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  • 1

    Yolk Sac Surprise!Ovarian and Extraovarian Pelvic Yolk Sac Tumors in

    WomenCharles Zaloudek, M.D.

    Professor, Department of PathologyUniversity of California, San Francisco

    505 Parnassus Ave., M563San Francisco, CA 94143

    charles.zaloudek@ucsf.edu

    Malignant Germ Cell Tumors in Females

    9 SEER Registries, 1973-2002

    Tumor N %Immature Teratoma 449 35Malignancy in Benign Teratoma 37 3Dysgerminoma 414 33Yolk Sac Tumor 183 15Embryonal Carcinoma 52 4Choriocarcinoma 27 2Mixed Germ Cell Tumor 100 8

    Obstet Gynecol 2006;107:1075-1085

    Yolk Sac Tumor Most occur in girls and young women; average

    age 19 Rare cases in elderly women associated with

    endometrioid carcinoma Clinical presentation: abdominal pain, palpable

    mass Stage

    70% stage I (none bilateral) 30% stage > I (peritoneum, distant)

    Differentiates toward yolk sac or vitelline structures secretes -fetoprotein, AAT

    Yolk Sac Tumor

  • 2

    Yolk Sac Tumor

    Yolk Sac Tumor Histologic Patterns

    Microcystic (aka Reticular)Macrocystic

    SolidEndodermal sinus (aka festoon)

    PapillaryGlandular/alveolar (including intestinal,

    endometrioid)Polyvesicular vitelline

    Myxoid (aka myxomatous)Parietal

    Hepatoid

    Embryological Classification of Yolk Sac TumorHistologic patterns correspond to types of

    differentiation of primitive endoderm

    Site Tissue PatternExtraembryonal endoderm

    Primary endoderm and secondary yolk sac

    MicrocysticSolidEndodermal sinus

    Allantois PVVMurine parietal yolk sac

    Parietal

    Somatic endoderm

    Primitive intestine and lung

    GlandularYolk sac tumor - reticular

    pattern

  • 3

    Yolk sac tumor - solid pattern

    Yolk sac tumor - yolk sac, or festoon pattern

    Yolk Sac Tumor Schiller Duval Bodies

    PASD

  • 4

    Papillary YST

    Glandular-alveolar pattern

    Yolk sac tumor - polyvesicular vitelline pattern

  • 5

    Myxoid YST Basement membrane material - Parietal pattern

    Yolk Sac TumorSurvival Rates

    Stage Pre-ChemoModernChemo

    I 16% 100%

    II-IV 8% > 60%

    Yolk Sac Tumor Immunohistochemistry

    Stain ResultCytokeratin AE1/AE3 + CytoplasmEMA, CK7 -OCT4, NANOG -SALL4 +Glypican 3 +Alpha-fetoprotein +CD30 -CD117 (c-kit) -D2-40, PLAP - or minimal

  • 6

    CKAE1/AE3

    -fetoprotein

    Glypican-3 SALL4

  • 7

    Yolk Sac TumorUCSF Staining Panel

    Stain Expected ResultCytokeratin AE1/AE3 + CytoplasmicEMA -CK7 -SALL4 +OCT4 -Glypican 3 +Alpha-fetoprotein +

    Yolk Sac TumorDifferential Diagnosis

    Clear cell carcinoma Endometrioid adenocarcinoma Hepatoid carcinoma Juvenile granulosa cell tumor Embryonal carcinoma Polyembryoma

    Mesonephroma

    Yolk Sac Tumor Clear Cell Carcinoma

    Gonocytoma; homologous ovarian and testicular tumours. 1. With discussion of mesonephroma ovarii (Schiller: Am. J. Cancer, 1939) Teilum, G. Acta Pathol Microbiol Scand 1946;23:243-251.

    Mesonephroma of the ovary: tumor of Mlleriannature related to endometrioid carcinoma. Scully RE, Barlow JF. Cancer 20: 1405-1417,1967.

  • 8

    CK 7 Clear Cell Carcinoma vs YST

    CK CK7 EMASALL

    4GLY3 AFP

    CCCa + + + - - -

    YST + - - + + +

    Yolk Sac Tumors in Older Women

    Ovarian epithelial tumors with a yolk sac tumor component

    First described by Rutgers and Scully Most patients postmenopausal Most carcinomas endometrioid, but

    carcinosarcoma also described Typical yolk sac tumor patterns Usual immunohistochemical findings in

    the two components Poor response to chemotherapy

    Nogales FF et al. Am J Surg Pathol 1996;20:1056-1066

    Our Case

    59 year old postmenopausal woman G4P4

    Abdominal distention, pressure. Various GI symptoms CT scan showed L adnexal mass

    suggestive of malignancy CA125 elevated at 531 IU/ml

  • 9

    L

    Solid left adnexal mass with at least 4 implants.

    Surgery

    2 liters of hemorrhagic ascites removed 10 cm extensively necrotic tumor resected

    from region of left adnexa; grew to involve the anterior abdominal wall, left side of uterus, anterior aspect of the bladder, the sigmoid colon, the cul de sac and the small bowel mesentery

    Did not involve the ovary or fallopian tube

  • 10

    TIC

    p53

    Clear cell carcinoma, fallopian tube

    Left pelvic tumor, with clear cells

  • 11

    Hyaline globules HNF-1 positive

    What we learned later

    Patient had an elevated alpha-fetoprotein

    Additional immunostains performed

    Yolk Sac TumorUCSF Staining Panel

    Stain Result in CaseCytokeratin AE1/AE3 + CytoplasmicEMA -CK7 -SALL4 +OCT4 -Glypican 3 +Alpha-fetoprotein +HNF 1- +

    Revised Diagnosis for Pelvic Tumor

    Extraovarian Yolk Sac Tumor

  • 12

    What did we learn from this case?

    Yolk sac tumors, in or out of the ovary, can occur in older women, and the diagnosis should not be dismissed based only on the patients age without performing appropriate stains

    Hepatocyte nuclear factor 1 staining cannot be used to differentiate between yolk sac tumor and clear cell carcinoma

    We have now stained about 20 yolk sac tumors for HNF-1and all have been positive except one, which also did not stain for AFP

    HNF-1 is a good marker for yolk sac tumor, helpful for identifying it and differentiating it from embryonal carcinoma and dysgerminoma, which are HNF-1-

    HNF-1 positive in yolk sac tumors

    HNF 1 Staining in Germ Cell Tumors

    Yolk Sac Tumor +

    Embryonal carcinoma

    -

    Dysgerminoma -

    Teratoma+ in immature and

    some mature endodermal glands

    Immunohistochemistry in Yolk Sac Tumor Differential Diagnosis

    Stain Embryonal CA YST Dysgerminoma

    Cytokeratin + Membrane + Cytoplasm -EMA - - -OCT4, NANOG + - +SOX2 + - -SALL4 + + +HNF 1 - + -AFP -/+ + -Glypican 3 -/+ + -CD30 + - -CD117, D2-40 - - +

  • 13

    HNF-1 should be used with caution pending better delineation of its staining pattern

    Did this tumor originate from the fallopian tube carcinoma should it be viewed as a combined clear cell carcinoma/yolk sac tumor?

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