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In this scenario, we record that HT began for the second primary on the date of diagnosis, and the Systemic/Surgery Sequence ends up usually being coded 4 because the HT continues even if the specific agent may be changed. This does not seem to meet the definition of neoadjuvant therapy for the second primary so we approach the staging and grade coding as just clinical/pathological? For example, if the tumor size at surgery is a little larger than estimated on imaging, we would use the pathologic size for our staging. The tumor size and grade of the second primary are not being changed by the ongoing HT. Do we have the right approach?

Patient is diagnosed March 2021, with malignant pleural effusion, clinical impression supports either endometrial or tubo-ovarian primary and neoadjuvant chemotherapy is given. Subsequent total abdominal hysterectomy/bilateral salpingo-oophorectomy (TAH/BSO) in July, shows high-grade serous carcinoma involving the right fallopian tube and bilateral ovaries, as well as bilateral STIC. Summary Stage lists tumor site as right fallopian tube, with the serous tubal intraepithelial carcinoma (STIC) noted under “additional findings.”

Should the contralateral (left-sided) STIC be accessioned as an additional primary, per MP/H Rule M8, the since fallopian tubes are listed in Table 1 as Paired Organs with Laterality?

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)?

Patient was diagnosed on 01/2020, and primary site on the pathology report is Ampulla of Vater (C241). Synoptic Report states histology as: Intra ampullary papillary-tubular neoplasm in association with microinvasion.

I have reviewed the ICD-O-3 coding table and found histology Intraductal tubulopapillary neoplasm (C25_) code 8503/2. Based on the Matrix principle (Rule F on the ICD-O-3), I will change the behavior to 3 and code as 8503/3. If I look in ICDO-3, Tubulopapillary adenocarcinoma is coded 8263/3.

Ampulla, biopsy: High grade dysplasia with focal intramucosal carcinoma in a background of ulceration with acute and chronic inflammation.

Surgery pathology: Head of pancreas, duodenum, and distal stomach, pancreaticoduodenectomy-Ampulla, Adenocarcinoma, intestinal type, intra-ampullary and peri-ampullary (mixed type). Grade moderately differentiated, 1.5cm. Tumor invades into duodenal submucosa. Lymphovascular Invasion: Foci suspicious for lymphovascular invasion identified. Perineural Invasion: Present.

Synoptic report: Tumor Site Intra-ampullary and peri-ampullary (mixed type). Histologic Type Adenocarcinoma, intestinal type.

There is not enough information regarding site in radiology reports or operative report. CT-A/P/C:The patient's known ampullary mass is not well visualized on this exam. No significant intrahepatic or extrahepatic biliary ductal dilation is identified. The pancreatic duct is normal caliber.

2021 Bone marrow biopsy showed erythroid hyperplasia, increased histiocytes with hemophagocytosis and Factor XIIIa positive histocytic cells. Moderate cytoplasmic staining for ALK 1, consistent with bone marrow involvement of ALK-positive histiocytosis. A subsequent kidney lesion biopsy was also found to have ALK-positive histiocytosis. The patient was then treated with clofarabine.

Patient is 3 years old.

07/2020-Chart indicates patient presented in June with fevers and refusing to walk with pancytopenia, bone marrow biopsy showed no leukemia buthistiocytes. Impression: ALK positive histiocytosis involving BM and kidney.

10/2020 Bone marrow final diagnosis states right and left bone marrow aspirates and biopsies: No morphologic or immunohistochemical evidence of involvement by the patient's previously diagnosed ALK+ histiocytosis (see Comments) - Multiple histiocytic collections with prominent hemosiderin; favor reactive - background normocellular bone marrow with maturing trilineage hematopoiesis.

The patient's prior bone marrow samples are reviewed (9/2020 and 7/2020). Similar to the September bone marrow sample, the current marrow shows numerous histiocyte collections with abundant associated hemosiderin deposition. These histiocytes have a stellate/dendritic appearance and lack the atypical features noted in the patient's marrow at diagnosis, favoring a reactive process. This impression is further supported by the lack of immunoreactivity for either Factor XIIIa or ALK1 among these cells. There is no convincing morphologic or immunohistochemical evidence of marrow involvement by the patient's previously diagnosed ALK+ histiocytosis within the sampled material. Of note, the marrow otherwise appears normocellular for the patient's age, indicative of ongoing marrow recovery post therapy.

It is not clear whether this would be equivalent to Langerhans cell histiocytosis, disseminated (9751/3) as there is not a statement of Langerhans cell or whether this is just histiocytosis, NOS and not reportable.

Table 3 (Breast Equivalent Terms and Definitions) lists “Neuroendocrine tumor, well-differentiated” of the breast as histology 8246/3. There is no entry for a grade 2 neuroendocrine tumor of the breast in Table 3.  

The pathologist did not indicate the neuroendocrine tumor was poorly differentiated (or it would otherwise be a small cell carcinoma). The pathologist noted “By current WHO criteria, this tumor is characteristic of a mammary neuroendocrine tumor, grade 2.  These invasive tumors have similar prognostic and predictive features of invasive ductal carcinoma of the same grade and stage.”

Example: Breast case where first course treatment plan is neoadjuvant therapy and surgery after. The patient was hospitalized during neoadjuvant therapy, elected hospice, and later died, so the neoadjuvant therapy was never completed, surgery not done. How are the 2021 neoadjuvant therapy fields coded in this situation as neoadjuvant therapy and surgery were part of first course plans. I coded neoadjuvant therapy to 2 - started but not completed, but there are no codes to properly explain the clinical response and therapy treatment effect as the patient did not complete neoadjuvant therapy. Should I use code 9 for clinical response and treatment effect or should this be left blank for this particular case?