Malignant Tumors of Ovary and Tubes PDF

TUMORS OF THE OVARIUM AND TUBES ASENA AYAR MADENLİ, MD, ASST. PROF. ISU MEDICAL SCHOOL 2024-2025 LIV HOSPITAL VADİSTANBUL DEPARTMENT OF OBSTETRICS AND GYNECOLOGY  OVARIAN CANCERS EPITHELIAL GERM CELL SEX CORD STROMAL METASTATIC  1. EPITHELIAL (%90) 3. GERM C...

TUMORS OF THE OVARIUM AND TUBES ASENA AYAR MADENLİ, MD, ASST. PROF. ISU MEDICAL SCHOOL 2024-2025 LIV HOSPITAL VADİSTANBUL DEPARTMENT OF OBSTETRICS AND GYNECOLOGY  OVARIAN CANCERS EPITHELIAL GERM CELL SEX CORD STROMAL METASTATIC  1. EPITHELIAL (%90) 3. GERM CELL SEROUS (HGSOC, LGSOC) DYSGERMINOMA MUCINOUS YOLK SAC BRENNER CHORIOCARCINOMA ENDOMETRIOID TERATOMA OVARIAN CLEAR CELL EMBRYONAL CANCER CARCINOMA 2. SEX CORD GRANULOSA CELL 4. METASTATIC SERTOLI LEYDIG CELL KRUKENBERG  EPIDEMIOLOGY EPITHELIAL OVARIAN CANCERS ARE A SIGNIFICANT CAUSE OF MORTALITY WORLDWIDE. IN DEVELOPED COUNTRIES, THERE ARE 190,000 NEW CASES, 114,000 DEATHS EACH YEAR. THEY ACCOUNT FOR 3% OF ALL CANCERS. THEY RANK 5.TH AMONG CANCER-RELATED DEATHS (AFTER LUNG, BREAST, COLORECTAL AND PANCREATIC CANCERS) IT IS THE SECOND MOST COMMON GYNECOLOGICAL MALIGNANCY, APPROXIMATELY 23% OF GYNECOLOGIC CANCERS ARE OVARIAN IN ORIGIN. THE ESTIMATED LIFETIME RISK OF A WOMAN DEVELOPING OVARIAN CANCER IS BETWEEN 1.4% AND 1.7%. (1/70) THE AVERAGE AGE OF DIAGNOSIS FOR OVARIAN CANCER IS 63.  EPIDEMIOLOGY THEY MAY OCCUR AT ALL AGES WITH VARIATION IN HISTOLOGIC SUBTYPE BY AGE: IN WOMEN YOUNGER THAN 20 YEARS OF AGE, GERM CELL TUMORS PREDOMINATE, WHILE BORDERLINE TUMORS TYPICALLY OCCUR IN WOMEN IN THEIR 30S AND 40S INVASIVE EPITHELIAL OVARIAN CANCERS MOSTLY OCCUR AFTER THE AGE OF 50 YEARS. NO EFFECTIVE SCREENING TESTS FOR OVARIAN CANCER AND FEW NOTABLE EARLY SYMPTOMS MAY BE PRESENT  EPITHELIAL OVARIAN CANCER (EOC) ORIGINATE FROM THE COELOMIC EPITHELIUM 90% OF OVARIAN CANCER CASES. Serous (hgsoc, lgsoc) Mucinous Brenner EndometrIoId Clear cell THESE TUMORS ARE CLASSIFIED BASED ON THEIR CELLULAR STRUCTURE AND BEHAVIOR PATTERNS AS FOLLOWS: BENIGN BORDERLINE (LOW MALIGNANT POTENTIAL) MALIGNANT  THE CARCINOGENESIS ACCEPTED TO DATE: SURFACE EPITHELIUM INCLUSION CYSTS METAPLASIA OCCURRING IN INCLUSION CYSTS GENETIC PREDISPOSITION IN %5-10 CASES  RISK FACTORS AGE (>40 YEARS) REPRODUCTIVE HISTORY: NULLIPARITY, INFERTILITY FERTILITY MEDICATIONS, HRT >10 YEARS PERSONAL HISTORY OF BREAST CANCER RACE FAMILY HISTORY (HEREDITARY NONPOLIPOSIS COLORECTAL CANCER HNPCC –LYNCH 2 SYND ) EARLY MENARCHE - LATE MENOPAUSE ENVIRONMENTAL FACTORS GENETIC PREDISPOSITION (BRCA MUTATIONS)  RISK FACTORS FOR EPITHELIAL OVARIAN TUMORS : REPRODUCTIVE RISK FACTORS: WOMEN WHO HAVE NEVER HAD CHILDREN ARE TWICE AS LIKELY TO DEVELOP THIS DISEASE. FIRST PREGNANCY AT AN EARLY AGE, EARLY MENOPAUSE, AND THE USE OF ORAL CONTRACEPTIVES HAVE BEEN ASSOCIATED WITH LOWER RISKS OF OVARIAN CANCER. THE RELATIONSHIP OF THESE VARIABLES TO FALLOPIAN TUBE CANCER IS UNCLEAR.  BRCA 1 AND 2 THESE ARE TWO TUMOR-SUPPRESSOR GENES BRCA1 GENE IS LOCATED ON CHROMOSOME 17Q21. PATIENTS WITH A PROVEN MUTATION HAVE A DRAMATICALLY ELEVATED RISK OF DEVELOPING OVARIAN CANCER (39 TO 46 PERCENT). BRCA2 IS LOCATED ON CHROMOSOME 13Q12 AND IN GENERAL IS LESS LIKELY TO LEAD TO OVARIAN CANCER (12 TO 20 PERCENT). MANIFESTATIONS OF BRCA1 OR BRCA2 MUTATIONS CAN APPEAR TO SKIP GENERATIONS  BRCA 1 AND 2 IN BRCA1 OR BRCA2 CARRIERS, PROPHYLACTIC BILATERAL SALPINGO-OOPHORECTOMY (BSO) MAY BE PERFORMED EITHER UPON COMPLETION OF CHILDBEARING OR BY AGE 40 YEARS P RO P HYL ACTIC BS O REDUCES THE RIS K O F DEVELOPING BREAST CANCER BY 50 PERCENT  PROTECTIVE FACTORS MULTIPARITY ORAL CONTRACEPTIVES LACTATION HYSTERECTOMY TUBAL LIGATION BSO (BILATERAL SALPINGO-OOPHORECTOMY)  FIGO STAGING CLASSIFICATION FOR CANCER OF THE OVARY, FALLOPIAN TUBE, AND PERITONEUM STAGE I: TUMOR CONFINED TO OVARIES OR FALLOPIAN TUBE(S) T1‐N0‐M0 IA: TUMOR LIMITED TO 1 OVARY (CAPSULE INTACT) OR FALLOPIAN TUBE; NO TUMOR ON OVARIAN OR FALLOPIAN TUBE SURFACE; NO MALIGNANT CELLS IN THE ASCITES OR PERITONEAL WASHINGS T1A‐N0‐M0 IB: TUMOR LIMITED TO BOTH OVARIES (CAPSULES INTACT) OR FALLOPIAN TUBES; NO TUMOR ON OVARIAN OR FALLOPIAN TUBE SURFACE; NO MALIGNANT CELLS IN THE ASCITES OR PERITONEAL WASHINGS T1B‐N0‐M0 IC: TUMOR LIMITED TO 1 OR BOTH OVARIES OR FALLOPIAN TUBES, WITH ANY OF THE FOLLOWING: IC1: SURGICAL SPILL T1C1‐N0‐M0 IC2: CAPSULE RUPTURED BEFORE SURGERY OR TUMOR ON OVARIAN OR FALLOPIAN TUBE SURFACE T1C2‐N0‐M0 IC3: MALIGNANT CELLS IN THE ASCITES OR PERITONEAL WASHINGS T1C3‐N0‐M0 STAGE II: TUMOR INVOLVES 1 OR BOTH OVARIES OR FALLOPIAN TUBES WITH PELVIC EXTENSION (BELOW PELVIC BRIM) OR PERITONEAL CANCER T2‐N0‐M0 IIA: EXTENSION AND/OR IMPLANTS ON UTERUS AND/OR FALLOPIAN TUBES AND/OR OVARIES T2A‐N0‐M0 IIB: EXTENSION TO OTHER PELVIC INTRAPERITONEAL TISSUES T2B‐N0‐M0  STAGE III: TUMOR INVOLVES 1 OR BOTH OVARIES OR FALLOPIAN TUBES, OR PERITONEAL CANCER, WITH CYTOLOGICALLY OR HISTOLOGICALLY CONFIRMED SPREAD TO THE PERITONEUM OUTSIDE THE PELVIS AND/OR METASTASIS TO THE RETROPERITONEAL LYMPH NODES T1‐3/N0‐1/M0 IIIA1: POSITIVE RETROPERITONEAL LYMPH NODES ONLY (CYTOLOGICALLY OR HISTOLOGICALLY PROVEN): T1/T2‐N1‐M0 IIIA1(I) METASTASIS UP TO 10 MM IN GREATEST DIMENSION IIIA1(II) METASTASIS MORE THAN 10 MM IN GREATEST DIMENSION IIIA2: MICROSCOPIC EXTRAPELVIC (ABOVE THE PELVIC BRIM) PERITONEAL INVOLVEMENT WITH OR WITHOUT POSITIVE RETROPERITONEAL LYMPH NODES T3A2‐N0/N1‐M0 IIIB: MACROSCOPIC PERITONEAL METASTASIS BEYOND THE PELVIS UP TO 2 CM IN GREATEST DIMENSION, WITH OR WITHOUT METASTASIS TO THE RETROPERITONEAL LYMPH NODES T3B‐N0/N1‐M0 IIIC: MACROSCOPIC PERITONEAL METASTASIS BEYOND THE PELVIS MORE THAN 2 CM IN GREATEST DIMENSION, WITH OR WITHOUT METASTASIS TO THE RETROPERITONEAL LYMPH NODES (INCLUDES EXTENSION OF TUMOR TO CAPSULE OF LIVER AND SPLEEN WITHOUT PARENCHYMAL INVOLVEMENT OF EITHER ORGAN) T3C‐N0/N1‐M0 STAGE IV: DISTANT METASTASIS EXCLUDING PERITONEAL METASTASES ANY T, ANY N, M1 STAGE IVA: PLEURAL EFFUSION WITH POSITIVE CYTOLOGY STAGE IVB: PARENCHYMAL METASTASES AND METASTASES TO EXTRA‐ABDOMINAL ORGANS (INCLUDING INGUINAL LYMPH NODES AND LYMPH NODES OUTSIDE OF THE ABDOMINAL CAVITY)  SYMPTOMS IRREGULAR BLEEDING IN PREMENOPAUSE PRESSURE SYMPTOMS LOWER ABDOMINAL DISTENSION DYSPAREUNIA ACUTE PAIN DISTENSION DUE TO ASCITES DISTENSION RELATED TO OMENTAL AND INTESTINAL METASTASIS POSTMENOPAUSAL BLEEDING  PHYSICAL FINDINGS PELVIC EXAMINATION REVEALS A SOLID, IRREGULAR FIXED MASS UPPER ABDOMINAL MASS ASCITES PALPABLE OVARIES IN POSTMENOPAUSAL PATIENTS PLEURAL EFFUSION  DIAGNOSTIC EVALUATION PHYSICAL EXAMINATION ULTRASOUND , MRI, CT TUMOR MARKERS (CA 125, CA 15-3, CA 19-9) CERVICAL CYTOLOGY ENDOMETRIAL SAMPLING IN PATIENTS WITH IRREGULAR MENSTRUATION AND POSTMENOPAUSAL BLEEDING COMPLAINTS INVESTIGATION OF LIVER AND PANCREATIC PATHOLOGIES IN PATIENTS WITHOUT PELVIC MASSES WITH ASCITES LAPAROTOMY/LAPARASCOPY FOR DEFINITIVE DIAGNOSIS  WHY A SUCCESSFUL SCREENING IS DIFFICULT? ANATOMICAL LOCALIZATION OF OVARIES NO WELL-DEFINED PRECURSOR LESION NO SPECIFIC VALIDATED DIAGNOSTIC METHOD THAT WOULD REDUCE UNNECESSARY INTERVENTION.  CA 125 IN THE EVALUATION OF OVARIAN CANCER, IT IS POSITIVE IN 80-85% OF THE SEROUS EPITHELIAL OVARIAN CANCER CASES. ACCORDING TO SERIAL VALUES, IT RISES BEFORE CLINICAL CANCER. IN POSTMENOPAUSAL WOMEN OVER THE AGE OF 50, AN ANNUAL CA 125 TEST WILL YIELD A FALSE POSITIVE RESULT FOR EVERY 30 WOMEN FOR EACH CASE OF OVARIAN CANCER DETECTED. IN 90% OF ADVANCED STAGE CASES, IT INCREASES, BUT IT HAS LOW SENSITIVITY (IT ONLY INCREASES IN 50% OF STAGE 1 CASES). IT HAS LOW SPECIFICITY BECAUSE IT INCREASES IN MANY GYNECOLOGICAL AND NON-GYNECOLOGICAL CONDITIONS. SENSITIVITY IS HIGHER IN POSTMENOPAUSE  CA 125 MALIGNANT - INCREASES IN ENDOMETRIAL, FALLOPIAN TUBE, GERM CELL, CERVICAL, PANCREATIC, BREAST, AND COLON CANCERS. BENIGN - INCREASES IN ENDOMETRIOSIS, FIBROIDS, PID, ADENOMYOSIS, FUNCTIONAL OVARIAN CYSTS, AND PREGNANCY. ALSO INCREASES IN OTHER DISEASES (KIDNEY, HEART, LIVER). HIGH IN 1% OF NORMAL WOMEN. NORMAL VALUE 100-200.  TREATMENT SURGERY CHEMOTHERAPY RADIOTHERAPY IMMUNOTHERAPY HORMONE THERAPY  TREATMENT IN BORDERLINE TUMORS, THE PRIMARY TREATMENT IS THE RESECTION OF THE PRIMARY TUMOR STAGE IA AND IB G1 TUMORS, SURGERY ALONE IS SUFFICIENT (FERTILITY-SPARING SURGERY CAN BE PERFORMED). STAGE IA AND IB G2-3 AND STAGE IC TUMORS, SURGERY PLUS CHEMOTHERAPY IS REQUIRED. ADVANCED STAGES, CYTOREDUCTIVE SURGERY (DEBULKING) PLUS CHEMOTHERAPY IS INDICATED.  CYTOREDUCTIVE SURGERY THE GOAL IS TO ACHIEVE A STATE WHERE THERE ARE NO RESIDUAL TUMORS LARGER THAN 1 CM, AS THIS IS THE BEST INDICATOR OF SURVIVAL IN ADVANCED-STAGE DISEASE. IT IS NOT THE VOLUME OF THE REMAINING TUMOR CELLS THAT DETERMINES PROGNOSIS, BUT RATHER THE DIAMETER OF THE LARGEST RESIDUAL TUMOR.  MOST IMPORTANT FAVORABLE PROGNOSTIC FACTORS FOR OVARIAN CANCER YOUNGER AGE GOOD PERFORMANCE STATUS CELL TYPE OTHER THAN MUCINOUS AND CLEAR CELL WELL-DIFFERENTIATED TUMOR SMALLER DISEASE VOLUME PRIOR TO SURGICAL DEBULKING NO ASCITES SMALLER RESIDUAL TUMOR AFTER PRIMARY CYTOREDUCTIVE SURGERY  NON EPITHELIAL OVARIAN TUMOURS II – GONAD STROMASI KÖKENLI TÜMÖRLER I- GERM HÜCRESI KÖKENLI TÜMÖRLER A. GRANULOZA – TEKA HÜCRELI TÜMÖRLER A. TERATOM 1. GRANULOZA HÜCRELI TÜMÖR 2. TEKOMA 1. MATÜR TERATOM B. SERTOLI – LEYDIG HÜCRELI TÜMÖRLER 2. İMMATÜR TERATOM 1. ARRHENOBLASTOMA B. DISGERMINOM 2. SERTOLI HÜCRELI TÜMÖR C. GYNANDROBLASTOMA C. EMBRIYONAL KARSINOM D. LIPID HÜCRELI TÜMÖRLER D. ENDODERMAL SINUS TÜMÖRÜ I I I – N ON S PE S I FI K M E Z E N KI M KÖKE N L I TÜMÖRLER E. KORYOKARSINOM A.FIBROM, HEMANJIOM, LEIOMYOM, LIPOM F. GONADOBLASTOMA B. LENFOMA C. SARKOM IV - METASTATIK OVER TÜMÖRLERI  GERM CELL OVARIAN TUMORS THE MOST IMPORTANT FEATURE OF GERM CELL TUMORS OF THE OVARY WHICH ORIGINATE FROM PRIMITIVE GERM CELLS, IS THAT THEY ACCOUNT FOR 70% OF OVARIAN TUMORS SEEN BEFORE THE AGE OF 20. AFTER THE THIRD DECADE, IT IS VERY RARE. THE MOST COMMONLY SEEN MALIGNANT GERM CELL TUMOR IS "DYSGERMINOMA" IT ACCOUNTS FOR 40% OF ALL GERM CELL TUMORS. WHEN DIAGNOSED, 60-75% ARE IN STAGE I.  MALİGN GERM HÜCRELİ OVER TÜMÖRLERİ Histogenetik ve İmmünohistolojik Sınıflama PRİMORDİYAL GERM HÜCRESİ EMBRİYONAL KARSİNOM AFP ( - ) veya (+) DİSGERMİNOM hCG ( - ) (+) AFP ( - ), hCG ( - / + ) EKSTRAEMBRİYONİK EMBRİYONAL DİFERANSİYASYON DİFERANSİYASYON Teratom Poliembrioma TROFOBLAST YOLK KESESİ MATÜR TERATOM KORİYOKARSİNOM AFP ( - ), hCG ( - ) ENDODERMAL SİNÜS AFP ( - ), hCG ( + ) TÜMÖRÜ İMMATÜR TERATOM AFP ( + ), hCG ( - ) AFP ( - ), hCG ( - )  DYSGERMINOMA IT ACCOUNTS FOR 1-3% OF OVARIAN CANCERS AND 30-45% OF ALL GERM CELL OVARIAN TUMORS. 75% O F IT IS O BS ERV ED BET W EEN THE AG ES O F 10 AND 30. IN PARAL L EL , IT CONSTITUTES 20-30% OF THE OVARIAN CANCERS OBSERVED DURING PREGNANCY. ALONG WITH DYSGERMINOMA, OTHER GERM CELL OVARIAN TUMORS ARE OBSERVED, WITH "IMMATURE TERATOMA" BEING THE MOST COMMON. 5% OF DYSGERMINOMAS ARE ASSOCIATED WITH ABNORMAL GONADS. IT IS SEEN IN PATIENTS WITH GONADAL DYSGENESIS AND ANDROGEN INSENSITIVITY. 10-15% IS BILATERAL. WHEN DIAGNOSED, 75% OF CASES ARE STAGE I. TUMOR MARKERS IN GERM CELL TUMORS  TREATMENT THE MOST COMMONLY ENCOUNTERED GERM CELL OVARIAN CANCER, OCCURS IN 75% OF CASES BETWEEN THE AGES OF 10 AND 30, WITH 75% BEING IN STAGE I. THE TREATMENT OUTLINES THAT PRESERVE THE PATIENT'S FERTILITY AS MUCH AS POSSIBLE AND PROVIDE THE LONGEST SURVIVAL INCLUDE: OPTIMAL SURGICAL STAGING, UNILATERAL SALPINGO-OOPHORECTOMY CHEMOTHERAPY.  TREATMENT THE OTHER OVARY SHOULD BE EXAMINED WITH AN INSPECTION; IF A SUSPICIOUS LESION AND/OR GROWTH IS DETECTED IN THIS OVARY, A BIOPSY SHOULD BE TAKEN, AND THE SUSPICIOUS LESION SHOULD BE EXCISED WHILE PRESERVING THE NORMAL OVARIAN TISSUE. WHAT SHOULD WE DO IF THERE IS A TUMOR IN THE OTHER OVARY? THE LESION SHOULD BE RESECTED, BUT OVARY MUST BE PROTECTED. IF FERTILITY IS NOT DESIRED, RADICAL SURGERY.  TREATMENT WHAT SHOULD WE DO IF THERE ARE DYSGENETIC GONADS (IF IT CARRIES A Y CHROMOSOME)? IN CASES OF DETECTING DYSGENETIC GONADS, A BILATERAL OOPHORECTOMY SHOULD BE PERFORMED, ESPECIALLY IN STAGE I, BUT THE UTERUS SHOULD BE PRESERVED. IS PELVIC - PARAAORTIC LYMPHADENECTOMY NECESSARY? DYSGERMINOMAS MOST COMMONLY SPREAD THROUGH THE LYMPHATIC SYSTEM. THEY PARTICULARLY AFFECT THE LYMPH NODES AROUND THE AORTA, ESPECIALLY NEAR THE RENAL VEIN. BECAUSE IT IS SENSITIVE TO CHEMOTHERAPY, SAMPLING IS SUFFICIENT.  TREATMENT HOW SHOULD MANAGEMENT BE IN A PREGNANT PATIENT?? IN STAGE I A, THE TUMOR IS REMOVED AND THE PREGNANCY CONTINUES. IN OTHER STAGES, TREATMENT IS BASED ON GESTATIONAL AGE. IN STAGES II AND III, CONSERVATIVE SURGERY IS PERFORMED, FOLLOWED BY CHEMOTHERAPY. WHICH PATIENT HAS A HIGH RISK OF RECURRENCE? UNDER 20 YEARS OLD MASS LARGER THAN 15 CM RUPTURE OF THE MASS DURING SURGERY LYMPHATIC INVOLVEMENT Disgerminom + Gebelik  IMMATURE TERATOMA THEY ACCOUNT FOR 20% OF GERM CELL OVARIAN TUMORS. OVARIAN CANCERS SEEN IN INDIVIDUALS UNDER 20 ACCOUNT FOR 10-20% OF CASES AND ARE RESPONSIBLE FOR OVER 30% OF DEATHS FROM OVARIAN CANCER IN THIS AGE GROUP. THE AVERAGE AGE OF ONSET IS 18. WHEN DIAGNOSED, 70% OF CASES ARE STAGE I. IT IS RARELY BILATERAL. IN 5% OF CASES, A BENIGN CYSTIC TERATOMA IS DETECTED IN THE OTHER OVARY. THIS SITUATION MAY LEAD TO ERRORS IN TERMS OF METASTASIS.  THE IMPORTANCE OF "GRADE" IN IMMATURE TERATOMA THE HISTOLOGICAL CLASSIFICATION (GRADE) OF THE TUMOR IS BASED ON THE MATURATION OF NEURAL TISSUES. GRADE I: 0-1 IMMATURE CELLS GRADE II: IMMATURE CELLS IN LESS THAN 3 AREAS GRADE III: IMMATURE CELLS IN MORE THAN 3 AREAS THE PROGNOSIS IS POOR IN IMMATURE TERATOMAS CONTAINING MALIGNANT SQUAMOUS COMPONENTS !! TREATMENT SURGICAL STAGING + UNILATERAL SALPINGO-OOPHORECTOMY BIOPSY FROM THE OTHER OVARY IS UNNECESSARY. CHEMOTHERAPY SHOULD BE ADMINISTERED FOR ALL TUMORS OTHER THAN OF STAGE I.  ENDODERMAL SINUS TM THEY ACCOUNT FOR 20% OF GERM CELL OVARIAN TUMORS. 15% IS ASSOCIATED WITH IPSILATERAL OVARY, WHILE 5% IS ASSOCIATED WITH THE OPPOSITE OVARY ALONG WITH A DERMOID CYST. THE AVERAGE AGE OF ONSET IS 18. ONE-THIRD OF THE CASES ARE PREMENSTRUAL. 75% OF CASES INVOLVE PELVIC PAIN. SURGICAL TREATMENT: UNILATERAL SALPINGO-OOPHORECTOMY. IT IS MEANINGLESS TO REMOVE THE OTHER OVARY AND PERFORM A TOTAL ABDOMINAL HYSTERECTOMY (TAH). THE FORECAST WILL NOT BE AFFECTED. THE LIFESPAN AFTER CHEMOTHERAPY HAS BEEN EXTENDED. AFP IS A VERY GOOD TUMOR MARKER.  SEX CORD STROMAL TUMORS I - GRANULOZA – STROMAL HÜCRELI TÜMÖRLER III – ANNULER TUBULUSLU SEKS A.GRANULOZA HÜCRELI TÜMÖRLER KORD TÜMÖRLER A. ADULT TIP IV – GYNANDROBLASTOMA B. JUVENIL TIP B.FIBRO – TEKOMA GRUBU V – STEROID HÜCRELI TÜMÖRLER A. TEKOMA B. FIBROMA-FIBROSARKOMA A. STROMAL LUTEOMA C.SKLEROZAN STROMAL TÜMÖR B. LEYDIG HÜCRELI TÜMÖR II – SERTOLI – STROMAL HÜCRELI TÜMÖRLER * HILUS HÜCRELI TIP TÜMÖR A. SERTOLI HÜCRELI TÜMÖR **NONHILAR TIP TÜMÖR B. LEYDIG HÜCRELI TÜMÖR C. SERTOLI-LEYDIG HÜCRELI TÜMÖR C. NONSPESIFIALIZE STEROID HÜCRELI TÜMÖRLER IV – SINIFLANDIRILAMAYANLAR  SEX CORD STROMAL OVARIAN TM THEY ACCOUNT FOR 5% OF ALL OVARIAN TUMORS AND 90% OF HORMONALLY ACTIVE TUMORS. THEY ORIGINATE FROM UNDIFFERENTIATED CELLS FOUND IN THE GONADAL MESENCHYME. ESTROGEN ANDROGENS GRANULOSA CELL TUMORS SERTOLİ-LEYDİG CELL TUMORS LİPİD CELL TUMORS THECA CELL TUMORS SERTOLI CELL TUMORS ESTROGEN – ANDROGEN GYNANDROBLASTOMA  GRANULOSA CELL TM THEY MAKE UP 70% OF SEX CORD STROMAL CELL TUMORS. THEY CAN BE SEEN IN EVERY PERIOD FROM CHILDHOOD TO SENILITY. THERE ARE TWO TYPES: ADULT AND JUVENILE. ADULT TYPE 95% OF ALL CASES AND CAN ALSO BE SEEN IN THE PREPUBERTAL PERIOD. DEFINED AS "TUMORS WITH LOW MALIGNANCY POTENTIAL." 30% İS ASSOCIATED WITH ENDOMETRIAL HYPERPLASIA WHEREAS 10% IS ASSOCIATED WITH ENDOMETRIAL CANCER. 2-5% BILATERAL 80-90% ARE STAGE I TUMORS AT THE TIME OF DIAGNOSIS. IN THE LONG TERM, METASTASIZE TO THE LUNGS AND BRAIN THROUGH HEMATOLOGICAL PATHWAYS. THEY CAN CAUSE RUPTURE AND LEAD TO HEMOPERITONEUM.  GRANULOSA CELL TM JUVENILE TYPE: AT THE TIME OF DIAGNOSIS, 90% ARE STAGE IA - IB. CHILDHOOD BRAIN TUMORS ACCOUNT FOR 5-7% OF ALL TUMORS. IT IS 5% BILATERAL. IT IS SEEN BEFORE 90% OF PUBERTY. WHEN OBSERVED BEFORE PUBERTY, IT IS ASSOCIATED WITH PERIPHERAL PRECOCIOUS PUBERTY (PSEUDO-PRECOCIOUS PUBERTY). E2 ↑, PROGESTERONE ↑, TESTOSTERONE ↑; FSH-LH ↓ IN 10% OF CASES, THERE IS ACID, AND IN 10% OF CASES, THERE IS TUMOR RUPTURE.  TM MARKERS E2 İNHIBIN FOLLICLE REGULATORY PROTEIN (FRP) MÜLLERIAN INHIBITING FACTOR (MİF)  TREATMENT-PROGNOSIS TREATMENT FOR GRANULOSA CELL TUMORS IS ADJUSTED ACCORDING TO THE AGE AT DIAGNOSIS AND THE DESIRE FOR FERTILITY. FRACTIONAL CURETTAGE IS ESSENTIAL DURING THE PERIMENOPAUSAL AND POSTMENOPAUSAL PERIODS. IF FERTILITY IS TO BE PRESERVED: USO + SURGICAL STAGING 5-YEAR SURVIVAL IN ADULT TYPE: STAGE I: 92%-100% STAGE II - III: 33% - 53% JUVENILE TYPE: THE 10-YEAR SURVIVAL RATE IN STAGE I IS 97%, AND THE PROGNOSIS IS BETTER IN CASES WITH FALSE PRECOCIOUS PUBERTY (EARLY DIAGNOSIS).  FIBRO-THECOMAL TM THEY ARE CONSIDERED OF BENIGN NATURE. THEY ARE ALMOST ALWAYS ONE-SIDED. CONSERVATIVE SURGERY SHOULD BE PERFORMED. THECOMA: THEY ARE SEEN TOGETHER WITH ENDOMETRIAL HYPERPLASIA AND CANCER. FIBROMAS: THEY MAY CAUSE MEIGS SYNDROME AND GORLIN SYNDROME.  SERTOLI-LEYDIG CELL TM VERY RARE 0.5% OF ALL OVARIAN TUMORS. THE AVERAGE AGE OF ONSET IS 25. 10% IS SEEN AFTER THE AGE OF 50. 1% BILATERAL. DEFEMINIZATION OR MASCULINIZATION OCCURS IN 75-85% OF CASES. TREATMENT WITH USO + SURGICAL STAGING IS SUFFICIENT. IF THE TUMOR CONTAINS A "RETIFORM PATTERN," IT IS SEEN IN INDIVIDUALS UNDER 20 YEARS OLD AND THE PROGNOSIS IS POOR. PROGNOSTIC FACTORS TUMOR RUPTURE EXTRA-OVARIAN SPREAD AT THE TIME OF DIAGNOSIS RETIFORM (RETICULAR) PATTERN HETEROLOG MESENCHYMAL DIFFERENTIATION, THE PROGNOSIS IS POOR.  METASTATIC TUMORS OF OVARY THE OVARIES ARE THE ORGANS MOST FREQUENTLY METASTASIZED BY DIFFERENT TUMORS AMONG THE GENITAL ORGANS. METASTATIC TUMORS CAN ORIGINATE FROM DIFFERENT ORGANS SUCH AS THE THYROID, STOMACH, BREAST, COLON, AND GALLBLADDER. THE INCIDENCE OF METASTATIC OVARIAN CANCER AMONG ALL OVARIAN TUMORS IS REPORTED TO BE AROUND 10%.  METASTATIC TUMORS OF OVARY 50% OF CASES OF GASTROINTESTINAL SYSTEM-DERIVED METASTATIC OVARIAN TUMORS AND 83% OF CASES OF BREAST-DERIVED METASTATIC OVARIAN TUMORS, ARE IN THE REPRODUCTIVE PERIOD AT THE TIME OF DIAGNOSIS. IN YOUNG PATIENTS PRESENTING WITH ASCITES, EITHER WITH BILATERAL OVARIAN LESIONS OR WITHOUT ANY LESIONS DETECTED IN THE OVARIES, THE POSSIBILITY OF METASTATIC OVARIAN TUMOR SHOULD ALWAYS BE CONSIDERED. LAPAROSCOPY IS THE METHOD OF CHOICE FOR DIFFERENTIAL DIAGNOSIS.  COMMON METASTATIC SITES GASTRIC KRUKENBERG TUMOR: PRIMARY COLORECTAL CARCINOMAS THAT HAVE A COMPONENT OF MUCIN FILLED SIGNET RING CELLS BREAST LOOSELY APPLIED TO ALL METASTATIC TMS TO THE OVARY GYNECOLOGIC CARCINOMAS GRANULOSA CELL SERTOLI LEYDIG CALL EXNER BODY REINKE BODY GROSS YELLOW APPEREANCE(LIPIDS) GROSS YELLOW APPEREANCE(LIPIDS) ESTROGEN, INHIBIN B ANDROGENS PMB, PRECOCIOS PUBERTY VIRILIZATION (HAIR GROWTH, VOICE DEEPENING)  SCREENING EFFECTIVE SCREENING STRATEGIES FOR THE EARLY DETECTION OF OVARIAN CANCER DO NOT EXIST !!! WOMEN WITH HIGH RISK OF DEVELOPING OVARIAN CANCER SUCH AS THOSE WITH GERMLINE MUTATIONS IN BRCA1 OR BRCA2 OR OTHER GENES ASSOCIATED WITH A HIGH RISK OF DEVELOPING OVARIAN CANCER CAN BE IDENTIFIED. FOR THESE INDIVIDUALS, STRATEGIES TO REDUCE THE RISK OF OVARIAN CANCER HAVE BEEN IMPLEMENTED THROUGH RISK-REDUCING SURGERY, SUCH AS BILATERAL SALPINGO-OOPHORECTOMY. SCREENING STRATEGIES IN WOMEN WITH AN AVERAGE RISK OF DEVELOPING OVARIAN CANCER HAVE PRIMARILY FOCUSED ON THE BIOMARKER CA125 (ALSO KNOWN AS MUCIN 16) AND THE USE OF TRANSVAGINAL ULTRASONOGRAPHY. COMBINATIONS OF THESE SCREENING MODALITIES HAVE SHOWN SUCCESS IN DETECTING EARLY-STAGE CANCERS, BUT HAVE NOT YET DEMONSTRATED DEFINITIVE IMPROVEMENTS IN PATIENT MORTALITY  PRIMARY FALLOPIAN TUBE CARCINOMA (FTC) TUMORS PRIMARILY LOCATED IN THE FALLOPIAN TUBE, SPECIFICALLY ARISING FROM THE ENDOSALPINX, WITH A PAPILLARY PATTERN OF TUBAL MUCOSAL INVOLVEMENT, AND WITH IDENTIFIABLE TRANSITION FROM BENIGN TO MALIGNANT EPITHELIUM IN THE PRESENCE OF TUBAL WALL INVOLVEMENT. IF TUMORS ARE CONCURRENTLY PRESENT IN THE OVARIES OR ENDOMETRIUM, THEY SHOULD BE SMALLER THAN THOSE IN THE FALLOPIAN TUBE. DUE TO ITS CLINICAL AND HISTOLOGICAL SIMILARITIES TO OVARIAN CANCERS AND THE DIFFICULTY IN MAKING A CLEAR DISTINCTION, TUBAL CANCERS ARE CURRENTLY APPROACHED AND MANAGED SIMILARLY TO OVARIAN CANCERS.  FALLOPIAN TUBE CARCINOMA TWO-THIRDS OF CASES OF TUBAL CANCER ARE IN THE POSTMENOPAUSAL PERIOD. THE AVERAGE AGE RANGES BETWEEN 60 AND 69. HISTOLOGICAL TYPES: SEROUS MUCINOUS ENDOMETRIOID UNDIFFERENTIATED TRANSITIONAL CARCINOMAS  DIAGNOSTIC CRITERIA DIAGNOSIS OCCURS IN ADVANCED STAGES, MOSTLY POSTOPERATIVELY (1) THE MAIN TUMOR ARISES FROM THE ENDOSALPINX, (2) THE HISTOLOGIC PATTERN REPRODUCES THE EPITHELIUM OF TUBAL MUCOSA, (3) TRANSITION FROM THE BENIGN TO MALIGNANT TUBAL EPITHELIUM IS DEMONSTRABLE (4) IF BOTH THE TUBE AND THE OVARY ARE AFFECTED BY THE TUMOR, THEN THE LARGE PART OF THE TUMOR MUST BE IN THE TUBE. BY SEDLIS ET AL (MODIFIED HU CRITERIA)  INCIDENCE FALLOPIAN TUBE CARCINOMAS SHARE CLINICAL AND HISTOPATHOLOGIC FEATURES WITH PRIMARY PERITONEAL AND HIGH-GRADE SEROUS CARCINOMAS OF THE OVARY, AND HAVE RECENTLY BEEN IDENTIFIED AS THE ORIGIN FOR MANY OF THE HIGH- GRADE SEROUS CARCINOMAS, INCLUDING EXTRAUTERINE AND PERITONEAL CANCERS. FALLOPIAN TUBE CANCER IS (THOUGHT TO BE) A RARE PRIMARY MALIGNANCY REPRESENTING 1% TO 2 % OF ALL GYNECOLOGIC MALIGNANCIES. THE INCIDENCE RATE FOR FALLOPIAN TUBE CANCER HAS BEEN REPORTED 0.41 PER 100, 000 ANNUALLY  EPIDEMIOLOGY AND RISK FACTORS PRIMARY FALLOPIAN TUBE CANCER IS THOUGHT TO EMERGE FROM CHRONIC TUBAL INFLAMMATION. BECAUSE OF ITS RARE INCIDENCE, DATA ON RISK FACTORS ARE LIMITED STUDIES HAVE SUGGESTED POSTMENOPAUSAL PROGESTIN-BASED HORMONAL THERAPIES, SPECIFICALLY ESTRADIOL COMBINED WITH LEVONORGESTREL‐RELEASING INTRAUTERINE SYSTEM AND SEQUENTIAL ESTRADIOL‐PROGESTIN THERAPY, ARE ASSOCIATED WITH A HIGHER RISK FOR PRIMARY FALLOPIAN TUBE CANCER. DEMOGRAPHIC RISK FACTORS INCLUDE AGE AND RACE, WITH THE MAJORITY OF CASES OCCURRING IN WHITE WOMEN AGED 50 TO 60YEARS. SIMILAR TO OVARIAN CANCER, A FAMILY HISTORY OF FALLOPIAN TUBE CANCER, AS WELL AS BRCA 1 MUTATIONS, AND THE PRESENCE OF GENES LINKED TO HEREDITARY NONPOLYPOSIS COLORECTAL CANCER, OR LYNCH SYNDROME, ARE ALSO ASSOCIATED WITH INCREASED RISK FOR PRIMARY FALLOPIAN TUBE CANCER. THE USE OF BIRTH CONTROL PILLS AND AN INCREASED NUMBER OF LIVE BIRTHS, PREFERABLY WITH BREAST FEEDING MAY LOWER THE RISK  RISK FACTORS BRCA1 AND BRCA2 MUTATIONS (IN THE PRIMARY FTC : BRCA1 MUTATIONS WERE FOUND IN 11% OF CASES, WHILE BRCA2 MUTATIONS WERE DETECTED IN 5%) CHRONIC TUBAL INFLAMMATION, INFERTILITY TUBERCULOUS SALPINGITIS TUBAL ENDOMETRIOSIS FAMILIES WITH A HIGH RISK OF BREAST AND OVARIAN CANCER. ETHNICITY (ASHKENAZI JEWS)  SIGNS AND SYMPTOMS PRESENTING SYMPTOMS OF FALLOPIAN TUBE CARCINOMA ARE VAGUE, BUT MAY INCLUDE ABDOMINAL BLOATING, FULLNESS, DIFFICULTY EATING, BOWEL OR BLADDER CHANGES, PELVIC OR ABDOMINAL PAIN, AND BLEEDING OR WATERY VAGINAL DISCHARGE. TOGETHER WITH A PALPABLE PELVIC MASS THESE SYMPTOMS ARE REFERRED TO AS THE LATZKO TRIAD, WHICH IS REPORTED IN APPROXIMATELY 15% OF CASES. OF PARTICULAR CONCERN IS POSTMENOPAUSAL VAGINAL BLEEDING, WHICH MAY SUGGEST EITHER ENDOMETRIAL OR FALLOPIAN TUBE CARCINOMA. APPROXIMATELY 5% OF CASES PRESENT WITH A WATERY VAGINAL DISCHARGE REFERRED TO AS HYDROPS TUBAE PROFLUENS.  DIAGNOSIS DIAGNOSIS OF FALLOPIAN TUBE CANCER MAY INVOLVE IMAGING STUDIES, INCLUDING PET AND CT SCANS, AS WELL AS MRI, BLOOD WORK (INCLUDING CA-125 LEVELS), AND IS ULTIMATELY CONFIRMED WITH BIOPSY. APPEARANCE OF A FALLOPIAN TUBE MASS ON IMAGING HAS BEEN REPORTED TO HAVE A SAUSAGE SHAPE OR COG-AND-WHEEL APPEARANCE. BECAUSE OF THE POTENTIAL PROGRESSION OF FALLOPIAN TUBE CANCER WITHOUT SYMPTOMS, OR THE PRESENCE OF SYMPTOMS THOUGHT TO BE ASSOCIATED WITH OVARIAN CANCER, DIAGNOSIS CAN BE DELAYED UP TO 48 MONTHS. THE RISK OF OVARIAN MALIGNANCY ALGORITHM (ROMA) : ASSESSMENT OF CA-125, HUMAN EPIDIDYMIS PROTEIN 4 (HE4), AND MENOPAUSAL STATUS, IS APPROVED TO DISTINGUISH BETWEEN MALIGNANT AND BENIGN MASSES  STAGING AND GRADING SHOWS SIMILARITY TO OVARIAN CARCINOMA FIGO PROVIDES THE STAGING FOR FALLOPIAN TUBE CANCER AND WAS MOST RECENTLY UPDATED IN 2014 (AS SHOWN PREVIOUSLY) SEROUS TYPE IS THE MOST COMMON TYPE FALLOPIAN TUBE CANCER, LIKE OVARIAN CANCER, IS CATEGORIZED AS EITHER LOW- GRADE OR HIGH-GRADE SEROUS CARCINOMA. HIGH-GRADE CARCINOMAS TYPICALLY PRESENT AT A MORE ADVANCED STAGE AND ARE ASSOCIATED WITH TP53 AND BRCA MUTATIONS; WHEREAS LOW-GRADE CARCINOMAS ARE LESS COMMON AND PRESENT WITH KRAS AND BRAF MUTATIONS.  TREATMENT SINCE TUBAL CANCER IS EXTREMELY RARE, THERE IS INSUFFICIENT DATA IN THE LITERATURE REGARDING TREATMENT APPROACHES, AND MANAGEMENT IS GENERALLY DETERMINED BASED ON DATA OBTAINED FROM OVARIAN CANCER SERIES. THE PRIMARY TREATMENT IS SURGICAL RESECTION, AND EXCEPT FOR SOME CASES IN THE EARLY STAGE, THEY REQUIRE ADJUVANT THERAPY. TAH + BSO + OMENTECTOMY +PELVIC AND PARAAORTIC LND  KEYNOTES FOR FTC THE TRIAD OF PAIN, METRORRHAGIA, AND LEUKORRHEA IS CONSIDERED PATHOGNOMONIC FOR FTC. BRCA1 AND BRCA2 CARRIERS ARE AT INCREASED RISK AND SHOULD UNDERGO EXTENSIVE SECTIONING OF THE FALLOPIAN TUBES DURING RISK-REDUCING SURGERY. SEROUS TUBAL INTRAEPITHELIAL CARCINOMA IS A PRECURSOR LESION TO HIGH-GRADE SEROUS CARCINOMA. EARLY SALPINGECTOMY WITH DELAYED OOPHORECTOMY IS CURRENTLY BEING STUDIED AND MAY BECOME AN OPTION IN HIGH-RISK WOMEN TO DECREASE MORTALITY AND MAINTAIN QUALITY OF LIFE  GENETIC SCREENING WHO TO REFER ?!! ACCORDING TO AMERICAN CANCER SOCIETY GENETIC TESTING IS RECOMMENDED FOR: 1. WOMEN WHO HAVE A KNOWN FAMILIAL HISTORY OF BRCA MUTATION 2. DIAGNOSIS OF OVARIAN CANCER, PANCREATIC CANCER 3. FAMILY HISTORY OF BREAST CANCER AT A YOUNGER AGE 4. MORE THAN ONE FAMILY MEMBER WITH BREAST CANCER 5. BREAST CANCER IN A MALE FAMILY MEMBER THANK YOU