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Staging of Ovarian, Fallopian Tube, and Primary Peritoneal Carcinoma

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Staging of Ovarian, Fallopian Tube, and Primary Peritoneal Carcinoma Appendix A: Staging of Ovarian, Fallopian Tube, and Primary Peritoneal Carcinoma Introduction correct determination of tumor subtype is there- fore of as much signifi cance as disease stage, if Cancer staging is vital for correct patient man- not of greater signifi cance, a factor even more agement, is based on the biology of the individual relevant in the evolving era of personalized medi- tumor type, and is determined by internationally cine and targeted treatment. The histotype of agreed protocols which enable comparison and ovarian cancer is therefore included in the 2013 sharing of experience. The major staging body staging system as it is recognized to be funda- for gynecological cancers is FIGO, and the UICC mental to successful treatment. The fi ve major has broadly developed parallel and concordant histotypes of ovarian carcinoma are listed below: systems of TNM staging. The FIGO staging for • High-grade serous carcinoma (HGSC) ovarian cancer has been revised in 2013 • Clear cell carcinoma (CCC) (Table A1 ). The previous staging system was • Endometrioid carcinoma (EC) developed in 1988, and two major changes led to • Low-grade serous carcinoma (LGSC) recognition of the need to change the existing • Mucinous carcinoma (MC) system: fi rstly, the knowledge that ovarian can- cers are not a single disease with varying mor- phology but fall into fi ve separate discrete major Pathogenesis of High-Grade disease categories and, secondly, insight into the Serous Carcinoma origins of the most common of the subtypes, high-grade serous carcinoma (HGSC). These The second signifi cant discovery infl uencing the factors are considered below. current approach to staging was the recognition in 2001 that a high percentage of HGSC arise in the fi mbrial end of the fallopian tube. This dis- Histological Types covery was made in risk reducing salpingo- of Ovarian Carcinoma oophorectomy specimens (RRSO) in BRCA1 mutation carriers which were found to harbor The majority (90 %) of ovarian malignancy is of serous tubal intraepithelial carcinoma (STIC). epithelial origin, i.e., carcinoma, with the remain- Since then STIC has been found in signifi cant ing 10 % being predominantly of sex cord- numbers of sporadic “ovarian” and “primary stromal or germ cell origin. It is now clear that peritoneal” carcinomas. Although there is com- there are fi ve major subtypes of ovarian carcinoma pelling evidence that the majority of HGSC arise that differ in their origins, morphology, immuno- from the fallopian tube through studies on RRSO histochemical staining characteristics, and specimens, it is also possible that in some cases molecular genetics. These tumor subtypes differ STIC represents secondary involvement or evi- in their behavior and response to treatment. The dence of multicentric tumor origin. These N. Wilkinson (ed.), Pathology of the Ovary, Fallopian Tube and Peritoneum, 497 Essentials of Diagnostic Gynecological Pathology, DOI 10.1007/978-1-4471-2942-4, © Springer-Verlag London 2014 498 Appendix A: Staging of Ovarian, Fallopian Tube, and Primary Peritoneal Carcinoma Table A1 FIGO staging to be used from January 2014: for ovary, fallopian tube, and peritoneum [ 4 ] Existing FIGO stage group 2013 description Stage 1 Stage 1 : Tumor confi ned to ovaries or fallopian tube ( s ) IA 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 IB 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 IC ( Stage 1C ) Tumor limited to 1 or both ovaries or fallopian tubes , with any of the following : IC1: Surgical spill IC2: Capsule ruptured before surgery or tumor on ovarian or fallopian tube surface IC3: Malignant cells in the ascites or peritoneal washings Stage 2 ( Stage 2 ) Tumor involves 1 or both ovaries or fallopian tubes with pelvic extension ( below pelvic brim ) or primary peritoneal cancer IIA IIA: Extension and/or implants on uterus and/or fallopian tubes and/or ovaries IIB IIB: Extension to other pelvic intraperitoneal tissues Stage 3 Stage 3 : Tumor involves 1 or both ovaries or fallopian tubes , or primary peritoneal cancer , with cytologically or histologically confi rmed spread to the peritoneum outside the pelvis and / or metastasis to the retroperitoneal lymph nodes IIIA IIIA1: Positive retroperitoneal lymph nodes only (cytologically or histologically proven) 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 IIIB IIIB: Macroscopic peritoneal metastasis beyond the pelvis up to 2 cm in greatest dimension, with or without metastasis to the retroperitoneal lymph nodes IIIC 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) Stage 4 Stage 4 : Distant metastasis excluding peritoneal metastases IV IVA: Pleural effusion with positive cytology IVB: Parenchymal metastases and metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside of the abdominal cavity) The stage values shown in bold on the left hand column correspond to the 2013 stage values shown in the right hand column and should be used until the options have been updated and aligned to the 2013 classifi cation alternative possibilities cannot be ruled out at tube, even if this is not demonstrable pathologi- present. It is also relevant that some cases of cally at the time of presentation. Furthermore ovarian and primary peritoneal carcinoma do not HGSC, which accounts for the majority of “ovar- show STIC lesions despite complete examination ian” carcinomas and also the majority of fatal of the fallopian tube using a standard protocol. cases, shows identical clinical behavior, includ- While the putative precursor of HGSC is eas- ing progression patterns and responses to chemo- ily identifi ed in RRSO specimens, HGSCs gener- therapy, irrespective of apparent site of origin. ally present at high stage, at which point any For these reasons the FIGO 2013 staging system precursor lesions are largely obscured. It is how- has been unifi ed for primaries of each of these ever clear that HGSC, whether presenting as three sites. Proposals to unify HGSC of ovarian, “tubal,” “ovarian,” or “primary peritoneal” carci- tubal, and peritoneal origins as “pelvic serous noma based on criteria formerly used to assign carcinoma” were rejected to avoid confusion by primary site, represents a single disease with the introducing new and ambiguous terminology. As majority of cases probably arising in the fallopian part of staging, however, the primary site for each Appendix A: Staging of Ovarian, Fallopian Tube, and Primary Peritoneal Carcinoma 499 case needs to be designated as ovarian, tubal, or origin is uncertain, the convention of designating primary peritoneal. It is recognized that this may all serous carcinoma as originating in the ovary not be possible in some cases and these should be should not be used” [ 2 ] and “the term HGSC of categorized as undesignated. ovary should be kept until the different origins The remaining minority of ovarian carcinoma are better understood” [ 1 ]. subtypes, namely, clear cell, endometrioid, low- In this scenario there is high potential for grade serous, and mucinous carcinomas, have categorizing identical cases differently. For distinct precursors and origins. The 2013 FIGO example, high-grade serous carcinoma with staging system is applicable to these subtypes. low-volume ovarian involvement, widespread peritoneal spread, and STIC in the fallopian tube could potentially be categorized as being of Determining the Site of Origin fallopian tube, ovary, primary peritoneal, or of High-Grade Serous Carcinoma undesignated origin by different pathologists. Similarly it is also possible for an ovarian mass Complete Sampling with complete obscurement of the fallopian tube of the Fallopian Tube where demonstration of a precursor lesion is not possible and an identical case of advanced ovar- A fundamental step in pathological evaluation of ian carcinoma in the presence of demonstrable cases of ovarian, tubal, and primary peritoneal STIC to be categorized differently as ovarian/ carcinoma has emerged from the aforementioned undetermined and fallopian tubal, respectively, developments, which is detailed examination of if STIC is taken to be proof of origin whenever the fi mbrial end of the fallopian tube. The recom- it is found. Another diffi cult scenario is assign- mended protocol, designated SEE-FIM, enables ing origin to a large ovarian mass which obscures examination of the fi mbrial end for the presence the ipsilateral fallopian tube in the presence of of STIC, as detailed in Chap. 20 , Specimen STIC on the contralateral fallopian tube. One Cutup . This step is essential for correct staging as further confounding factor is the aggressive the changes are not visible to the naked eye and nature of STIC; it is accepted that this lesion, there is a potential to under-stage disease if these although intraepithelial, is capable of wide- are missed. In addition, although currently there spread metastasis and presentation as
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