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Abstract a single primary and assign code 9700/3 for MF.  According to our subject matter expert, this is all MF.  When MF progresses, there can be large cd30 positive T cells. This is not the same as anaplastic large cell lymphoma.

Assign histology code 8254/3 (mixed invasive mucinous and non-mucinous adenocarcinoma) to this lung tumor using Lung Solid Tumor Rules, Rule H4. This is a new code/term approved by IARC/WHO for ICD-O. Rule H4 instructs one to code the histology when only one histology is present. In this case, the pathologist indicates the tumor is mixed mucinous and non-mucinous histologies. The non-mucinous carcinoma that is seen in this mixed histology may be identified as: Adenocarcinoma in situ, minimally invasive adenocarcinoma, or lepidic predominant adenocarcinoma. In this case it is lepidic predominant adenocarcinoma. Lepidic is a recognized histology in lung. It is not unusual for the pathologist to indicate mixed non-muncinous and mucinous adenocarcinoma AND also list the non-mucinous subytpe. It is important to capture both mucinous and non-mucinous histologies which drives treatment, etc. 

Abstract multiple primaries using the Solid Tumor Rules, Other Sites, Rule M13.  The topography differs at the second and third characters (C54.1 Endometrium; C15 Esophagus).  Rule M12 refers to being disease-free vs. recurrence of a tumor, where Note 1 states that clinically disease-free means no evidence of recurrence in the same site on follow up.

A note can be added to clarify that M12 applies to new tumors in the SAME site.

Abstract this case as a single primary. Code as 8044/3 (small cell carcinoma, hypercalcemic type) listed in the Other Sites Solid Tumor Rules, Table 13. Small cell carcinoma, large cell variant, is a subtype of small cell carcinoma, hypercalcemic type. This table does not include all possible histologies.

WHO Classification of Female Genital Tumors, 5th edition, states: Small cell carcinoma of the ovary, hypercalcemic type, is rare, accounting for < 1% of ovarian tumors. Small cell carcinomas, hypercalcemic type, are usually large, with a mean size of 15 cm (range: 6–26 cm). Large cells are present (in varying numbers) in half of these tumors, which are designated “small cell carcinoma, large cell subtype” if the large cells are predominant (which is rare).

Abstract two primaries using the 2024 Malignant Central Nervous System (CNS) and Peripheral Nerves Solid Tumor Rules, Rule M13. Glioblastoma, IDH-wild-type (9440/3) and astrocytoma, IDH-mutant, grade 4 (9445/3) are on two separate rows in Table 3 of the Malignant CNS and Peripheral Nerves Solid Tumor Rules.

WHO Classification of Central Nervous System, 5th edition, lists the subtypes of glioblastoma, IDH-wild-type as giant cell glioblastoma; gliosarcoma; and epithelioid glioblastoma. The term glioblastoma multiforme is not recommended for glioblastoma, IDH-wildtype in the 5th edition, and lists astrocytoma, IDH-mutant, grade 4 as a subtype of astrocytoma, IDH-mutant.

Report this case of leukemia cutis and code to bone marrow (C421) and leukemia NOS (9800/3) based on the information provided. Update the abstract if new information becomes available.

Leukemia cutis is the rare infiltration of neoplastic leukocytes into the epidermis, dermis, or subcutis from an existing leukemia that results in clinically identifiable cutaneous lesions. Leukemia cutis may precede, follow, or occur concurrently with the diagnosis of systemic leukemia. It is an advanced phase of the leukemia having a poor prognosis that also strongly correlates with additional sites of extramedullary involvement. This can alter the appropriate treatment regimen for a patient. It is a type of "metastasis" or spread of the leukemia cells. The "conventional" definition for leukemia cutis is the infiltration of skin from a bone marrow primary. It is most often diagnosed via skin biopsy—punch, shave, etc., utilizing IHC/biomarker testing and is commonly associated with CMML and acute myeloid leukemia (AML). As such, it a reportable condition especially when preceding a confirmed systemic leukemia diagnosis. In this situation, the diagnosis date would be the date of the positive leukemia cutis skin bx—punch, shave, etc. The case should be coded to C421; 9800/3 Leukemia NOS until the official systemic leukemia diagnosis is rendered. If possible, follow back should be conducted to determine the specific systemic leukemia histology (CMML; AML) and the treatment received. If the leukemia cutis follows or occurs concurrently with the diagnosis of a systemic leukemia, it is NOT a separate primary but merely an advanced stage of the systemic leukemia diagnosis.

Stop at the first rule that applies which is M12. Per note 3: When it is unknown/not documented whether the patient had a recurrence, use date of diagnosis to compute the time interval. This means there are two primaries.

There is a genetic syndrome that causes multiple carcinoid tumors in the GI tract, per our GI expert, and they should be treated as new primaries per M12.

SINQ 20110046 describes a unique situation whereby the subject matter expert felt that the occurrence of multiple tumors was due to an unknown underlying condition driving the proliferation of neuroendocrine cells.

Code as an oropharyngeal primary site and histology as solitary plasmacytoma (9734/3) based on consultation with our hematological expert.

The WHO Classification of Hematopoietic and Lymphoid Tissues defines multiple myeloma as "bone marrow plasma cell percentage >60%." There are several other factors, but the bone marrow involvement is the key point for your case. The pathologist also states that the bone marrow is consistent with "plasma cell neoplasm," which by itself is not the same as multiple myeloma.

This case has 5-10% involvement by plasma cell neoplasm. This does not meet the bone marrow qualifications for multiple myeloma and is consistent with the pathologist's statement that there is minimal bone marrow involvement.

We will be updating the Hematopoietic and Lymphoid Neoplasms Database and Manual to clarify this (2025 updates).

The example used in Rule M15 of the Urinary Solid Tumor Rules should refer to Table 2. We will update this in the next revision of the Rules.

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.)