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The code for retinoic acid depends upon the primary site and histology of the tumor. Code retinoic acid (also called Vitamin A, tretinoin, ATRA, all-transretinoic acid or Vesanoid) in the Immunotherapy field as 01 [Immuno administered as first course therapy] for acute promyelocytic leukemia. This drug is given to patients as an alternative to chemotherapy.

For all other sites/histologies, code retinoic acid in the Other Cancer-Directed Therapy Field. Use code 2 [Other experimental cancer-directed therapy] or 3 [Double-blind clinical trial, code not yet broken] if the drug is given as part of a protocol. If the drug is not being given as part of a protocol or you don't know whether it is part of a protocol, use code 1 [Other cancer-directed therapy].

For cases diagnosed 1998-2003:

Code the EOD-Lymph Nodes field to 6 [Axillary regional lymph nodes, NOS], if the size of the metastasis within the lymph node is not known. "Extension into perinodal soft tissue" does not imply that the lymph nodes are fixed to one another or to other structures. AJCC stage for lymph nodes is coded to N1 [Metastasis to moveable ipsilateral axillary lymph nodes].

In order to code the EOD-Lymph Nodes field to 5 [Fixed/matted ipsilateral axillary nodes] which is the equivalent to AJCC equivalent N2, there must be some clinical or pathologic statement of fixation or matting. There can be extension through the capsule without fixation or matting. "Fixation" is a clinical term and "matting" can be either clinical or pathologic. A pathologist can recognize two or more lymph nodes stuck together by tumor.

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.

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/

This answer was provided in the context of CSv1 coding guidelines. The response may not be used after your registry database has been converted to CSv2.Lymphatic channels/vessels carry lymph fluid throughout the organs and tissues of the body. Lymph channels/vessels within an organ are not nodes. Lymph channels/vessels outside an organ are not nodes.

Updated answer

Assign code 9413/0. 

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 2003 and later: For cryosurgery alone, without a pathology specimen, assign site-specific surgery code 19 [Local tumor destruction, NOS]. Cryosurgery, cryotherapy or cryoablation uses extreme cold to destroy the tumor cells.

If a specimen is sent to pathology use code 20 [Partial mastectomy, NOS] rather than code 19.

If cryosurgery is followed by further surgery, do not use code 19.

For tumors diagnosed prior to 2007:

Abstract the case above as one primary according to multiple primary rule 3a. Code the primary site to C579 [Female genital, NOS] according to the table on page 9 of the 2004 SEER Manual.

Multiple tumors of the same site and same histology diagnosed at the same time are abstracted as one primary. Multiple independent tumors of the vulva and vagina are abstracted as a single site when diagnosed simultaneously. VAIN III and VIN III have the same histology code [8077].

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.