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The Solid Tumor Rules instruct us not to use differentiation for coding histology unless it is specifically listed in the table. The terminology non-small cell carcinoma with neuroendocrine differentiation is not in lung histology Table 2.

However, SINQ 20150033, prior to Solid Tumor rules, indicates this diagnosis should be coded to 8574 (adenocarcinoma/carcinoma with neuroendocrine differentiation).

This presentation appears to represent distinctly different histologies. However, because the 2018 histology diagnosis is not in the table and the prior SINQ appears to disagree with current instruction, it is not clear how to apply the M rules to this case. The outcome of the histology coding will affect the number of primaries reported in this case.

For example, 9/12/18 left lung upper lobe lobectomy: 1.5 cm, 0.8 cm invasive component, lepidic predominant adenocarcinoma with acinar and lepidic patterns, G2, no visceral pleural invasion, no LVI, 0/14 LNS positive. An additional minimally invasive adenocarcinoma, 1 mm, was seen away from the main tumor. The correct coding of the minimally invasive adenocarcinoma will ultimately determine if we have one tumor (using rule M7) versus two primaries (using rule M6).

IMPRESSION/PLAN: Patient is a previously healthy middle aged woman with a diagnosis of adenocarcinoma of the rectum, clinical stage II (T3N0M0). We will proceed with a neoadjuvant course of radiation and concurrent chemotherapy (5-FU) to maximize local regional control and survival, and hopefully facilitate a sphincter-sparing resection in the future. The primary tumor and the pelvic nodes at risk will receive 4500 cGy delivered over 25 treatments. The primary tumor will subsequently receive an additional 1080 cGy delivered over 5 treatments, for a cumulative dose of 580 cGy.

PATHOLOGY: Adenocarcinoma of the rectum, clinical stage II (T3N0M0). The patient is referred by (dr) for a neoadjuvant course of chemoradiotherapy.

HPI: Patient presented recently with rectal bleeding and a change in bowel habits. Colonoscopy revealed an ulcerated mass located 4.0 cm above the anal verge. A biopsy was positive for invasive well-differentiated adenocarcinoma that arose from a tubular adenoma. A staging work-up demonstrated no evidence of metastatic disease.

Rule PH28 confirms the more specific histologies are ignored if this is truly a low grade B-cell lymphoma (i.e., non-Hodgkin lymphoma, NOS) since both MALT lymphoma and LPL are more specific types of low grade B-cell lymphomas. This leaves only a diagnosis of low grade B-cell lymphoma with plasmacytic differentiation to consider.

SINQ 20130033 states a low grade B-cell lymphoma with plasmacytic differentiation should be coded as 9680/3 (diffuse large B-cell lymphoma (DLBCL)). However, DLBCL is a high grade B-cell lymphoma, not a low grade B-cell lymphoma.

If the pathologist classifies this as a non-specific low grade B-cell lymphoma, and clarifies that this may represent a more specific type of low grade B-cell lymphoma (MALT lymphoma or LPL), should the histology be coded to a high-grade lymphoma (DLBCL) or non-Hodgkin lymphoma, NOS?

Example 1: Operative report states the surgery is a right breast simple mastectomy. There is no lymph node removal documented or attempted; however, a single incidental intramammary node is found in the final pathology results. How should these nodes be captured in the Scope of Regional Lymph Node Surgery field?

CAnswer Forum states to code Scope of Regional Lymph Node Surgery as 0 (No regional lymph nodes removed), see Scope LN surgery, incidental LN found on path, Breast.

However, SEER Program Coding and Staging Manual 2018 instruction states: Code the removal of intra-organ lymph nodes in Scope of Regional Lymph Node Surgery. Example: Local excision of breast cancer. Specimen includes an intra-mammary lymph node. Assign code 4 (1 to 3 regional lymph nodes removed).

The STORE 2018 Manual does not provide instruction for incidental nodes specifically, but does appear to be focused on capturing procedural intent.

Example 2: Patient has bilateral breast primaries. Operative report states the surgery is bilateral simple/skin-sparing mastectomies with bilateral sentinel node biopsies and immediate reconstruction. However, pathology shows that the left breast specimens are labeled: (a) Left breast mastectomy, (b) Left sentinel lymph node biopsy, (c) Additional left lymph nodes biopsy, and (d) Left axillary contents biopsy. The total nodes removed for this case are: 2/2 positive SLN, 0/1 positive intramammary nodes, 1/1 positive additional lymph node, and 3/3 positive axillary contents nodes. How should these nodes be captured in the Scope of Regional Lymph Node Surgery field?

Example: Left breast total mastectomy final diagnosis is incidental microscopic findings suspicious for early Paget disease of the nipple. The diagnosis comment states: The left breast mastectomy shows mildly atypical cells within the nipple epidermis which are suspicious for early Paget disease of the nipple. Additional sampling of the left breast was performed, and no evidence of atypical hyperplasia, in situ carcinoma, or invasive carcinoma within the left breast tissue was identified.

Would this case be non-reportable using rationale similar to an early/evolving melanoma per SINQ 20180029?

The 2018 Breast Solid Tumor Rules Equivalent Terms and Definitions - Changes from 2007 Multiple Primaries/Histology Rules states: Mammary carcinoma is a synonym for carcinoma no special type (NST)/duct carcinoma not otherwise specified (NOS) 8500. It will no longer be coded as carcinoma NOS 8010. Should metastatic carcinomas of breast origin be 8500, or is code 8010 (carcinoma NOS) more applicable because histology coding from metastatic sites is not as reliable?

The physicians do not use the Lugano system as we are instructed to stage chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) as lymphomas. I had always been instructed that this qualifies as "bone marrow involvement," or "diffuse disease," and therefore is a Stage IV. Our experts advise that there is not enough information to code it to bone marrow, but do not elaborate as to whether you can actually code Extent of Disease (EOD), SEER Summary Stage, and AJCC Staging?

Patient has a history of breast cancer and diffuse large B-cell lymphoma (DLBCL), both treated with chemotherapy and radiation.

On 6/26/19, bone marrow biopsy: MDS with excess blasts-2 (18% dysplastic blasts) in a normocellular marrow (overall 40% cellularity) with trilineage dysplasia. Comment: least myelodysplastic syndrome with excess blasts-2. However, an early/evolving AML cannot be completely excluded. The findings likely represent therapy-related myeloid neoplasm.

MD note on 7/15/19: Diagnosis: MDS, high grade borderline AML with complex karyotype secondary disease. Patient has high grade MDS which is bordering on AML transformation with 20% blasts by IHC and areas higher than this. This is likely secondary to the treatment she has received for her other cancers particularly pelvic radiation for her DLBCL. Given her very high IPSS score, it is likely she will eventually develop AML. No treatment given.

On 7/15/19, bone marrow biopsy: Persistent acute leukemia in a marrow with trilineage dyspoiesis and 23% blasts.

According to the literature, CIC gene rearrangement sarcomas in young patients are soft tissue sarcomas with an aggressive clinical course and may have previously been grouped under the Ewing-like family of tumors or as undifferentiated round cell sarcomas. There is currently no guideline in the solid tumor rules for coding a CIC gene rearrangement sarcoma. However, coding the histology to 8800 (sarcoma, NOS) seems unlikely to capture the more aggressive nature of these tumors. Can a more specific histology be coded?