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For cases diagnosed 2007 or later:

Yes. This is an example of a site-specific exception to the general rule to code only from the final diagnosis. The Colon Histology Rules specifically state that "other parts of the pathology report" may be used to identify a tumor arising from a polyp, adenomatous polyp, villous adenoma, or tubulovillous adenoma.

Do not report primary acquired melanosis with atypia.

According to our expert pathologist consultant, "There has been a lot of debate in the literature about the diagnostic criteria, terminology, and natural history of primary acquired melanosis [PAM]. Your case comes down squarely on the main issue, which is whether PAM with atypia should be regarded as melanoma in situ. In most studies it appears that PAMs with no atypia or mild atypia do not progress to melanoma, and only a small percentage of those with severe atypia do so."  "PAM, even with atypia, is not melanoma in situ, and should not be reported."

For further information, see this article for a review of a large number of patients: Shields, Jerry A, Shields, Carol L, et al. Primary Acquired Melanosis of the Conjunctiva: Experience with 311 Eyes. Trans. Am Ophthalmol Soc 105:61-72, Dec 2007.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Per Rule PH30, use the Heme DB to determine the primary site and histology when rules PH1-PH29 do not apply. Code the primary site to C419 [bone, NOS], assuming there are multiple bones involved in this case. If only one bone is involved, code the primary site to the specified bone.

In the Abstractor Notes section in the Heme DB, it indicates the primary site may differ for LCH in the solitary disease and multisystem disease. This patient has multisystem disease with involvement of the bone, liver, spleen and retroperitoneum. The most common sites for multisystem involvement include three of the four above sites (bone, liver, and spleen).

Determine the primary site based on the knowledge of the usual sites of involvement for this disease, the actual sites of involvement for the case presented, and identifying which sites of involvement are likely metastatic and which are the potential primary sites. There are two potential primary sites of involvement: the bone and the retroperitoneum. Bone is a common site of involvement for LCH while the retroperitoneum is not. Code the primary site to C419 [bone, NOS] because multiple bones are involved for this patient and bone is the most common site for LCH based on the documentation in the Abstractor Notes.

The spleen and liver are typically not primary sites for this disease process. They become involved when there is multisystem involvement because they filter the blood. They are typically sites of metastatic involvement. This information will be added to the ABSTRACTOR NOTE section.

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

We consulted an expert genitourinary pathologist who advises that micropapillary serous borderline tumor of the testis is reportable. He states "it is the same neoplasm as in the ovary. It arises from tissue (tunica vaginalis) surrounding the testis so is a paratesticular neoplasm." 

 Please note: not all borderline tumors are reportable and this diagnosis is an exception because it is assigned /2 in ICD-O-3.2. It is reportable for cases diagnosed Jan 1, 2021 and later.

For cases diagnosed 2010 and forward, access the Hematopoietic Database at http://seer.cancer.gov/seertools/hemelymph.

Code the histology to 9823/3 [chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL)] per Rule PH15. Code the histology to the non-Hodgkin lymphoma (NHL) with the numerically highest ICD-O-3 code when two or more NHLs are present in the same present in the same lymph node(s) or lymph node region(s), tissue(s), organ(s), or bone marrow. Both follicular lymphoma [9690/3] and SLL [9823/3] are types of NHL. Therefore, the histology is coded to 9823/3.

This composite histology represents a single primary per Rule M4. The rule states to abstract a single primary when two or more types of non-Hodgkin lymphoma are simultaneously present in the same anatomic location(s), such as the same lymph node or lymph node region(s), the same organ(s), and/or the same tissue(s).

SEER*Educate provides training on how to use the Heme Manual and DB. If you are unsure how to arrive at the answer in this SINQ question, refer to SEER*Educate to practice coding hematopoietic and lymphoid neoplasms. Review the step-by-step instructions provided for each case scenario to learn how to use the application and manual to arrive at the answer provided. https://educate.fhcrc.org/LandingPage.aspx.

For tumors diagnosed prior to 2007:

If the histology of the bladder primary is "transitional cell carcinoma" or "papillary transitional cell carcinoma," do not code the prostatic urethra as a new primary. This is probably a case of intraluminal (mucosal) spread of the original tumor, rather than separate primaries. The clinical diagnosis supports this view.

For tumors diagnosed 2007 or later, refer to the MP/H rules. If there are still questions about how this type of tumor should be coded, submit a new question to SINQ and include the difficulties you are encountering in applying the MP/H rules.