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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?

11/01/2018, lung, left upper lobe, wedge resection: Invasive moderately differentiated adenocarcinoma, predominantly papillary subtype, with minor acinar and lepidic subtypes. Would this be 8260/3 since the acinar and lepidic subtypes are described as minor or would this be 8255/3 because there is papillary plus two other subtypes/variants described as subtypes?

Example: Patient has a secretory carcinoma of minor salivary gland tissue (mammary analogue secretory carcinoma [MASC]) of the mucosal lower lip; it is unclear which table to use and how to arrive at the correct histology using the H Rules.

Rule H1 (code the histology when only one histology is present) states, Note 1: Use Tables 1-9 to code histology. There is no table that includes the lip. The correct histology should be 8502 which is listed in Table 6 (Tumors of Salivary Glands) however this does not correspond to minor salivary glands of the mucosal lip (site C003 per ICD-O-3 coding instruction).

The 2018 ICD-O-3 Update table does not include this histology, however Table 6 indicates code 8502 (secretory carcinoma) is a new code that was approved by IARC/WHO.

The ICD-O-3 only includes this histology as secretory carcinoma of breast. Therefore, in order to arrive at the correct histology, one must be aware of previous SINQ entries 20160036 and 20130003 that indicate secretory carcinoma (or MASC) is histology 8502. However, these are related to MP/H Rules, so registrars may be hesitant to apply this guideline to cases coded using Solid Tumor Rules.

In the April 2019 Breast Solid Tumor Rules update, the Priority Order for Using Documentation to Identify Histology was changed, giving equal priority to the Final diagnosis / synoptic report as required by CAP (item 2B).

There are technically two histologies documented for the case above; a Not Otherwise Stated (NOS)/No Special Type (NST) (invasive mammary carcinoma, per final diagnosis text) and subtype/variant (invasive cribriform carcinoma, per CAP report). If we do not use the synoptic report with priority over the final diagnosis, Rule H14 indicates the histology would be the NOS histology (invasive mammary carcinoma) because the percentage of tumor is not given for the subtype. However, SINQ 20180045 states, In the CAP protocol, the term Histologic Type is a label where the histology that corresponds to the largest carcinoma is collected. According to the CAP protocol for invasive breast cancer, the histologic type corresponds to the largest carcinoma.

If the pathologist summarizes the findings in a synoptic report, should the specific Histologic Type identified have priority?

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).

The patient is a 34 year old who presented with testicular pain radiating into the abdomen approximately 1 month before orchiectomy in 2018. CT abdomen/pelvis: Multiple focal sclerotic bone lesions. Given the lack of change from July 2014, these are likely benign bone islands. No adenopathy mentioned. He has no prior history of germ cell tumor nor any surgery for any tumor/cancer before this. Pathology: Testis, left, radical orchiectomy: - Demarcated scar tissue (1.3 cm), with atrophic seminiferous tubules and cortical bone consistent with burnt-out germ cell tumor. No evidence of germ cell neoplasia in situ (GCNIS). - Margins are unremarkable.

7/25/17

Part A: Left breast at 8:00, 5 CFN: Specimen type: Stereotactic biopsy. Tumor type: Ductal carcinoma in situ (DCIS), cribriform type. Tumor size: The largest focus of DCIS measures 1 mm in greatest dimension as measured on the slide. Nuclear grade: 2 (Intermediate grade). Microcalcifications: Present. Other findings: Stromal fibrosis, microcalcification and fat necrosis.

11/1/17

A. Sentinel lymph node, left: One lymph node, negative for metastatic tumor on three levels of routine H\T\E and pan cytokeratin immunohistochemical stains.

B. Left breast: Procedure: Total mastectomy with skin and nipple. Specimen Laterality: Left. Lymph Node Sampling: Yes, portion A. Specimen Integrity: Intact. Histologic Type: Extensive ductal carcinoma in situ and one focus of Invasive ductal carcinoma with mucinous features. Histologic Grade (Nottingham Histologic Score): Glandular Differentiation: Score 3 Nuclear Grade: Score 2. Mitotic Count: Score 1. Total Nottingham score 6 (grade 2, moderately differentiated). Tumor Size: 3.3 x 2 mm (0.33 x 0.2 cm) measured on slide (B3). Tumor Site: Lower inner quadrant of left breast. Tumor Focality: Unifocal. Ductal Carcinoma In Situ (DCIS): Present, cribriform, solid and micropapillary types with focal necrosis and calcifications. Size of DCIS: Number of blocks examined: Thirty (30). Number of blocks with DCIS: Thirteen (13). Lobular Carcinoma In Situ (LCIS): Not identified, Lymphovascular Invasion: Present. Perineural Invasion: Not identified. Other Findings: Changes consistent with previous biopsy site. Cysts, foci of atypical ductal hyperplasia, focal ductal hyperplasia, adenosis, stromal fibrosis and microcalcifications. Skin (epidermis): Uninvolved. Nipple: Uninvolved. Margins: 1 mm from DCIS to the closest deep margin (slide B12). At least 10 mm (1 cm) from invasive carcinoma to deep margin. Estrogen receptor (ER, clone 1D5) by immunohistochemistry performed on this material: Positive (invasive and in situ carcinoma), high intensity, in greater than 95% of carcinoma cells. Progesterone receptor (PR, clone 16) by immunohistochemistry performed on this material: Positive (invasive and in situ carcinoma), moderate intensity in about 80% of the carcinoma cells. Her 2 by FISH performed on this material: Pending, an addendum to follow. Pathologic staging: pT1aN0(sn)MX (AJCC 7th edition). Dictated by: (Pathologist), MD Intradepartmental review.

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.

Example: Patient has a left cerebral meningioma that is meningothelial meningioma (9531) and two right-sided cerebral meningiomas: one that is transitional meningioma (9537) and the other that is meningioma, transitional and angiomatous, WHO Grade I. If the histology for the mixed tumor is 9534 (angiomatous meningioma), then there are three primaries. If the histology is 9537 (transitional meningioma), then there are two primaries.

Per Table 6, angiomatous meningioma is 9534/0 and transitional meningioma is 9537/0. There is no mixed histology coding rule, or mixed histology meningioma code. There is also no default rule that would instruct registrars to code the numerically higher ICD-O code or to default to a meningioma (NOS) histology code.