Dysgerminoma
# nonepithelial tumors of ovary
-ºóµµ: Àüü ³¼Ò¾ÏÀÇ 10% Á¤µµ
-Á¾·ù
1) germ cell tumor
2) sex-cord stromal tumor
3) metastatic cancer
4) sarcoma, lipoma
¥°. germ cell malignancies
-ºóµµ: ovarian tumorÀÇ ¾à 20~25%
-³¼Ò¾ÏÀÇ 5~15% Â÷Áö
-È£¹ß¿¬·É: children & °¡ÀÓ±âÀÇ ÀþÀº ¿©¼º
10¼¼ÀÌÇÏ¿¡¼ ³¼Ò Á¾¾çÀÇ 70%´Â germ cell tumor
20´ë ÀÌÈÄ¿¡´Â µå¹°´Ù
1. º´ÀÎ
-embryonic gonad¿¡ ÀÖ´Â primitive germ cell·ÎºÎÅÍ »ý°Ü³ °ÍÀ¸·Î »ý°¢
primordial germ cell
germinoma:dysgerminoma AFP(-) hCG(-) embryonal carcinoma AFP(+) hCG(+)
extraembryonic differentiation embryonic differentiation
trophoblast yolk sac immature teratoma AFP(-) hCG(-)
choriocarcinoma AFP(-) hCG(+) mature teratoma AFP(-) hCG(-)
endodermal sinus tumor AFP(+) hCG(-)
2. ºÐ·ù
-histologic typing of ovarian germ cell tumor
1) dysgerminoma
2) teratoma
A. immature teratoma
B. mature teratoma
(1) solid
(2) cystic
a. dermoid cyst: mature cystic teratoma
b. dermoid cyst with malignant transformation
C. monodermal and highly specialized
(1) struma ovarii
(2) carcinoid
(3) struma ovarii and carcinoid
(4) others
3) endodermal sinus tumor
4) embryonal carcinoma
5) polyembryoma
6) choriocarcinoma
7) gonadoblastoma
3. clinical features
1) sign & symptom
-»óÇǼ¼Æ÷ Á¾¾ç°ú ´Þ¸® »ý½Ä¼¼Æ÷Á¾Àº ÁøÇàÀÌ ºü¸£´Ù
¨ç pelvic pain ¡çcapsular distention, hemorrhage, necrosis
¨è gastrointestinal or genitourinary symptom
¨é menstrual irregularities
¨ê acute symptoms due to torsion & rupture
¨ë abdominal distention due to ascites, mass
¨ì palpable mass
2) diagnosis & management
¨ç history: age, growing rate
¨è physical examination
¨é tumor marker (AFP, hCG, CEA)
dysgerminoma(-,-,-)
immature teratoma(-,-,-)
endodermal sinus tumor(+,-,-)
embryonal carcinoma(+,+,+)
polyembryoma(+,+,-)
choriocarcinoma(-,+,-)
¨ê ultrasonography
¨ë chest PA: lung or mediastinal metastasis °¡´É¼º
¨ì CT, MRI: retroperitoneal LN metastasis
4. dysgerminoma
1) Á¤ÀÇ: early gonadal developmental stage¿¡¼ sexually undifferentiated neutral germinal tissue·ÎºÎÅÍ originÇÏ´Â tumor
2) ¿ªÇÐ ¹× Ư¡
-°¡Àå ÈçÇÑ ¡®malignant¡¯ germ cell tumor (30~40%)
-È£¹ß¿¬·É: young women (75%, under the age of 30yrs)
-ÀÓ½ÅÁß ¾Ç¼ºÁ¾¾çÀÇ 20~30%
-most radiosensitive tumor
3) pathology
-size: ÀϹÝÀûÀ¸·Î 5~15cm
-solid nature
-hyalinized connective tissue septum with lymphocyte infiltration
-PAS stain(+): cytoplasmic glycogen ¶§¹®
rim of alkaline phosphatase by histochemical techniques
syncytiotrophoblastic giant cells, hCG ºÐºñ
? may, precocious puberty or virilization
4) À°¾ÈÀû ¼Ò°ß
-´ë¸®¼® ¸ð¾ç: pink & yellow
-cut surface: solid & ¸Å²ô·¯¿î ¾ç»ó
5) clinical characteristics
-tumor of early life
-mass & pain in lower abdomen(°¡Àå ÈçÇÑ Ãʱâ Áõ»ó)
-no characteristic effect on menstruation
-phenotypically normal females
5%¿¡¼´Â phenotypic female with abnormal gonads
? Ãʰæ ÀÌÀü¿¡ °ñ¹ÝÁ¾±«°¡ °üÂûµÇ¸é ¿°»öü °Ë»ç°¡ Çʼö
46XY or 46XY pure gonadal dysgenesis
45X/46XY mixed gonadal dysgenesis
46XY androgen insensitivity syndrome
-¾çÃø¼º:10~15%
-75%´Â Áø´Ü ´ç½Ã stage ¥°
-ÀüÀ̹æ½Ä
¨ç lymphatics (most common) ? LN, mediastinum
¨è ±×¿Ü Ç÷¾×, º¹¸·ÀüÀÌ, °ñÀüÀÌ(ÇÏÃø ôÃß°ñ)
6) treatment
-ÀÌ ÁúȯÀº ¾î¸°¾ÆÀ̳ª ÀþÀº ¿©ÀÚ¿¡¼ ¹ß»ýÇϹǷΠÇâÈÄÀÇ Àӽſ¡ ´ëÇØ °í·Á
(1) localized disease
-salpingo-oophorectomy(¥°a) + focal resection and chemotherapy(¥°b)
¨ç young
¨è well encapsulated unilateral tumor and resectable metastasis or small focus in contralateral ovary
-stage¥°b or postoperative occult metastasis (+) ÀÎ °æ¿ì
? chemotherapy (BEP 4 cycles)
(2) metastatic or recurrent disease
-TAH & BSO with surgical staging and
: chemotherapy (BEP, VBP, VAC, POMB-ACE) or
: postoperative radiation (2500~3000cGy)
-recurrent cancer
: Àç¹ßȯÀÚÀÇ 75%´Â Ä¡·á½ÃÀÛ ÈÄ 1³â À̳»¿¡ Àç¹ß
: Àç¹ßÀÌ Àß µÇ´Â Àå¼Ò: º¹°³», Èĺ¹¸· ¸²ÇÁÀý
(3) ÀӽŰúÀÇ °ü·Ã
-¹ÌºÐÈ ¼¼Æ÷Á¾Àº ÀþÀº ¿©¼º¿¡¼ ¹ß»ýÇϹǷΠÀӽŰú µ¿¹ÝµÉ ¼ö ÀÖ´Ù
¨ç stage¥°a: Á¾¾ç¸¸ Á¦°Å, ÀӽŠÀ¯Áö
¨è advanced stage
: ÀÓ½ÅÁÖ¼ö¿¡ µû¶ó ÀӽŠÁö¼Ó¿©ºÎ ÆÇ´Ü
: Ç×¾ÏÁ¦ÀÇ °æ¿ì ÀӽŠÁ߱⠶Ǵ Èı⿡´Â ºñÀÓ½Å½Ã¿Í µ¿ÀÏÇÑ ¾àÁ¦, ¿ë·® Åõ¿© °¡´É
(4) ¿¹ÈÄ
-ovarian malignancy Áß °¡Àå ¿¹Èİ¡ ÁÁ´Ù
-stage¥°a: disease free 5 year survival rate´Â 95%
advanced disease: surgery with radiotherapy or chemotherapy
(80%ÀÌ»ó cure rate)
-ºÒ·®ÇÑ ¿¹Èĸ¦ º¸ÀÌ´Â °æ¿ì
¨ç mixed with other germ cell tumor
¨è large tumor (>10~15cm)
¨é young patient(<20yrs)
¨ê Á¶Á÷ÇÐÀûÀ¸·Î numerous mitotic, anaplastic, medullary pattern
-ºóµµ: Àüü ³¼Ò¾ÏÀÇ 10% Á¤µµ
-Á¾·ù
1) germ cell tumor
2) sex-cord stromal tumor
3) metastatic cancer
4) sarcoma, lipoma
¥°. germ cell malignancies
-ºóµµ: ovarian tumorÀÇ ¾à 20~25%
-³¼Ò¾ÏÀÇ 5~15% Â÷Áö
-È£¹ß¿¬·É: children & °¡ÀÓ±âÀÇ ÀþÀº ¿©¼º
10¼¼ÀÌÇÏ¿¡¼ ³¼Ò Á¾¾çÀÇ 70%´Â germ cell tumor
20´ë ÀÌÈÄ¿¡´Â µå¹°´Ù
1. º´ÀÎ
-embryonic gonad¿¡ ÀÖ´Â primitive germ cell·ÎºÎÅÍ »ý°Ü³ °ÍÀ¸·Î »ý°¢
primordial germ cell
germinoma:dysgerminoma AFP(-) hCG(-) embryonal carcinoma AFP(+) hCG(+)
extraembryonic differentiation embryonic differentiation
trophoblast yolk sac immature teratoma AFP(-) hCG(-)
choriocarcinoma AFP(-) hCG(+) mature teratoma AFP(-) hCG(-)
endodermal sinus tumor AFP(+) hCG(-)
2. ºÐ·ù
-histologic typing of ovarian germ cell tumor
1) dysgerminoma
2) teratoma
A. immature teratoma
B. mature teratoma
(1) solid
(2) cystic
a. dermoid cyst: mature cystic teratoma
b. dermoid cyst with malignant transformation
C. monodermal and highly specialized
(1) struma ovarii
(2) carcinoid
(3) struma ovarii and carcinoid
(4) others
3) endodermal sinus tumor
4) embryonal carcinoma
5) polyembryoma
6) choriocarcinoma
7) gonadoblastoma
3. clinical features
1) sign & symptom
-»óÇǼ¼Æ÷ Á¾¾ç°ú ´Þ¸® »ý½Ä¼¼Æ÷Á¾Àº ÁøÇàÀÌ ºü¸£´Ù
¨ç pelvic pain ¡çcapsular distention, hemorrhage, necrosis
¨è gastrointestinal or genitourinary symptom
¨é menstrual irregularities
¨ê acute symptoms due to torsion & rupture
¨ë abdominal distention due to ascites, mass
¨ì palpable mass
2) diagnosis & management
¨ç history: age, growing rate
¨è physical examination
¨é tumor marker (AFP, hCG, CEA)
dysgerminoma(-,-,-)
immature teratoma(-,-,-)
endodermal sinus tumor(+,-,-)
embryonal carcinoma(+,+,+)
polyembryoma(+,+,-)
choriocarcinoma(-,+,-)
¨ê ultrasonography
¨ë chest PA: lung or mediastinal metastasis °¡´É¼º
¨ì CT, MRI: retroperitoneal LN metastasis
4. dysgerminoma
1) Á¤ÀÇ: early gonadal developmental stage¿¡¼ sexually undifferentiated neutral germinal tissue·ÎºÎÅÍ originÇÏ´Â tumor
2) ¿ªÇÐ ¹× Ư¡
-°¡Àå ÈçÇÑ ¡®malignant¡¯ germ cell tumor (30~40%)
-È£¹ß¿¬·É: young women (75%, under the age of 30yrs)
-ÀÓ½ÅÁß ¾Ç¼ºÁ¾¾çÀÇ 20~30%
-most radiosensitive tumor
3) pathology
-size: ÀϹÝÀûÀ¸·Î 5~15cm
-solid nature
-hyalinized connective tissue septum with lymphocyte infiltration
-PAS stain(+): cytoplasmic glycogen ¶§¹®
rim of alkaline phosphatase by histochemical techniques
syncytiotrophoblastic giant cells, hCG ºÐºñ
? may, precocious puberty or virilization
4) À°¾ÈÀû ¼Ò°ß
-´ë¸®¼® ¸ð¾ç: pink & yellow
-cut surface: solid & ¸Å²ô·¯¿î ¾ç»ó
5) clinical characteristics
-tumor of early life
-mass & pain in lower abdomen(°¡Àå ÈçÇÑ Ãʱâ Áõ»ó)
-no characteristic effect on menstruation
-phenotypically normal females
5%¿¡¼´Â phenotypic female with abnormal gonads
? Ãʰæ ÀÌÀü¿¡ °ñ¹ÝÁ¾±«°¡ °üÂûµÇ¸é ¿°»öü °Ë»ç°¡ Çʼö
46XY or 46XY pure gonadal dysgenesis
45X/46XY mixed gonadal dysgenesis
46XY androgen insensitivity syndrome
-¾çÃø¼º:10~15%
-75%´Â Áø´Ü ´ç½Ã stage ¥°
-ÀüÀ̹æ½Ä
¨ç lymphatics (most common) ? LN, mediastinum
¨è ±×¿Ü Ç÷¾×, º¹¸·ÀüÀÌ, °ñÀüÀÌ(ÇÏÃø ôÃß°ñ)
6) treatment
-ÀÌ ÁúȯÀº ¾î¸°¾ÆÀ̳ª ÀþÀº ¿©ÀÚ¿¡¼ ¹ß»ýÇϹǷΠÇâÈÄÀÇ Àӽſ¡ ´ëÇØ °í·Á
(1) localized disease
-salpingo-oophorectomy(¥°a) + focal resection and chemotherapy(¥°b)
¨ç young
¨è well encapsulated unilateral tumor and resectable metastasis or small focus in contralateral ovary
-stage¥°b or postoperative occult metastasis (+) ÀÎ °æ¿ì
? chemotherapy (BEP 4 cycles)
(2) metastatic or recurrent disease
-TAH & BSO with surgical staging and
: chemotherapy (BEP, VBP, VAC, POMB-ACE) or
: postoperative radiation (2500~3000cGy)
-recurrent cancer
: Àç¹ßȯÀÚÀÇ 75%´Â Ä¡·á½ÃÀÛ ÈÄ 1³â À̳»¿¡ Àç¹ß
: Àç¹ßÀÌ Àß µÇ´Â Àå¼Ò: º¹°³», Èĺ¹¸· ¸²ÇÁÀý
(3) ÀӽŰúÀÇ °ü·Ã
-¹ÌºÐÈ ¼¼Æ÷Á¾Àº ÀþÀº ¿©¼º¿¡¼ ¹ß»ýÇϹǷΠÀӽŰú µ¿¹ÝµÉ ¼ö ÀÖ´Ù
¨ç stage¥°a: Á¾¾ç¸¸ Á¦°Å, ÀӽŠÀ¯Áö
¨è advanced stage
: ÀÓ½ÅÁÖ¼ö¿¡ µû¶ó ÀӽŠÁö¼Ó¿©ºÎ ÆÇ´Ü
: Ç×¾ÏÁ¦ÀÇ °æ¿ì ÀӽŠÁ߱⠶Ǵ Èı⿡´Â ºñÀÓ½Å½Ã¿Í µ¿ÀÏÇÑ ¾àÁ¦, ¿ë·® Åõ¿© °¡´É
(4) ¿¹ÈÄ
-ovarian malignancy Áß °¡Àå ¿¹Èİ¡ ÁÁ´Ù
-stage¥°a: disease free 5 year survival rate´Â 95%
advanced disease: surgery with radiotherapy or chemotherapy
(80%ÀÌ»ó cure rate)
-ºÒ·®ÇÑ ¿¹Èĸ¦ º¸ÀÌ´Â °æ¿ì
¨ç mixed with other germ cell tumor
¨è large tumor (>10~15cm)
¨é young patient(<20yrs)
¨ê Á¶Á÷ÇÐÀûÀ¸·Î numerous mitotic, anaplastic, medullary pattern