MP/H Rules/Multiple Primaries--Urinary: How many primaries are there and which MP rules apply in this scenario? See discussion.
Patient has 2 tumors in the left ureter; one is transitional cell (8120) and one is papillary transitional cell (8130). Rule M6 says BLADDER tumors with any combination of the following histologies ... are a single primary. But this is not a bladder case. Rule M8 says urothelial tumors in 2 or more of the following sites are a single primary... but this is not in 2 or more sites. Rule M9 then says histologies different at the 1st, 2nd, or 3rd digit are separate primaries. That makes this 2 primaries, but I do not think this should be 2 primaries.
Rule M9 applies. Abstract 2 primaries.
We will evaluate this scenario for the next version of the multiple primary rules.
Reportability/MP/H Rules/Histology: Is this kidney tumor diagnosis reportable? If so, what is the correct histology? See discussion.
Left radical nephrectomy: Tumor histologic type: Renal angiomyoadenomatous tumor (see Note). Note: The a clear cell papillary renal cell tumor and a renal angiomyoadenomatous tumor (""RAT"") (reval cell carcinoma with angioleiomyoma-like stroma). Although some authors consider RAT tumors to represent a pattern of clear cell papillary RCC we believe that this represents a dstinct entity. The combined findings ...confirm the diagnosis of renal angiomyoadenomatous (RAT) tumor. These tumors are also known as renal cell carcinoma within angioleiomyoma-like stroma. To date none of these tumors have developed metastases. Given the small number of reported cases we would consider these to have at worst a low malignant potential.
According to our expert pathologist adviser, renal angiomyoadenomatous tumor ("RAT") is not reportable. He states "l would be reluctant to consider the entity malignant. The authors of the papers describing it do not seem ready to call it malignant either. I agree with calling it LMP, or in this case uncertain malignant potential."
Histology--Breast: Please confirm the morphology code for a diagnosis of "encapsulated papillary carcinoma" of the breast. Several articles on the internet lead me to believe it is the same as an intracystic carcinoma, code 8504/2 (our case shows no evidence of invasion).
You are correct in coding 8504/2 for this case. Per the 4th Edition WHO Tumors of the Breast, encapsulated papillary carcinoma (EPC) of the breast is synonymous with intracystic or encysted papillary carcinoma. It is a variant of ductal carcinoma in situ (DCIS).
Surgery of Primary Site--Corpus uteri: What is the correct surgery code to assign for dilation and curettage (D&C) for an in-situ endometrium (C541) primary? The code to use for the cervix uteri (C530-C539) is specified, but not for the corpus uteri (C540-C549).
Assign code 20 for endometrial D&C for in situ cancer of endometrium.
Reportability--Pancreas: Is a solid pseudopapillary neoplasm of the pancreas reportable?
Solid pseudopapillary neoplasm of the pancreas is reportable. According to the WHO classification, it is a "low-grade malignant neoplasm…[which] frequently undergoes hemorrhagic-cystic degeneration and occurs predominantly in young women."
Assign topography code C25 with the appropriate 4th digit. Code the histology as 8452/3.
MP/H Rules/Histology/Multiple primaries--GE junction: How is histology coded for a goblet cell carcinoma in the GE junction? See discussion.
The patient was diagnosed with GE junction signet ring adenocarcinoma (8490/3) in 5/2012, treated with radiation. GE junction biopsy on 9/20/2012 showed residual signet ring carcinoma. Subsequent biopsies on 7/8/2013 showed GE junction biopsy of invasive adenocarcinoma, signet ring cell type along with “Esophagus, distal and GE junction biopsies” (site not further clarified in available documentation) with Goblet cell carcinoma. The histology code for the goblet cell carcinoma is needed to determine the number of primaries.
According to our expert pathologist consultant, goblet cell is a descriptive term and not a specific histology in this context. There is no ICD-O-3 code for it. The "goblet cell carcinoma" in this case is not a new primary.
Goblet cell is used to describe some cells containing mucin. In addition to individual tumor cells containing mucin which compresses the nucleus to give the appearance of signet rings, the mucin is present in columnar cells with the nuclei at one end -- this latter is a pattern often seen when glandular structures are formed by the tumor cells. It is also often intermixed with the signet ring cells in the surrounding stroma.
MP/H Rules/Histology--Testis: How should histology be coded for a testicular teratoma with somatic type malignancy (adenocarcinoma)? See discussion.
11/8/2013 Rt orchiectomy: teratoma with somatic type malignancy (adenocarcinoma).
5/2/2014 Abdominal mass excision: metastatic teratoma involving matted lymph nodes. Patient age at diagnosis is 31.
Per web search, a teratoma with somatic type malignancy is a rare type of tumor. Should the histology be coded to 8140/3? This seems to conflict with SINQ 20120085, which indicates a testicular mature teratoma in an adult is malignant, and in this example, it was also the portion of tumor that metastasized.
Assign code 9084/3, listed in ICDO as teratoma with malignant transformation.
Our expert pathologist consultant states that this is a very rare situation. The non-germ cell components are believed to arise out of the teratoma portions, and are seen in only of few percent of teratomas. They are given the designation "teratoma with somatic type malignancies" (WHO).
MP/H Rules/Histology--Breast: What is the correct histology code for this final diagnosis of a breast tumor: INVASIVE POORLY DIFFERENTIATED DUCTAL CARCINOMA WITH SQUAMOUS DIFFERENTIATION (METAPLASTIC FEATURES)?
Code the histology to 8575/3.
The instruction for coding duct and another non-duct histology not listed in Table 3 was inadverantly left out of the rules. The default is to code to the histology with the numerically higher ICD-O-3 code which is 8575/3.
Reportability--Appendix: Is a pathologic final diagnosis of an appendix with "well-differentiated neuroendocrine tumor (carcinoid)" reportable? See discussion.
SINQ 20130027 states that "well-differentiated neuroendocrine tumor" of the appendix is reportable (8240/3) while "carcinoid" tumors of the appendix are not reportable (8240/1). Please explain the difference between "well-differentiated neuroendocrine tumor" of the appendix and a "carcinoid" of the appendix.
Well-differentiated neuroendocrine tumor of the appendix is reportable. The difference is terminology. "Carcinoid" is listed in ICD-O-3 as a /1 for appendix making it non-reportable.
When both terms are used, ask for clarification from the pathologist. Failing that, accept the reportable terminology and report the case.
Behavior--Breast: Is behavior for encapsulated papillary carcinoma (EPC) of the breast coded as noninvasive or invasive?
The pathologist has the final say on behavior. Code behavior based on the pathologist's final diagnosis. See Rule F in ICD-O-3.
According the WHO Classification of Breast Tumors, encapsulated papillary carcinoma of the breast is in situ, /2. Encapsulated papillary carcinoma with invasion is assigned /3. WHO describes "frank invasive carcinoma" for this histology as "neoplastic epithelial elements infiltrate beyond the fibrous capsule of encapsulated papillary carcinomas." WHO cautions that true infiltration should be "differentiated from entrapment of neoplastic epithelial cells in the fibrous capsule and from epithelial displacement into the biopsy site, which is frequently encountered following needle-core procedures of papillary lesions."
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