Primary site: How is this field coded for an adenocarcinoma arising in a chronic perianal fistula without extension to the anal canal, but stated to arise in "ectopic rectal tissue"? See Discussion.
The patient underwent a resection of a perineal mass. Per review of slides it was stated to be "primary mucinous adenocarcinoma arising in a chronic perianal fistula." The adenocarcinoma was invasive into the dermal connective tissue and skeletal muscle, but there was no extension into the anal canal. The discharge diagnosis from the reporting facility called this adenocarcinoma of "ectopic rectal tissue in perianal area."
Should the primary site be coded to skin based on the dermal involvement and lack of anal or rectal involvement? Or, should the primary site be coded to rectum based on the physician's assessment that this adenocarcinoma arose in ectopic rectal tissue?
For cases diagnosed 2007-2014:
Code the Primary Site field to C210 [Anus, NOS]. This is an unusual and rare presentation. According to our expert pathologist, "There is no ideal site code [for] this case. I would code to C210. In this location it can at least be located by anyone who wants to get a look at such lesions. Because of the unusual location of this tumor, I would like to be able to code it to perineum, but it will be totally lost in those site codes as they represent extensive areas beyond perianal (skin of trunk, soft tissue of pelvis, and pelvis, respectively)... I would not code to rectum [because it would be] lost among too many primary rectal carcinomas."
Primary Site: How is this field coded for cholangiocarcinoma involving the intrapancreatic bile duct?
Code the primary site as C24.0 [Extrahepatic bile duct, includes Common bile duct] for a cholagiocarcinoma originating in the common bile duct. A portion of the common bile duct is within the head of the pancreas: The intrapancreatic segment of the common bile duct.
Primary site: If text supports a pancreatobiliary primary with no other information what primary site code would be assigned? C249 biliary tract NOS, or C269 GI tract nos, or C809 unknown?
Assign C269 in the absence of any additional information.
Primary Site: Is an "angiosarcoma" stated as arising in the skin of the breast and treated with a mastectomy, coded to the primary site of skin or breast?
Code the primary site as skin of breast when skin of breast is documented as the site of origin.
According to the WHO classification of soft tissue tumors, the majority of angiosarcomas "develop as cutaneous tumors...less than one quarter present as a deep soft tissue mass."
Primary Site: Should we code C80.9 [unknown primary] or code C34.9 [Lung] according to the terminology, "most likely site of origin is lung"? See Description.
We have a case of metastatic keratinizing squamous cell ca. The work-up shows small densities in the lung that may represent inflammatory or chronic changes. No other imaging that shows origin. Physical exam states 2 months of left axillary mass. H/O SCCA of the skin involving chest wall.
Path reads: Metastatic w/d keratinizing SCCA. This lesion almost undoubtedly represents mets. The most likely site of origin is lung followed by esophageal primary or head & neck. The final discharge states, "Metastatic SCCA to Left Axilla".
Code the primary site according to the physicians' opinion, especially the treatment decision. If the physician treats the patient for a lung primary, code primary site as lung. If the primary site cannot be determined, code C80.9.
According to the pathologist, the most likely primary site for the example above is lung. The final discharge diagnosis does not reflect the pathologist's opinion, and does not contradict it either. If there is no conflicting medical opinion, code primary site to C34.9 [lung].
Primary Site: What code should be used to represent the site for an "extraovarian" papillary serous adenocarcinoma located in the "rectal muscle sheath"? See discussion.
The location of the tumor in the rectus muscle sheath is unusual and suggests an origin within a preexisting mullerian.
Code the Primary Site field to C49.4 [connective, subcutaneous and other soft tissues of the abdomen].
Primary site: What is the correct primary site code for angiosarcoma of the spleen with mets to bone marrow C42.2 vs C49x? See Discussion.
Robbins Pathology states the following about liver angiosarcomas: Hepatic angiosarcomas are rare but of interest because they are associated with distinct carcinogens, including arsenic (exposure to arsenical pesticides), Thorocast (a radioactive contrast medium previously widely used in radiology), and polyvinyl chloride (PVC) (widely used in plastics). The increased frequency of angiosarcomas among works in the PVC industry is one of the truly well-documented instances of chemical carcinogenesis in humans. With all these agents, there is a very long latent period of many years between exposure and the development of tumors.
Could the same apply to the spleen?
Code C422 [Spleen] as the primary site for angiosarcoma of spleen with metastasis to bone marrow.
Primary site: What is the single primary site used for a patient with multiple tumors in the duodenum and jejunum? See discussion.
The patient has a tumor in the jejunum and another tumor in the duodenum. Both tumors have the same histology. This disease process is a single primary per Other Sites Rule M18. Is the primary site coded to the more invasive tumor? If the tumors are equally invasive, is the primary site coded to C179?
Code the primary site to C179 [small intestine, NOS] for multiple invasive tumors of the small intestine accessioned as a single primary.
The steps used to arrive at this decision are:
Step 1: Go to the Primary Site subsection located in Section IV of the 2012 SEER Manual titled "Description of This Neoplasm."
Step 2: Apply instruction 5. "Code the last digit of the primary site code to '9' for single primaries, when multiple tumors arise in different subsites of the same anatomic site and the point of origin cannot be determined." Code the primary site to C179 [small intestine, NOS].
When multiple tumors arising in different subsites are accessioned as a single primary, the primary site is coded to the NOS code, in this case small intestine, NOS [C179]. The level of invasion does not determine the primary site, unless one or more of the tumors is in situ and another is invasive.
Primary Site: What site code is used to classify a femur biopsy with pathologic diagnosis of "Ewing sarcoma/primitive neuroectodermal tumor (PNET)"? See discussion.
ICD-O-3 lists PNET as being site specific to C71._. The pathology report states "some authors consider both Ewing sarcoma and PNET to be the same histologic entity given that they share the same translocation between chromosomes 11 and 23."
Code the Primary Site field to C40.2 [femur] based on Rule H in the ICD-O-3 that states, "Use the topography code provided when a topographic site is not listed in the diagnosis. This topography code should be disregarded if the tumor is known to arise at another site."
Primary Site: What would the primary site be for carcinoma of the renal pelvis, status post transplant? Please see details below. See Discussion.
The patient has a renal pelvis urothelial carcinoma confined to the pelvis but is status post renal pelvic transplant of the same renal pelvis.
Code the primary site to renal pelvis [C659]. Code the site in which the primary tumor originated. The transplant status in this example does not affect the primary site.
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