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Patient has a 2021 skin excision showing an atypical spindle cell neoplasm, most consistent with dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation.

Per the ICD-O-3.2 Coding Table, DFSP, NOS has a behavior code of /1, and DFSP, fibrosarcomatous has a behavior code of /3. There is no code listed for DFSP with fibrosarcomatous transformation. Transformation is not included as a term that can/cannot be used for the Other Sites Schema, but this type of DFSP is often described as DFSP with fibrosarcomatous transformation. How do we code DFSP when transformation is used to describe fibrosarcomatous?

Patient is diagnosed July 2021 with high-grade serous carcinoma on ascites cytology. Tumor debulking total abdominal hysterectomy/bilateral salpingo-oophorectomy in August shows high-grade serous carcinoma involving the right ovary (capsule intact, right fallopian tube is negative), left ovary (capsule ruptured), and fallopian tube. Pathologist has chosen tumor site to be bilateral ovaries in the staging summary, with the left fallopian tube listed as “other tissue/organ involvement” along with uterus, peritoneum, and omentum. Additional findings in staging summary includes serous tubal intraepithelial carcinoma (STIC).

Our interpretation of SINQ 20210025 is that any case with both ovarian and tubal involvement would be coded as a fallopian tube primary if STIC is present, even when the pathologist is clearly calling the case ovarian. If this is correct, then the previous SINQ 20120093 may need to be updated with a date restriction reference since it would be in disagreement with this instruction.

If our interpretation is incorrect, then the STIC would be an additional primary per MP/H Rule M11.

Unless there is a biopsy that proves in situ tumor (EOD code 000, Tis) or extratesticular invasion into the scrotum, penis, or further contiguous extension (EOD code 700, T4), EOD Primary Tumor must be coded based on the PATHOLOGICAL assessment (orchiectomy). There are no other CLINICAL codes because the AJCC indicates imaging is not used for local T-categorization, and the EOD derives the AJCC TNM staging. If the case can not be coded to either EOD Primary Tumor codes 000 or 700 clinically, the only clinical code that seems to apply is 999 (Unknown).

We are seeing more cases treated with neoadjuvant chemotherapy prior to orchiectomy, especially in patients with distant metastatic disease. The EOD Manual indicates that clinical evidence takes priority over pathological evidencewhen neoadjuvant treatment is given, unless the extent of disease following neoadjuvant treatment is greater than pre-treatment clinical findings. If the clinical and pathological information are the same, code the extension based on the clinical information.

Do these general rules also apply to testis even though we cannot code CLINICAL findings for these tumors? If so, will EOD Primary Tumor be coded to 999 (Unknown) for any testis primary that is not in situ or invasive into the scrotum, etc., that is treated with neoadjuvant therapy? Or should the post-neoadjuvant PATHOLOGICAL assessment be coded for these tumors because the CLINICAL assessment would otherwise be unknown?

How is the EOD Primary Tumor coded for the following two cases?

1. Left testicular mixed germ cell tumor, biopsy-proven metastasis to a supraclavicular lymph node. The left testis contained a small mass on scrotal ultrasound. The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved no residual primary tumor (ypT0). Is EOD Primary Tumor 999 because it is clinically unknown (even though it was clinically limited) or 800 (No evidence of primary tumor) because there was no pathological evidence of tumor following neoadjuvant treatment?

2. Right testicular mixed germ cell tumor with biopsy-proven inguinal lymph node metastasis. There was a palpable mass in right testis on physical exam (not described as fixed or involving scrotum). The patient underwent neoadjuvant chemotherapy, and the post-treatment orchiectomy proved a residual 2 cm tumor limited to the testis without lymphovascular invasion (LVI). Is EOD Primary Tumor 999 because it is clinicallyunknown or 200 (PATHOLOGICAL assessment only - Limited to testis WITHOUT LVI)?

Rule H6 states: Code 8742/3 (Lentigo maligna melanoma) when the diagnosis is lentigo maligna melanoma with no other histologic types. However, if the diagnosis was strictly lentigo maligna or lentigo maligna melanoma, the first rule that applies is Rule H1 because lentigo maligna melanoma is a single, specific histologic type and Rule H1 states, Code the histology when only one histologic type is identified. Following the current rules, one would never arrive at Rule H6. Should the H Rules be reordered? Or should an example of when one would use Rule H6 be added to clarify when to use this rule?

The Breast EOD Regional Nodes notes contain new clarification regarding the clinical assessment vs. pathological assessment codes, but the new Note 2 does not specifically indicate an exception for neoadjuvant therapy. However, if the pre-treatment lymph node core biopsy proved cN1 disease, and the post-treatment resection proved ypN0 disease, should the clinical assessment code (code 150) have priority over any pathological assessment code (including 200) since the involved lymph node was only clinically positive and not pathologically positive? Should an exception be added to Note 2 to address cases where neoadjuvant therapy is given, but the clinical assessment is greater than the pathological assessment?

A physician stated the patient likely had secondary polycythemia vera due to cardiac and pulmonary conditions but that a polycythemia vera could not be ruled out. A JAK2 was ordered that was positive for JAK2 V617F mutation. The patient was treated with hydrea.

According to SEER SINQ 20120049, secondary polycythemia vera is not reportable. However, in this case, the patient was positive for JAK2 V617F mutation. Therefore, is this reportable? We looked for guidance in the Hematopoietic and Lymphoid Neoplasms Database and found it confusing that secondary polycythemia vera was not mentioned or discussed under polycythemia vera in the database. The only thing we could find was secondary polycythemia NOS that was discussed under polycythemia.

We are preparing to send our hospitals a reminder that the behavior changes from 2 to 3 at the bottom of the basement membrane, and the T category changes from Tis to T1 at the bottom of the mucosa for colon and rectum carcinomas. We are confused by the wording of the Exception.

Distinguishing In Situ and Localized Tumors for the Digestive System

1.b. If the tumor has penetrated the basement membrane to invade the lamina propria, in which case it is localized and assigned Summary Stage 1 (localized) and for invasion of the lamina propria

Exception: Code 0 (behavior code 2) includes cancer cells confined within the glandular basement membrane (intraepithelial); includes in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.)

The text following (intraepithelial) is unclear. The question is: Does the text include in situ plus intramucosal carcinoma (involvement of the lamina propria and may involve but not penetrate through the muscularis mucosa) (penetration through the muscularis mucosa is behavior code 3.) mean the following:

Code 0 (behavior code 2) includes in situ plus intramucosal carcinoma. In situ plus intramucosal carcinoma is involvement of the lamina propria, which may involve (but not penetrate through) the muscularis mucosae. Penetration through the muscularis mucosa is behavior 3. If that is what the text above means, then it seems that the 2018 SEER Summary Stage Manual is saying colorectal tumors reported as: adenocarcinoma in situ, at least intramucosal adenocarcinoma in situ, high grade dysplasia/intramucosal adenocarcinoma in situ, focally intramucosal at the margin are to be coded behavior 2 and SEER Summary stage In situ (0) like the intraepithelial carcinoma tumors. However, it conflicts with the EOD Data for Colon and Rectum, Note 2, and SINQ 20210006. The text for both EOD Data for Colon and Rectum and SINQ 20210006 is clear. According to them, the above bulleted adenocarcarcinoma examples are coded SEER Summary Stage localized (1) and behavior 3. SINQ 20210006 states that: For purposes of Summary Stage, intramucosal carcinoma is a localized lesion So, intramucosal carcinoma is coded SEER Summary Stage 1 (localized) and (behavior code 3).

According to the text for EOD Primary Tumor, Colon and Rectum, Note 2 below, intramucosal, NOS involvement is invasive.

Note 2: Code 050 (behavior code 3) includes the following:

Intramucosal, NOS

Lamina propria

Mucosa, NOS

Confined to, but not through the muscularis mucosa

Thank you for your help clarifying the 2018 SEER Summary Manual Exception text above.