choice for all malignant germ cell tumorsexcept grade I, stage I immature teratoma, where surgery alone is adequate, andperhaps early-stage dysgerminoma, where surgery and radiation therapy are used. Dysgerminoma is the ovarian counterpart of testicular seminoma. Thetumor is very sensitive to radiation therapy. The 5-year disease-free survival is100% in early-stage patients and 61% in stage III disease. Unfortunately, the useof radiation therapy makes many patients infertile. BEP chemotherapy is equallyor more effective and does not cause infertility. In incompletely resected patientswith dysgerminoma treated with BEP, the 2-year disease-free survival is 95% andinfertility is not observed. Combination chemotherapy (BEP) has replacedpostoperative radiation therapy as the treatment of choice in women with ovariandysgerminoma. Ovarian Stromal Tumors Stromal tumors make up frequently in the first three decades of life. Granulosa cell tumors frequentlyproduce estrogen and cause menstrual abnormalities, bleeding, and precociouspuberty. Endometrial carcinoma can be seen in 5% of these women, perhapsrelated to the persistent hyperestrogenism. Sertoli and Leydig cell tumors, whenfunctional, produce androgens with resultant virilization or hirsutism. Some 75%of these stromal cell tumors present in stage I and can be cured with totalabdominal hysterectomy and bilateral salpingo-oophorectomy. Stromal tumorsgenerally grow slowly, and recurrences can occur 5–10 years after initial surgery;serum markers such as estradiol, inhibin, and müllerian inhibitory substance maybe useful in monitoring patients. Neither radiation therapy nor chemotherapy hasbeen documented to be consistently effective, and surgical management remainsthe primary treatment. Ovarian stromal and germ cell tumors are sometimes components ofcomplex genetic syndromes. Peutz-Jeghers syndrome (mucocutaneouspigmentation and intestinal polyps) is associated with ovarian sex cord stromaltumors and Sertoli cell tumors in men. Patients with gonadal dysgenesis (46XYgenotype or mosaic for Y-containing cell lines) develop gonadoblastomas, andwomen with nevoid basal cell carcinomas have an increased risk of ovarianfibromas. Carcinoma of the Fallopian Tube The fallopian tube is a very rare site of cancer in the female genital tract,although its epithelial surface far exceeds that of the ovary, where epithelial canceris 20 times more common. Approximately 300 new cases occur yearly; 90% arepapillary serous adenocarcinomas, with the remainder being mixed mesodermal,endometrioid, and transitional cell tumors. BRCA1 and -2 mutations are found in16% of cases. The gross and microscopic characteristics and the spread of tumorare similar to those of ovarian cancer but can be distinguished if the tumor arisesfrom the endosalpinx, where the tubal epithelium shows a transition betweenbenign and malignant, and the ovaries and endometrium are normal or minimallyinvolved. The differential diagnosis includes primary or metastatic ovarian cancer,chronic salpingitis, tuberculous salpingitis, salpingitis isthmica nodosa, andcautery artifact. Unlike patients with ovarian cancer, patients often present with earlysymptoms, usually postmenopausal vaginal bleeding, pain, and leukorrhea.Surgical staging is similar to that used for ovarian cancer, and prognosis is relatedto stage and extent of residual disease. Patients with stages I and II disease aregenerally treated with surgery alone or with surgery and pel ...