Coding for esophageal cancer in ICD-10-CM begins with understanding that esophageal cancer is a malignant neoplasm that occurs in the esophagus. Neoplasms can be malignant or benign. Terms such as cancer, carcinoma, adenocarcinoma, and malignancy mean the tumor or mass is malignant.
Read on to learn the facts about this disease, anatomy of the esophagus, types of esophageal cancer, risk factors, symptoms, and how a diagnosis is made. We’ll also cover some of the ICD-10-CM coding guidelines related to neoplasms and how to look up the correct code using the Alphabetic Index and the Table of Neoplasms.
At the end of this article, see if you can assign the appropriate ICD-10-CM codes for the five related coding exercises based on the coding guidelines. The answers and rationales are also provided.
Esophageal cancer of the esophagus is a malignant disease in which cells in the esophageal lining begin to grow out of control. These cancerous cells can spread, or metastasize, to other areas of the body.
According to the National Library of Medicine, esophageal cancer is the sixth most common cause of cancer-related deaths and the eighth most common cancer worldwide, with a 5-year survival rate of less than 25%.
The American Cancer Society reports that about 20,640 new esophageal cancer cases will be diagnosed in 2022. The estimated number of deaths due to esophageal cancer in 2022 will be 16,410.
Anatomy of the Esophagus
The esophagus is a long muscular tube that moves food from the pharynx (throat) to the stomach.
According to Cancer.org. the esophagus lies behind the trachea (windpipe) and in front of the spine. It is approximately 10 to 13 inches long in adults and about an inch wide at its smallest point.
A ring of muscle known as the upper esophageal sphincter lies at the opening of the upper esophagus. The upper esophageal sphincter relaxes to open the esophagus when food or liquid approaches it.
When swallowing, food and liquid move through the inside of the esophagus called the lumen to get to the stomach.
The lower part of the esophagus, called the gastroesophageal (GE) junction, connects to the stomach. A lower esophageal sphincter is a ring of muscle close to the GE junction that controls the movement of food from the esophagus into the stomach. The lower esophageal sphincter closes between meals to prevent the stomach’s acid and digestive juices from entering the esophagus.
There are two ways to describe the subsites of the esophagus, and they are not equal.
Subsite Description 1
According to StatPearls, the first way to describe the subsites of the esophagus is based on the anatomical segments.
- Cervical vertebrae begin at the lower end of the pharynx (C6 or lower) and extend to the thoracic inlet (suprasternal notch).
- Thoracic vertebrae include the upper, middle, and lower thoracic vertebrae.
- Upper: This is from the thoracic inlet to the level of the tracheal bifurcation.
- Middle: This is from the tracheal bifurcation midway to the gastroesophageal junction.
- Lower: This is from midway between the tracheal bifurcation and gastroesophageal (GE) junction to the GE junction, including abdominal esophagus.
- Abdominal vertebrae are considered part of the lower thoracic esophagus.
Subsite Description 2
The second way to describe the subsites of the esophagus is based on thirds.
- Upper (proximal) third is located at approximately C7-T2 and accounts for 10% of esophageal cancers.
- Middle third is located at approximately T3-T7 and accounts for 40% of esophageal cancers.
- Lower (distal) third is located at approximately T8-T10 and accounts for 50% of esophageal cancers.
Cancer may overlap at least two contiguous (adjacent) sites in some cases.
Types of Esophageal Cancer
According to the National Cancer Institute, there are two main types of esophageal cancer: adenocarcinoma and squamous cell carcinoma.
- Adenocarcinoma is the most common type of esophageal cancer. It starts in glandular cells and usually develops in the lower (distal) part of the esophagus.
- Squamous cell carcinoma is made up of flat, thin cells that line the surface of the esophagus. This type usually occurs in the upper or middle parts of the esophagus and is the most common type of esophageal cancer worldwide.
Other rare types of esophageal cancer include small cell carcinoma, sarcoma, lymphoma, melanoma, and choriocarcinoma.
According to Cleveland Clinic, the specific cause of esophageal cancer is unknown. However, what is clear is that certain factors increase a person’s risk of developing the disease. They include:
- Gender and age: Men are three times more likely to develop the disease than women, and they are more likely to be older than 60 years of age.
- Ethnicity: Adenocarcinoma occurs more often in Caucasians. Squamous cell esophageal cancer occurs more often in African Americans and Asians.
- Tobacco use: Smoking and smokeless tobacco
- Alcohol use: Chronic and/or heavy alcohol
- Barrett’s esophagus and chronic acid reflux: Barrett’s esophagus develops in the lower end of the esophagus and occurs from chronic untreated acid reflux known as gastroesophageal reflux (GER). GER may progress into a more severe form called gastroesophageal reflux disease (GERD). A person without Barrett’s esophagus with long-term heartburn (pain in the chest due to irritation of the esophagus) also has a higher risk of esophageal cancer.
- Human papillomavirus (HPV): HPV is a common virus that can cause changes in the vocal cords and mouth, on the hands and feet, and sex organs. In areas such as Asia and South Africa, where there is a high incidence of esophageal cancer, infection with HIV increases the risk of developing esophageal squamous cell cancer.
Other conditions that have been linked to esophageal cancer include:
- Achalasia: an uncommon disease that causes difficulty swallowing
- Tylosis: a rare, inherited disorder in which excess skin grows on the palms of the hands and soles of the feet
- Occupational exposure to certain chemicals: exposure to dry cleaning solvents over long periods
- History of cancer: previous cancer of the neck or head
Esophageal cancer often has no symptoms in the early stages, according to Cancer.org. As the disease progresses, however, problems may include:
- Difficulty swallowing (dysphagia)
- Chest pain
- Unintentional weight loss
- Chronic cough
- Bone pain if cancer has spread to the bone
- Bleeding into the esophagus
Diagnosing Esophageal Cancer
A physician may use the following tests and procedures to make a diagnosis of esophageal cancer:
- Physical examination and health history: An exam will be performed to check overall health. Any sign of disease will be checked, such as lumps that seem unusual. The patient’s history will be taken.
- Chest X-ray: An X-ray may be taken that shows a picture of the organs and bones inside the chest.
- Esophagoscopy: An endoscope is a long tube with a light on it. It is passed through the mouth, throat, and into the esophagus for viewing while the patient is asleep.
- Biopsy: During the esophagoscopy, a physician may remove a piece of tissue to view under a microscope for any cancer cells.
- Esophageal endoscopic ultrasound: Sound waves are used to create images of internal structures using an esophagoscope.
- Computed tomography (CT): This is often used to determine how far the tumor has spread to the chest and abdomen.
ICD-10 Coding for Neoplasms
The codes for most benign and all malignant neoplasms are in Chapter 2 of the ICD-10-CM coding manual. When coding for a neoplasm, the site needs to be chosen based on whether the documentation indicates the neoplasm is benign, in situ, malignant, or of uncertain histologic behavior. If malignant, any secondary (metastatic) sites should be determined. An unspecified code should be assigned If the specific behavior is not documented.
The ICD-10-CM Official Guidelines for Coding and Reporting instructs us to first go to the Table of Neoplasms in the Alphabetic Index to find the correct neoplasm code. That is unless you have the histological term, in which case you can look that term up first.
For example, if the diagnosis is “adenoma,” look up the term in the Index and review the entries beneath this term. Also, look at the instructional note to “see also neoplasm, by site, benign” at guidelines such as adenocarcinoma. It refers us to Neoplasm, malignant, by site.
Esophageal Cancer Codes and Table of Neoplasms
The codes for esophageal cancer are found in the Table of Neoplasms under the anatomical site and subsites. When we look up “esophagus” in the Table of Neoplasms, we see many subsites.
These subsites are classified into six different categories, as shown in the following table. The categories are based on whether the neoplasm is malignant primary, malignant secondary, CA in situ, benign, uncertain behavior, or unspecified behavior. The dash – shown next to “overlapping lesion” under five of the six categories means there is no code for that category.
Categories and Their Definitions
The definitions for each category are as follows:
- Malignant primary: This is the original site where cancer began.
- Malignant secondary: Cancer that has spread from the place where it first started to another part of the body.
- Ca (carcinoma) in situ: Cancer remains in the place where the abnormal cells first formed.
- Benign: This is a noncancerous tumor.
- Uncertain behavior: The documentation from a pathologist states the neoplasm is of uncertain behavior. This is based on the tissue having characteristics of both benign and cancerous cells.
- Unspecified behavior: The documentation does not indicate if the neoplasm is benign or malignant.
An unspecified code is assigned when the part of the esophagus where the neoplasm is located is not indicated. For example, “primary cancer of the esophagus” without mention of the upper, middle, or lower third of the esophagus is assigned C15.9, Malignant neoplasm of esophagus, unspecified (see table below). C15.9 must be verified in the Tabular List before we can code it.
Do not confuse C15.9, Malignant neoplasm of esophagus, unspecified, with D49.0, Neoplasm of unspecified behavior of digestive system. D49.0 is reported when the documentation does not indicate the type of neoplasm, such as benign or malignant.
Carcinoma in situ of the esophagus is reported with D00.1 no matter which subsite of the esophagus contains cancer. “In situ” means the cancer cells are only located where they first formed.
Uncertain behavior and unspecified behavior of the esophageal neoplasm are reported with codes D37.8 and D49.0, respectively. The subsite of the neoplasm does not change the coding.
If a malignant neoplasm overlaps two or more contiguous sites of the esophagus, it is reported with C15.8, Malignant neoplasm of overlapping sites of esophagus.
Neoplasm Coding Scenarios
Assign the correct ICD-10-CM codes for the following five coding scenarios and then review the answers and rationales below. The answers to these questions are based on your knowledge of the coding guidelines related to neoplasms. There are more coding guidelines related to neoplasms than are covered here. For a complete set of the related guidelines, refer to the ICD-10-CM Official Guidelines for Coding and Reporting.
1. A 50-year-old male with complaints of chest pain, chronic cough, and trouble swallowing is diagnosed with primary squamous cell esophageal cancer of the upper part of the esophagus.
2. A patient with primary esophageal adenocarcinoma of the lower esophagus is diagnosed with liver metastasis. The patient is being treated today for liver cancer.
3. A 62-year-old African American male is diagnosed with neoplasm of uncertain behavior of the esophagus.
4. Patient is diagnosed with carcinoma of the upper and middle third of the esophagus.
5. Mr. Blue is diagnosed with abdominal esophagus. He has a long history of alcohol abuse and dependence.
Answers and Rationales
1. Answer: C15.3
Rationale: This is a primary malignancy (cancer). There is no mention of metastasis or secondary sites. In the Table of Neoplasms, look up esophagus/upper (third)/Malignant Primary C15.3. This code can be verified in the Tabular List as:
C15.3, Malignant neoplasm of upper third of esophagus
Review the instructional notes at this classification. For example, a “Use additional” note instructs us to code to identify alcohol abuse and dependence (F10.-). In this case, the documentation does not indicate alcohol abuse and dependence, so we cannot code for it.
Chest pain, chronic cough, and trouble swallowing (dysphagia) are routinely associated with esophageal cancer. Per the coding guidelines, these symptoms should not be assigned as additional codes unless instructed by the classification.
2. Answer: C78.7, C15.5
Rationale: Esophageal adenocarcinoma of the lower esophagus is documented as the primary site. In the Table of Neoplasms, look up esophagus/lower (third)/Malignant Primary C15.5. This code can be verified in the Tabular List as:
C15.5, Malignant neoplasm of lower third of esophagus
Metastasis is to the liver, so we need to locate the code for this secondary site. In the Table of Neoplasms, look up liver – see also index to disease, by histology/Malignant Secondary C78.7. This code can be verified in the Tabular List as:
C78.7, Secondary malignant neoplasm of liver and intrahepatic bile duct
The coding guidelines state that “when a patient is admitted because of a primary neoplasm with metastasis, and treatment is directed toward the secondary site only, the secondary neoplasm is sequenced as the principal diagnosis even though the primary malignancy is still present.” Therefore, the correct sequencing is C78.7 followed by C15.5.
3. Answer: D37.8
Rationale: Histologic confirmation as to whether the neoplasm is malignant or benign could not be made. In the Table of Neoplasms, look up esophagus/middle (third)/Uncertain Behavior D37.8. This code can be verified in the Tabular as:
D37.8, Neoplasm of uncertain behavior of other specified digestive organs
Note: Coding for Unspecified Behavior is different from Uncertain Behavior. When a definitive diagnosis cannot be made at the time of an encounter, it is coded as “unspecified,” not “uncertain.”
4. Answer: C15.8
Rationale: The cancer covers two contiguous sites: the upper and middle third of the esophagus. In the Table of Neoplasms, look up esophagus; overlapping lesion/Malignant Primary C15.8. This code can be verified in the Tabular List as:
C15.8, Malignant neoplasm of overlapping sites of esophagus
5. Answer: C15.5, F10.20
Rationale: In the Table of Neoplasms, look up esophagus/abdominal/Malignant Primary C15.5. This code can be verified in the Tabular List as:
C15.5, Malignant neoplasm of lower third of esophagus
The abdominal esophagus is also considered part of the lower esophagus. Therefore, if the documentation states “malignancy of the lower (third) of the esophagus” or “malignancy of the abdominal esophagus,” the code assigned is C15.5 (as shown in the above table).
We need a second code. There is a “Use additional” note to identify alcohol abuse and dependence (F10.-). When we go to F10.- in the Tabular List, we can verify our correct code as:
F10.20, Alcohol dependence, uncomplicated
Alcohol use disorder, moderate
Alcohol use disorder, severe
The coding guidelines state:
“When the provider documentation refers to use, abuse, and dependence of the same substance (e.g., alcohol, opioid, cannabis, etc.), only one code should be assigned to identify the pattern of use based on the following hierarchy:
- If both use and abuse are documented, assign only the code for abuse
- If both abuse and dependence are documented, assign only the code for dependence
- If use, abuse, and dependence are all documented, assign only the code for dependence
- If both use and dependence are documented, assign only the code for dependence”
There is no indication of another problem other than alcohol dependence, such as intoxication or delirium, so it is coded as “uncomplicated.”
So, our two codes are sequenced as C15.5 followed by F10.20.