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Germ Cell Tumors ) Systemicist Pathology.. Lecture # 13 Title : FGT 5 ( Germ Cell Tumors ) Done by: Dema Mhmd Khdier A man may die, nations may rise and fall…….But an idea lives on Teratoma :tumor contain fully developed tissues and organs, including hair, teeth, muscle, and bone. Germ Cell Tumors 1)Teratoma _ Immature teratoma _ Mature teratoma : ** Cystic (dermoid cyst) ** Solid ** Monodermal teratoma 2) Dysgerminoma 3) Yolk sac tumor 4) Choriocarcinoma 5) Embryonal carcinoma 6) Mixed germ cell tumors ~Teratoma _15% to 20% of ovarian tumors _ in the first two decades of life _ Young age ↑ incidence of malignancy _ > 90% are benign mature cystic teratomas. Benign Mature Cystic Teratomas (Dermoid Cysts ) _Most common ovarian tumors in childhood _90% are unilateral, more on the right. Complications: 1) In 1%, malignant transformation of one of the tissue elements, usually SCC. 2)10-15% undergo torsion due to long pedicle. Torsion: twisting around,,, may cause obstruction and abdominal pain Gross: _Multiloculated cyst filled with sebum & matted hair. _Teeth protruding from a nodular projection. _ Occasionally foci of bone and cartilage. Microscopic : _Mature tissues representing all three germ cell layers. _A cyst lined by epidermal type epithelium with adnexal appendages. Monodermal _Specialized _ teratoma _Usually solid and unilateral (one type of tissues ) *Struma ovarii _Composed of mature thyroid tissue. _ May produce hyperthyroidism. _Thyroid tumors may arise . *Ovarian carcinoid : Rarely produce carcinoid syndrome. ***Combined struma ovarii and carcinoid ~ Metastasis to Ovary Formation of fibrosis , 1)older ages 2) bilateral and multinodular 3) solid gray-white masses collagen around tumor 4)Malignant tumor cells arranged into cords and glands in a desmoplastic stroma cells 5) Cells may be "signet-ring" mucin-secreting 6) Primaries: GI (Krukenberg tumors), breast, lung. Placenta is the place where gas & nutrients exchange Diseases of the Placenta 1)Ectopic pregnancy 2)Gestational trophoblastic disease ~~Ectopic pregnancy “Implantation of the fertilized ovum in any site other than the uterine cavity” 1% of pregnancies Site: --In > 90% tubal pregnancy -- The cervix, the ovaries, the abdominal cavity --Abdominal pregnancy occasionally reach term . Predisposing factors and pathogenesis Tubal pregnancy: The ovum cant move normally 1)Chronic salpingitis and obstruction of fallopian tubes 2)Congenital tubal anomalies 3)Functional tubal disturbances 4)Intrauterine tumors 5)Endometriosis Ovarian pregnancy: Fertilization of ovum within its follicle at the time of rupture Abdominal pregnancy: Dropping of the fertilized egg from fallopian tube and implantation on the peritoneum ~Tubal pregnancy Most common in the mid-portion (ampulla) --Gestational sacs develop in the tube --Chorionic villi are partially attached causing hemorrhage in the tube (hematosalpinx) --Chorionic villi penetrate the BVs and the wall causing tubal rupture and intra-abdominal hemorrhage -- The uterine cavity shows decidual reaction with the absence of villi or fetal parts Gross morphology: The effects of hormones of pregnancy on stromal cells which lay between glands The tube is distended by freshly clotted blood, gray placental tissue and fetal parts. Histologic diagnosis: -- Depends on identification of placental villi or, rarely the embryo within the fallopian tube. -- Rarely, death of the embryo with spontaneous proteolysis and absorption occurs. ~~Gestational Trophoblastic Disease GTD A group of diseases characterized by proliferation of gestational trophoblasts. Classification: Benign GTD 1)Complete hydatidiform mole 2)Partial hydatidiform mole Malignant GTD 1)Invasive mole “chorioadenoma destreuns” 2)Gestational choriocarcinoma 3)Placental site trophoblastic tumors (PSTT) is growth of an abnormal fertilized egg or an overgrowth of tissue from the placenta. Hydatidiform Mole : 1)Complete mole 2)Partial mole (15-35%) _High in Asia. _Most common < 20 and > 40 years _ A history of molar pregnancy increases the risk in subsequent pregnancies. Gross appearance: _ Voluminous mass of swollen chorionic villi appearing as grapelike structures _Fetal parts may be seen in partial mole. Microscopic appearance: _ The swollen villi are covered by varying amounts of proliferating trophoblasts. 1) Complete mole look at the last page _Hydropic swelling of all chorionic villi _ Absence of fetal RBCs or fetal parts Cistern formation: the center of the villi is loose and myxomatous with broken strands. Trophoblastic hyperplasia with atypia _Proliferation of cyto- and syncytiotrophoblast _Diffuse and circumferential 2)Partial mole _Edema of some of the villi & have scalloped margin _Focal trophoblastic proliferation Evidence of an embryo or fetus _ Normal villi _ Fetal red blood cells in the villi _ A fully formed fetus in some cases Clinical Pic For 2 both Complete mole was discovered at 12-14 weeks due to vaginal bleeding & large for date uterus. Antenatal care has lowered age of detection. Complete & partial mole: Elevated B-HCG in the blood and urine 1) Aid the diagnosis 2) Monitor response to therapy Management: Suction curettage + serial B-HCG. Feature Complete Partial Karyotype Diploid 46,XX (46,XY) Triploid (69,XXY) Paternal chromosomes Paternal & maternal Fetal Absent Fetal parts, some normalvilli & fetal RBCs Villous edema All villi Some villi Trophoblast proliferation Diffuse; circumferential Focal; slight Atypia Often present Absent Serum HCC Elevated Less elevated HCC in tissue ++++++++++++++ ++ Behavior 10% invasive mole2% choriocarcinoma Rare choriocarcinoma Gestational Choriocarcinoma A very aggressive malignant tumor arises from gestational chorionic epithelium. Incidence: _Rare in the West,1/ 30,000 pregnancies _ More common in Asia and Africa, 1/2000 _The risk ↑< age 20 and > age 40 _50% arise in complete moles. _25% arise after an abortion. _ 22% in a normal pregnancy. The remainder in ectopic pregnancy Clinical picture 1)Bloody vaginal discharge 2) Absence of marked uterine enlargement 3) Very high titer of B-HCG in blood and urine 4) X-ray of chest & bone detect mets Gross 1) Hemorrhagic and necrotic masses within the uterus 2)The necrosis may be complete. Microscopic _Dimorphic tumor composed of anaplastic cyto- & syncytiotrophoblasts _Chorionic villi are absent Prognosis & Treatment 1) Widespread blood dissemination to the lungs(50%), vagina, brain, liver, and kidneys 2) Lymphatic invasion is uncommon 3) MAC regimen of CTX (methotrexate, actinomycin D and chlorambucil) Survival rates: ∼ 100% for tumors confined to uterus. ∼ 83% for metastatic tumors The End .
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