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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.

Sequence the brain tumor in the 60-87 series when you do not know the behavior. If you have reason to believe the brain tumor was malignant, sequence it in the 00-59 series.

BCC of lip C00_ is rare and requires a statement that the tumor is on the vermilion border (rather than skin) to be coded C00_ and to be reported. Our expert pathologist consultant refers to an article in the Am Acad Dermatol 2004; 50(3): 384-387.

An extracranial schwannoma is not reportable. The schwannoma must arise on the intracranial part of the nerve to be reportable.

Report this case as a basal cell carcinoma, noninvasive, of the nasal cavity, based on the information provided.

The polyp was removed from the nasal cavity (C300) which is a reportable site for basal cell carcinoma.

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.

Assign surgery of primary site code 42. It is possible to obtain lymph nodes in a mastectomy specimen without an axillary dissection. Remember to capture the excised lymph nodes in the scope of lymph node surgery field.

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).

Rule M13 applies, abstract as two primaries. Since both DLBCL and FL were present in 2011, rule M2 does not fit -- not a single histology. Rule M13 reflects the situation in this case much better: an acute neoplasm which was treated and a chronic neoplasm diagnosed later.

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.