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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.

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.

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?

Pathology Diagnosis: Left temporal lesion - Low grade glial/glioneuronal neoplasm BRAF mutant. Pathologist Comment: The histopathological appearance of this lesion does not allow for a definitive diagnosis. However, the low-grade appearance, fibrillary nature, immunohistochemical profile, and the presence of a BRAF V600E mutation allow this to be categorized as a low-grade glial or possibly glioneuronal tumor. Despite the lack of exact classification this neoplasm can be expected to behave in a very indolent manner consistent with a WHO grade I classification.

Example: Small intestine wedge resection with GIST, 1.8 cm in mid small intestine, single nodule. Stomach nodule biopsy: GIST, 0.3 cm. Pathology report comment section indicates the gastric GIST is not staged due to the small size and incidental nature.

Core biopsy of left breast at 2:00: Invasive ductal carcinoma, Nottingham score 6/9.

Core biopsy of left breast at 4:00: Invasive mucinous carcinoma (variant of ductal carcinoma), Nottingham score 5/9.

Post neo-adjuvant mastectomy: Main (largest tumor): Invasive ductal carcinoma, upper outer quadrant grade 2. Secondary tumor: mucinous carcinoma, grade 1 at 4:00.

Patient has a 1.7 cm encapsulated papillary carcinoma staged as pTis located 2 cm from the nipple and Paget disease of the nipple on mastectomy pathology.

There is no indication in Table 3: Specific Histologies, NOS/NST, and Subtypes/Variants that encapsulated papillary carcinoma is a subtype of ductal carcinoma. Rule M8 notes that if the histology of the underlying tumor is any histology OTHER THAN duct or subtypes of duct, one should continue through the rules. But if M9 applies to this case, then incidence reporting will be increased in comparison to prior years.