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The bone marrow biopsy proved bone marrow with blasts comprising 15-19%. Neither the pathologist nor the physician specifically diagnosed this as AML, calling this only high risk disease or early evolving AML prior to starting the patient on Vidaza.

No further information can be obtained from the pathologist or the physician for this case. Should this early evolving AML be accessioned as an additional primary per Rule M10, or is this the same MDS that is now high risk as the blast count is up to 19%, but has not yet reached the threshold of 20% blasts usually required for AML per the Hematopoietic and Lymphoid Neoplasm Database?

Is an M rule needed to address multiple tumors and Note 2 in Table 3? Does Note 2 in Table 3 apply when multiple tumors exist and one tumor contains only ductal carcinoma?

The M Rules currently confirm that a metaplastic carcinoma (whether it is involved with ductal or lobular) and a separate ductal carcinoma are separate primaries because these histologies are on different rows in Table 3 (separate primaries per M14). There is no specific rule regarding metaplastic carcinomas in the Multiple Tumors (M Rules) module, so presumably, the presence of a separate ductal carcinoma is not lumped into Note 2 in Table 3 for metaplastic carcinoma.

However, the note is confusing when there are multiple tumors involved because it appears to the registrars there are two options for coding the histology. To some registrars, the rules indicate it does not matter if the tumor is predominantly ductal carcinoma as long as some percentage of metaplastic carcinoma is present, code histology to metaplastic carcinoma. For other registrars, the presence of solely a ductal carcinoma in a second tumor is a separate primary from the separate metaplastic carcinoma.

The M rules and Note 2 need to clarify this issue to promote consistency.

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.

There are four invasive tumors in the right lung:

Large cell undifferentiated carcinoma in the right lower lobe (8012/3, C343);

Adenocarcinoma, acinar-predominant in the right lower lobe (8551/3, C343) that was 0.7 cm in size and limited to the lung;

Mucinous adenocarcinoma in the right upper lobe (8253/3, C341) that was 0.9 cm and limited to the lung;

Adenocarcinoma, NOS also in the right upper lobe (8140/3, C341) that was 1 cm and limited to the lung.

The Lung M Rules confirm the large cell undifferentiated carcinoma is a separate primary from the three adenocarcinoma tumors (Rule M8). The acinar adenocarcinoma and mucinous adenocarcinoma tumors are separate primaries (Rule M6). The adenocarcinoma, NOS tumor is the same primary as both the acinar and mucinous are adenocarcinomas (Rule M7).

How is Primary Site coded for both the acinar and mucinous adenocarcinomas if they represent multiple tumors reported as a single primary (when compared to the adenocarcinoma, NOS tumor)?

Should the adenocarcinoma, NOS tumor also be included when coding EOD Primary Tumor for both the right lower lobe acinar adenocarcinoma and right upper lobe mucinous adenocarcinoma primaries? Further follow-up with the physician is not possible.

We think this should be a single primary because the Solid Tumor rules do not apply to metastases. However, we are not sure whether or not the instructions outlined for prostate (SINQ 20180088, 20130221), that indicate we are to accession a new metastatic tumor only with a small cell neuroendocrine histology after an adenocarcinoma, also applies to lung primaries.

We are aware of a phenomenon in which lung adenocarcinoma cases treated with Erlotinib can transform to small cell, but do not know whether it impacts the number of reportable primaries.

Pathology report dated 10/17/2019: Final Diagnosis: Fallopian tubes and gonads, right and left, excision: Dysgenetic gonadal tissue with nests and tubules of atypical germ cells suspicious for gonadoblastoma and at least germ cell neoplasia in situ; and segments of fallopian tube (pending expert consultation).

For example, chemotherapy and immunotherapy administered first, followed by surgery, then immunotherapy and hormone therapy after surgery. Or is code 4 used for two administrations of chemotherapy before surgery and two more courses after surgery?

SINQ 20130140 indicates a histologic diagnosis that is characteristic of a specified malignancy is reportable because this is equivalent to the term, diagnostic of. Does the same logic apply to a clinical diagnosis that is strongly characteristic of a malignancy on imaging?

SINQ 20180104 indicates the term, almost certainly, is not a reportable ambiguous term. If a radiologist notes a mass was most certainly malignant, is this adequate to accession this as reportable? Is a clinically certain diagnosis equivalent to diagnostic of? Or are the modifiers almost and most irrelevant because the terms certainly and certain are not on the ambiguous terminology list?

The Hematopoietic and Lymphoid Neoplasms Database (Heme DB) definition for systemic mastocytosis with an associated hematological neoplasm (SM-AHN, 9741/3) states SM-AHN is a variant of systemic mastocytosis that arises with a myeloid disease of non-mast cell lineage (e.g., MDS, MPN, etc.) and that,

However, SINQ 20130172 conflicts with the Heme DB stating the systemic mastocytosis and the associated hematological neoplasm are a single primary coded to a single histology (9741/3) per Rule M2.

After a little research, it appears as though Endometrial Dedifferentiated carcinoma is a relatively new term and is set to be included in ICD-O-3.2:

http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

If you look at the link on that page for All Additions, Changes, and Revisions to the ICD-O-3, 1st Revision for ICDO-3.2, there is 8020/3 Dedifferentiated carcinoma. Currently, 8020/3 is Carcinoma, undifferentiated, NOS. For 2018 diagnosis, would you use 8020/3 for a predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma as stated in the pathology: Uterus, bilateral ovaries and fallopian tubes; supracervical hysterectomy/BSO: Predominantly dedifferentiated carcinoma with focal well-differentiated endometrioid adenocarcinoma in the endometrium, FIGO grade 1 Portion of omentum, omental/anterior abdominal wall/ round ligament/uterine/small bowel mesenteric tumor nodules all involved by dedifferentiated carcinoma. Synoptic reads as follows: Histological Type: Endometrioid carcinoma, NOS Dedifferentiated carcinoma predominantly Histological Grade: Endometrioid carcinoma, FIGO grade 1.