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The neoplasm is reportable if it originated in the spinal cord or is intradural (within the spinal dura; spinal nerve roots are intradural). If there is not enough information to determine the exact site of origin, do not report the case.

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).

Assign primary site code C340 when a lung tumor is described as a hilar mass.

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.

Code carcinoma with neuroendocrine differentiation (8574/3). MP/H rule H7 applies: code the higher ICD-O-3 code. There is non-small cell lung carcinoma (8046/3) and a carcinoma, NOS with neuroendocrine differentiation present (8574/3).

Noninvasive low grade (micropapillary) serous carcinoma (MPSC) of the ovary is reportable. Assign code 8460/2, applying the ICD-O-3 matrix concept to this noninvasive carcinoma. Noninvasive can be used as a synonym for in situ, ICD-O-3 behavior code /2. See page 66 in the softcover ICD-O-3. Low malignant potential (LMP) means that the neoplasm is not malignant, but has some chance of behaving in a malignant fashion. LMP can be used as a synonym for ICD-O-3 behavior code /1, see page 66.

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.

Accession the new tumor in the transplanted organ as you would any other new/second primary. As transplants have become more common especially for liver, lung, and kidney, we are seeing more of these types of cases. We are adding instructions to the revised MP/H rules on coding subsequent primaries when they occur in a transplanted organ. We are also looking at adding a data field that will identify cancers/tumors which arose in a transplanted organ. We feel this is important to track for analysis. Until the revised MP/H rules are implemented, we will look at adding general coding instructions to the SEER Program Manual for transplants.

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.

Code cutanteous leiomyosarcoma to skin. Leiomyosarcoma can originate in the smooth muscle of the dermis. The WHO classification designates this as cutaneous leiomyosarcoma. The major portion of the tumor is in the dermis, although subcutaneous extension is present in some cases.