Chapter 093. Gynecologic Malignancies (Part 5)

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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 Before the advent of chemotherapy, extensive surgery was routine, but it has now been replaced by careful evaluation of extent of spread, followed by resection of bulky disease and preservation of one ovary, the uterus, and the cervix, if feasible.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) 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 Before the advent of chemotherapy, extensive surgery was routine, but it has now been replaced by careful evaluation of extent of spread, followed by resection of bulky disease and preservation of one ovary, the uterus, and the cervix, if feasible.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 staging, 60–75% of women have stage I disease and 25–30% have stage III disease. Stages II and IV are infrequent.
Most of the malignant germ cell tumors are managed with chemotherapy after surgery. Regimens similar to those used in testicular cancer, such as BEP (bleomycin, etoposide, and cisplatin), with three or four courses given at 21-day intervals, 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 tumors except grade I, stage I immature teratoma, where surgery alone is adequate, and perhaps early-stage dysgerminoma, where surgery and radiation therapy are used. Dysgerminoma is the ovarian counterpart of testicular seminoma. The tumor is very sensitive to radiation therapy. The 5-year disease-free survival is 100% in early-stage patients and 61% in stage III disease. Unfortunately, the use of radiation therapy makes many patients infertile. BEP chemotherapy is equally or more effective and does not cause infertility. In incompletely resected patients with dysgerminoma treated with BEP, the 2-year disease-free survival is 95% and infertility is not observed. Combination chemotherapy (BEP) has replaced postoperative radiation therapy as the treatment of choice in women with ovarian dysgerminoma. Ovarian Stromal Tumors Stromal tumors make up
frequently in the first three decades of life. Granulosa cell tumors frequently produce estrogen and cause menstrual abnormalities, bleeding, and precocious puberty. Endometrial carcinoma can be seen in 5% of these women, perhaps related to the persistent hyperestrogenism. Sertoli and Leydig cell tumors, when functional, produce androgens with resultant virilization or hirsutism. Some 75% of these stromal cell tumors present in stage I and can be cured with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Stromal tumors generally grow slowly, and recurrences can occur 5–10 years after initial surgery; serum markers such as estradiol, inhibin, and müllerian inhibitory substance may be useful in monitoring patients. Neither radiation therapy nor chemotherapy has been documented to be consistently effective, and surgical management remains the primary treatment. Ovarian stromal and germ cell tumors are sometimes components of complex genetic syndromes. Peutz-Jeghers syndrome (mucocutaneous pigmentation and intestinal polyps) is associated with ovarian sex cord stromal tumors and Sertoli cell tumors in men. Patients with gonadal dysgenesis (46XY genotype or mosaic for Y-containing cell lines) develop gonadoblastomas, and women with nevoid basal cell carcinomas have an increased risk of ovarian fibromas. 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 cancer is 20 times more common. Approximately 300 new cases occur yearly; 90% are papillary serous adenocarcinomas, with the remainder being mixed mesodermal, endometrioid, and transitional cell tumors. BRCA1 and -2 mutations are found in 16% of cases. The gross and microscopic characteristics and the spread of tumor are similar to those of ovarian cancer but can be distinguished if the tumor arises from the endosalpinx, where the tubal epithelium shows a transition between benign and malignant, and the ovaries and endometrium are normal or minimally involved. The differential diagnosis includes primary or metastatic ovarian cancer, chronic salpingitis, tuberculous salpingitis, salpingitis isthmica nodosa, and cautery artifact. Unlike patients with ovarian cancer, patients often present with early symptoms, usually postmenopausal vaginal bleeding, pain, and leukorrhea. Surgical staging is similar to that used for ovarian cancer, and prognosis is related to stage and extent of residual disease. Patients with stages I and II disease are generally treated with surgery alone or with surgery and pelvic radiation therapy, although radiation therapy does not clearly improve 5-year survival (5-year survival, stage I: 74% vs. 75%; stage II: 43% vs. 48%). Patients with stages III and IV disease are treated with the same chemotherapy regimens used in advanced ovarian cancer; 5-year survival is similar (stage III, 20%; stage IV, 5%).