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In the 2018 ICD-O-3 Update Table, undifferentiated high-grade pleomorphic sarcoma and undifferentiated high-grade pleomorphic sarcoma of bone (C40_) were both listed as a New Term for histology 8830/3. There was no site restriction for a diagnosis of undifferentiated high-grade pleomorphic sarcoma. Therefore, it appears the diagnosis could easily be applied to a soft tissue tumor. This histology is used by pathologists in our region for soft tissue tumors as well as bone tumors. However, in the ICD-O-3.2 Table an entry (or synonym) was not provided for a tumor outside the bone.

The ICD-O-3.2 Table only lists undifferentiated high-grade pleomorphic sarcoma of bone for site codes C40_ and C41_ as a synonym for histology 8830/3. This also is not listed in the ICD-O-3.2 Implementation Guidelines. As a result, it is unclear whether a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue can be coded to 8830/3 and/or can be a synonym for the preferred term (8830/3, Malignant fibrous histiocytoma). Can a diagnosis of undifferentiated high-grade pleomorphic sarcoma of the soft tissue be coded to 8830/3, C49_ as it was per the 2018 ICD-O-3 Update Table?

This question was prompted from preparing SEER*Educate coding exercises. We will use the answer as a reference in the rationales.

Pathology report diagnosis: Urethral lesion: Intraductal carcinoma of the prostate, see microscopic. Clinical Information: Urethral Lesion/Hematura. Microscopic Description: The biopsy shows dilated ductal structures filled with anaplastic epithelium showing areas of comedo-type necrosis. The tumor cells have enlarged nuclei prominent nucleoli and mitoses are identified. Surrounding benign prostatic tissue is also present. Immunostains show that the tumor cells stain for PSA, PSAP, P504s but are negative for GATA-3. The other components of the PIN 4 stain CK5/14 and P63 stain the basal cells surrounding the tumor confirming the intraductal nature of the process. Intraductal carcinoma should not be confused with high grade PIN as the former is usually associated with high grade invasive tumor. Is this C619 and 8500/2?

Example 1: Endometrial biopsy final diagnosis is high-grade serous adenocarcinoma. Should we code this endometrial primary with histology 8441 (serous adenocarcinoma) because C54.X topography code is not listed in the applicable 2018 ICD-O-3 codes Histology Update for the new morphology, or should we apply the new histology code 8461 (high-grade serous carcinoma)?

The NAACCR implementation guideline section 2.3 includes an important reminder that: Many of the new codes, terms, and behaviors listed in this update are site-specific and do not apply to all sites. Applicable C codes will be noted next to the term in bold font.

However, this is followed by the more ambiguous instruction for edits that appear to imply the combination with non-listed sites is possible: These site- and histology-specific combinations will not be added to the Impossible combination edit. However, if a site other than the one listed with the morphology code is assigned, the result will be an edit requiring review. This is Interfield Edit 25.

The SEER Coding and Staging Manual instructs one to code the primary site to the location where the tumor originated, in this case the liver. However, there is no CS Extension code for in situ tumors found in the CS Manual Liver Schema.

In our registry, we code the primary site for such cases to the extra-lymphatic site if there is one extra-nodal site involved with disease and the patient does not have disseminated involvement of multiple extra-nodal sites. Is this correct? Example: A patient with a submandibular lymphoma and involved nodes undergoes a salivary gland excision and a modified radical neck dissection yielding 100 nodes.

A patient had posterior cervical lymphadenopathy status post biopsy and subsequent lymph node dissection showed metastatic melanoma in 2018. Workup showed no skin lesions or primary site. Final diagnosis is melanoma of unknown primary (unknown if cutaneous or non-cutaneous). Should C760 be used as the primary site for this case since the histology codes of 8700-8790 are included in the Cervical Lymph Nodes and Unknown Primary Tumors of the Head and Neck schema in SEER*RSA?

The pathology report says: Right knee tumor:

A. discrete subcutaneous mass 3.5x5.2x1.4 cm malignant spindle cell neoplasm (see Comment)

B. A focus of subcutaneous malignant neoplasm is identified in the superior resection margin.

C.All other margins are clear.

The comment mentions that the specimen has been sent to Mayo Clinic and the Mayo clinic consult says, "we still believe that the diagnosis of spindle cell carcinoma is correct. Obviously the differential diagnosis involves melanoma and sarcoma also. The results of the immunoperoxidase stains strongly support the prior diagnosis of a carcinoma."

The previous SEER Program Coding and Staging Manual included Appendix C that has Coding Guidelines for some sites. The breast guidelines specifically instructed one to code the subsite with the invasive tumor when the pathology report identifies invasive tumor in one subsite and in situ tumor in a different subsite or subsites. The current Breast Solid Tumor Rules Table 1: Primary Site Codes refers one back to the SEER Manual and COC Manual for a source document priority list but does not make mention of invasive vs. in situ on that final version of the source document.

In addition, the Colon Solid Tumor Rules currently contains no Site Coding Section/Table. However, the Lung Solid Tumor Rules do and also refer one to the SEER/COC Manuals for document priority lists. The Urinary Solid Tumor Rules has both the Primary Site Codes Table and an additional section called Priority for Coding Primary Site, which does not reference a document priority list or other manuals. Unfortunately, there is additional information in Appendix C Bladder Coding Guidelines that may have been used in the past regarding site source priority.

Could the remaining applicable Appendix C information be consolidated into the Solid Tumor Rules consistently among all the sites to lessen the need for additional manual referencing? Also, is there a reason one site includes the Priority Site Coding instructions and others do not?