2009 USCAP Gyn Pathology Evening Session Case #3. Richard J. Zaino, MD Hershey Medical Center Penn State University Hershey, PA

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1 2009 USCAP Gyn Pathology Evening Session Case #3 Richard J. Zaino, MD Hershey Medical Center Penn State University Hershey, PA

2 Clinical history Middle aged woman with an exophytic mass of the vagina

3 biopsy

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5

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7 Differential diagnosis of adenocarcinoma in the vagina Primary vaginal adenocarcinoma (clear cell ca in adenosis DES, other) Secondary adenocarcinoma of the vagina Locally advanced endometrial adenocarcinoma Recurrent endometrial adenocarcinoma Non-endometrial adenocarcinoma

8 Carcinoma in the vagina* Primary 16% Secondary 84% Cervix 32% Corpus 18% Colon 9% Ovary 6% Vulva 6% Urinary 4% *all cell types

9 Differential diagnosis Primary vaginal adenocarcinoma (clear cell ca in adenosis DES, non-des) Secondary adenocarcinoma of the vagina Locally advanced endometrial adenocarcinoma Recurrent endometrial adenocarcinoma Non-endometrial adenocarcinoma

10 Insert photo

11 Vaginal adenocarcinoma DES related (3rd decade of life) DESAD project thru cases cases of clear cell carcinoma 11 DES exposed Non-DES related (7th decade of life) rare, with unknown risk factors majority of newly diagnosed cases of vaginal adenocarcinoma since 2000 occasionally arise in endometriosis

12 Differential diagnosis Primary vaginal adenocarcinoma (clear cell ca in adenosis DES, other) Secondary adenocarcinoma of the vagina Locally advanced endometrial adenocarcinoma Recurrent endometrial adenocarcinoma Non-endometrial adenocarcinoma

13 Surgical Staging of Corpus Cancer (FIGO,1988) Stage IA G123 IB G123 IC G123 IIA IIB IIIA IIIB IIIC IVA IVB Characteristics tumor limited to endometrium invasion to inner half of myometrium invasion to outer half of myometrium endocervical gland involvement cervical stromal invasion tumor invades serosa, adnexa, or + peritoneal cyto vaginal metastases pelvic or para-aortic lymph node metastases tumor invades bladder or bowel mucosa distant, intraabdominal or inguinal node metastases

14 Stage IIIB Corpus Cancer: vaginal metastasis Vaginal extension or metastasis often associated with nodal or distant metastases (Stage IIIC or IVB) Isolated vaginal metastases are rare

15 Stage IIIB endometrial adenocarcinoma Author Frequency Creasman* 1 % Nicklin 1 % Greven 3 % Aoki 2 % *FIGO data ~ total 8000 cases

16 Stage IIIB endometrial adenocarcinoma Author Frequency 5 yr survival Creasman* 1 % 30% Nicklin 1 % 13% *FIGO data ~ total 8000 cases

17 A slight diversion

18 Surgical Staging of Corpus Cancer (FIGO,1988) Stage IA G123 IB G123 IC G123 IIA IIB IIIA IIIB IIIC IVA IVB Characteristics tumor limited to endometrium invasion to inner half of myometrium invasion to outer half of myometrium endocervical gland involvement cervical stromal invasion tumor invades serosa, adnexa, or + peritoneal cyto vaginal metastases pelvic or para-aortic lymph node metastases tumor invades bladder or bowel mucosa distant, intraabdominal or inguinal node metastases

19 Tentative staging conclusions Stage IA cannot reliably be distinguished from Stage IB pathologically in many cases Stage IIA and IIB are poorly defined pathologically and may not differ prognostically Stage II is probably not prognostically significant Stage III disease is very heterogeneous Stage IIIA alone is heterogeneous + cytology alone is rare and probably significant but with small effect (85%) + adnexa is more significant (70%) + uterine serosa carries a poor prognosis (30%)

20 Tentative staging conclusions Stage IIIB (vaginal mets) very rare, poor prognosis (25%) Stage IIIC + pelvic nodes significant (70%) + paraaortic nodes significantly worse (30%) grossly positive nodes; capsular invasion and desmoplasia; other extrauterine sites associated with a much worse outcome (Stage IIIC limited to nodes usually fails in nodal area)

21 Surgical Staging of Corpus Cancer (EGO, 2008) Stage IA G123 IB G123 IIA IIB IIIA1 IIIA2 IIIB1 IIIB2 IVA IVB Characteristics invasion to endometrium/inner half of myometrium invasion to outer half of myometrium positive peritoneal cytology uterine serosa, adnexal or other pelvic spread pelvic nodal metastasis (micro) pelvic nodal metastasis (gross) paraaortic nodal metastasis (micro) paraortic nodal metastasis (gross) vaginal metastases, invasion of bladder or bowel mucosa distant, intraabdominal or inguinal node metastases

22 Beyond EGO ISGP working with FIGO Proposed 2008 FIGO Staging of uterine corpus carcinoma Jaime Pratt (ISGP president) Lora Hedrick Ellenson

23 Surgical Staging of Corpus Cancer (proposed FIGO, 2008) Stage IA G123 IB G123 II IIIA IIIB IIIC1 IIIC2 IVA IVB Characteristics tumor to endometrium/inner half of myometrium invasion to outer half of myometrium endocervical cervical stromal invasion tumor invades serosa, adnexa vaginal metastases or parametrial extension pelvic lymph node metastases para-aortic lymph node metastases tumor invades bladder or bowel mucosa distant, intraabdominal or inguinal node metastases

24 Differential diagnosis Primary vaginal adenocarcinoma (clear cell ca in adenosis DES, other) Secondary adenocarcinoma of the vagina Locally advanced endometrial adenocarcinoma Recurrent endometrial adenocarcinoma Non-endometrial adenocarcinoma

25 Recurrent/metastatic endometrial adenocarcinoma Local/regional recurrence Vaginal cuff (most common) Pelvic Distant recurrence

26 Vaginal recurrence of early stage endometrial adenocarcinoma Author frequency Creutzberg 5% Wylie 5% Salihoglu 2% Kohlberger 12% Ng 9%

27 Vaginal recurrence of endometrial adenocarcinoma Author frequency 5 yr survival* Creutzberg 5% ~ 60% Wylie 5% ~ 65% ~ 80% upper third ~ 20% lower third *radiation or surgery

28 Differential diagnosis Primary vaginal adenocarcinoma (clear cell ca in adenosis DES, other) Secondary adenocarcinoma of the vagina Locally advanced endometrial adenocarcinoma Recurrent endometrial adenocarcinoma Non-endometrial adenocarcinoma

29 Carcinoma in the vagina Primary 16% Secondary 84% Cervix 32% Corpus 18% Colon 9% Ovary 6% Vulva 6% Urinary 4%

30 Additional history Patient sent to Gyn Oncologist for evaluation of vaginal mass about the size of a flattened golf ball Remote history of Hodgkin s Disease Long history of Crohn s Disease with crampy abdominal pain, intermittent diarrhea and perianal fistulas

31 biopsy

32 ER

33 CK7

34 CK20

35 Cdx-2

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38 Total procto-colectomy, posterior vaginectomy, TAH, BSO 7x4x3 cm polypoid mass arising in the anterior rectum, extending thru a rectovaginal fistula Extensive perineural and lymphatic invasion Extension into paravaginal skeletal muscle, within 1 mm of margin 72 lymph nodes, negative for tumor

39 Final diagnosis Adenocarcinoma of the rectum arising in Crohn s Disease with a fistulous extension into the vagina

40 Gyn involvement in Crohn s disease common about 25% of women vaginal/vulvar pain Labial swelling Recto-vaginal fistulas Ano-vaginal fistulas Carcinoma arising in vaginal fistula (3 cases reported) (Moore-Maxwell, 2004, Buchanon, 1980)

41 Rectal/anal adenocarcinoma in Crohn s disease Epidemiological studies are contradictory Increased risk if severe proctitis/perianal disease Outcome same stage for stage Often of advanced stage at diagnosis High risk groups for surveillance: extensive colitis, severe proctitis, rectal remanent, strictures, bypassed segments, sclerosing cholangitis

42 Conclusion 1) Mucinous adenocarcinoma of the colon may mimic primary mucinous adenocarcinomas of the ovary 2) Non-mucinous adenocarcinomas of the colon may mimic advanced or recurrent adenocarcinoma of the endometrium