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SINQ 20160023 and the Solid Tumor Rules indicate NET G1 (or well differentiated NET) is coded as 8240 and NET G2 is coded as 8249.

Clarification regarding grade coding in the CAnswer Forum indicates well differentiated neuroendocrine tumor refers to the histologic type, and not the grade. Therefore, the term well differentiated is ignored for the purpose of grade coding.

Neither of these sources clarifies how to code histology for a tumor diagnosed as well differentiated neuroendocrine tumor, grade 2.

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.

This question involves positive cervical lymph nodes with an unknown primary site. The SEER Manual says under the coding instructions for Primary Site:

14. b.Use the NOS category for the organ system or the Ill-Defined Sites (C760-C768) if the physician advisor cannot identify a primary site.

Note: Assign C760 for Occult Head and Neck primaries with positive cervical lymph nodes.

Schema Discriminator 1: Occult Head and Neck Lymph Nodes is used to discriminate between these cases and other uses of C760. Does SEER agree with AJCC that cases with positive cervical lymph nodes that are EBV+ should be coded to the nasopharynx and cases with positive cervical lymph nodes that are p16+ should be coded to the oropharynx, if no primary site is identified?

Example: Patient has left thigh skin excision with final diagnosis of atypical smooth muscle cell proliferation, inked peripheral margin is involved and inked deep margin is free of disease in the sections examined. See Comment.

Diagnosis comment states: The terminology regarding this lesion is controversial. Lesions with identical features are designated as leiomyosarcoma in the dermatopathology literature, whereas, the preferred classification in the soft tissue pathology is atypical intradermal smooth muscle neoplasm. Although the lesion appears predominantly dermal based, since the margin is involved, the lesion cannot be entirely evaluated, and therefore the final designation is deferred to the findings in the excisional specimen. (This slide was read by bone and soft tissue pathologist.)

There has been no excision of this tumor and, as a central registry, we have no access to the pathologist for clarification.

Is this skin case reportable based on the dermatopathology interpretation when further documentation is not available?

For example, pathologic diagnosis is non-small cell carcinoma with squamous features. The medical oncologist describes this as squamous cell carcinoma and begins treatment regimen. As I interpret the rules, we would use code 8046, non-small cell carcinoma, because of instruction 2 and the fact that features is not included in the list of ambiguous terminology.

Patient was diagnosed with Stage IV colon cancer (liver metstasiss) and started on Folfox with Avastin. The medical oncologist decided to continue maintenance treatment with Xeloda and Avastin.

Per Colon NCCN Guidelines Version 3.2019, interest in the use of maintenance therapy approach after first-line treatment of unresectable, metastatic colorectal cancer is growing. In general, this approach involves intensive first-line therapy, followed by less intensive therapy until progression in patients with good response to initial treatment.

Colon Therapy

5/1/18 Colonoscopy biopsy: mod diff colon adenoca, MMR proficient, BRAF wild type

5/5/18 Liver biopsy: mets from colon cancer

6/18/18 " 11/20/2018 Med Onc: started 12 cycles Chemo - Folfox (Fluorouracil, leucovorin, Oxaliplatin) with Avastin

11/28/18 CT Pelvis: continued improvement in the liver mets; no residual tumor involving colon; no new mas or adenopathy in the chest, abdomen or pelvis

12/02/18 Med Onc follow up: Pt had tremendous response to chemotherapy and Avastin, cancer is not curable. Is amenable to maintenance therapy with Xeloda and Avastin; also amenable to descending colectomy in the future

1/7/19 Med Onc: starting maintenance treatment Xeloda + Avastin.

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?

There is a discrepancy between Notes 3 and 6 in the schemas Fallopian Tube, Ovary, and Primary Peritoneal Carcinoma for EOD Primary Tumor. Note 3 describes extension/discontinuous metastasis to the pelvic sites (code 450) and includes the sigmoid colon, rectosigmoid and rectum since these are all pelvic sites. However, Note 6 also includes rectosigmoid and sigmoid colon.

Note 6 is describing extension/discontinuous metastasis to the abdominal sites (600-750), so it should include rectosigmoid or sigmoid colon (since those are pelvic sites). Note 6 indicates, Intestine, large (except rectum). In the previous Collaborative Stage, the corresponding note used to also include: except sigmoid colon, rectosigmoid and rectum.

Did sigmoid colon and rectosigmoid get removed from the list here? That is, should Note 6 read, Intestine, large (except sigmoid colon, rectosigmoid, rectum)? Involvement of the sigmoid, rectosigmoid, or rectum via peritoneal seeding/metastasis is consistent with T2b disease and would correlate with code 450 (pelvic sites), not codes 600-750 (abdominal sites). Those codes only correlate with T3 and greater disease (i.e., peritoneal seeding/metastasis of the abdomen).