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NONEPITHELIAL OVARIAN CANCER Ovarian Germ Cell Tumors
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What percent of all ovarian neoplasms are germ cell tumors (GCTs) From what embryologic origin do ovarian GCTs (OGCTs) arise What are the OGCT subtypes 20 25% They arise from the totipotential germ cells that normally differentiate into the three germ layers Dysgerminoma; endodermal sinus tumor (yolk sac tumor); immature teratoma; mature teratoma; embryonal carcinoma; choriocarcinoma; mixed GCTs It is a disease of children and young women (usually between 10 and 30 years) Acute abdominal pain (from rupture or torsion); rapid abdominal enlargement (because of either the mass or ascites); fever; vaginal bleeding Patients usually present at Stage Ia For each of the following scenarios, identify the correct GCT: A 20-year-old woman presents with acute onset of lower abdominal pain and pelvic mass Serum tests reveal an elevated level of alpha-fetoprotein
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At what age do these malignancies occur
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What are the typical clinical symptoms and signs
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How does the time of diagnosis differ from that of EOC
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5: Gynecologic Oncology
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(AFP) Intraoperative examination reveals bilateral ovarian masses and intraperitoneal dissemination Tumor biopsy reveals Schiller-Duval bodies Endodermal sinus tumor (yolk sac tumor) These tumors are rare, found in young females They may secrete estrogen, causing precocious puberty and abnormal vaginal bleeding b-hCG is commonly elevated and serves as a tumor marker Embryonal carcinoma and choriocarcinoma A young female presents with abdominal enlargement Radiographic films reveal a unilateral mass with calcification Tumor markers are not present Histology of the removed specimen reveals the presence of a poorly differentiated tumor consisting of hair, teeth, cartilage, bone, and muscle Immature (malignant) teratoma This dysgerminoma is the female counterpart of the male seminoma and commonly occurs in adolescents and young females It is the most common of the malignant OGCTs and typically presents as a rapidly growing unilateral mass Lactic dehydrogenase (LDH) may be elevated Dysgerminoma A young female presents with tachycardia, palpitations, anxiety, and tremors The pituitary and thyroid appear normal on examination and radiographic films The patient is later found to have a mature teratoma consisting of thyroid tissue Mixed GCTs These neoplasms contain two or more germ cell elements A dysgerminoma and endodermal sinus tumor occur together most frequently LDH, AFP, and b-hCG may be elevated Struma ovarii
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Section II: Topics in Gynecology
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Which GCTs are benign What laboratory test should be ordered prior to definitive diagnosis and treatment What is the treatment plan for malignant OGCTs How are OGCTs staged How effective is chemotherapy in malignant GCTs
Gonadoblastomas and mature cystic teratomas Serum ovarian tumor marker panel for b-hCG, AFP, LDH Unilateral adnexectomy (fertility preserving) and complete surgical staging; adjuvant chemotherapy Similar to EOC staging GCTs are very chemosensitive 90% of patients with early-stage OGCTs and 80% of patients with advanced disease are long-term survivors For all resected Stage I GCTs except Stage Ia dysgerminoma and Stage Ia, grade 1 unruptured immature teratoma Bleomycin, etoposide, cisplatin is the preferred regimen
For which tumors is chemotherapy recommended
What chemotherapeutic agents are used List the chemotherapeutic agent that causes the toxicities listed below: Nephrotoxicity Pulmonary fibrosis Blood dyscrasias How do you monitor the response to chemotherapy What is the prognosis of GCTs
Cisplatin Bleomycin Etoposide Based on the physical examination and decrease in the serum tumor marker levels (if initially elevated) Much better than EOC Depending on the GCT, 5-year survival ranges from 60% to 95%
Ovarian Sex Cord-Stromal Tumors
What percent of all ovarian tumors are sex cord-stromal tumors From what embryologic origin do ovarian sex cord-stromal tumors arise What are the ovarian sex cord-stromal tumor subtypes 5 8% of all primary ovarian neoplasms They develop from the cells that surround the oocytes, including those that produce ovarian hormones Granulosa cell tumors; ovarian thecoma; ovarian fibroma; SertoliLeydig cell tumors
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5: Gynecologic Oncology
What are the typical clinical symptoms and signs
Estrogen-producing tumors may cause precocious puberty in young girls, and endometrial hyperplasia and abnormal vaginal bleeding in postmenopausal women Androgenproducing tumors cause virilization, acne, and gynecomastia Most women present with a unilateral adnexal mass between the second and third decade Virilization, hirsutism, acne, and menstrual abnormalities commonly occur Testosterone levels may be elevated Sertoli-Leydig cell tumors
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