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December 5, 2011

Coding for Carcinoid Tumors
For The Record
Vol. 23 No. 22 P. 28

Carcinoid tumors, a subgroup of neuroendocrine tumors, are biologically different from other types of cancer and can be either malignant or benign. They usually grow slowly and usually appear in the gastrointestinal tract, such as the appendix, stomach, small intestine, large intestine, or rectum. They can also appear in the lungs, thymus, and kidneys. Risk factors include smoking and familial tendency, with the average age at onset being 60 years old.

Signs and symptoms do not typically appear in the early stages of the disease process. When symptoms do appear, they are vague depending on the location of the tumor. Symptoms may include abdominal pain, bowel obstruction, chest pain, cough, hemoptysis, wheezing, shortness of breath, tachycardia, diarrhea, rectal bleeding, rectal pain, recurrent pneumonia, and skin flushing.

Carcinoid tumors are classified to ICD-9-CM category 209. The fourth and fifth digits identify the specific location of the carcinoid tumor and whether it is malignant or benign. If the physician documents only carcinoid tumor, assign the default code 209.60. However, it is always appropriate to query the physician for specificity if not available in the record.

There is an instructional note under category 209 that states “code first any associated multiple endocrine neoplasia syndrome (258.01-258.03).” Although this coding directive provides sequencing guidance in the presence of both conditions, it is appropriate to code only the carcinoid tumor (category 209) if the patient does not have multiple endocrine neoplasia (MEN) syndrome. According to Coding Clinic, it is acceptable to assign a code from category 209, Neuroendocrine tumors, as the principal diagnosis. A code from subcategory 258.0, Polyglandular activity in multiple endocrine adenomatosis, should be used and sequenced as the principal diagnosis only when documented by the physician (AHA Coding Clinic for ICD-9-CM, 2009, fourth quarter, pages 150-151).

MEN syndrome is caused by mutations in genes that regulate cell growth. Depending on the type, patients develop carcinoid tumors due to the absence of cell regulation. In MEN I (Wermer’s syndrome; 258.01), patients develop parathyroid, pancreatic, and adrenal tumors as well as gastrinomas and insulinomas. Patients with MEN IIA (Sipple’s syndrome; 258.02) develop thyroid, parathyroid, and adrenal tumors. In MEN IIB (258.03), thyroid and adrenal tumors develop. Treatment depends on the type and severity of the tumors and includes the management of complications related to tumors and may include surgical resection.

The following complications may arise from carcinoid tumors due to secreting hormones: stomach ulcers, carcinoid syndrome, carcinoid heart disease, or Cushing’s syndrome.

Carcinoid syndrome (259.2) describes the hormonal effects of carcinoid tumors. Not all patients with carcinoid tumors have carcinoid syndrome. Symptoms may include skin flushing, chronic diarrhea, and difficulty breathing. Treatment includes medications to prevent tumor cells from secreting hormones responsible for the syndrome’s symptoms. Octreotide (Sandostatin), lanreotide (Somatuline Depot), and dexamethasone have been found effective. The carcinoid tumor (category 209) would be sequenced as the principal diagnosis, and carcinoid syndrome (259.2) would be listed as the secondary diagnosis.

Common diagnostic tests the physician may order to confirm the diagnosis of carcinoid tumor include blood tests to detect elevated levels of a protein caused by hormones secreted by the carcinoid tumor, urine tests to identify increased levels of a chemical from hormones broken down by the carcinoid tumor, imaging tests, endoscopy, or biopsy.

Treatment for a carcinoid tumor will depend on the size and location of the tumor and whether it has spread to other parts of the body. Tumor resection is preferable if it is localized and can be completely removed. Other types of surgery include embolization to cut off the blood supply to the cancer cells, radio-frequency ablation to deliver heat, or cryoablation to freeze the cancer cells.

Coding and sequencing for carcinoid tumors are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding.

— This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to more than 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447.


Carcinoid Tumors in ICD-10-CM
Classify benign carcinoid tumors to category D3a, Benign neuroendocrine tumors. Fourth, fifth, and sixth characters are necessary to identify tumor location. Classify malignant carcinoid tumors to category C7a, Malignant neuroendocrine tumors, and secondary neuroendocrine tumors to category C7b, with fourth, fifth, and sixth characters once again needed.

As in ICD-9-CM, an instructional note is included under the category headers to assign an additional code for carcinoid syndrome (E34.0), if present. The coding note related to multiple endocrine neoplasia (MEN) syndrome states “code also” instead of “code first.” Therefore, MEN syndrome associated with carcinoid tumors is assigned to codes E31.20 to E31.23, with sequencing depending on the circumstances of admission.