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We are aware that squamous intraepithelial neoplasia, grade II (e.g., AIN II), 8077/2 is reportable for 2021. However, because this is also called rather than high grade squamous dysplasia (8077/2), we are unsure about reportability. There is no known histology and behavior code for moderate squamous dysplasia, the classifications available are only low grade (8077/0) or high grade (8077/2).

In 2018, patient has lymph node metastasis confined to left retroperitoneal area; core biopsy was done which showed metastatic adenocarcinoma, unknown primary site. There are no other sites of disease found.  Should I code Mets at Diagnosis--Distant Lymph Node(s) as 1, and the others such as bone and lung as 0?

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.

11/24/20 Adnexal mass, cyst fluid: Positive for malignant cells. Clusters of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells. Groups of inhibin-negative, CK7-positive epithelial cells consistent with serous borderline tumor cells. Peritoneal washing: Positive for malignant cells. Small groups of inhibin-positive, CK7-negative cells, consistent with adult granulosa cell tumor cells.

A. Left ovarian mass: Adult granulosa cell tumor (AGCT) of ovary (see note). pTNM Stage: pT1c3 pNX - Serous borderline tumor (SBT) of ovary (see note). pTNM Stage: pT1a pNX. Fallopian tube; unremarkable.

B. Right ovary: - Serous cystadenofibroma of ovary. Fallopian tube; unremarkable.

C. Left pelvic wall nodule: Fibro-calcified nodule, consistent with necrotic appendix epiploica.

D. Uterus (hysterectomy): Uterine leiomyomas. Endosalpingiosis of uterine serosa and paracervical tissue. Atrophic endometrium.

Note: The left ovarian mass is involved by a combined adult granulosa cell tumor and a serous borderline tumor. The AGCT mainly involves the thick-walled cystic area while the SBT the thin-walled cyst/s. The 2 neoplastic elements do, however, demonstrate areas of intimate and close intermingling. From the current literature, it appears that, based on FOXL2 mutation, the AGCT component of combined AGCT and ovarian epithelial tumors is either a true neoplastic processes or an AGCT- like proliferation morphologically indistinguishable from AGCT. To further evaluate the nature of the AGCT component, a FOXL2 analysis is in progress and an addendum will follow.

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)?

HL-7 e-path report, Final Diagnosis

High normocellular marrow with maturing trilineage hematopoiesis, multilineage dyspoiesis, compatible with MDS-MLD and involvement by plasma cell neoplasm/myeloma, IgA kappa positive, approximately 20-25% of total cellularity present. See comment.

Comments

Correlation with other relevant laboratory (amount and type of serum and urine paraprotein levels, renal function tests, serum calcium level, and anemia) and radiologic (lytic bone lesions) findings is recommended for complete interpretation.  Dyspoiesis of all lineages is seen and the findings are compatible with low grade myelodysplastic syndrome (MDS-MLD), assuming that other possible causes are excluded. Correlation with cytogenetic and molecular studies is recommended for complete characterization

4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored.

4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH.

5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia.

6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma.

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.