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Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

Malignant PTT (8103/3) of the skin is not reportable. A neoplasm originating in the skin with histology coded to 8103 is not reportable. See 1.b.i. on page 7 in the 2018 SEER manual for a complete list, https://seer.cancer.gov/manuals/2018/SPCSM_2018_maindoc.pdf

Mammary analogue secretory carcinoma (MASC) is reportable. MASC is a recently described tumor that predominantly arises in the parotid gland. In this case, if the primary site is submandibular gland, assign C080.  We contacted our expert pathologist and he stated that the best code to use for MASC is 8502/3.  Override any edits triggered by the combination of C080 and 8502/3.

Mammary fibromatosis is not reportable. The WHO classification for breast tumors assigns mammary fibromatosis a behavior code of /1. According to WHO, mammary fibromatosis "is a locally infiltrative lesion without metastatic potential…"

Many of these terms are not specified in the codes and definitions in ICD-10-CM. This is because ICD-10-CM does not have the same granularity as ICD-O-3.2. There are a few sites where intraepithelial neoplasia II and/or III are mentioned.

Even though ICD-O-3.2 classifies these as /2 (in-situ), for the intraepithelial neoplasia that are listed in ICD-10-CM, Grade II is designated as benign, while Grade III is designated as in-situ. It is not clear if medical coding will change the Grade II to an in-situ code.

All the in-situ codes (except cervix) are included in the casefinding list. Grade III is included with the in-situ codes; however, there is no guarantee that medical coders will code them as in situ. High grades are coded as in-situ in ICD-10-CM.

For those where there is no specific intraepithelial neoplasia code, the benign codes will cover any benign lesion for that site. This would make for a lot of review using the codes for casefinding. Most of the benign codes were removed from the casefinding list a couple of years ago to make it more manageable. 

Use the casefinding list as a guide for these neoplasias. It is not the most definitive source due to the lack of specificity of ICD-10-CM. It is not possible to map every single histology to a specific code. It is also not known how medical coders across the U.S. are coding these neoplasias. For that reason, pathology should remain the foremost casefinding resource used.

The casefinding team will need to review the prepared list below and determine what codes to add. Any updates will be incorporated in the FY2025 updates (October 2024.)

Marital Status is self-reported because the information is recorded in the medical record based on information obtained from the patient. Use text fields to document relevant information.

Examples

1. Assign code 2 for the first tumor and assign code 1 for the subsequent tumor unless the available information indicates the patient is divorced at the time of the subsequent tumor diagnosis. Patient may self-report single after a divorce. Assign code 4 in that situation.

The code assigned for marital status reflects the patient's marital status at the time of diagnosis for the tumor being abstracted. It is possible that marital status may be different for each tumor if the patient has multiple tumors.

2. If marital status is stated to be single, assign code 1.

3. If marital status is stated to be common law marriage, assign code 2.

Common Law Marriage is defined as a couple living together for a period of time and declaring themselves as married to friends, family, and the community, having never gone through a formal ceremony or obtained a marriage license.

Mast Cell Activation Syndrome (MCAS) is not a reportable neoplasm unless it is specifically stated to be a result of a mast cell proliferative disorder that is reportable.

Per our expert pathologist, MCAS is a relatively new term used for conditions in which patients experience the symptoms of mast cell mediators in the absence of an increase/proliferation of mast cells. The diagnosis of this group of disorders is based in part on a complex of symptoms and on the demonstration of no increase in mast cells. Some of these diseases are difficult to separate from mastocytosis (which is reportable). Currently, this group of disorders is not part of the systemic mastocytosis/mast cell leukemia/mast cell sarcoma spectrum.

Mature teratomas in the ovary are benign [9080/0].

For testis, mature teratoma in an adult is malignant (9080/3); however, mature teratoma in a child is benign (9080/0).

With regard to the thyroid issue, from the information above, the medullary carcinoma in the patient's thyroid is clearly a separate event. According to our expert pathologist consultant, "thyroid tissue is one of the many tissue types that may be seen in teratomas. When the teratoma has exclusively or predominantly thyroid tissue the term struma ovarii is used Adenoma or carcinoma of the thyroid type may be seen in this thyroid tissue. If medullary carcinoma were present in the thyroid tissue in the ovary/teratoma, there would be mention of it in the path report."

Meningioangiomatosis is not reportable. It is a cortical lesion which may occur sporadically or in NF2 (neurofibromatosis type 2). It is not listed in ICD-O-3.

Microcarcinoid and carcinoid tumors are reportable. The ICD-O-3 histology code is 8240/3. Microcarcinoid is a designation for neuroendocrine tumors of the stomach when they are less than 0.5 cm. in size. Neuroendocrine tumors of the stomach are designated carcinoid when they are 0.5 cm or larger.

The term microcarcinoid tumor is not equivalent to carcinoid tumorlet.