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Updated Answer July 2024

Code histology as myelodysplastic neoplasm with increased blasts (9983/3) based on the WHO Classification of Hematolymphoid Tumors, 5th edition, Beta version 2.  WHO lists MDS with increased blasts-2 (MDS-IB2) as a subtype of 9983/3.

Terms coded to 9983/3 include

• Myelodysplastic neoplasm with increased blasts
• Myelodysplastic neoplasm with increased blasts-1
• Myelodysplastic neoplasm with increased blasts-2
• Myelodysplastic neoplasm with increased blasts and fibrosis
• Subtypes: MDS with increased blasts-1 (MDS-IB1); MDS with increased blasts-2 (MDS-IB2); MDS with increased blasts and fibrosis (MDS-F)

When differences exist between WHO and ICC, assign the histology based on the WHO Classification.

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.

If you are unable to follow up with the physician, do not report this case until or unless more information becomes available.

Assign 8013/3 (combined large cell neuroendocrine carcinoma). There are two histologies present: large cell NEC and urothelial. Literature search found primary large cell NEC of the bladder is extremely rare with less than 20 reported cases. This case does not fall into the site-specific rules and given it's raity, a specific rule for this situation was not and will not be added to the Bladder rules. See #1, Example 2, in the general instructions for coding histology.

Assign histology code, 8077/2 (squamous intraepithelial neoplasia, high grade) for LIN III and for LIN II. ICD-O-3.2 lists squamous intraepithelial neoplasia, grade II and grade III as 8077/2 indicating it is reportable. ICD-O-3.2 does not list every site-specific type of intraepithelial neoplasia. Check the SEER manual for reportable and non-reportable examples.

Please understand that AJCC sets the standards for TNM Staging and the Cancer PathCHART (CPC) initiative sets standards for the validity of site and morphology combinations. The statement in the AJCC manual “8240 and 8249 are excluded for topography code C160” means that these two histologies are not staged using the AJCC Staging System. As with numerous other reportable entities that are not staged by AJCC, the case is reportable and a Summary Stage should be assigned. Combinations of C160 with 8240 or 8249 are valid site/histology combinations for registry reporting and should not be discouraged from use if they correspond to the pathologist’s diagnosis. This goes for any other similar note in the AJCC manual. All CPC standards are enforced via the Primary Site, Morphology-Type, Beh ICDO3, 2024 (SEER) N7040 and Histologic Type ICDO3, Primary Site, Date of Diagnosis (NAACCR) N4911 data quality edits. Registrars can also look up the validity of site and morphology combinations using the CPC*Search tool:  https://seer.cancer.gov/cancerpathchart/search/tool/. 

It is important to remember the following.

• ALWAYS code the tumor histology stated by the pathologist/physician
• NEVER change the tumor histology to assign TNM
• Not all tumors or histologies can be staged per TNM
• Cases that cannot be assigned TNM are assigned a summary stage

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).

We will add the same definition for teratoma with malignant transformation found in the ovary table: 9084/3 Teratoma with malignant transformation when a malignant (/3) histology arises in a benign teratoma.

Teratoma with malignant transformation and teratoma with somatic-type malignancy are synonoyms. The term teratoma with somatic-type malignancy is outdated and no longer recommended.

Abstract two primaries using Urinary Solid Tumor Rules, Rule M12. The histologies include non-invasive papillary urothelial carcinoma (8130/2) and non-invasive micropapillary urothelial carcinoma (8131/3). The two histology codes are listed as subtypes of Papillary urothelial (transitional cell) carcinoma in column 3 of Table 2.

WHO Classification of Urinary and Male Genital Tumors, 5th edition classifies micropapillary urothelial carcinoma as an aggressive subtype of urothelial carcinoma with carcinoma in situ present in more than half of all micropapillary carcinomas.

Rule 7 Note 3 of the Urinary Solid Tumor Rules states that there are no /2 subtypes for urothelial carcinoma with the exception of papillary urothelial carcinoma and applies to multiple occurrences of /2 urothelial carcinoma of the bladder.

Rule 8 applies to 8131/3 and 8120/3.

Report these high grade dysplasia of the following organs as stated below.

Stomach: Assign code 8148/2 glandular intraepithelial neoplasia, high grade using the Other Sites Solid Tumor Rules, Table 6: Stomach Histologies and as described in the WHO Classification of Digestive Tumors, 5th edition.

Small intestine and Esophagus: Assign code 8148/2 glandular intraepithelial neoplasia, high grade, using the Other Sites Solid Tumor Rules, Other Sites Histology Rules, Rule H4/H26. The following note is listed for both of these rules.

Note: This list may not include all reportable neoplasms for 8148/2. See SEER Program Coding and Staging Manual or STORE manual for reportable neoplasms

The Other Sites Solid Tumor Rules, Table 5: Esophagus Histologies and Table 7: Small Intestine and Ampulla of Vater Histologies will be updated to reflect this code as time permits.