# Presentation: 132454

**Centers for Disease Control and Prevention**
**National Program of Cancer Registries**
**Atlanta, Georgia**
**www.cdc.gov/cancer/npcr**

**NPCR Education and Training Series (NETS)****Module 6: GYN Malignancies**
**Part 1**
**Prepared by Scientific Applications International Corporation (SAIC)**** CDC Contract Number 200–2002–00576–0004**

## Advanced Abstracting: Assumptions

**This presentation assumes**
**You have a good grasp of basic abstracting**
**You know many of the common abbreviations **
**There’s no need to cover topics completely **
**That it’s OK to focus on the abstracting rules and situations that cause problems for experienced abstractors**

##  I. INTRODUCTION

** ****I. INTRODUCTION**
**Site Profiles, Abstracting Issues, Data Sets **

**Notes:**
This section of advanced abstracting for the gynecologic cancers discusses incidence, symptoms, diagnostic procedures, the anatomy of the female genital tract, abstracting issues, and other aspects of the diagnosis of GYN cancers.

## GYN SITES

**C51 – Vulva**
**C52 – Vagina**
**C53 – Cervix**
**C54 – Corpus Uteri**
**C55 – Uterus, NOS**
**C56 – Ovary**
**C57 – Fallopian tube, other and unspecified GYN**
**C58 – Placenta**

**Vulva **
**                **

**Notes:**
Cancers can occur in any site within the female genital tract.  

C51 -- The vulva is the external organ of the female genital tract.  It includes the labium majus, labium minus, the clitoris and the skin of the vulva.  Important to know: a skin cancer of the vulva is not coded to C44 skin.

C52 – The vagina is a hollow, cylindrical organ leading from the uterus to the vulva.

C53 – The cervix or cervix uteri is the narrow neck of the uterus and partially protrudes into the vagina.  It is the lower 1/3 of the uterus.

C54 – The body or corpus of the uterus is the upper 2/3 of the uterus.

C55 – Uterus, NOS, refers to uterine sites not specified as being cervix or uterine corpus primaries.

C56 – Ovary, one of the few cancer sites where bilateral involvement may be abstracted as a single site.

C57 – Catch-all topographical category for uterine adnexal sites such as the fallopian tubes and other specified and unspecified GYN sites.

C58 – Placenta, of course, is a specialized organ that is present only during pregnancy.

## Types of GYN Cancers

**Epithelial carcinoma**
**Squamous cell carcinoma**
**Adenocarcinoma**
**Sarcoma**
**Germ cell tumor**
**Mullerian mixed tumor**
**Melanoma**

**Notes:**
The types of cancers that develop within the female genital tract usually depend on the specific GYN primary site that produces the malignant cells.  The following are most common:

Squamous cell carcinoma – most frequent for vulva, vagina, and exocervix.

Adenocarcinoma – most frequent for endocervical glands, endometrium, and ovaries.

Sarcoma – leiomyosarcomas arise from smooth muscle (myometrium) in the uterine wall, mullerian mixed tumors and endometrial stromal sarcomas arise in stromal tissue in the endometrial epithelium, and carcinosarcomas have features of both carcinomas and sarcomas, although categorized as a sarcoma. 

Germ cell tumors – occur almost always in the ovaries.

GYN melanomas – usually occur in the skin of the vulva.

As we look at each GYN site, we will review the types of cancers most associated with that site. This information can be found in Book 4, SEER Self Instructional Manuals, the SEER Web site Cancer Stat Fact Sheets and the American Cancer Society Web site Cancer Reference Information.

## Incidence of GYN Cancers

** ****74,788 new cases occurred in 2004**
**Almost half were uterine corpus (including NOS)**
** ****Almost 30% were ovary**
** ****Of all cancer sites among women**
** ****Uterine corpus is 4****th**** most common**
** ****Ovary is 8****th**** most common**

**Notes:**
Data are from selected statewide and metropolitan area cancer registries that meet the data quality criteria for all invasive cancer sites combined. Counts cover approximately 98% of the U.S. population.  

Source: U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2004 Incidence and Mortality Web-based Report. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2007. Available at: www.cdc.gov/uscs.

## GYN Incidence Comparisons, 2004

| CANCER SITE | RATE* |
| --- | --- |
| GYN total | 47.1 |
| Uterine-Corpus and NOS | 23.1 |
| Ovary | 12.5 |
| Cervix |   7.9 |
| Vulva |   2.2 |
| Vagina |   0.7 |
| Other GYN |   0.7 |
| *Rates are per 100,000 persons and are age-adjusted to the 2000 U.S. standard population (19 age groups – Census P25–1130)  |  |

**Notes:**
Notice that the uterine, ovarian and cervical cancers are by far the most common GYN sites.  Vulvar cancers occur much less frequently, about a fourth as frequently as cervical cancers.  Vaginal cancers are less than a third as common as vulvar cancers.  All other GYN cancers combined, which include fallopian tube and placenta, occur about as frequently as vaginal cancers.  Uterine NOS cancers, where the abstractor was unable to determine from the medical record documentation where in the uterus the cancer originated are frequently lumped together with uterine corpus in published statistics.  Uterus NOS accounts for approximately 3% of the uterine cancers combined.

These incidence statistics were obtained from the United States Cancer Statistics: 2004.

U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2004. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2007. Available at: www.cdc.gov/uscs.
Data are from selected statewide and metropolitan area cancer registries that meet the data quality criteria for all invasive cancer sites combined. Rates cover approximately 98% of the U.S. population. 

## Prevalence of GYN Cancers

**The number of GYN cancer patients alive at any given time**
**Prevalence on 1/1/04**
**568,407 –  Uterus**
**250,726 – Cervix**
**172,765 – Ovary**

**Notes:**
Prevalence includes all GYN cancer patients alive on a specific date.  It includes the following populations:
Those diagnosed on or before the date
Those with active GYN cancer on the date
Those who previously had GYN cancer, but were in remission or considered cured on the date.

Note that while the incidence of ovarian cancer is higher than cervical cancer on the previous slide, many more cervical cancer patients are alive at any point in time.  This results from the higher mortality of ovarian cancer.  

These figures for selected GYN sites are from the SEER Web site, Fast Stats, U.S. Estimated Complete Prevalence Counts on January 1, 2004.

## GYN Mortality

** ****27,049 deaths due to GYN cancers occurred in 2004**
** ****54% ****– ovarian cancer**
** ****26% ****– uterine cancer**
** ****Of all cancer deaths among women**
** ****Ovary is 5****th**** most common**
** ****Uterine corpus is 8****th**** most common**

**Notes:**
Ovarian cancer will account for over half of GYN cancer deaths.  Ovarian cancer ranks 5th behind lung, breast, colorectal, and pancreatic cancers as the most common cause of cancer deaths among women.  Uterine cancer will account for over one-fourth of GYN cancer deaths.

Data are from selected statewide and metropolitan area cancer registries that meet the data quality criteria for all invasive cancer sites combined. Counts cover approximately 98% of the U.S. population. 

Source: U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2004 Incidence and Mortality Web-based Report. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2007. Available at: www.cdc.gov/uscs.

## Treatment of GYN Cancers

**Standards of care**
**Example: College of American Pathology (CAP) protocols for path reports**
**National treatment guidelines**
**Web resources**
**National Cancer Institute (cancer.gov)**
**NCCN Clinical Practice Guidelines (nccn.org)**
**Treatment varies with the GYN site**

**Notes:**
The College of American Pathology developed protocols for standardizing the information in path reports. The protocols provide a practical tool for ensuring the most important information needed for diagnosis, staging, and treating are included.  They also provide a format that makes this information more quickly visualized and assimilated. And the protocols enable the information to be transferred from computer to computer.

The National Comprehensive Cancer Network (NCCN) designs and maintains clinical practice guidelines used by many if not most cancer programs. These site-specific treatment care maps already cover all major sites and more are being developed for less common sites.  These cancer management plans are evidence-based and are modified as research develops new understandings and new therapies of cancers. GYN guidelines have been developed for cervical, uterine and ovarian cancers. 

The National Cancer Institute (NCI) Web site has a list of cancers A-Z with information on site-specific standards of care by stage of disease, plus information on prevention and screening.  GYN cancers covered by the NCI Web site include cervical, uterine, ovarian, vaginal, vulvar, and gestational trophoblastic tumors (placental).

The American Society of Clinical Oncology  (ASCO) also makes treatment recommendations. The American Cancer Society Web site has good general information on cancer sites.

When looking for GYN information on Web sites, check also for terms like endometrium or germ-cell tumors, which sometimes are GYN topics covered separately from articles named for body organs.

It is sometimes difficult to find information on these web sites for fallopian tube and other non-major GYN cancer sites. 

## Clinical Follow-up 

**Follow-up critical to GYN management**
**Data from clinical follow-up provides**
**Understanding of disease processes**
**Evaluation of treatment modalities**
**Indication of areas for research**
**Clinical follow-up is just good medicine**

**Notes:**
Cancer is a chronic disease that requires life-time management. Management of cancer includes treatment at the time the cancer is diagnosed and clinical follow-up after treatment is completed.  

Different types of cancers have different recurrence rates.  Even among GYN cancers recurrence rates vary from site to site and histology to histology. Times to recurrence vary even among individual patients.  How much of the difference can be attributed to variations in the cancers or in cancer therapy or the general health of the patient is the subject of many clinical studies.

Data collected by cancer registries help establish probabilities of recurrence for different cancers and for different characteristics for the same cancer.  For example, cancer registry data collected over many years by multiple cancer registries helps establish probable rates of recurrence for different stages of GYN cancers. 

## Corpus Uteri

**Upper 2/3 of uterus**
**Most common GYN cancer site**
**50% of GYN cancers**
**6% of female cancers**

- © 2004. A.D.A.M., Inc.

**Notes:**
The corpus uteri is the upper 2/3 or body of the uterus.  The cervix is the lower 1/3. The corpus is the most common GYN cancer site.  Approximately 50% of all GYN cancers occur here.  By itself, corpus accounts for about 6% of all cancers in women.

The word cervix means “neck” and is the lower, neck-shaped part of the uterus. This also explains why cervical (neck) lymph nodes are located very distant from the cervix.  Corpus means “body” and is the bulkier, main part of the uterus.

## Corpus Uteri: Anatomy

**A**** Endometrium**
**B**** Myometrium**
**C ****Fundus**
**D**** Internal os (cervix)**

**Notes:**

Endometrium – the lining of the uterine body (corpus uteri)
 95% or more of uterine cancers occur in the endometrium, which also means...
Largest number of GYN cancers occur in the endometrium
Per NCI Cancer Topics, endometrial cancer by itself is the 6th most common female cancer and is the reason uterine corpus is the 6th most common female cancer site.

Myometrium – the thick muscular wall of the uterine body
Fewer than 5% of uterine cancers originate in the myometrium—also per NCI Cancer Topics.

Fundus – the area of the uterus superior to (above) the fallopian tube orifices

Internal os – marks the point at which the cervix uteri begins.  The topography code for a cancer of the internal os is endocervix (C53.0)

## Corpus Uteri: Profile

**Lifetime risk – 1 in 41 **
**Median age at diagnosis – 63**
**Typical symptoms**
**Vaginal bleeding**
**Pelvic pain and/or mass**
**Screening available – No**

**Notes:**
Because the myometrium is the site of very few cancers, few statistics are available for this corpus subsite.  

Per SEER Stat Fact Sheets (on-line), the lifetime risk of developing cancer of the corpus is about 2.5%, which equates to 1 in 41.  However, only 1 in 200 women die of uterine cancer.  The dramatic difference between incidence and mortality is an indication of this cancer’s curability.

Per NCI Cancer Topics (on-line), higher risk for endometrial cancer has been associated with long-term estrogen exposure, Tamoxifen therapy, obesity, first pregnancies later in life or no pregnancies, hormone therapy for menopause and long-term use of birth control pills that do not include progesterone.

Based on NCI data from the years 2000 to 2004, the median age for developing uterine cancer is 63, which means 50% of women are diagnosed at ages younger than 63 and 50% are diagnosed at age 63 or older.

There is no effective screening tool for discovering occult cancers of the uterus.  Studies have been done using pelvic ultrasound, but this test results in too many false positives and too many false negatives to be of screening value.  Fortunately, per SEER Stat Fact Sheets, 70% of the diagnoses of this cancer occur while the tumor is still localized (confined to the uterus).  Localized cancer of the corpus is highly curable with a five-year survival rate of 95%.

## Corpus Uteri: Histologies

**Endometrial carcinomas**
**Endometrioid adenoCa**
**Mixed**
**Uterine papillary serous**
**Sarcomas**
**Endometrial stromal sarcoma **
**Mixed mesodermal **
**Leiomyosarcoma**

**Notes:**
Endometrial carcinomas (NCI Cancer Topics on Endometrial Cancer Treatment)
75 – 80% of endometrial cancers are some type of endometrioid adenocarcinoma, including ciliated, secretory, papillary, villoglandular, adenoacanthoma or adenosquamous.
10% of endometrial cancers are some type of mixed histology.
Less than 10% are uterine papillary serous.
About 4% are clear cell.
The remaining less than 5% are mucinous adenocarcinoma. squamous cell, undifferentiated and other histologies.

Sarcomas (NCI Cancer Topics on Uterine Sarcoma Treatment)
Less than 1% of GYN cancers are uterine sarcomas.
Sarcomas comprise only 2%-5% of uterine cancers.
50% of uterine sarcomas are mixed mesodermal sarcomas such as carcinosarcoma and mullerian mixed tumor that originate in the stroma of the endometrial epithelium.
30% of uterine sarcomas are leiomyosarcomas that originate in the myometrium.
15% are endometrial stromal sarcomas.

## Ovary

** ****Bilateral ovoid glands, 2****–4 cm in size**
** ****2****nd**** most common GYN cancer site**
** ****27% of GYN cancers**
** ****3% of all cancers among women**

- © 2004. A.D.A.M., Inc.

**Notes:**
The ovaries are oval-shaped organs on each side of the uterus, about the size of an English walnut.
Functions of ovaries:
Produce reproductive eggs
Primary source of female hormones estrogen and progesterone

Ovary is second to corpus uteri as the most common GYN cancer site.  It accounts for 27% of all GYN cancers and 3% of all cancers among women.

Data are from selected statewide and metropolitan area cancer registries that meet the data quality criteria for all invasive cancer sites combined. Counts cover approximately 96% of the U.S. population. 

Source: U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2004 Incidence and Mortality Web-based Report. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2007. Available at: www.cdc.gov/uscs.

## Ovary: Anatomy

**Types of tissue**
**Germ cell**
**Stromal**
**Epithelial**
**Graafian follicles**
**Corpus luteum**

**Notes:**
Ovaries consist of three main types of tissues:
Germ cell tissue – exists inside of the ovary; makes the eggs or ova. 
Stromal cell tissue – exists inside of the ovary; connective tissue that makes most of the progesterone and estrogen for the body.
Epithelial cell tissue – 
Makes up the covering of the ovary,
Makes up the lining of most ovarian cysts—most ovarian malignancies develop from these cells.

Graafian follicles – structures imbedded in stromal connective tissue that mature on a monthly cycle and expel eggs; while the follicle is maturing, certain cells that surround the developing egg produce estrogen hormones.

Corpus luteum  – a temporary endocrine gland that develops from the graafian follicle wall after ovulation and produces progesterone until a programmed degenerative process shrinks the structure and eliminates it; progesterone is produced for about two weeks if the egg is not fertilized and for an extended period of time during the pregnancy if the egg is fertilized.

## Ovary: Profile

**Lifetime risk – 1 in 67 **
**Median age at diagnosis – 63**
**Typical symptoms**
**Asymptomatic in early stages**
**Pelvic pressure (late stages)**
**Abdominal bloating (late stages)**
**Screening available – No**

**Notes:**
Per SEER Stat Fact Sheets, the lifetime risk of getting ovarian cancer is about 1.4%, or 1 oin67 women, much less than the risk of getting uterine cancer.  Lifetime risk of dying of ovarian cancer is 1 in 95, more than twice the risk of dying of uterine cancer, even though the incidence of uterine cancer is higher. 

Higher risk has been associated with age (specifically being post-menopausal), obesity, early menses, late menopause, first pregnancy after age 30, never having children, long-time use of the fertility drug clomiphene citrate, a family history of ovarian cancer in first or second-degree relatives, and a history of breast cancer. Some studies have shown a slightly higher incidence among women who took estrogen therapy after menopause, but not among those taking both estrogen and progesterone.  Some studies have also shown a slightly higher risk for women who smoke and use alcohol.

Based on data from the years 2000 to 2004, the median age for women developing ovarian cancer is 63 (50% diagnosed younger than 63 and 50% at age 63 or older). 

There is no effective screening tool for discovering occult cancers of the ovaries among the general population. Regular pelvic exams are important in discovering ovarian cancer, but early-stage tumors are difficult to feel. Women who are at high risk may have routine pelvic ultrasounds and blood tests, but these tests have not been shown effective in lowering the mortality of ovarian cancer or in finding early-stage tumors while still being potentially curable. While 70% of uterine cancers are found while the tumor is still localized and highly curable, almost the same percentage (69%) of ovarian cancers aren’t found until the cancer has metastasized. 

## Protein Patterns for Ovarian Cancer

**NCI-sponsored research**
**Simple blood test **
**Used to identify ovarian cancer**
**Have found stage I ovarian cancers**
**More research needed**

**Notes:**
Some promising research has identified a method of detecting early-stage ovarian cancer with a simple, noninvasive blood test.  Rather than using a single tumor marker, the test looks at a pattern of proteins. Already in testing, the investigators were able to identify all stage I ovarian cancers in a small sample of patients.  More research is needed to confirm the accuracy of the test and establish its sensitivity and specificity. If this test proves successful, it will open an entire new line of research to see if protein patterns can be used to identify other types of cancers. 

An article on this research is posted on the NCI Web site on the News Center page. Internet addresses for sources used in this presentation are provided on the final slides of this presentation.

## Ovary: Histologies

**Epithelial**
**Serous cystadenoCa**
**Mucinous cystadenoCa**
**Endometrioid adenoCa**
**Germ cell**
**Malignant teratoma**
**Dysgerminoma**
**Stromal **
**Granulosa cell**
**Theca cell**

**Notes:**
The histology of an ovarian cancer depends on which of the three types of ovarian tissue generated the cancer (following information from ACS Cancer Reference Information and SEER Book Four).
Epithelial tissue:
Approximately 90% of ovarian cancers arise from epithelial tissue.
Cystadenocarcinomas come from epithelial tissue lining ovarian cysts.
1.  Serous type (the most common) secrete serous fluid
2.  Mucinous type secrete mucin
Clear cell cystadenocarcinomas (CCC are less common.)
Endometrioid adenocarcinomas resemble uterine endometrioid cancer.
Germ cell tissue:
About 5% of ovarian tumors arise from germ cells.
Malignant teratoma (teratocarcinoma) is comprised of germ cell tissue that develops into bone, teeth, hair etc.  
Dysgerminoma is the female equivalent of male seminoma.
Stromal tissue:
Less than 5% of ovarian cancers arise from stromal tissue.
Granulosa cell and theca cell tumors are types of stromal tumors.
These tumors produce estrogen.
Other:
Borderline serous and mucinous adenomas and borderline endometrioid and clear cell adenomas have low potential to develop into frank malignancies and are not currently required to be reported to most central registries.
Adenomas, dermoids and mature teratomas, among others, are benign ovarian tumors.

## Cervix

**Lower 1/3 of uterus**
**3rd most common GYN cancer site**
**14% of GYN cancers**
**1.5% of female cancers**

- © 2004. A.D.A.M., inc.

**Notes:**
Information on cervical cancer relates only to invasive cancer unless specifically mentioned to be noninvasive.
Slide statistics derived from SEER Stat Fact Sheets

## Cervix: Anatomy

**A**** Internal os**
**B**** Endocervix**
**C ****External os**
**D**** Exocervix**

- © 2004. A.D.A.M., inc.

**Notes:**
Internal os (opening) – opening from uterus into cervical canal
Endocervix – area of cervix closest to the uterus, lined with mucus-producing glandular (columnar) tissue
External os – opening from cervical canal into vaginal canal
Exocervix – area of cervix closest to the vagina covered with squamous epithelium, joined to the endocervix at the squamous-columnar junction, which is located at the external os.

About 90% of cervical cancers are squamous cell carcinomas arising in the exocervix at the squamous-columnar junction (per ACS Cancer Reference Information).

## Cervix: Profile

**Lifetime risk – 1 in 142 **
**Median age at diagnosis – 48**
**Typical symptoms**
**Abnormal vaginal bleeding**
**Pain during intercourse**
**Screening available – Yes**

**Notes:**
Per SEER Cancer Stat Facts and ACS Cancer Reference Information, the lifetime risk of developing cervical cancer is less than 1% (about 0.7%), or approximately 1 in 142 women.  Only 1 in 417 women dies of cervical cancer. The major risk factor for cervical cancer is the human papilloma virus (HPV).  Most women with HPV will not get cervical cancer, but 2/3 of cervical cancer is caused by HPV.  Women who begin having sex at an early age, have multiple sexual partners, or have a partner who has had multiple partners are at higher risk of being infected with HPV. Higher risk of cervical cancer has also been associated with smoking, HIV infection, Chlamydia infection, obesity, poor diet, long-term oral contraceptive use, multiple pregnancies, having a mother who took diethylstilbestrol (DES), and a family history of cervical cancer. 

The median age for developing cervical cancer is 48 (50% diagnosed younger than 48 and 50% diagnosed age 48 or older). Localized cervical cancer is usually asymptomatic.  The most common sign is abnormal vaginal bleeding. Pain during intercourse could be another indication.  These symptoms are nonspecific and could indicate many causes other than cervical cancer. If caused by cervical cancer, they usually mean the cancer is no longer localized.
Regular GYN exams with PAP smears are very effective for screening for cervical cancer. NCI estimates 90% of cervical cancer could be detected early if every woman got regular PAP smears, but up to 33% of women who should get PAP smears don’t. The FDA has approved HPV DNA testing for women over 30 in conjunction with PAP smears.  The test looks for certain forms of the virus known to cause cervical cancer. Earlier screening is not effective  Fortunately, there is now a vaccine for the types of HPV viruses that cause cervical cancer.
Only 51% of invasive cervical cancer is diagnosed while still localized (confined to the cervix). The five-year survival rate for localized cervical cancer is 92%.  Another 34% of cervical cancer is regional when diagnosed, and the five-year survival rate for regional cervical cancer is only 56%. 

## Cervix: Histologies

**Squamous cell carcinoma**
**Adenocarcinoma**
**Adenosquamous carcinoma**

**Notes:**
Per ACS Cancer Reference Information:

About 80% to 90% of cervical cancers are squamous cell carcinomas, most of which arise in the squamous-columnar junction of the cervix.

The other 10% to 20% are adenocarcinomas arising in the endocervix.

A few are mixed squamous cell carcinomas and adenocarcinomas.

Other carcinomas, lymphomas and sarcomas are rare, but do occur.

## Vulva and Vagina

**Vulva**
**4th most common GYN cancer site**
**4.8% of GYN cancers**
**Vagina**
**5****th**** most common GYN cancer site**
**1.5% of GYN cancers**

**Notes:**
Vulvar, vaginal and other GYN cancers combined are less than 1% of all cancers among women. 

Data are from selected statewide and metropolitan area cancer registries that meet the data quality criteria for all invasive cancer sites combined. Counts cover approximately 96% of the U.S. population. 

Source: U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2004 Incidence and Mortality Web-based Report. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2007. Available at: www.cdc.gov/uscs.

## Vulva  and Vagina: Anatomy

**Vagina**
**Fornix**
**Vault**
**Vulva**
**Labia majora**
**Labia minora**
**Mons pubis**
**Clitoris**
**Bartholin’s and Skene’s glands**

- © 2004. A.D.A.M., inc.

**Notes:**
Vagina
The anatomy of the vagina is fairly simple.  It’s a hollow organ with a muscular wall and a lumen lined mostly with squamous epithelium.
The vagina cups around the exocervix, where it connects to the uterus forming a circular fold called the vaginal fornix.
Vulva
The vulva is the external genital organ and has a more complex anatomy.
Labia majora means large lips; they are the more prominent folds of hair-bearing skin. 
Labia minora means small lips; they are located within the labia majora, surround the opening to the vagina and are covered with a modified epithelium. The area circumscribed by the labia minora around the vaginal opening is called the vaginal vestibule.
The mons pubis is a skin-covered fat pad covering the pubic bone.
The clitoris is a firm nodule of sensitive tissue located just outside of where the anterior portions of the labia minora join.
The two Bartholin’s glands are located on each side of the vaginal opening; their purpose is to lubricate the vagina.
The two Skene’s glands secrete mucous and are within the vaginal vestibule but are located near the opening of the urethra.

## Vulva and Vagina : Profile

**Lifetime risk – very small**
**Typical symptoms**
**Visible lesion**
**Abnormal bleeding or spotting**
**Pain during intercourse**
**Screening available – Yes**

**Notes:**
These two cancer sites are much less common than endometrial, ovarian or cervical cancers. Less than one-third as many women are diagnosed with vulvar cancer as with cervical cancer.  Vaginal cancers and all “other” GYN cancers combined occur about one-fifth as frequently as cervical cancer.  

More than 50% of vulvar cancers occur on the labia majora, the most external part of the organ. Another 20% occur on the labia minora. The vulva’s accessibility is one reason that about 61% of vulvar cancer are discovered while still localized. 

Per SEER Cancer Stat Fact Sheets, the five-year survival rate for localized vulvar cancer is 92%. The five-year survival rate for regional vulvar cancer drops to 56%. The overall survival rate is 78%.  Vaginal cancer is also highly curable if treated while still in early stages.

## Vulva: Histologies

**Squamous cell Ca**
**Basal cell Ca**
**Sarcoma**
**Histiocytosis X**
**Melanoma**

**Notes:**
Per NCI Cancer Topics, the majority of vulvar cancers are squamous cell carcinoma
Sarcoma, histiocytosis X and melanoma histologies are not very common, but they do occur.
VIN III, intraepithelial neoplasia of the vulva, is not reportable to the Commission on Cancer-Approved Registries, although it is reportable to the state registries and required by both SEER and NPCR.
Carcinoma in situ of the vulva is reportable, is fairly common, and is almost 100% curable.
Skin of the vulva is a vulvar site (C51.9), not a skin site (C44), so basal cell of the skin of the vulva is reportable.

## Vagina: Histologies

**Squamous cell carcinoma**
**Adenocarcinoma**
**Adenosquamous (mixed) carcinoma**
**Sarcoma**
**Melanoma**

**Notes:**
Per NCI Cancer Topics, 
Squamous cell carcinoma 
Account for approximately 85% of vaginal cancers
Normally spreads superficially within the vaginal wall and later directly invades regional tissues and adjacent organs.
Most common distant sites are lungs and liver
Adenocarcinoma 
About 15% of vaginal cancers
More aggressive and more likely to become non-localized earlier
Peak age of incidence is 17-21 years of age
More likely than squamous cell to spread to regional lymph nodes
Most common distant sites are lungs and supraclavicular lymph nodes
Adenosquamous carcinomas
Mixed histology tumor
Approximately  1% - 2% of vaginal tumors
Aggressive
Sarcomas and melanomas are rare but they do occur

## Other GYN Cancer Sites

**Fewer than 1% of GYN cancers**
**Include**
**Fallopian tube**
**Gestational trophoblastic tumors (placenta)**
**Round ligament**
**Uterine ligament**
**Uterosacral ligament**

**Notes:**
Fallopian tube tumors are very rare and behave similarly to ovarian cancers. The primary site is often not determined until the surgical specimen is examined. The histology is almost always a type of adenocarcinoma, including histologies associated with ovarian carcinomas. Survival rates are also similar to ovarian cancers.  Because of their rarity and similarity to ovarian carcinomas, information on fallopian tube malignancies is sometimes included in discussions on ovarian cancers.

Gestational trophoblastic tumors arise from the embryonic trophoblast tissue that forms the placenta for a fertilized cell.  These tumors are very rare, occurring in approximately 1 of every 1000 pregnancies. The term “pregnancy” is a little misleading in that there is no viable fetus.  Gestational trophoblastic disease (GTD) results from a genetic accident and replaces a normal pregnancy.  There are several types of GTD, most of which are benign.  Even those that are invasive and non-local are usually highly curable with chemotherapy.

The round, uterine and uterosacral ligaments support the GYN organs within the pelvis.  Cancers of these sites are extremely rare and aren’t covered further in this presentation.

## Gestational Trophoblastic Tumors (GTT): Benign Histologies

**Hydatidiform mole**
**Complete ****(9100/0)**
**Partial ****(9103/0)**
**Invasive****/****malignant****/****persistent ****(9100/1)**
**Trophoblastic tumor, placental site ****	****(9104/1)**

**Notes:**
Because the histologies of trophoblastic tumors are complicated and the behavior of benign tumors can include invasion and metastases, information on this slide and the next discuss both benign and malignant variants of gestational trophoblastic tumors (ACS Cancer Reference Information):

Hydatidiform moles
The two types of benign tumors include:
Complete mole (M-9100/0) – occur when a sperm fertilizes an egg that has no female DNA. A small number can become truly malignant (cancerous).  When that happens, the histology is choriocarcinoma (M-9100/3).
Partial mole (M-9103/0) – occur when two sperm fertilize an egg so that the DNA has two male copies. These only very rarely become cancerous.
There is one type of invasive/ malignant hydatidiform mole (M-9100/1) – uncertain behavior in spite of being called malignant and invasive. 
Approximately 20% of complete moles and 5% of partial moles invade into the myometrium of the uterus. 
These might also be called persistent (not an ICDO-3 morphology term) because residual tumor remains in the uterine wall after the initial standard D&C therapy.
If left untreated, an invasive mole could perforate the wall of the uterus and cause extensive bleeding or might regress and disappear on its own.
Invasive moles could even metastasize and require chemotherapy.

Trophoblastic tumor, placental site (M-9104/1)
Uncertain behavior (not reportable)
Rare tumor that originates where the placenta attaches to the uterus
Occurs after a normal pregnancy or abortion
Can penetrate the wall of the uterus but rarely metastasize.

## Gestational Trophoblastic Tumors (GTT): Malignant Histologies

**Choriocarcinoma ****(9100/3)**
**Malignant trophoblastic teratoma ****(9102/3)**
**Trophoblastic tumor, epithelioid ****(9105/3)**

**Notes:**
  Choriocarcinoma (M-9100/3 or M-9101/3 if mixed with other germ cell histologies) may have the following characteristics:
  Very rare, highly aggressive gestational trophoblastic tumor
  Metastasizes fairly quickly to organs outside of the uterus
  Usually develops from a complete mole, but can arise independently in       the placenta after a pregnancy.
  Can originate in other organs besides the placenta, but rarely does.

  Other malignant trophoblastic tumors
Malignant trophoblastic teratoma (M-9102/3)
Trophoblastic tumor, epithelioid type (M-9105/3) 

## Difficulties in Abstracting

**Biopsies of multiple GYN sites**
**GYN cancer management**
**Determining amount of data**
**Importance of support text**

**Notes:**
There are assuredly many more areas of difficulty than on this slide.  You could probably add a few more or a lot more from your own experience.  We’ll talk more about each of these on the next slides.
.

## Multiple Biopsies

**Problem: where did the cancer originate?**
**Issues**
**Proximity of sites**
**Few natural barriers**
**Similar histologies**

**Notes:**
GYN sites provide unique problems in determining the primary site.  Multiple biopsies don’t always clarify the search.

## Site of Origin

**Origin of cancer suggests**
**How best to treat**
**Usual pattern of spread**
**Probability of metastasis**
**Prognosis**
**Biopsy site may not be the primary site**

**Notes:**
Most cancers are named for their site of origin.  Experience has shown that the site of origin indicates the probable histology and the probable pattern of spread – whether the cancer usually spreads superficially or quickly metastasizes—and where it most likely will metastasize—to regional nodes or distant organs. The origin helps the physician determine what treatment is usually most effective.  The origin also provides strong clues to prognosis, knowledge that will help the physician interact with the patient and the patient’s family.

It’s important to remember that the biopsy site might not be the site of origin, even if the biopsy histology is common to the site of biopsy.  For example, a biopsy of a squamous cell carcinoma found in the vagina might be from a cancer that originated from the vulva, vagina, or cervix—all sites for which squamous cell is common. On the other hand, the histology of the biopsy site could direct the physician to a more probable origin. For example, a serous cystadenocarcinoma found in the vagina might indicate an ovarian primary site.

## Proximity of GYN Sites

**All are connected **
**Most are hollow**
**All located in the pelvis **

**Notes:**
All GYN organs are connected to each other.  Only the organs at each end of the tract, the ovaries and vulva, would not be considered hollow. 

All GYN organs are located in the pelvis where involvement of pelvic nodes is of little help in indicating which GYN site could be the primary site.

## Few Natural Barriers

**GYN sites connect via narrowed openings**
**Most lined with epithelial tissue**
**Histology changes mark some divisions**

**Example of T3 cancer of corpus uteri**

- AJCC Cancer Staging Atlas, 2006.  Used with permission.

**Notes:**
Most GYN organs connect to each other through a narrowed opening or os.  The cervix has an os at each end. But the epithelial tissue lining one organ also lines the os. The cervix has a circumferential “line,” the squamous columnar junction, where the cell type changes from squamous cell in the exocervix to glandular cells in the endocervical canal.  This divides the two areas of the cervix.  The glandular epithelium lining the endocervix continues on to merge with the glandular epithelium lining the body of the uterus.  

The ovary is suspended by its own ligament and doesn’t actually touch the fallopian tube, which is connected to the uterus, but the fallopian tube is designed to capture the eggs produced by the ovary.  You can see how easily cancers can cross from one GYN organ to another and how difficult it is sometimes for surgeons and pathologists to determine the primary site with certainty. 

## Similar Histologies

**Most frequent histologies**
**Squamous cell carcinoma**
**Adenocarcinoma**
**Few histologies suggest a single site**
**Multiple biopsies may not help**

**Notes:**
The most common histologies for all GYN cancer sites as a group are squamous cell carcinomas and adenocarcinomas. Squamous cell is more common in the lower or distal GYN organs. Adenocarcinoma is more common in the proximal GYN organs. Those differences are not dramatic, especially in a poorly differentiated cancer. Some histologies, like those characteristic of ovarian cancers, are also characteristic of fallopian tube cancers, but are not unique to those two sites.

Multiple biopsies of several GYN sites could conceivably show the same histology and not help to identify the primary site.  Even if surgery is done, it could be difficult to determine the primary site for a single squamous cell carcinoma that involves the exocervix and the upper vagina.  Is it cervical cancer that spread to the vagina or vaginal cancer that spread to the cervix?  For these reasons, documentation of the GYN primary site could be lacking in a patient record.  If the physician determines all involvement can best be treated with brachytherapy, or even regional radiation therapy, it might not be as important to the physician precisely where the cancer originated as it will be to the cancer registrar who has to assign a site code. The registrar might have to assign an overlapping (C57.8) or NOS (C55.9, C57.9) GYN code, depending on the extent of the tumor.

## GYN Cancer Management

**Causes of abstracting difficulties**
**Screening/prevention efforts**
**Comorbidities and complications**
**Inaccessible sites**
**Research protocols**
**Non-compliant patients**

**Notes:**
These are a few of the cancer management situations that could result in difficulties in abstracting GYN cases.  We’ll discuss each of them individually.

## Screening and Prevention Efforts

**Screening may be off-site**
**HPV vaccine**
**Invasive vs. non-invasive**

- © 2004. A.D.A.M., Inc.

**Notes:**
Routine GYN exams and PAP smears for cervical cancer screening are often done in physicians’ offices, cancer screening centers, or women’s centers. Using outpatient facilities off-site provides convenience for patients, but it’s more difficult for cancer registrars to get objective information on screening results and dates of diagnosis.  Often the facility that did the screening is not named in the patient’s medical record. In many cases, for the more accessible GYN sites, the initial surgical treatment could also be done off-site.  The patient’s available documentation could begin with the patient’s admission for more extensive therapy.

Developing a vaccine that is effective against some types of cervical cancer was an exciting breakthrough for research.  Prior to establishing a causal link between the human papilloma virus (HPV) and cervical cancer, HPV was known only to increase women’s risk of getting cervical cancer. HPV vaccine is not treatment. Although data on HPV vaccines is not required, your organization might decide on its own to collect these data. It is thought that HPV might be implicated in several other GYN cancers. Research is ongoing.

Most nationally published statistics do not include GYN in situ cancers.  Noninvasive cancer is often presented as “not quite” cancer.  Registrars are still required to abstract most noninvasive GYN cancers and need to be aware that sometimes physicians do not call a lesion cancer or malignant unless it is invasive. For example, PAP smears that find CIS are sometimes referred to as “precancerous”. Cervical carcinoma in situ (CIS) is not required to be accessioned, but abstracting CIS is required for the other GYN sites. Intraepithelial neoplasms of the vulva (VIN III) and vagina (VAIN III) are required to be abstracted by NPCR and SEER. Registrars need to be aware of these differences in terminology and make sure they do not miss any reportable non-invasive cases. 

## Comorbidities and Complications

**Might force deviations from standard treatment**
**Coded from secondary diagnoses**
**May prevent a specific treatment**

**Notes:**
Registrars in Commission on Cancer-approved programs are required to collect comorbidities and complications exactly as they are coded in the patient record.  That’s direct entry from the medical chart to the abstract.  Some registry software products could even download secondary diagnoses codes over a computer interface.

Sometimes the comorbidity or complication is the reason that a particular type of treatment could not be given to a patient. The “reason for no” treatment data item isn’t specific to any complications  or comorbidities, but a code in this field might indirectly be the result of a comorbidity or complication. For example, a patient suffering from congestive heart failure might have to forego chemotherapy that would otherwise be recommended. “Reason no chemo” would simply be 82 “contraindicated due to patient’s risk factors.” The specific reason for selecting this code or for any deviation from standard care should be documented in the text.

## Inaccessible GYN Sites

**Corpus uteri and ovaries **
**Vagina accessible with instruments**
**Only vulva accessible w/o instruments**

**Notes:**
Physicians are limited in their examination of inaccessible sites, which then limits the documentation available to the cancer registrar and increases the ambiguities. Inaccessibility also limits the ability of physicians to take biopsies.  Not only do inaccessible sites hinder physicians’ ability to determine origin of cancers, but they also hinder their ability to stage extent of disease. 

Special endoscopic instruments allow physicians to look inside the vagina, cervical canal and uterus, but access for palpation of these sites and their regional lymph nodes is very limited. The vagina can be sufficiently dilated with a speculum to allow visual examination of it and the exocervix.  Because the vulva is an external organ, it is the only GYN site that is visible and accessible without instruments.

## Research Protocols: Patients

**Clinical trials enroll patients**
**Might require deviations from standard care**
**Blinded studies have unidentified agents**
**Chemopreventive agents are not treatment**

**Notes:**
Clinical trials in hospitals are research investigations that enroll patients in prospective studies of potentially more effective treatment.  Patients can be randomized to one arm of standard therapy or to another arm of a new therapy under study that has already been shown effective in animals.  The purpose is to find out if the new treatment is more effective than the standard treatment. Clinical trials not only “might” require deviations from standard care, the deviation is often the focus of the study.  Planned deviations might make it more difficult for you to anticipate and code treatment.
Blinded studies are particularly troublesome to abstract.  Until the therapeutic agents are known, blinded studies are coded as “other treatment.”  FORDS instructs us to “code the treatment actually administered when the double-blind trial code is broken.”  Treatment can be coded before the trial code is broken if it is known that the agents are all chemotherapy or all hormones.  Registries should set policies on how to handle blinded studies, whether to attempt to identify the type of modality through the pharmacy or principal investigator or simply code as “other treatment” until the treatment codes are broken. A tickler procedure might be needed to remind registrars to revise the treatment codes once the therapies are known.
Chemopreventive agents are sometimes given to high-risk patients who do not yet have cancer or who are in remission, but at risk for recurrence or at risk for additional primaries. These patients do not have active cancer and are not required to be accessioned in most states.

## Research Protocols: Tissue

**Some protocols do research on tissue**
**Example: research on targeted therapies**
**Might require modifications to standard care—especially surgery**
**Some facilities contribute only tissue**
**Might require collection of additional data**

**Notes:**
Research on targeted therapies is going on now at research institutions.  The idea is to more effectively and specifically treat the cancer cells while more effectively protecting normal cells. Radioactive iodine to treat thyroid cancer is one of the oldest types. Monoclonal antibodies that carry radioactive isotopes to specific types of cells is another.

Another concept is to group cancer patients by their outcomes, analyze their DNA—specifically their gene expression—and find points of similarity for creating patient profiles.  When new patients are diagnosed with cancer, their DNA profiles are compared to the database of profiles in hopes that profile matches will indicate the best individualized treatment that will provide optimum outcomes.

Only a few major institutions actually design research treatment protocols. Physicians at community hospitals can participate in research studies by following a study’s procedures under the direction and monitoring of a principal investigator located at a research institution. An example would be supplying tissue for analysis. Registries can be asked to collect additional data and avoid unknown values for data needed for the studies. Another result of targeted therapies, which will challenge cancer registrars, will be more complex treatment algorithms.

## Non-Compliant Patients

**Problem for physicians and registrars**
**Missing documentation**
**Partial care at multiple facilities**

**Notes:**
Non-compliant patients cause problems for treating physicians and for registrars, as well as for the patient. At what point should a delay by a patient become “patient-refused” for first-course treatment and standard treatment becomes subsequent.  There isn’t a consensus of opinion.  Some experienced registrars think that as long as the delayed therapy was part of the initial treatment plan and there was no obvious progression of the cancer, it should be counted as first course.  Others think that mixing delayed-treatment cases in with other analytic cases would skew outcomes. SEER made a rule to resolve the issue, but reasons for delays should be documented in the text.

SEER sets one year as the time limit for delaying first-course treatment. If the patient delays more than a year before starting treatment, it is coded as subsequent.  On the other hand, once treatment has started, it can continue beyond a year and still be first course—as long as there is no progression. If the delay is less than a year without documented progression, code treatment as first course. An untreated cancer that doesn’t progress in a year would be a very indolent cancer.  Read your manuals to confirm what your state requires. 

Expected documentation for non-compliant patients might not be in the chart because the procedure, although planned, was never done. Some non-compliant patients “hop” from facility to facility never completing an entire treatment plan making attempts to collect complete data almost impossible.

## Determining How MuchData to Collect

**Required data set(s)**
**Current data requests**
**Anticipating future data requests**
**Avoiding “unknowns”**

**Notes:**
You should be aware of which data items are required and which are simply useful.  Most cancer registry software systems collect more than just required data.  This is a matter for cancer committee discussion to decide whether the facility’s registry will simply be a reporting mechanism for the central registry or whether the registry should collect enough information to be a useful tool for the facility to evaluate its own treatment patterns and outcomes.

## Required Data Set(s)

**Differ by type of registry**
**Central registry (NPCR, SEER, state)**
**Hospital and freestanding treatment centers**
**Differ by CoC approval status**
**Approved cancer programs also use FORDS**
**Differ by state**
**Some states may require additional data**

**Notes:**
Which data sets are required depends on the state reporting law, and whether the facility is approved by the American College of Surgeons Commission on Cancer (COC).

Some state cancer registries can require reporting of data items in addition to those required by the COC and NPCR.  Requirements are set as follows:
State legislatures mandate data items for reporting cancer cases.
COC-approved facilities collect data items listed in the FORDS Manual.
Facilities in SEER locations collect SEER data items.  
Your facility might require collecting reportable-by-agreement data.

Different types of facilities can collect different data items. For example, free-standing radiation facilities and SEER registries collect slightly different data sets than those collected at full-service hospitals. 

Fortunately, most of the data items in these data sets are the same in every set, but there are some differences that abstractors will need to know. Know where your data goes. Refer to your state’s data collection manuals and any other manuals that apply to your facility.

## Current Data Requests

**Who uses your data at your facility?**
**Administration**
**Physicians**
**Researchers**
**Regulatory reporting (NCDB, state, etc)**
**Know your facility’s data needs!**
**Do required data sets provide sufficient data?**

**Notes:**
Know who uses cancer registry data at your facility. Assess your data sets for required fields and requested data, both internal and external.

Some non-required data items might be very useful to individual facilities that are engaged in special studies.
Example: facilities can decide to track the severity of the cancer when a patient is first seen at their facility.  The registry might be asked to collect the stage of disease at presentation for patients treated for recurrence.

Physicians might want to have a statistical profile of their patients or a survival analysis on a certain procedure they performed on their patients. Some types of facilities, such as research and teaching facilities, may also require additional data items to support their research. Staying aware of the goals of your facility is helpful in anticipating its data needs.

## Future Data Requests

**Anticipate future data requests**
**Stay current on changes in oncology**
**Collect now for future use**
**Use software user-defined fields**
**Think creatively**

**Notes:**
Anticipate which data you might be called upon to provide and begin collecting the data now. Remember you have to collect the data today that will be requested tomorrow—or two years from today. Sometimes having the data available at the time they are requested is as simple as avoiding unknown values. Pick a date to start collecting special data that will give you a good statistical base.  This is most often the first day of a year, but can be the first day of a quarter or the first day of a month.  Just remember the time frame must make sense when doing statistical reports.  Compare data from date ranges of equal lengths.

An example of being proactive in collecting data is using a user-defined field to record HPV virus in your database—if your facility documents it in patients’ records.  Physicians and administrators may request information on how often it occurs. Your facility can use the data to provide education on the cervical cancer vaccine to the community.

Most cancer registry software systems have user-defined data fields; use them for your special data needs.

## Avoiding “Unknowns”

**Might pass edits but useless for analysis**
**Seek data that’s not immediately available**
**Focus on data with value for analyses**
**Be proactive asking physicians and others to document data that has value**

**Notes:**
Some “unknown” values might be accepted by state registry edits but are useless for analysis—theirs and yours. Make an honest effort to find important data that’s not immediately available, such as postoperative radiation at another facility after the surgery was done at yours.

Make a special effort to collect data that has research or prognostic value, even if it means contacting the pathologist or treating physician for clarifications. For example, the evaluation of surgical margins has importance in analyzing vulvar cancer survivals. All required data items have value, but your state’s manual might list situations and data fields where unknown values are more accepted. As cancer research moves more toward targeted therapies, detailed information on individual cancer cases becomes increasingly more relevant.

Ask the people who document information in the medical record to make sure the data you need are noted. These include physicians, admission personnel and other healthcare professionals who document the data you collect.  Most will be cooperative once they learn the importance and utilization of the information.

## Text Validates Codes

**Supports code selections**
**Easier to do visual review of abstract**
**Facilitates re-abstracting QA **
**Provides clear summary of case **
**Prevents re-researching case to explain anomalies**
**Required by NPCR state registries**

**Notes:**
It’s easier and quicker to do quality control on an abstract if there is good text documentation along with the code selections. 
It’s easier to answer questions about the case from the text documentation than from the codes.
It’s easier to do re-abstracting QA because you know what data to look for in chart.
Text documentation can explain anomalies in the case, saving endless hours if someone down the line requests and then questions the data.
Text documentation is also required by state registries, for these same reasons.
(Go to part 2)

## 

    The findings and conclusions in this presentation are those of the authors and do not necessarily represent the views of the Centers for Disease Control and Prevention.

**    ****The findings and conclusions in this presentation are those of the authors and do not necessarily represent the views of the Centers for Disease Control and Prevention.**

## For information about CDC’s  
Cancer Prevention and Control Programs
and the 
National Program of Cancer Registries

Please visit www.cdc.gov/cancer/npcr

**Cancer Prevention and Control Programs**
**and the **
**National Program of Cancer Registries**
**Please visit**_** **__**www.cdc.gov/cancer/npcr**_