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Chapter 12

Chapter 12. Germ cell, stromal, and Other ovarian tumors. Germ cell tumors. Classification. Clinical profile. Relatively small proportion of all ovarian tumor(~20%) Occur in young women Recent developments of in chemotherapy -> prognosis 변화 Initial symptoms. Dysgerminoma.

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Chapter 12

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  1. Chapter 12 Germ cell, stromal, and Other ovarian tumors

  2. Germ cell tumors • Classification

  3. Clinical profile • Relatively small proportion of all ovarian tumor(~20%) • Occur in young women • Recent developments of in chemotherapy -> prognosis 변화 • Initial symptoms

  4. Dysgerminoma • Uncommon tumor - 1-2% of primary ovarian neoplasms - 3-5% of ovarian malignancies • Occur at any age from infancy to old age : 7 Mo-70years - most cases occur in adolescence and early adulthood • Present at a relatively early stage - Ia 65-75% - Ib 10-15% - II+III 15% - IV 5%

  5. Stroma • - infiltrated with lymphocytes • - often contains granulomas similar to sarcoid • - occasionally, contains isolated gonadotropin producing syncytiotrophoblastic giant cells

  6. Gross finding - firm or flesh and cream colored or pale tan - both external and its cut surfaces may be lobulated

  7. Sexually abnormal females : a few dysgerminoma • - pure or mixed gonadal dysgenesis or testicular feminization. • - develops in a previously existing gonadoblastoma • Symptoms • - not distinctive • - similar to other solid ovarian neoplasms • - duration of Sx : usually short • - abdominal enlargement and presence of a mass in the lower abdomen(m/c) • One of the two most common malignant ovarian neoplasm in pregnancy • - serous cystadenoca. of low maliganant potential • - nonspecific finding • - relates to the age rather than to the pregnant states

  8. the opposite ovary may be involved with the tumor process - only germ cell tumor (10-15%) • Metastatic spread - lymphatic spread - hematogenous spread : occur later - any organ can affected : liver, lungs, bone

  9. Treatment • Historically, surgery followed by radiation • Recently, the use of multiple-agent chemotherapy • Chemotherapy • Recently appears to have become the treatment of choice • Success rate : as good as that of radiation • Preservation of fertility • used more frequently with significant success in patients who advanced disease • Optimal drug combination : not yet been determined • Doxorubicin and cyclophosphamide orcisplatin, vinblastin, and bleomycin (VBP) • Today, the treatment of choice after surgery in patients with advanced disease

  10. Conservative management - in the young woman with a unilateral encapsulated tumor - who desirous of future childbearing • Conservative surgery without radiation in stage Ia results

  11. In patient whom unilateral salpingo-oophoretomy is performed • careful inspection of the other ovary • rule out disseminated disease • assessment of the retroperitoneal lymph nodes • should be followed closely and have periodic examinations : 90% of recurrences appear in the first 2 years • most recurrences can be successfully eradicated by radiation therapy or chemotherapy • Several prognostic factors • Tumor size (>10cm) • Patient age : older than 40 years or younger than 20 years • Presence of other germ cell elements

  12. Report • Schwartz : 4 patients with metastasis to the contralateral ovary and preservation of ovary with subsequent CTx • All patients were alive and had no disease 14-56 Mo after diagnosis • De palo …. : 56 patients who had pure dysgerminomas - 5 year relapse-free survival rates • Stage Ia, Ib, Ic : 91% • Stage III retorperitoneal disease :74% • Stage III peritoneal disease : 24% • peritoneal involvement of any kind was associated a poor prognosis

  13. Recurrences • Should be treated aggressively with reexploration and tumor reduction • Removed tissue : examined carefully for evidence of germ cell elements other than dysgerminoma • Tx of Dysgerminoma associated with gonadoblastoma • Radical because of the frequent occurrence of bilateral tumors and the absence of normal gonadal function • Investigation of the genotypes and karyotypes of all patients • Preserve the uterus : IVF can be utilized • Tx of dysgerminoma contain small areas of more malignant histology(embryonal ca or EST) • The prognosis and therapy are determined by the more malignant germ cell elements

  14. Endodermal Sinus Tumor (Yolk Sac Tumor) • second most common form of malignant germ cell tumor • 22% of germ cell lesions • ¾ of the patients : combination of abdominal pain and abdominal or pelvic mass • Median age : 19years • Sx • Acute pain : torsion • Almost always unilateral • Size : 10-30 cm

  15. Cut surface : gray yellow with areas of hemorrhage and cystic gelatinous changes • Consist of • scattered tubules or spaces lined by single layers of flattened cuboidal cells • loose reticular stroma • Numerous scattered para-amnosalicylic-positive globules • Characteristic invaginated papillary structure with a central blood vessel

  16. Highly malignant • Metastasize early and invade the surrounding structures • Intraabdominal spread : extensive involvement of abdominal structures with tumor deposits • Metastases also occur via the lymphatic system • AFP level are often elevation • Characterized by extreme rapid growth and extensive intra-abdominal spread

  17. prognosis • Historically : Unfavorable, died within 12-18MO of diagnosis Treatment • In the past : frustrating • Kurman and Norris : no logn-term survirors in 17 pts with stage I tumor who were receiving adjuvant RTx and single alkylating agents, dactinomycin or MTX • Gallion : review the literature in 1979 - only 27% of 96 patients with stage I EST were alive at 2 years • not sensitive to radiation therapy • Optimal surgical extirpation of the disease : advocated

  18. In later years : optimistic reports of sustained remission in some pts treated by surgery and multiple agent chemotherapy

  19. GOG : VAC, pure EST 31 pts • 15(48%) failed, including 11 of 24(46%) who had complete resection • GOG : VAC, mixed germ cell tumors containing EST 15 pts • 8(53%) failed • GOG : VAC, stage I-III completely resected EST 48 pts • 35(73%) pts were free of disease with a median follow time of 4 years • Gershenson and associates : VAC, pure EST • 18 of 26(69%) pts were free • Gallion and associates : VAC, stage I disease • 17 of 25(68%) pts were alive and well 2 years or more after • Sessa… : VBP, pure EST • 13 pts, unilateral oophorectomy(12) • All received VBP and are alive at 20 months to 6 years

  20. Schwartz …: used VAC for stage I disease but prefer VBP for stage II-IV • 12 of 15 pts are alive and have no evidence of disease. • treat at least one course beyond a normal AFP titer • one recurrence was treated successfully with BEP • GOG : evaluated VBP in stage III and IV • 16 of 29(55%) ESTs were long-term disease-free survivor • Induced a substantial number of durable complete responses, even in Pts with prior CTx • Toxicity was significant • Smith….: disease were resistant to MAC and VBP • complete remission with VP-16 and cisplatin contained regimen • Williams • In disseminated germ cell tumors, BEP was more effective and had less neuromuscular toxicity than had VBP

  21. Williams : reported the GOG experience • 93 patients who were given BEP postoperatively in an adjuvant setting for malignant germ cell cancers of the ovary • Immature teratomas(42), EST(25), mixed germ cell tumors(24) • 91 of 93 had no evidence of disease after three course of BEP with median follow-up of 39 months • Dimopoulos • 40 pts with nondysgerminomatous tumor were treated BEP or PVB • Median follow-up of 39 months • 5 pts developed progressive disease and died • Fujita • 41 pts with EST, either pure or mixed • More aggressive surgery did not increase survival • Survival was similar whether VAC or PBV • All stage I pts given either VAC or PBV following surgery survived without evidence of recurrence

  22. Serum AFP • Useful diagnostic tool in patients who have EST • Ideal tumor marker • Can be useful when monitoring the results of therapy and for detecting metastasis and recurrences after therapy • Levels of hCG and its b subunit • Normal

  23. Embryonal carcinoma • One of the most malignant cancer in the ovary • Only 4% of the malignant ovarian germ cell tumors • A mean age : 15 years • More than half of the pts have hormonal abnormalities • Precocious puberty, irregular uterine bleeding, amenorrhea, or hirsutism

  24. Consist of large primitive cells with occasional papillary or gland-like formations(f12-7) • The cell have eosinophilic cytoplasm with distinct borders and round nuclei with prominent nucleoli • Numerous mitotic figures, many atypical multinucleated giant cells

  25. EST vs Embryonal ca • Contain hCG, syncytiotrophoblast-like cells and AFP in the large primitive cells • Kurman and Norris • Actual survival rate of 30% for the entire group • Stage I tumor, survival rate was 50% • With modern therapy, survival rates should greatly improved

  26. Optimal therapy • Not yet established • Probably similar to that for EST • VAC • Definitely active in this disease • Not appear to be as reliable for advanced cases as VBP • VBP • Can be used at that point in the hope of salvage • Total number of courses of VAC therapy • Really not known • GOG : evaluated the effectiveness of the VBP in stage III, IV, recurrent malignant germ cell tumors • Overall progression-free interval at 24 months : 55% • hCG and AFP : progression monitor

  27. Polyembryoma • Rare ovarian germ cell neoplasm • In most cases, associated with other neoplastic germ cell elements(immature teratoma) • Highly malignant germ cell neoplasm • Invasion of adjacent structures and organs • Not sensitive to RTx • Response to CTx : unknown

  28. Choriocarcinoma • Rare • Highly malignant tumor • May be associated with sexual precocity • Arise in one of three ways • Primary gestational choriocarcinoma associated with ovarian pregnancy • Metastatic choriocarcinoma form a primary gestational choriocarinoma arising in other parts • A germ cell tumor differentiating in the direction of trophoblastic structures • In the most cases, the tumor is admixed with other neoplastic germ cell elments • Occurs in children and young adults

  29. Secrete hCG • Precocious puberty • Mammary development, growth of pubic and axillary hair, and uterine bleeding • Adult patients : signs of ectopic pregnancies • Associated with an increased production of hCG • Estimation of urinary or plasma hCG levels • useful diagnostic test • Prognosis • Historically : unfavorable • Modern CTx regimen : effective • Creasman….: MAC • Prolonged remissions • Combination CTx using MTX or dactinomycin and alkylating agent

  30. Mixed Germ Cell Tumors • Contain at least two malignant germ cell elements • Dysgerminoma(m/c, 65-80%) • MD anderson Hospital • 9 pts(surgery alone), 6 pts(RTx) : all recurrences • Of 17 pts (VAC) : 9 were placed into remission • 5 pts (VBP after surgery) : 4 are alive and well • 20 pts are alive and well • 14 pts who had stage I disease and were treated with combination CTx after surgery : 11(79%) survived • Creasman… : treated 5 pts stage I lesion, MAC • 3 pts also received RTx • All 5 were long-term survivors • GOG • 10 completely resected tumor, VAC : 7 are long-term survivors • 5 incompletely resected disease, VAC : 4 recurrence

  31. Schwartz • 8 pts, VAC • 7 long-term survivors

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