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Patient has biopsy of prostate 12/28/2018 showing Gleason 5+5 adenocarcinoma. Liver biopsy on same date is metastatic small cell carcinoma consistent with prostate primary. Oncology consult states that liver biopsy is likely neuroendocrine conversion from prostate carcinoma. Patient also has bone metastasis and receives radiation, Lupron, Casodex, and chemotherapy of carboplatin and etopiside.

Per Solid Tumor Rules, we code histology from primary site over a metastatic site. Thus, the small cell carcinoma, which appears to be the focus of the chemotherapy is lost. Is it correct to code this as a single primary with an adenocarcinoma histology?

Both SINQ 20130221 and 20180088 instruct us to abstract multiple primaries when patient develops a metastatic small cell carcinoma of the prostate after being previously diagnosed with adenocarcinoma of the prostate.

Core biopsy of left breast at 2:00: Invasive ductal carcinoma, Nottingham score 6/9.

Core biopsy of left breast at 4:00: Invasive mucinous carcinoma (variant of ductal carcinoma), Nottingham score 5/9.

Post neo-adjuvant mastectomy: Main (largest tumor): Invasive ductal carcinoma, upper outer quadrant grade 2. Secondary tumor: mucinous carcinoma, grade 1 at 4:00.

SINQ 20160023 and the Solid Tumor Rules indicate NET G1 (or well differentiated NET) is coded as 8240 and NET G2 is coded as 8249.

Clarification regarding grade coding in the CAnswer Forum indicates well differentiated neuroendocrine tumor refers to the histologic type, and not the grade. Therefore, the term well differentiated is ignored for the purpose of grade coding.

Neither of these sources clarifies how to code histology for a tumor diagnosed as well differentiated neuroendocrine tumor, grade 2.

IMPRESSION/PLAN: Patient is a previously healthy middle aged woman with a diagnosis of adenocarcinoma of the rectum, clinical stage II (T3N0M0). We will proceed with a neoadjuvant course of radiation and concurrent chemotherapy (5-FU) to maximize local regional control and survival, and hopefully facilitate a sphincter-sparing resection in the future. The primary tumor and the pelvic nodes at risk will receive 4500 cGy delivered over 25 treatments. The primary tumor will subsequently receive an additional 1080 cGy delivered over 5 treatments, for a cumulative dose of 580 cGy.

PATHOLOGY: Adenocarcinoma of the rectum, clinical stage II (T3N0M0). The patient is referred by (dr) for a neoadjuvant course of chemoradiotherapy.

HPI: Patient presented recently with rectal bleeding and a change in bowel habits. Colonoscopy revealed an ulcerated mass located 4.0 cm above the anal verge. A biopsy was positive for invasive well-differentiated adenocarcinoma that arose from a tubular adenoma. A staging work-up demonstrated no evidence of metastatic disease.

Table 3 (Specific Histologies, NOS, and Subtypes/Variants) lists Ewing sarcoma as a synonym for Peripheral primitive neuroectodermal tumor 9364. Presumably, this is to be used for the reportable malignant peripheral nerve tumors when diagnosed as pPNET or Ewing sarcoma. However, this patient has a type of central (or CNS) primitive neuroectodermal tumor (histology 9473). Table 3 does not list central primitive neuroectodermal tumor (PNET or CPNET) as a valid histology for CNS tumors.

While Table 3 does not list all the possible histologies for the CNS, it currently is not clear how one would arrive at the histology code for a CNS Ewing sarcoma family tumor with CIC alteration, as this is recognized as a new entity for primitive neuroectodermal tumors of the CNS (i.e., PNET, histology 9473) per multiple journal articles. Ewing sarcoma family tumors include both peripheral PNET and central PNET tumors, but to code this histology as a peripheral PNET (9364) in this case seems incorrect when the primary tumor is stated to be of central nervous system origin, not peripheral nervous system origin.

Example: Left breast total mastectomy final diagnosis is incidental microscopic findings suspicious for early Paget disease of the nipple. The diagnosis comment states: The left breast mastectomy shows mildly atypical cells within the nipple epidermis which are suspicious for early Paget disease of the nipple. Additional sampling of the left breast was performed, and no evidence of atypical hyperplasia, in situ carcinoma, or invasive carcinoma within the left breast tissue was identified.

Would this case be non-reportable using rationale similar to an early/evolving melanoma per SINQ 20180029?

Example: Patient has multiple biopsies with final diagnosis of in situ papillary urothelial carcinoma in the prostatic urethra and invasive papillary urothelial carcinoma in the bladder.

How should primary site be coded in this type of mixed in situ and invasive situation?

Regarding the answer to SINQ 20180093: This is a single primary; coded 8140/3 adenocarcinoma. In the biopsy and the two tumors found on lobectomy, the specific adenocarcinoma histologies are described as acinar predominant pattern, solid growth pattern and lepidic predominant pattern. You do not code a pattern, so rule M7 above applies and this is a single primary.

My question is based on Note 2 in Coding Multiple Histologies for lung cancers that says: Predominantly describes the greater amount of tumor. Predominant and majority are synonyms. Per the CAP protocol, the term predominant is acceptable for the following specific subtypes of adenocarcinoma. For these subtypes only, the word predominant is used to describe both the subtype and the grade of the tumor.

In our region, pathologists often describe histology using the term phenotype. However, the use of the term phenotype is not discussed in the Solid Tumor Manual.

Example: Final Diagnosis of a colon tumor is invasive adenocarcinoma with a mixed phenotype, and the Diagnosis Comment states: The majority of the disease is poorly differentiated/signet ring cell phenotype.

Would the histology be coded to 8490 (signet ring cell carcinoma), if the majority of the tumor is a more specific histology described by the term phenotype?

Patient has a 1.7 cm encapsulated papillary carcinoma staged as pTis located 2 cm from the nipple and Paget disease of the nipple on mastectomy pathology.

There is no indication in Table 3: Specific Histologies, NOS/NST, and Subtypes/Variants that encapsulated papillary carcinoma is a subtype of ductal carcinoma. Rule M8 notes that if the histology of the underlying tumor is any histology OTHER THAN duct or subtypes of duct, one should continue through the rules. But if M9 applies to this case, then incidence reporting will be increased in comparison to prior years.