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Patient has a 2021 skin excision showing an atypical spindle cell neoplasm, most consistent with dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous transformation.

Per the ICD-O-3.2 Coding Table, DFSP, NOS has a behavior code of /1, and DFSP, fibrosarcomatous has a behavior code of /3. There is no code listed for DFSP with fibrosarcomatous transformation. Transformation is not included as a term that can/cannot be used for the Other Sites Schema, but this type of DFSP is often described as DFSP with fibrosarcomatous transformation. How do we code DFSP when transformation is used to describe fibrosarcomatous?

Autopsy/Pathology Report (2020) excerpts

External Examination

Nervous System: There is an 8.5 cm mass located in the right thoracic paraspinal area.

Final Anatomic Diagnosis

Clinical History: Paraspinal mass suspicious for neuroblastic tumor (detected by imaging studies)

Nervous System: Right thoracic paraspinal neuroblastoma, poorly differentiated

For example, total thyroidectomy specimen shows right lobe papillary thyroid carcinoma, 4.2 cm, unencapsulated, with numerous psammoma bodies in non-tumoral thyroid parenchyma, without angioinvasion; left lobe with papillary thyroid carcinoma, 0.6 cm, encapsulated, with capsular invasion, with intralymphatic psammoma bodies in non-tumoral thyroid parenchyma, without angioinvasion. The synoptic summary documents vascular invasion present (psammoma bodies only).

Patient has a history of B-cell lymphoma with multimodality treatment in 2002. Lab work in 2015 showed multilineage dysplasia leading to a diagnosis of therapy-related myelodysplastic syndrome. Patient presents in 2020 for a bone marrow biopsy now showing low-grade MDS. The MDS appears to have the same multilineage dysplasia previously identified.

MDS, NOS is not listed in the Heme DB as a possible transformation of t-MDS, nor is it listed as a Same Primary for t-MDS. Likewise, t-MDS is not listed as a more specific myelodysplastic syndrome, a transformation of MDS NOS, or a Same Primary as MDS, NOS.

The first M rule that applies to this case is M15, and the Multiple Primaries Calculator indicates that the MDS, NOS should be a new primary.

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?

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.

In Table 3 (Specific Histologies, NOS/ NST, and Subtypes/Variants), the entry for papillary carcinoma, NOS includes a change in column 3 of the 2018 Breast Solid Tumor Rules that conflicts with the H Rules. It is not accounted for in the change log. No explanation is offered as to why this change was made. This is a major change because encapsulated papillary carcinoma is frequently associated with carcinoma NST, and we have not been collecting these as such.

Encapsulated papillary carcinoma (8504) in column 3 now includes an indented entry, with invasive carcinoma, NST/invasive duct carcinoma 8504/3. However, most encapsulated papillary carcinomas are in situ or there is no definitive statement of invasive encapsulated papillary carcinoma, so when in situ and invasive tumors are present, we are instructed to code the invasive histology (which would be the invasive carcinoma (NST), 8500/3). How are registrars to arrive at the correct histology without a new H rule or a clarification regarding this update being documented in the change log?

Does the same change/addition apply to solid papillary carcinoma? These are often also associated with carcinoma, NST. Again, without an explanation regarding the change mentioned above, it is difficult to understand why the change was made.

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

It is unclear if code 300 is appropriate, since technically the fissure is comprised of pleura, involvement of the fissure appears to imply a tumor that is no longer localized.

An argument could be made for code 400, since the term traverses could be interpreted as crossing into adjacent lobe, however the lower lobe is not mentioned in this scan.

Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.

Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.