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Most often when the invasive tumor and the in situ component are in the same lung and are the same histology, rule M12 (example 3) applies and this is a single primary. If the first wedge resection included part of the tumor and the in situ was not separate from the tumor, it is a single primary. We suspect that the margins were positive on the first wedge specimen which prompted the second wedge resection where the in situ was found. In addition, terminology for lung malignancies is undergoing change: what was called BAC (invasive) is now called adenocarcinoma in situ.

Do not report primary acquired melanosis with atypia.

According to our expert pathologist consultant, "There has been a lot of debate in the literature about the diagnostic criteria, terminology, and natural history of primary acquired melanosis [PAM]. Your case comes down squarely on the main issue, which is whether PAM with atypia should be regarded as melanoma in situ. In most studies it appears that PAMs with no atypia or mild atypia do not progress to melanoma, and only a small percentage of those with severe atypia do so."  "PAM, even with atypia, is not melanoma in situ, and should not be reported."

For further information, see this article for a review of a large number of patients: Shields, Jerry A, Shields, Carol L, et al. Primary Acquired Melanosis of the Conjunctiva: Experience with 311 Eyes. Trans. Am Ophthalmol Soc 105:61-72, Dec 2007.

SCC of skin, signet ring cell type, is not reportable to SEER. SCC's of skin classifiable to 8050-8084 are not reportable to SEER. See page 11 in the SEER manual, http://seer.cancer.gov/manuals/2015/SPCSM_2015_maindoc.pdf

Signet ring is a rare histological variant of SCC and is coded to 8070/3 according to the WHO classification for skin tumors.

Follow rule H3 and code squamous cell carcinoma for these cases unless you can obtain confirmation that these cases should be coded keratinizing squamous cell carcinoma from the lab and/or pathologist. Document this confirmation in your policies and procedures.

The MP/H rules were written with input from leading pathologists in each specialty area. Based on their expert opinion, we instruct registrars to code histology based on the information in the final diagnosis. The microscopic description may contain other terms, but the pathologist lists only the pertinent terms in the final diagnosis.

Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

Code the primary site C621 (descended testis). The primary site of this neoplasm is a scrotal (descended) testis. The history of orchiopexy can be noted in a text field, but does not change the primary site in this case.

The code in Surgical Procedure of Primary Site should correspond to the most invasive, extensive, or definitive surgery when the patient has multiple surgical procedures of the primary site even if there is no residual tumor found in the pathologic specimen from the more extensive surgery. The timing of the procedures does not affect the code choice.

Assign code 27 for the first example. Assign code 24 for the second example.

Assign 8480/3.

There is no mixed ICD-O-3 code for EMPSCG. Both histologies are in the mucinous family: mucinous adenocarcinoma (8480/3) and sweat gland carcinoma (8400/3). Apply Other sites rule H17 and code the numerically higher ICD-O-3 code (8480/3).

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma with a strong predilection to the eyelid region. It is histologically analogous to endocrine ductal carcinoma/solid papillary carcinoma of the breast and is characterized by a multinodular solid cystic mucinous tumor with immunoreactivity to neuroendocrine markers. Only 20 cases of this unusual tumor have been reported.

If there is no further information about where the cancer originated, assign C220. Use ICD-O-3 as the source for coding topography. The topography code associated with combined hepatocellular and cholangiocarcinoma (8180/3) is C220 when there is no other information available, according to ICD-O-3.

Assign histology code 8312, renal cell ca, NOS. There is no specific code for the solid variant of renal cell carcinoma.