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Novak 33. Ovarian ca

Novak 33. Ovarian ca. Nonepithelial Ovarian Cancers 부산백병원 산부인과 R2 박영미. Nonepithelial Ovarian Cancers . Compared with epithelial ovarian cancers, other malignant tumors of the ovary are uncommon about 10% of all ovarian cancers 1. malinancies of germ cell origin,

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Novak 33. Ovarian ca

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  1. Novak 33. Ovarian ca Nonepithelial Ovarian Cancers 부산백병원 산부인과 R2 박영미

  2. Nonepithelial Ovarian Cancers • Compared with epithelial ovarian cancers, other malignant tumors of the ovary are uncommon • about 10% of all ovarian cancers 1. malinancies of germ cell origin, sex cord-stromal cell origin, 2. metastatic carcinoma to ovary 3. extremely rare ovarian cancers (sarcoma, lipoid cell tumors)

  3. Germ-Cell Malignancies

  4. Germ-Cell Malignancies

  5. Germ-Cell Malignancies ◆ Serum tumor markers in malignant germ cell tumors :can be clinically useful in the diagnosis of a pelvic mass and in monitoring course of patient after surgery • α-fetoprotein(AFP), human chorionic gonadotropin(hCG) : secreted by germ cell malignancy • Placental alkaline phosphatase (PLAP), lactate dehydrogenase(LDH) : produced by dysgerminoma

  6. Germ-Cell Malignancies ◆Symptoms • Germ-cell malignancies grow rapidly, in contrast to the relatively slowgrowing epithelial ovarian tumors : often are characterized by subacute pelvic pain related to capsular distension, hemorrhage, or necrosis : may produce pressure symptoms on the bladder or rectum

  7. Germ-Cell Malignancies • In menarchal patients, menstrual irregularities may also occur • Some young patients may misinterpret the early symptoms of a neoplasm as pregnancy --> This can lead to a delay in the diagnosis • Acute symptoms associated with torsion or rupture of the adnexa • In more advanced cases, • ascites and abdominal distension may develop

  8. Germ-Cell Malignancies ◆ Diagnosis • Adnexal masses will usually require surgical exploration • 2 cm or larger in premenarchal patient • 8 cm or larger in other premennopausal patient • Predominantly cystic lesions up to 8cm in diameters in postmenarcheal patients : may be observed or given oral contraceptives for two menstrual cycles

  9. Germ-Cell Malignancies • For young patient • Blood test - serum hCG and AFP titers, CBC, LFT • Chest x-ray - evaluation for metastasis to the lung or mediastinum • Karyotype - preoperatively for all premenarcheal girl because of the propensity of these tumors to arise in dysgenetic gonads • CT or MRI - may document retroperitoneal lymphadenopathy or liver metastases but, such expensive and time- consuming evaluation is unnecessary because the patients require surgical exploration

  10. Dysgerminoma ◆ Clinical Characteristics • The most common malignant germ-cell tumor (30-40%) • 1-3% of all ovarian cancer • 5-10% of ovarian cancers in patients younger than 20 years of age * 5% : before the age of 10 years, * 75% : between the ages of 10 and 30 years * rarely occur after 50 years of age

  11. Dysgerminoma • 20-30% of ovarian malignancies associated with pregnancy are dysgerminomas • Found in both sexes and may arise in gonadal or extragonadal sites • Size varies widely, but usually 5-15cm in diameter • Capsule is slightly bosselated, the cut surface consistency is spongy, the color is gray-brown

  12. Dysgerminoma • The histologic characteristics • The large round, ovoid, or polygonal cells • abundant, clear, very pale staining cytoplasm, large and irregular nuclei, and prominent nucleoli

  13. Dysgerminoma • Approximately 5% of dysgerminomas are discovered in phenotypic females with abnormal gonads. • pure gonadal dysgenesis (46XY, bilateral streak gonads), • mixed gonadal dysgenesis (45X/46XY, unilateral streak gonad, contralateral testis), • androgen insensitivity syndrome (46XY, testicular feminization) ∴the karyotype should be determined in premenarcheal patients with a pelvic mass

  14. Dysgerminoma • About 75% of dysgerminomas are stage I at diagnosis : 85-90 % are confined to one ovary : 10-15% are bilateral • Dysgerminoama is the only germ-cell malignancy that has this significant rate of bilaterality • Other germ-cell tumor are rarely bilateral • Contralateral ovary has been preserved : diseases can develop in 5-10% of the retained gonads over next 2 years

  15. Dysgerminoma • In the 25% of patients who present with metastatic disease • the tumor most commonly spreads via lymphatic system • Metastases to the lungs, liver, brain : with long standing or recurrent disease • Metastasis to the mediastinum and supraclavicular lymph nodes : a late manifestation of disease

  16. Dysgerminoma ◆ Treatment • The treatment of early dysgerminoma : primarily surgical, including resection of the primary lesion and proper surgical staging • Chemotherapy or radiation is administered to patients with metastatic disease • Special consideration must be given to the preservation of fertility • because the disease principally affects girls and young women

  17. Dysgerminoma • Surgery • Minimum operation : unilateral oophorectomy • If there is a desire to preserve fertility, even in the presence of metastatic disease : contralateral ovary, fallopian tube, and uterus should be left in situ : because of the sensitivity of the tumor to chemotherapy • If fertility need not be preserved : TAH and BSO with advanced disease

  18. Dysgerminoma • Karyotype analysis reveals a Y chromosome --> both ovaries should be removed • Dysgerminoma is the only germ-cell tumor that tends to be bilateral --> bisection of the contralateral ovary and excisional biopsy of any suspicious lesion • If a small contralateral tumor is found --> resect it and preserve some normal ovay

  19. Dysgerminoma • Radiation • Dysgerminoma are very sensitive to radiation therapy • Doses of 2500-3500cGy may be curative • Loss of fertility is a problem • radiation should rarely be used as first-line treatment

  20. Dysgerminoma • Chemotherapy • Metastatic dysgerminomas with systemic chemotherapy • the treatment of choice • preservation of fertility • The most frequently used regimens • BEP (bleomycin, etoposide, cisplatin) • VBP (vinblastine, bleomycin, cisplatin) • VAC (vincristine, actinomycin, cyclophosphamide)

  21. Dysgerminoma • Cysplatin-based combination chemoTx • Advanced stage, incompletely resected dysgerminoma have an excellent prognosis • The best regimen : Four cycles of BEP • Macroscopic disease has all been resected at the primary operation -> No need to perform a second-look laparotomy • Extensive macorscopic residual disease at the start of chemotherapy -> a second-look operation could be used • effective second-line therapy is available • the earlier persistent disease is identified, the better the prognosis

  22. Dysgerminoma

  23. Dysgerminoma ◆ Recurrent Disease • About 75% of recurrences occur within the first year after initial treatment • most common site : peritoneal cavity, retroperitoneal LN • Patients with recurrent disease who have had no therapy other than surgery -> should be treated with chemotherapy

  24. Dysgerminoma • If prior chemotharapy with BEP regimen • POMB-ACE may used • Vincristine, bleomycin, cisplatin, etoposide, actinomycin D, cyclophosphamide • The use of high-dose chemotherapy • Carboplatin, etoposide • Radiation therapy is effective for this disease ; major disadvantage is loss of fertility

  25. Dysgerminoma ◆ Pregnancy • Dysgerminomas tend to occur in young patient -> may coexist with pregnancy • Stage Ia • the tumor can be removed intact & the pregnancy continued • More advanced diseases • continuation of the pregnancy depend on gestational age • Chemotherapy • in 2nd & 3rd trimester • in the same dosages as given for the nonpregnant patients • without apparent detriment to the fetus

  26. Dysgerminoma ◆ Prognosis • Stage Ia(unilateral encapsulated dysgerminoma) -> unilateral oophorectomy alone : 5yr disease-free survival rate of greater than 95% • Higher tendency to recurrence • lesions larger than 10-15 cm in diameter • age younger than 20 years • a microscopic pattern that includes numerous mitoses, anaplasia, medullary pattern

  27. Dysgerminoma • In the past, Surgery for advanced disease followed by pelvic and abdominal radiation • resulted in a 5-year survival rate of 63-83%, • Now With the use of VBP or BEP combination chemotherapy • cure rates of 85-90% for this same group

  28. Immature teratomas • Fewer than 1% of all ovarian cancer • 2nd most common germ-cell malignancy • 10-20% of all ovarian malignancy in younger than 20 years ago of age • 30% of deaths from ovarian cancer in younger than 20 years ago of age • About 50% of pure immature teratoma in women between 10 and 20 years • Rarely occur postmenopausal women

  29. Immature teratomas - Pathology – • Neural tissues : demonstrate most clearly the importance of the ability to mature • Immature teratomas are classified : according to a grading system based on the degree of differentiation and the quantity of immature neural tissue • Grade 1 tumor : <1 LPF contains immature neural elements • Grade 2 tumor : 1-3 LPFs with immature elements • Grade 3 tumor : >3 LPFs with these elements

  30. Immature teratomas • The prognosis can be correlated with the grade of these immature neural elements • With a higher grade, there is a poorer prognosis • Malignant change in benign cystic teratomas • occuring in 1-2% of cases • usually after the 40 years of age

  31. Immature teratomas - Diagnosis - • Some of these lesions will contain calcification similar to mature teratomas • Calcification can be detected by an x-ray of the abdomen or by ultrasonography • Tumor markers are negative unless a mixed germ-cell tumor is present

  32. Immature teratomas - Treatment - • Surgery • For premenopausal patient, confined to a single ovary : unilateral ooporectomy and surgical staging • For postmenopausal patients : TAH and BSO • Contralateral involvement is rare and routine resection or wedge biopsy of the contralateral ovary is unnecessary

  33. Immature teratomas • Chemotharapy • Stage Ia, grade 1 : an excellent prognosis : no adjuvant therapy is required • Stage Ia,grade 2 or 3 : adjuvant chemotherapy should be used • Ascites : Chemotherapy is also indicated regardless of tumor grade

  34. Immature teratomas • Regimen : VAC (most frequently used in the past) :BEP, VBP(the newer approach) • the BEP regimen is superior to the VAC regimen in the treatment of completely resected nondysgerminomatous germ cells tumors of the ovary • The switch from VBP to BEP • replacement of vinblastine with etoposide • a better therapeutic index, especially less neurologic and gastrointestinal toxicity

  35. Immature teratomas • Radiation therapy • Generally not used in the primary treatment • No evidence that the combination of chemotherapy and radiation has a higher rate of disease control than chemotherapy alone • For patients with localized persistent disease after chemotherapy

  36. Immature teratomas - Second-Look Laparotomy - • The need for second-look operation has been questioned • It seems not to be justified in patient who have received chemotherapy in an adjuvant setting, because chemotherapy in these patients is so effective • Sampling of any peritoneal lesions and the retroperitoneal lymph nodes • Only mature elements : chemoTx should be discontinued • Persistent immature elements : alternative chemoTx

  37. Immature teratomas - Prognosis - • The most important prognostic feature : the grade of the lesion • the stage of disease and the extent of tumor at the initiation of treatment -> have an impact on the curability • the 5-year survival rate • all stage : 70-80% • surgical stage I : 90-95 % • The 5yr survival rates for all stages with grade 1, 2, 3 : 82%, 62%, 30%

  38. Endodermal Sinus Tumor • Yolk sac carcinoma • 3rd most frequent malignant germ-cell tumor • Median age ; 16-18 year • Abdominal or pelvic pain • The most frequent initial symptom • about 75% • Asymptomatic pelvic mass • In 10%

  39. Endodermal Sinus Tumor - Pathology - • The gross : soft grayish-brown • Cystic areas : degeneration of the rapidly growing lesions • The capsule is intact • Unilateral in 100% • Biopsy of the opposite ovary in such young patients is contraindicated

  40. Endodermal Sinus Tumor • Schill-Duval body • Microscopically, the characteristic feature • The cystic space is lined with a layer of flattened or irregular endothelium • into which projects a glomerulus-like tuft with a central vascular core

  41. Endodermal Sinus Tumor • Most EST secrete AFP • There is a good correlation between the extent of disease and the level of AFP • AFP is useful in monitoring the patient's response to treatment

  42. Endodermal Sinus Tumor - Treatment - • Surgery • Unilateral salpingo-oophorectomy and a frozen section for diagnosis • Any gross metastases should be removed • Surgical staging is not indicated • All patients need chemotherapy

  43. Endodermal Sinus Tumor • The tumors tend to be solid and large • In size from 7 to 28 cm (median 15 cm) • Bilaterality is not seen • Most patients have early stage disease • Stage I : 71% • Stage II : 6% • Stage III : 23%

  44. Endodermal Sinus Tumor • Chemotherapy • All patients with EST are treated with either adjuvant or therapeutic chemotherapy • VBP regimen • more effective regimen in the treatment of measurable of incompletely resected tumor • POMB-ACE regimen • Primary therapy for patients with liver or brain metastases • Moderately myelosuppressive • the intervals between each course can be kept to a maximum of 14days (usually 9 to 11 days)

  45. Endodermal Sinus Tumor • Cisplatin-containing combination chemotherapy, BEP or POMB-ACE • primary chemotherapy for EST • 3 cycles : stage I & completely resected disease • 2 further cycles after negative tumor marker status : macroscopic residual disease before chemotherapy

  46. Endodermal Sinus Tumor - Second-Look Laparotomy - • The value of a second-look operation has yet to be established in patients with EST • It seems reasonable to omit the operation • Pure low stage lesions • AFP values return to normal • Remain normal for the balance of their treatment

  47. Embryonal Carcinoma • Extremely rare tumor • Distinguished from a choriocarcinoma • By the absence of syncytiotrophoblastic and cytotrophoblastic cells • The patients are very young • Between 4 and 28 years (median 14yr) • Estrogen secretion • precocious pseudopuberty • Irregular bleeding

  48. Embryonal Carcinoma • The primary lesions tend to be large • About two-thirds are confined to one ovary at the time of diagnosis • The treatment of embryonal carcinoma is the same as for the EST • Unilateral oophorectomy followed by combination chemotherapy with BEP

  49. Choriocarcinoma of the Ovary • Extremely rare tumor • The same appearance as gestational choriocarcinoma metastatic to the ovaries • Most patients are younger than 20 years • High hCG • Isosexual precocity in about 50% of patients before menarche

  50. Choriocarcinoma of the Ovary • MAC regimen : complete responses have been reported • Methotrexate • Actinomycin D • Cyclophosphamide • The prognosis has been poor • Most patients having metastases to organ parenchyma at the time of diagnosis

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