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The pathologist has the final say on behavior. Code behavior based on the pathologist's final diagnosis. See Rule F in ICD-O-3.

According the WHO Classification of Breast Tumors, encapsulated papillary carcinoma of the breast is in situ, /2. Encapsulated papillary carcinoma with invasion is assigned /3. WHO describes "frank invasive carcinoma" for this histology as "neoplastic epithelial elements infiltrate beyond the fibrous capsule of encapsulated papillary carcinomas." WHO cautions that true infiltration should be "differentiated from entrapment of neoplastic epithelial cells in the fibrous capsule and from epithelial displacement into the biopsy site, which is frequently encountered following needle-core procedures of papillary lesions."

For cases diagnosed 2007 to 2017 ONLY: Code the histology as papillary urothelial carcinoma [8130].NOTE: In the CAP checklist, the statement that the tumor has a papillary configuration is a further description of this tumor. This is supported by the pathologist's stage of pTa [noninvasive papillary carcinoma]. Use the information from the CAP checklist when available. The MP/H Rules will be revised to include the term "configuration" in the specific histology terms for in situ tumors.

The steps used to arrive at this decision are

Step 1: Open the Multiple Primary and Histology Coding Rules manual. Choose one of the three (i.e., flowchart, matrix or text) and go to the Urinary Histo rules. The module you use depends on the behavior and number of tumors identified in the primary site. In this case, the patient has a single bladder tumor per the submitted information.

Step 2: Start at Rule H1 in the Single Tumor module. The rules are intended to be reviewed in consecutive order from Rule H1 to Rule H15. Stop at the first rule that applies to the case you are processing. Stop at Rule H7. Code the histology as 8130/2 (noninvasive papillary urothelial carcinoma) when the urothelial carcinoma is stated to have a papillary configuration.

For cases diagnosed 2018 or later, refer to the Solid Tumor Rules, https://seer.cancer.gov/tools/solidtumor/

For cases diagnosed 1998-2003:

Code the EOD-Extension field to 30 [Localized, NOS], unless more information is known about the extent of tumor involvement. Coding the Histology field to 8142/3 [Linitis plastica] and the Size of Primary Tumor field to 998 [Diffuse; widespread; 3/4 or more: Linitis plastica] identifies this diagnosis.

In the EOD-Extension field, the depth of invasion is the important characteristic to be coded. The 10 digit EOD corresponds to the AJCC Staging Manual in which the "T" is based on level of invasion. While a diagnosis of linitis plastica indicates a worse prognosis, it does not define the extent of infiltration. There is no luminal mass with linitis plastica. Instead, the entire gastric wall is thickened by tumor.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Hemophagocytic lymphohistiocytosis (HLH) is not a reportable disease because it is not listed in the Heme DB.

Per our expert pathologist consultant, "HLH is a lymphocyte driven hemophagocytic syndrome which may be either genetically based or caused by over-activated lymphoid cells, often in response to a viral infection. It is an abnormal immune response and is not considered a malignant disease, and is, therefore, not reportable. It is not synonymous with EBV-positive T-cell lymphoproliferative disease of childhood (9724/3)."

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

Yes, LCIS is reportable. Staging does not determine reportability. Follow the reportability requirements of your state and national standard setter. SEER reportability requirements are found in the SEER manual starting on page 5, https://seer.cancer.gov/manuals/2016/SPCSM_2016_maindoc.pdf

See the General Coding Rules on page 92 of the 2004 SEER Manual for instructions about coding grade.

If the only information available is "Grade 2," assign code 2 [Grade II].

This is a correction to the SEER manual. Lymphomas originating in lymph nodes (C77) could have distant metastases to any site except lymph nodes. The following corrections to the manual apply now and will appear in the next version of the manual.

Remove C770-C779 from the instruction for assigning code 8 on the following pages.

Page 135 Mets at Dx--Bone

Page 137 Mets at Dx--Brain

Page 139 Mets at Dx--Liver

Page 141 Mets at Dx--Lung

Page 145 Mets at Dx--Other

Example

Biopsy of axillary lymph node: Diffuse Large B-Cell lymphoma. Lymph nodes involved above and below the diaphragm, multiple nodules seen in lung, lesions in liver. Bone marrow biopsy positive for DLBLC. Per Hematopoietic manual, primary site would be C778 for multiple lymph node regions involved.

Mets at Dx--Bone-0

Mets at Dx--Brain-0

Mets at Dx--Liver-1

Mets at Dx--Lung-1

Mets at Dx--Distant Lymph Nodes-8

Mets at Dx--Other-1