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Example: Patient diagnosed following a resection of a cystic mass at L2-4 that proved an intradural tumor excision with final diagnosis of schwannoma, WHO grade 1.

Per new Solid Tumor Rules, the primary site in this case should be coded C476 (peripheral nerves of trunk, NOS) and histology is 9560/0 (schwannoma, NOS). However, there are currently no coding options in the Soft Tissue of Trunk and Extremities EOD schema relating to a benign tumor. Likewise there are no coding options in the Soft Tissue and Sarcoma Summary Stage 2018 schema relating to a benign tumor.

How should EOD 2018 and Summary Stage 2018 be coded for reportable benign schwannomas of the spinal nerve roots?

The patient has two, separate, non-contiguous tumors. One tumor is a benign meningioma and the other is a malignant oligodendroglioma.

The original plan was not to treat the asymptomatic meningioma. However, after worsening symptoms, imaging and resection proved a separate left frontal lobe malignant tumor.

Rule M5 is the only M Rule in the Malignant CNS Multiple Primary Rules, Multiple Tumors module that addresses separate non-malignant and malignant tumors. This rule provides only two criteria to follow when a malignant tumor follows a non-malignant tumor. The first criteria (for non-malignant tumor followed by malignant tumor) states:

--Patient had a resection of the non-malignant tumor (not the same tumor) OR

--It is unknown/not documented if the patient had a resection.

This patient did not have a resection of the original, separate, non-malignant tumor, but the treatment plan was known to not include a resection. Should Rule M5 also apply to cases where the patient never had treatment planned for the separate non-malignant tumor?

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

There are two histologies provided in the final diagnosis, malignant spindle cell neoplasm (8004/3) and atypical fibroxanthoma (8830/3). There is a definitive diagnosis of the non-specific histology, but the more specific histology is only described using ambiguous terminology.

The external ear (C442) is included in the Head and Neck schema for diagnosis year 2018 and later. The Head and Neck Histology Rules indicate ambiguous terminology cannot be used to code a more specific histology. So ignoring the atypical fibroxanthoma, because it is modified by ambiguous terminology, we are left with a non-reportable site and histology combination (C442, 8004/3).

Diagnoses of malignant atypical fibroxanthomas are regularly diagnosed using the syntax above in our area. Follow-up with the physician or pathologist is generally not possible as these cases are received from dermatopathology clinics only. The pathology report is the only information that will be received. If the reportable diagnosis of malignant atypical fibroxanthoma is ignored per the current Solid Tumor Rules, incidence cases will be lost.

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?

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.

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.

Patient has biopsy of prostate 12/28/2018 showing Gleason 5+5 adenocarcinoma. Liver biopsy on same date is metastatic small cell carcinoma consistent with prostate primary. Oncology consult states that liver biopsy is likely neuroendocrine conversion from prostate carcinoma. Patient also has bone metastasis and receives radiation, Lupron, Casodex, and chemotherapy of carboplatin and etopiside.

Per Solid Tumor Rules, we code histology from primary site over a metastatic site. Thus, the small cell carcinoma, which appears to be the focus of the chemotherapy is lost. Is it correct to code this as a single primary with an adenocarcinoma histology?

Both SINQ 20130221 and 20180088 instruct us to abstract multiple primaries when patient develops a metastatic small cell carcinoma of the prostate after being previously diagnosed with adenocarcinoma of the prostate.

Example: Operative report documents a left breast skin sparing mastectomy and sentinel node biopsy procedure. Pathology report lists left axillary sentinel nodes in specimen A) with 0/2 nodes positive, and left breast mastectomy without axilla in specimen B) yielding an additional 0/2 intramammary nodes positive. Would the Scope of Regional Node Surgery be coded as 2 (SLN biopsy) to capture the intent of the sentinel node procedure only, or 6 (code 2 + 4) to capture the actual type and number of nodes removed?

SEER Coding and Staging Manual includes Scope of Regional Lymph Node Surgery instruction 4.b. which mentions assigning code 4 to intra-organ node removal. Similarly, there is instruction for coding SLN biopsy as code 2 and SLN biopsy with axillary dissection at the same time (code 6) or during separate procedures (code 7). However, it is not clear this combination code is how we should also capture an incidental intra-organ node removal.