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Case 05- Ovarian oncology and cysts Physiological cysts Follicular cyst o Most common benign ovarian tumour o Diameter up to 10cm, smaller cysts do not need intervention o Can be caused by ovulation induction and gestational trophoblastic disease due to gonadotropin overstimulation o Pelvic ultrasound should be repeated in 4-6 weeks time o If symptomatic or not resolved in 8-16 weeks, treat with wait until half way through pregnancy before removing the cyst laparoscopically (lower chance of miscarriage) Symptoms are menstrual disturbances, endometrial hyperplasia Luteal cyst o More common on the right side, <3cm = corpora lutea o Can rupture causing intraperitoneal bleeding on days 20-26 of menstrual cycle o Sometimes, it fills with fluid or blood, creating a cyst that can grow to 6cm across. o can rupture causing hemoperitoneum, hypotension, and peritonitis haemorrhagic corpus luteal cysts are usually seen in the first trimester, with most resolving by 12 weeks' gestation o Treatment is laparoscopy if ruptured or torsion Germ cell tumours Happens more commonly in <30 years old All three germ layers are present Benign Dermoid cyst (25% of all ovarian tumours) o Bilateral in 11%, 60% are asymptomatic 3.5-10% can have torsion 1-4% can rupture, risk is higher in pregnancy and labour o Unilocular cyst <15 cm in diameter, has predominantly ectodermal structures Skin, teeth, hair, nervous tissue, thyroid, bronchus, intestine Mesodermal structures can be present too: bone, cartilage, muscle Mature solid teratoma o Rare, less cystic than dermoid cyst Malignant Dysgerminoma (2-5% of ovarian malignancy) o <30 yo, 10% bilateral o Mean diameter 15cm, spread via lymphatics o Can coexist with choriocarcinoma, endodermal tumour or teratoma (10%) Do beta hCG o Pure dysgerminoma is mostly Stage 1=good prognosis Choriocarcinoma o Secretes beta hCG, usually diagnosis is done when haematological spread has occurred Yolk sac (endodermal) o Usually in <40 yo o Can rupture causing pain, aggressive and fast growing Diagnosed late, with extensive metastases o Areas of necrosis, haemorrhage o Often makes AFP, can be measured to monitor treatment Teratoma o about 20% of all malignant tumors, mainly in <20 yo o gliomatosis peritonei, caused by rupture. Seeding of neural tissue in the peritoneum if the metastases are immature, it indicates a poorer prognosis Epithelial tumours (60 to 80% of ovarian tumors) Most ovarian neoplasia are of this category, they are mesothelial in nature Benign (more in <40 yo) Serous cystadenoma o Bilateral in 10%, inner surface is cuboidal or columnar and maybe ciliated o Usually smaller than mucinous cystadenoma o Sometimes psamoma bodies (round collections of calcium) are present Mucinous cystadenoma (10-15% of all ovarian tumours) o Large unilateral, multilocular cysts Has columnar mucin secreting cells, produces thick glutinous o pseudomyxoma peritonei is a rare complication, this follows preoperative rupture and spreading of tumour cells mucin in the peritoneum can cause bowel obstruction 50% 5 year survival, 18% 10 year survival Treatment is surgically debulking the metastases and abdominal chemotherapy Endometrioid cystadenoma (5%) o Most are malignant, edges are badly defined o Looks like and can co-exist with endometriosis Brenner tumours o Wolffian metaplasia, usually benign and <2cm in diameter o between 2 to 3 cm, solid, well circumscribed and may be calcified o 75% occur in >40 yo Clear cell (mesonephroid tumour) o Rarely benign, almost all are malignant Malignant Serous carcinoma o Commonest malignant ovarian tumor (33% of ovarian tumours) o Presents with ascites due to metastases o Often bilateral o Psamoma bodies often are seen Mucinous carcinoma o Multilocular, large like its benign counterpart but contains solid areas 25cm cyst is not uncommon o Pseudomyxoma peritonei Endometrioid carcinoma o Most are malignant, edges are badly defined o Looks like and can co-exist with endometriosis o 15% can have endometrial carcinoma, where both are separate primary tumours (not metastasis) Clear cell carcinoma o Cannot be distinguished from the other surface epithelial tumors by their gross appearances. They are partially cystic but may be entirely solid. Necrosis and haemorrhage may be seen in the solid areas. o May be a variant of endometrioid tumour, as they frequently co-exist o Strong association with endometriosis Borderline epithelial tumour o Most are confined in the ovary, usually serous or mucinuous o Good prognosis even if metastases are found, they often regress when primary lesion is removed Sex cord stromal tumours (3% of all ovarian tumor) Benign Theca cell tumour o Almost all are benign, incidence is high in 60yo 5-10 cm o Has systemic effects Precocious puberty, postmenopausal bleed, endometrial hyperplasia and cancer Fibroma o ~50yo o commonest ovarian stromal tumor o Meig’s syndrome is rare (1%): ascites and pleural effusion 40% of fibroma> 6cm Sertoli-leydig cell tumour o ~30yo o Rare, <0.2% of all ovarian tumours o 75% make androgen, many non-functional, some make oestrogen Malignant Granulosa cell tumour o Usually solid, but can develop cysts o 75% of pure granulosa cell tumors are functional and makes estrogen Fibrosarcoma o Very rare Sertoli-leydig cell tumour (rare) o 50% makes male hormones Gynandroblastoma o producing both ovarian (granulosa and/or theca) and testicular (Sertoli and/or Leydig) cells or tissue Natural history of Ovarian Cancers 2/3 present with cancer spread beyond the pelvis because of insidious and non-specific symptoms or fast growing tumour Metastases commonly happen via o Direct (peritoneum, omentum, peritoneal surface of bowels) o Lymphatic (pelvic and para-aortic nodes) o Haematological (brain, lungs) Risk factors o Nulligravida o Obesity (>30 BMI) o Tubal ligation (protective) o Clomiphene citrate for infertility treatment o HRT o Family history (BRCA 1 and 2) o History of breast cancer HOPC o Abdominal pain or discomfort o Abdominal distension or lump (77%) o Bowel changes (70%), urinary frequency (34%), weight loss, menstrual changes Examination findings o Pelvic mass +/- enlarged lymph nodes (groin, neck) o Hormonal changes Investigations o FBE, EUC o ultrasound pelvis + CA 125 can combine to give an estimate on the risk of malignancy o definitive diagnosis is made using laparotomy Treatment o Laparotomy with vertical incision Objective is to remove all macroscopically visible neoplastic tissues Usually involves total hysterectomy, bilateral salpingooophorectomy and infracolic omentectomy if the cancer has spread extensively If the tumour is unilateral and low stage (no spread): unilateral salpingo-oophrectomy will suffice Do sampling of peritoneal fluids or saline washing o Interval bulking For unresectable disease, it is possible to laparotomy after an initial chemotherapy. Chemotherapy is then resumed as soon as possible after surgery o Any ovarian cancers with Stage >1c should have adjuvant therapy Chemotherapy 5-6 cycles at 3-4 weekly intervals Platinum based chemo drugs are best o Caboplatin, paclitaxel (Taxol, usually in combination with carboplatin as first line regime and is not platinum based) Radiotherapy Experimental, not usually used in practice occasionally used for women with ovarian cancer, especially if it is confined to the pelvic cavity Prognosis o The 5-year survival rates are as follows: Stage I - 73%, Stage II - 45%, Stage III - 21%, Stage IV - Less than 5% Characteristics Benign Malignant Germ cell tumours <30 years old 2-3% malignant, but 33% for <20 yo Dermoid cyst Mature teratoma Epithelial tumours Most ovarian cancers are in this category Serous cystadenoma Mucinous cystadenoma Endometrioid cystadenoma Brenner tumours Clear cell Sex cord stromal tumours Most common ones are granulosa and theca tumours, produces steroid hormones (usually oestrogen) In general, have better prognosis compared to other ovarian tumours Granulosa cell tumour Theca cell tumour Fibroma Sertoli-leydig cell tumour Dysgerminoma Choriocarcinoma Yolk sac (endodermal) Teratoma Serous carcinoma Mucinous carcinoma Endometrioid carcinoma Clear cell carcinoma Borderline epithelial tumour Granulosa cell tumour Theca cell tumour Fibrosarcoma Sertoli-leydig cell tumour Gynandroblastoma References http://atlasgeneticsoncology.org/Tumors/OvarSexCordStromID5223.html http://www.babycenter.com.au/pregnancy/complications/ovariancystexpert/ http://www.med-ed.virginia.edu/courses/path/gyn/ovary3.cfm#germ http://www.cancer.org/cancer/ovariancancer/detailedguide/ovarian-cancer-risk-factors