19. Gynecological Oncology Flashcards

Question

Describe features: Leiomyosarcoma (3)

Answer 1

* Histologic distinction from leiomyoma * Increased mitotic count (\> 10 mitoses/10 high-power fields) * Tumour necrosis * Cellular atypia * Rapidly enlarging fibroids in a pre-menopausal woman * Enlarging fibroids in a postmenopausal woman

Answer 2

* Often post-operatively after uterusremoved for presumed fibroids * Stage using FIGO 2009 staging for leiomyosarcomas and ECC

Answer 3

* Hysterectomy/BSO usually * No routine pelvic lymphadenectomy * Chemotherapy is used in cases of metastatic disease * Radiation therapy does not improve local control or survival * Poor outcomes overall, even for early-stage disease

Answer 4

Usually presents in perimenopausal or postmenopausal women with abnormal uterine bleeding

Answer 5

* Abnormal uterine bleeding * Good prognosis

Answer 6

* Diagnosed by histology of endometrial biopsy or D&C * Stage using FIGO 2009 staging for leiomyosarcomas and ECC

Answer 7

* Hysterectomy & BSO (remove ovaries as ovarian hormones may stimulate growth) * No routine pelvic lymphadenectomy * Adjuvant therapy based on stage and histologic features (hormones and/or radiation) * Hormonal therapy (progestins) may be used for metastatic disease

Answer 8

Rare; less common than leiomyosarcoma, endometrial stromal sarcoma

Answer 9

* Severe nuclear pleomorphism, high mitotic activity, tumour cell necrosis, and lack smooth muscle or endometrial stromal differentiation * Poor prognosis

Answer 10

Often found incidentally post-operatively for abnormal bleeding

Answer 11

* Treatment primarily surgical * Radiation and/or chemotherapy for advanced diseased or unresectable disease

Answer 12

* The rarest of the uterine sarcoma * Mixed tumour of low malignancy potential

Answer 13

* Present with abnormal vaginal bleeding * Polypoid mass in uterine cavity

Answer 14

* Mixture of benign epithelium with malignant low-grade sarcoma * Often found incidentally at time of hysterectomy for PMB * Stage using FIGO 2009 staging for adenosarcoma

Answer 15

Treatment is surgical with hysterectomy and bilateral salpingo-oophorectomy

Answer 16

III disease

Answer 17

**Ovarian Tumour Markers** * Epithelial cell: CA-125 * Stromal * Granulosa cell: inhibin * Sertoli-Leydig: androgens * Germ cell * Dysgerminoma: LDH * Yolk sac: AFP * Choriocarcinoma: β-hCG * Immature teratoma: none * Embryonal cell: AFP + β-hCG

Answer 18

* many are asymptomatic * usually enlarge slowly, if at all * may rupture or undergo torsion, causing pain * pain associated with torsion of an adnexal mass usually originates in the iliac fossa and radiates to the flank * peritoneal irritation may result from an infarcted tumour (rare)

Answer 19

* lifetime risk 1.4% * in women \>50 yr, more than 50% of ovarian tumours are malignant * causes more deaths in North America than all other gynecologic malignancies combined * 4th leading cause of cancer death in women * 85% epithelial; 15% non-epithelial * 10-15% of epithelial ovarian cancers are related to hereditary predisposition

Answer 20

* early menarche and/or late menopause * personal history of breast, colon, endometrial cancer * family history of breast, colon, endometrial, ovarian cancer * Lynch syndrome and BRCA mutations * use of fertility drugs

Answer 21

* OCP: likely due to ovulation suppression (significant reduction in risk even after 1 yr of use) * pregnancy/breastfeeding

Answer 22

* salpingectomy (prophylactic) * BSO (prophylactic hysterectomy or tubal ligation performed for this reason in high-risk women [i.e. BRCA mutation carriers])

Answer 23

* no effective method of mass screening * routine CA-125 level measurements or U/S not recommended * high false positive rates * controversial in high-risk groups: transvaginal U/S and CA-125, starting age 30 (no consensus on interval) * familial ovarian cancer (\>1 first degree relative affected, BRCA-1 mutation) * other cancers (e.g. endometrial, breast, colon) * BRCA-1 or BRCA-2 mutation: recommendation is prophylactic bilateral oophorectomy after age 35 or when childbearing is completed

Answer 24

* most women with epithelial ovarian cancer present with advanced stage disease as patients often asymptomatic until disseminated (symptoms with early-stage disease are vague and non-specific) * when present, symptoms may include: * abdominal symptoms (nausea, bloating, pain, dyspepsia, anorexia, early satiety) * symptoms of mass effect * increased abdominal girth (from ascites or tumour itself) * urinary urgency and frequency * constipation

Answer 25

surgical pathology

Answer 26

malignant until proven otherwise

Answer 27

* a term for ascites plus a fixed upper abdominal and pelvic mass; * almost always signifies ovarian cancer

Answer 28

* Stage I 75-95% * Stage II 60-75% * Stage III 23-41% * Stage IV 11%

Answer 29

* a subcategory of epithelial ovarian cancer (~15% of all epithelial ovarian tumours) * pregnancy, OCP, and breastfeeding are protective factors * tumour cells with histologically malignant characteristics arise from the ovarian surface, but do not invade ovarian stroma * able to metastasize, but not commonly * treated primarily with surgery (BSO/omental biopsy ± hysterectomy) * chemotherapy has limited benefit: can be treated with hormonal manipulation (letrozole) * generally slow growing, excellent prognosis * 5 yr survival \>99% * recurrences tend to occur late, may be associated with low-grade serous carcinoma

Answer 30

* FUNCTIONAL TUMOURS (all benign) * Follicular Cyst * Corpus Luteum Cyst * Theca-Lutein Cyst * Endometrioma * Polycystic Ovaries * BENIGN GERM-CELL TUMOURS * Benign Cystic Teratoma (dermoid) * MALIGNANT GERM-CELL TUMOURS * Dysgerminoma * Immature Teratoma * Yolk Sac Tumour * Ovarian Choriocarcinoma * EPITHELIAL OVARIAN TUMOURS (malignant or borderline) * Serous * Mucinous * SEX CORD STROMAL OVARIAN TUMOURS * Fibroma/Thecoma (benign) * Granulosa-Theca Cell Tumours (benign or malignant) * Sertoli-Leydig Cell Tumour (benign or malignant) * METASTATIC OVARIAN TUMOURS * From GI Tract, Breast, Endometrium, Lymphoma

Answer 31

* Description: Follicle fails to rupture during ovulation * Presentation: * Usually asymptomatic * May rupture, bleed, tort, infarct causing pain ± signs of peritoneal irritation * Ultrasound/Cytology: 4-8 cm mass, unilocular, lined with granulosa cells * Treatment: * Symptomatic or suspicious masses warrant surgical exploration Otherwise if \<6 cm, wait 6 wk then re-examine as cyst usually regresses with next cycle OCP (ovarian suppression): will prevent development of new cysts * Treatment usually laparoscopic (cystectomy vs. oophorectomy, based on fertility choice)

Answer 32

* Description: Corpus luteum fails to regress after 14 d, becoming cystic or hemorrhagic * Presentation: * More likely to cause pain than follicular cyst * May delay onset of next period * Ultrasound/Cytology: * Larger (10-15 cm) and firmer than follicular cysts * Treatment: Same as for follicular cysts

Answer 33

* Description: Due to atretic follicles stimulated by abnormal β-hCG levels * Presentation: Associated with molar pregnancy, ovulation induction with clomiphene * Ultrasound/Cytology: - * Treatment: * Conservative * Cyst will regress as β-hCG levels fall

Answer 34

* Description: * Single most common ovarian germ cell neoplasm * Elements of all 3 cell lines; contains dermal appendages (sweat and sebaceous glands, hair follicles, teeth) * Presentation: * May rupture, twist, infarct * 20% bilateral * 20% occur outside of reproductive yr * Ultrasound/Cytology: * Smooth-walled, mobile, unilocular * Ultrasound may show calcification which is pathognomonic * Treatment: Treatment usually laparoscopic cystectomy; may recur

Answer 35

* Description: Rapidly growing, 2-3% of all ovarian cancers * Presentation: Usually children and young women (\<30 yr) * Treatment: Surgical resection (often conservative unilateral salpingo- oophorectomy ± nodes) ± chemotherapy

Answer 36

* Description: Produces LDH * Presentation: 10% bilateral * Treatment: When diagnosed at stage IA, no adjuvant treatment is indicated If diagnosed at advanced stage, very responsive to chemotherapy, therefore complete resection is not necessary for cure

Answer 37

* Description: No tumour marker identified * Presentation: Almost always unilateral * Treatment: * When diagnosed at stage IA Grade 1, no adjuvant treatment is indicated * When diagnosed at Grade 2-3, either adjuvant chemotherapy or surgical staging * If diagnosed at advanced stage, very responsive to chemotherapy, therefore complete resection is not necessary for cure

Answer 38

* Description: Produces AFP * Presentation: Abdominal pain and pelvic mass * Treatment: * When diagnosed at stage IA Grade 1, no adjuvant treatment is indicated * If diagnosed at advanced stage, very responsive to chemotherapy, therefore complete resection is not necessary for cure

Answer 39

* Description: Produces hCG * Presentation: Precocious puberty and irregular vaginal bleeding * Treatment: * When diagnosed at stage IA Grade 1, no adjuvant treatment is indicated * If diagnosed at advanced stage, very responsive to chemotherapy, therefore complete resection is not necessary for cure

Answer 40

* Description: * Derived from mesothelial cells lining peritoneal cavity * Classified based on histologic type 80-85% of all ovarian neoplasms (includes malignant) * Ultrasound/Cytology: Varies depending on subtype * Treatment: * Borderline: Cystectomy vs. unilateral salpingo-oophorectomy * Malignant * Early stage (stage I): Hysterectomy/BSO/staging (omentectomy, peritoneal biopsies, washings, pelvic and para-aortic lymphadenectomy) * Advanced stage: Upfront cytoreductive (debulking) followed by adjuvant chemotherapy consisting of IV carboplatic/paclitaxel vs. intraperitoneal chemotherapy (stage III) neoadjuvant chemotherapy with IV carboplatin/ paclitaxel, followed by delayed debulking with further adjuvant IV chemotherapy

Answer 41

* Description: * Most common ovarian tumour * 50% of all ovarian cancers * 75% of epithelial tumours * 70% benign * Presentation: 20-30% bilateral * Ultrasound/Cytology: * Lining similar to fallopian tube epithelium * Often multilocular Histologically contain * Psamomma bodies (calcified concentric concretions)

Answer 42

* Description: 20% of epithelial tumours * Presentation: * Rarely complicated by Pseudomyxoma peritoneii: implants seed abdominal cavity and produce large quantities of mucin * Ultrasound/Cytology: * Resembles endocervical epithelium * Often multilocular * May reach enormous size * Treatment: * Poor response to chemotherapy * If mucinous, remove appendix as well to rule out possible source of primary disease

Answer 43

* Surgical resection of tumour * Chemotherapy may be used for unresectable metastatic disease

Answer 44

* Description: From mature fibroblasts in ovarian stroma * Presentation: * Non-functioning * Occasionally associated with Meig’s syndrome (benign ovarian tumour and ascites and pleural effusion) * Ultrasound/Cytology: * Firm, smooth rounded tumour with interlacing fibrocytes

Answer 45

* Description: Can be associated with endometrial cancer Inhibin is tumour marker * Presentation: Estrogen-producing: feminizing effects (precocious puberty, menorrhagia, postmenopausal bleeding) * Ultrasound/Cytology: Histologic hallmark of cancer is small groups of cells known as Call-Exner bodies

Answer 46

* Description: Can measure elevated androgens as tumour markers * Presentation: Androgen-producing: virilizing effects (hirsutism, deep voice, recession of front hairline)

Answer 47

4-8% of ovarian malignancies Krukenberg tumour – metastatic ovarian tumour (usually GI tract, commonly stomach or colon, breast) with “signet-ring” cells

Answer 48

* women with suspected ovarian cancer based on history, physical, or investigations should be referred to a gynecologic oncologist * bimanual examination * solid, irregular, or fixed pelvic mass is suggestive of ovarian cancer * RMI (Risk of Malignancy Index) is best tool available to assess likelihood of ovarian malignancy and need for pre-operative gynecologic oncology referral (see sidebar) * physical exam findings largely dependent on stage of disease * blood work: CA-125 for baseline, CBC, liver function tests, electrolytes, creatinine * radiology * transvaginal ultrasound best to visualize ovaries * CT abdomen and pelvis to look for metastatic disease * bone scan or PET scan not indicated * try to rule out other primary source if suspected, based on: * occult blood per rectum: endoscopy ± barium enema * gastric symptoms: gastroscopy ± upper GI series * abnormal vaginal bleeding: endometrial biopsy to rule out concurrent endometrial cancer; abnormal cervix: need to biopsy cervix (not Pap smear); breast lesion identified or risk factors present: mammogram

Answer 49

RMI = U x M x CA-125 **Ultrasound Findings (1 pt for each)** * Multilocular cyst * Evidence of solid areas * Evidence of metastases * Presence of ascites * Bilateral lesions U = 1 (for U/S scores of 0 or 1) U = 4 (for U/S scores of 2-5) **Menopausal Status** * Postmenopausal: M = 4 * Premenopausal: M = 1 **Absolute Value of CA-125 Serum Level** * For RMI\>200: gynecologic oncology referral is recommended

Answer 50

* least common site for carcinoma of female reproductive system (0.3%) * usually serous epithelial carcinoma * new evidence shows that some serous ovarian cancers originate in the fallopian tube * more common in fifth and sixth decade

Answer 51

* classic triad present in minority of cases, but very specific * watery discharge (most specific): “hydrops tubae profluens” * vaginal bleeding or discharge in 50% of patients * crampy lower abdominal/pelvic pain * most patients present with a pelvic mass (see *Ovarian* *Tumours*, GY41 for guidelines regarding diagnosis/investigation)

Answer 52

* as for malignant epithelial ovarian tumours

Answer 53

* Nabothian cyst/inclusion cyst: no treatment required * endocervical polyps: treatment is polypectomy (office procedure)

Answer 54

* majority are SCC (95%); adenocarcinomas increasing (5%); rare subtypes include small cell, adenosquamous * 8000 deaths annually in North America * annual Pap test reduces a woman’s chance of dying from cervical cancer from 0.4% to 0.05% * average age at presentation: 52 yr old

Answer 55

* at birth, vagina is lined with squamous epithelium; columnar epithelium lines only the endocervix and the central area of the ectocervix (original squamocolumnar junction) * during puberty, estrogen stimulates eversion of a single columnar layer (ectopy), thus exposing it to the acidic pH of the vagina, leading to metaplasia (change of exposed epithelium from columnar to squamous) * a new squamocolumnar junction forms as a result * the transformation zone (TZ) is the area located between the original and the current squamocolumnar junction * the majority of dysplasias and cancers arise in the TZ of the cervix * must have active metaplasia in presence of inducing agent (HPV) to get dysplasia * dysplasia progresses to carcinoma in situ (CIS), which further progresses to invasion * slow process (~10 yr on average) * growth is by local extension * metastasis occurs late

Answer 56

* HPV infection * high risk of neoplasia associated with types 16, 18 * low risk of neoplasia associated with types 6, 11 * \>99% of cervical cancers contain one of the high risk HPV types * high-risk behaviours (risk factors for HPV infection) * multiple partners * other STs (HSVm trichomonas) * early age at first intercourse * high-risk male partner * smoking * poor screening uptake is the most important risk factor for cervical cancer in Canada * at-risk groups include: * immigrant Canadians * First Nations Canadians * geographically-isolated Canadians * sex-trade workers * low socioeconomic status Canadians

Answer 57

* Age influences reliability of test as a tumour marker * 50% sensitivity in early-stage ovarian cancer (poor), therefore not good for screening **Malignant** * Gyne: ovary, uterus * Non-Gyne: pancreas, stomach, colon, rectum **Non-Malignant** * Gyne: benign ovarian neoplasm, endometriosis, pregnancy, fibroids, PID * Non-Gyne: cirrhosis, pancreatitis, renal failure

Answer 58

developing countries and, therefore, is the only gynecologic cancer that uses clinical staging; this facilitates consistent international staging with countries that do not have technologies, such as CT and MRI

Answer 59

* SCC: exophytic, fungating tumour * adenocarcinoma: endophytic, with barrel-shaped cervix * early * asymptomatic * discharge: initially watery, becoming brown or red * postcoital bleeding * late * 80-90% present with bleeding: either postcoital, postmenopausal or irregular bleeding * pelvic or back pain (extension of tumour to pelvic walls) * bladder/bowel symptoms * signs * friable, raised, reddened, or ulcerated area visible on cervix

Answer 60

* colposcopy is a clinical procedure that facilitates identification and biopsy of suspicious cells * in colposcopy: * apply acetic acid and identify acetowhite lesions, punctation, mosaicism, and abnormal blood vessels to guide cervical biopsy * endocervical curettage (ECC) if entire lesion is not visible or no lesion visible * diagnostic excision (LEEP) if: * unsatisfactory colposcopy (poor visualization/access to transformation zone) * discrepancy between cytology, colposcopy, and histological findings * positive findings/glandular abnormalities in endocervical curettage * suspicious for adenocarcinoma in situ (consider cold-knife conization) * recurrence of lesion post-ablation or excision * inability to rule out invasive disease, i.e. large lesions (lesions extending into endocervical canal, extending widely on cervix, or onto vaginal epithelium) * consider cold-knife conization (in OR) if glandular abnormality suspected based on cytology or colposcopic findings due to concern for margin interpretation * tests permitted for FIGO clinical staging include: physical exam (including examination under anesthesia), cervical biopsy (including cone biopsy), proctoscopy/cystoscopy, intravenous pyelogram, ultrasound liver/kidneys, CXR, LFTs * MRI and/or CT and/or PET scan often done to facilitate planning of radiation therapy, results do not influence clinical stage

Answer 61

* is based on cytological results of a Pap test that permits the examination of cells but not tissue structure. * Cervical intraepithelial neoplasia (CIN) or cervical carcinoma is a histological diagnosis, requiring a tissue sample via biopsy of suspicious lesions seen during colposcopy

Answer 62

gestational trophoblastic disease

Answer 63

**Causes of Elevated CA-125** * Age influences reliability of test as a tumour marker * 50% sensitivity in early stage ovarian cancer (poor) – therefore not good for screening **Malignant** * Gyne: ovary, uterus * Non-Gyne: pancreas, stomach, colon, rectum **Non-Malignant** * Gyne: benign ovarian neoplasm, endometriosis, pregnancy, fibroids, PID * Non-Gyne: cirrhosis, pancreatitis, renal failure

Answer 64

* Stage 0 99% * Stage I 75% * Stage II 55% * Stage III 30% * Stage IV 7% * Overall 50-60%

Answer 65

2 vaccines currently approved (Gardasil®, Cervarix®)

Answer 66

* Viral Strains Covered: 16, 18 * Route of Administration: IM * Schedule of Dosing: 0, 1, 6 mo * Side Effects: * Local: redness, pain, swelling * General: headache, low grade fever, GI upset * Approved Age: Females age 10-25

Answer 67

* Viral Strains Covered: 6, 11, 16, 18 * Route of Administration: IM * Schedule of Dosing: 0, 2, 6 mo * Side Effects: * Local: redness, pain, swelling * General: headache, low grade fever, GI upset * Approved Age: Females age 9-45, males age 9-26 * Contraindications: Pregnant women and women who are nursing (limited data)

Answer 68

* should be administered before onset of sexual activity (i.e. before exposure to virus) for optimal benefit of vaccination * may be given at the same time as hepatitis B or other vaccines using a different injection site * not for treatment of active infections * most women will not be infected with all four types of the virus at the same time, therefore vaccine is still indicated for sexually active females or those with a history of previous HPV infection or HPV- related disease * conception should be avoided until 30 d after last dose of vaccination

Answer 69

* Preferred option for biopsy-proven CIN I is observation * Repeat assessment and cytology in 12 mo * Management according to cytology results * If after HSIL or AGC: * Cytology and histology should be reviewed * If discrepancy remains, excisional biopsy may be considered

Answer 70

* Women ≥25 yr * CIN II or III should be treated * Excisional procedures preferred for CIN III * Those with positive margins should have follow-up with colposcopy and directed biopsies and/or endocervical curettage * Women \<25 yr * Same treatment for CN II and CN III: observe with colposcopy at 6-mo intervals for up to 24 mo before treatment considered * During pregnancy * CIN II or III suspected or diagnosed during pregnancy: repeat colposcopy and treatment delayed until 8-12 wk after delivery

Answer 71

* LEEP if future fertility desired (and lesion ≤2 cm) * Simple hysterectomy if future fertility is not desired

Answer 72

* Typically treated with radical hysterectomy and pelvic lymphadenectomy (sentinel nodes under study) * If high chance of adjuvant radiation then consider primary chemoradiation as more morbidity occurs from double-modality treatment (surgery and radiation) * Equal cure rates may be obtained with primary radiation therapy; advantage of surgery: may accurately stage and grade and more targeted adjuvant therapy * Advantage is that ovaries can be spared if pre-menopausal * For fertility preservation (if tumour \<2 cm), may have radical trachelectomy (removal of cervix and parametria) and nodes instead of radical hysterectomy for early-stage disease * Chemoradiation therapy if adverse high-risk prognostic factors on radical surgical specimen, such as: positive pelvic lymph nodes, positive parametria, and/or positive margins or adverse cervical factors (2 or more): deep stromal invasion, size \>4 cm, LVSI

Answer 73

* Primary chemoradiation therapy * CT assess extent of disease: evaluate pelvic and para-aortic nodes * For positive nodes on PET: primary chemoradiation with extended field RT * Hysterectomy generally not suggested following primary treatment with curative intent

Answer 74

* incidence: 1/2200

Answer 75

* if abnormal Pap or suspicious lesion, refer to colposcopy * if diagnostic conization required, should be deferred until second trimester (T2) to minimize risk of pregnancy loss * if invasive cancer ruled out, management of dysplasia deferred until completion of pregnancy (may deliver vaginally) * if invasive cancer present, management depends on prognostic factors, degree of fetal maturity, and patient wishes * general recommendations in T1: consider pregnancy termination, management with either radical surgery (hysterectomy vs. trachelectomy if desires future fertility), or concurrent chemoradiation therapy * recommendations in T2/T3: delay of therapy until viable fetus and C-sectionfor delivery with concurrent radical surgery or subsequent concurrent chemoradiation therapy

Answer 76

* biopsy is necessary to make diagnosis and/or rule out malignancy: * Hyperplastic dystrophy (squamous cell hyperplasia) * Lichen sclerosis * Mixed dystrophy (lichen sclerosis with epithelial hyperplasia)

Answer 77

* surface thickened and hyperkeratotic * pruritus most common symptom * typically postmenopausal women * treatment: 1% fluorinated corticosteroid ointment bid for 6 wk

Answer 78

* subepithelial fat becomes diminished; labia become thin, atrophic, with membrane-like epithelium and labial fusion * pruritus, dyspareunia, burning * ‘figure of 8’ distribution * most common in postmenopausal women but can occur at any age * treatment: ultrapotent topical steroid 0.05% clobetasol x 2-4 wk then taper down, can consider long-term suppression twice a week

Answer 79

* hyperkeratotic areas with areas of thin, shiny epithelium * treatment: fluorinated corticosteroid ointment

Answer 80

* papillary hidradenoma * nevus, * fibroma * hemangioma

Answer 81

* 5% of genital tract malignancies * 90% SCC; remainder melanomas, basal cell carcinoma, Paget’s disease, Bartholin’s gland carcinoma * Type I disease: HPV-related (50-70%) * more likely in younger women * 90% of vulvar intraepithelial neoplasia (VIN) contain HPV DNA (usually types 16, 18) * Type II disease: not HPV-related, associated with current or previous vulvar dystrophy * usually postmenopausal women

Answer 82

* HPV infection * VIN: precancerous change which presents as multicentric white or pigmented plaques on vulva (may only be visible at colposcopy) * progression to cancer rarely occurs with appropriate management * treatment: local excision (i.e. superficial vulvectomy ± split thickness skin grafting to cover defects if required) vs. ablative therapy (i.e. laser, cauterization) vs. local immunotherapy (imiquimod) * history of cervical cancer * cigarette smoking * immunodeficiency

Answer 83

* many patients asymptomatic at diagnosis (many also deny or minimize symptoms) * most lesions occur on the labia majora, followed by the labia minora (less commonly on the clitoris or perineum) * localized pruritus or lesion most common * less common: raised red, white or pigmented plaque, ulcer, bleeding, discharge, pain, dysuria * patterns of spread * local * groin lymph nodes (usually inguinal, then spreading to pelvic nodes) * hematogenous

Answer 84

* ± vulvar biopsy * always biopsy any suspicious lesion * do not remove entire lesion (allows for site identification through sentinel LN injection if malignant)

Answer 85

* depends on stage: particularly nodal involvement (single most important predictor followed by tumour size) * lesions \>4 cm associated with poorer prognosis * overall 5 yr survival rate: 79%

Answer 86

* T1 lesions (tumour confined to vulva; no extension to adjacent perineal structures): radical local excision * T2 lesions (tumour of any size with extension to adjacent perineal structures): modified radical vulvectomy * T3 lesions (extension to any of: proximal 2/3 of urethra, proximal 2/3 of the vagina, bladder mucosa, rectal mucosa, or fixed to pelvic bone): chemoradiation followed by selective resection of residual primary * node positive disease: adjuvant chemoradiation or radiation therapy

Answer 87

Any suspicious lesion of the vulva should be **biopsied**

Answer 88

* inclusion cysts * endometriosis * Gartner’s duct cysts * urethral diverticulum

Answer 89

* cysts form at site of abnormal healing of laceration (e.g. episiotomy) * no treatment required

Answer 90

* dark lesions that tend to bleed at time of menses * treatment: excision

Answer 91

* remnants of Wolffian duct, seen along side of cervix * treatment: conservative unless symptomatic

Answer 92

* can lead to recurrent urethral infection, dyspareunia * treatment: surgical correction if symptomatic

Answer 93

* primary carcinomas of the vagina represent 2-3% of malignant neoplasms of the female genital tract * 80-90% are SCC * more than 50% diagnosed between 70-90 yr old

Answer 94

* associated with HPV infection (analogous to cervical cancer) * increased incidence in patients with prior history of cervical and vulvar cancer

Answer 95

* cytology * significant false negative rate for existing malignancy (i.e. if gross lesion present, biopsy) * colposcopy * Schiller test (normal squamous epithelium takes up Lugol’s iodine) * biopsy, partial vaginectomy (wide local excision for diagnosis) * rule out disease on cervix, vulva, or anus (most vaginal cancers are metastatic from one of these sites) * staging

Answer 96

Grades: analogous to cervical dysplasia

Answer 97

* Most common site is upper 1/3 of posterior wall of vagina * Asymptomatic * Painless discharge and bleeding * Vaginal discharge (often foul-smelling) * Vaginal bleeding especially during/post-coitus * Urinary and/or rectal symptom 2° to compression

Answer 98

* Most are metastatic, usually from cervix, endometrium, ovary, or colon Most primaries are clear-cell adenocarcinomas * 2 types: non-DES and DES syndrome

Answer 99

* radiation therapy: for tumours \>2 cm diameter or tumour involvement of the mid- to low-grade vagina * surgical excision: radical hysterectomy, upper vaginectomy, and bilateral pelvic lymphadenectomy

Answer 100

chemoradiation

Answer 101

* refers to a spectrum of proliferative abnormalities of the trophoblast

Answer 102

* 1/1000 pregnancies * marked geographic variation (as high as 1/125 in Taiwan) * 80% benign, 15% locally invasive, 5% metastatic * cure rate \>95%

Answer 103

* most common type of hydatidiform mole * diffuse trophoblastic hyperplasia, hydropic swelling of chorionic villi, no fetal tissues or membranes present * 46XX or 46XY, chromosomes completely of paternal origin (90%) * 2 sperm fertilize empty egg or 1 sperm with reduplication * 15-20% risk of progression to malignant sequelae

Answer 104

* geographic (South East Asia most common) * others (maternal age \>40 yr, β-carotene deficiency, vitamin A deficiency not proven)

Answer 105

* clinical features often present during apparent pregnancy with abnormal symptoms/findings * vaginal bleeding (97%) * hyperemesis gravidarum (26%) * excessive uterine size for LMP (51%) * hyperthyroidism (7%) * theca-lutein cysts \>6 cm (50%) * β-hCG \>100,000 IU/L * preeclampsia (27%) * no fetal heartbeat detected

Answer 106

gestational trophoblastic disease

Answer 107

* focal trophoblastic hyperplasia and hydropic villi are associated with fetus or fetal parts * often triploid (XXY, XYY, XXX) with chromosome complement from both parents * usually related to single ovum fertilized by two sperm * low risk of progression to malignant sequelae (\<4%) * associated with fetus, which may be growth-restricted, and/or have multiple congenital malformations

Answer 108

* typically present similar to threatened/spontaneous/missed abortion * pathological diagnosis often made after D&C

Answer 109

* quantitative β-hCG levels (tumour marker) abnormally high for gestational age * U/S findings * if complete: no fetus (classic “snow storm” due to swelling of villi) * if partial: molar degeneration of placenta ± fetal anomalies, multiple echogenic regions corresponding to hydropic villi, and focal intrauterine hemorrhage * CXR (may show metastatic lesions) * features of molar pregnancies at high risk of developing persistent GTN post-evacuation * local uterine invasion as high as 31% * β-hCG \>100,000 IU/L * excessive uterine size * prominent theca-lutein cysts

Answer 110

* suction D&C with sharp curettage and oxytocin * Rhogam® if Rh negative * prophylactic chemotherapy of no proven benefit * chemotherapy for GTN if develops after evacuation

Answer 111

* contraception required to avoid pregnancy during entire follow-up period * serial β-hCGs (as tumour marker) every week until negative x 3 (usually takes several wk), then monthly for 6-12 mo prior to trying to conceive again * increase or plateau of β-hCG indicates GTN: patient needs chemotherapy

Answer 112

* diagnosis made by rising or plateau in β-hCG, development of metastases following treatment of documented molar pregnancy * histology: molar tissue from D&C * metastases are rare (4%)

Answer 113

* often present with symptoms from metastases * highly anaplastic, highly vascular * no chorionic villi, elements of syncytiotrophoblast and cytotrophoblast * may follow molar pregnancy, abortion, ectopic, or normal pregnancy

Answer 114

* rare aggressive form of GTN * abnormal growth of intermediate trophoblastic cells * low β-hCG, production of human placental lactogen (hPL), relatively insensitive to chemotherapy

Answer 115

* non-metastatic * metastatic

Answer 116

* ~15% of patients after molar evacuation * may present with abnormal bleeding * all have rising or plateau of β-hCG * negative metastases on staging investigations

Answer 117

* 4% of patients after treatment of complete molar pregnancy * metastasis more common with choriocarcinoma, which tends toward early vascular invasion and widespread dissemination * if signs or symptoms suggest hematogenous spread, do not biopsy (they bleed) * lungs (80%): cough, hemoptysis, CXR lesion(s) * vagina (30%): vaginal bleeding, “blue lesions” on speculum exam * pelvis (20%): rectal bleeding (if invades bowel), U/S lesion(s) * liver (10%): elevated LFTs, U/S or CT findings * brain (10%): headaches, dizziness, seizure (symptoms of space-occupying lesion), CT/MRI findings * highly vascular tumour, which is more likely to bleed and result in anemia * all have rising or plateau of β-hCG

Answer 118

* divided into good prognosis and bad prognosis * features of bad prognosis * long duration (\>4 mo from antecedent pregnancy) * high pre-treatment β-hCG titre: \>100,000 IU/24 h urine or \>40,000 IU/L of blood * brain or liver metastases * prior chemotherapy * metastatic disease following term pregnancy * good prognosis characterized by the absence of each of these features

Answer 119

Lungs are the primary site for malignant GTN metastases; when pelvic exam and chest x-ray are negative, metastases are uncommon

Answer 120

* blood work: CBC, electrolytes, creatinine, β-hCG, TSH, LFTs * imaging: CXR, U/S pelvis only * if CXR shows lung metastasis then CT abdo/pelvis, MRI brain * if suspect brain metastasis but CT brain negative, consider lumbar puncture for CSF β-hCG * ratio of plasma β-hCG:CSF β-hCG \<60 indicates metastases

Answer 121

* contraception for all stages to avoid pregnancy during entire follow-up period * stage I, II, III * weekly β-hCG until 3 consecutive normal results * then monthly x 12 mo * stage IV * weekly β-hCG until 3 consecutive normal results * then monthly x 24 mo

Answer 122

* β-hCG plateau: \<10% drop in β-hCG over four values in 3 wk (e.g. days 1, 7, 14, and 21) OR * β-hCG rise \>20% in any two values over two wk or longer (e.g. measure at days 1, 7, 14) OR * β-hCG persistently elevated \>6 mo OR * metastases on work-up