Type I. Type II. Excess estrogen Lynch Endometrioid adenocarcinoma PTEN. High grade More aggressive Serous, Clear Cell p53

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2 Type I Excess estrogen Lynch Endometrioid adenocarcinoma PTEN Type II High grade More aggressive Serous, Clear Cell p53

3 Stage I IA IB Stage II Stage III IIIA IIIB IIIC IIIC1 IIIC2 Stage IV IVA IVB nodes Tumour confined to the corpus uteri No or less than half myometrial invasion Invasion equal to or more than half of the myometrium Tumour invades cervical stroma, but does not extend beyond the uterus Local and/or regional spread of the tumour Tumour invades the serosa of the corpus uteri and/or adnexae Vaginal and/or parametrial involvement Metastases to pelvic and/or para-aortic lymph nodes Positive pelvic nodes Positive para-aortic lymph nodes with or without positive pelvic lymph nodes Tumour invades bladder and/or bowel mucosa, and/or distant metastases Tumour invasion of bladder and/or bowel mucosa Distant metastases, including intra-abdominal metastases and/or inguinal lymph

4 IIIA- uterine serosal or adnexal involvement IIIB- vaginal and/or parametrial involvement IIIC- pelvic and/or para-aortic nodes (IIIC1- pelvic nodes; IIIC2- para-aortic nodes)

5 to be performed and reported separately ie not part of staging system significance to be discussed at MDTM

6 never had staging system previously carcinosarcomas staged as per uterine carcinomas staging system for leiomyosarcomas different system for ESS and adenosarcoma Local and hematogenous spread

7 Stage I IA IB Stage II IIA IIB Tumour limited to uterus <5 cm >5 cm Tumour extends to the pelvis Adnexal involvement Tumour extends to extrauterine pelvic tissue Stage III Tumour invades abdominal tissues (not just protruding into the abdomen) IIIA One site IIIB > one site IIIC Metastasis to pelvic and/or para-aortic lymph nodes Stage IV IVA IVB Tumour invades bladder and/or rectum Distant metastasis

8 Stage I IA IB IC Stage II IIA IIB Stage III IIIA IIIB IIIC Stage IV IVA IVB Tumour limited to uterus Tumour limited to endometrium/endocervix with no myometrial invasion Less than or equal to half myometrial invasion More than half myometrial invasion Tumour extends to the pelvis Adnexal involvement Tumour extends to extrauterine pelvic tissue Tumour invades abdominal tissues (not just protruding into the abdomen) One site > one site Metastasis to pelvic and/or para-aortic lymph nodes Tumour invades bladder and/or rectum Distant metastasis

9 Squamous Adenocarcinoma Adenosquamous Clinical staging

10 Stage I IA IA1 IA2 IB IB1 IB2 Stage II lower IIA IIA1 IIA2 IIB The carcinoma is strictly confined to the cervix (extension to the corpus would be disregarded) Invasive carcinoma which can be diagnosed only by microscopy, with deepest invasion <5 mm and the largest extension >7 mm Measured stromal invasion of <3.0 mm in depth and extension of <7.0 mm Measured stromal invasion of >3.0 mm and not >5.0 mm with an extension of not >7.0 mm Clinically visible lesions limited to the cervix uteri or pre-clinical cancers greater than stage IA Clinically visible lesion <4.0 cm in greatest dimension Clinically visible lesion >4.0 cm in greatest dimension Cervical carcinoma invades beyond the uterus, but not to the pelvic wall or to the third of the vagina Without parametrial invasion Clinically visible lesion <4.0 cm in greatest dimension Clinically visible lesion >4.0 cm in greatest dimension With obvious parametrial invasion

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12 SCC Melanoma AdenoCa BCC Sarcoma Surgically staged

13 Stage I IA IB Stage II Stage III nodes. IIIA Tumour confined to the vulva Lesions <2 cm in size, confined to the vulva or perineum and with stromal invasions <1.0 mm*, no nodal metastasis Lesions >2 cm in size or with stromal invasion >1.0 mm* confined to the vulva or perineum, with negative nodes Tumour of any size with extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with negative nodes Tumour of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguino-femoral lymph (i) With 1 lymph node metastasis (>5 mm), or (ii) 1-2 lymph node metastasis(es) (<5 mm) IIIB (i) With 2 or more lymph node metastases (>5 mm), or (ii) 3 or more lymph node metastases (<5 mm) IIIC With positive nodes with extracapsular spread Stage IV Tumour invades other regional (2/3 upper urethra, 2/3 upper vagina), or distant structures. IVA Tumour invades any of the following: (i) upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or (ii) fixed or ulcerated inguino-femoral lymph nodes IVB Any distant metastasis including pelvic lymph nodes * The depth of invasion is defined as the measurement of the tumour from the epithelialstromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion.

14 IA- < 2cm, stromal invasion <1mm, confined to vulval or perineum, no nodal metastasis IB- previous IB and II combined- >2cm size or with stromal invasion >1mm, confined to vulval or perineum, no nodal metastasis

15 any size with extension to lower third of urethra, lower third of vagina or anus and negative nodes

16 any size, with or without extension to lower third of urethra, lower third of vagina or anus and positive inguino-femoral nodes IIIA- 1 nodal metastasis > 5mm or up to 2 nodes <5mm IIIB- 2 or more nodes >5mm or 3 or more nodes <5mm IIIC- extracapsular spread

17 upper two thirds of urethra or vagina or distant structures various substages bilateral nodal involvement now not taken into account

18 Surgery WLE- Stage O, stage IA SCC, Pagets, Radical partial vulvectomy all others that are amenable to surgery

19 LNs For greater than stage IA disease Unilateral lesion- unilateral LNDx Central lesion- Bilateral LNDx Sentinel LN Biopsy Radioactive label- Technicium Isosulphan blue intraop injected Resect all hot and blue nodes

20 Locally advanced disease Neoadjuvant chemoradiation

21 Pain and itching Hyperemic, well demarcated lesions, cake icing coating Patchy leukoplakia Approx 10-20% with associated local or distant adenocarcinoma

22 Intraepithelial lesion. Large cells, clear cytoplasm, confined to epidermis, can involve hair follicles. Wide Local Excision Recurrence common regardless of margin status.

23 Epithelial Germ Cell Sex Cord Stromal

24 Serous Mucionous Endometrioid Clear Cell Ca125, CEA, Ca19-9 Endometriosis link to clear cell and endometriois

25 Most sporadic Familial Breast Ovarian type BRCA 1 & 2 Carboplatin- N/V, hypersensitivity Paclitaxel- alopecia, neuropathy

26 Dysgerminoma +LDH Choriocarcinoma + HCG Yolk Sac Tumor + AFP Immature Teratoma

27 Chemosensitive Affects younger patients Fertility preservation Bleomycin- Pulm Fibrosis Etoposide- Secondary malignancy Cisplatin- Renal impairment

28 Granulosa Cell tumors Increased estrogen, concurrent endometrial Ca, Inhibin Fibro-thecomas Sertoli-Leydig tumors Virilising Gynandroblastoma

29 Rare More likely to be secondary than primary ca. If cervix or vulva involved, then is classified as this primary Types- SCC Clear cell Melanoma Sarcoma

30 Staging- Clinical, not surgical, ie exam and imaging Stage 0-CIS Stage I- Vaginal Wall Stage II- Subvaginal wall Stage III-Pelvic wall Stage IVA- Invasion of rectal or bladder mucosa Stage IVB- Distant spread

31 Upper vagina, spread as for cervix ca Distal vagina, spread as for vulva ca Surgery an option for CIS and stage I disease. Radiation +/-chemo for others. External beam, tandem and ovoid or interstitial implant.

32 Clear cell ca Related to vaginal adenosis Linked to DES exposure, this population is aging out. Arise secondary to endometriosis.

33 Melanoma Typically advanced at diagnosis. Sarcoma Spindle cell- LMS, fibrosarcoma. Local excision and radiation

34 Sarcoma botryoides Children Anterior upper vagina Cluster of grapes, vaginal bleeding Rx- Chemotherapy, (VAC, vincrisitne, actinomycin D, cyclophosphimide), surgery, sometimes just chemoradiation if small lesion.

35 Partial Mole 69XXY Complete Mole 46XX

36 Invasive Mole Choriocarcinoma Placental site trophoblastic tumor Epithelioid trophoblastic tumor May follow any pregnancy, not just molar gestation

37 Stage I-Confined to uterus Stage II- Limited to genital structures Stage III- Lungs Stage IV- Any other spread WHO score Seven or greater is high risk disease (Age, Antecedant pregnancy, Time from pregnancy, HCG, Site, number and size of mets, prior chemo)

38 Non metastatic disease- single agent chemo Methotrexate vs actinomycin D Low risk metastatic- Single agents still, but more intense regimen High risk disease- Multiagent chemotherapy- EMACO

39 Treat until normal HCG then 2 further cycles. Survail for 12 months with monthly HCGs

40 Trophoblastic tumor- surgery, more chemoresistant.