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Code the histology to 8413/3 [eccrine adenocarcinoma]. This is the most specific code available for this diagnosis.

According to our expert pathologist advisor, "The adnexal glands in the skin, sweat (eccrine) glands and apocrine glands, all have ducts which connect the business portion of each gland to the skin surface. Some of the adnexal tumors have features of differentiation which appear to be duct-like, hence the designation 'ductal.'"

In addition, "The 'squamoid' simply indicates some degree of squamous differentiation, but doesn't alter the usefulness of [code 8413/3] because we have no way of coding anything more specific in this case anyway."

Well-differentiated neuroendocrine tumors and grade 1 neuroendocrine tumors of the appendix are reportable because these tumors have a morphology code 8240/3 per the WHO Classification of Tumors of the Digestive System. However, per the ICD-O-3, carcinoid tumors of the appendix have a behavior code of /1 [borderline].

The terminology of neuroendocrine tumors is evolving and current thinking at the international level is that carcinoid/WD NET of appendix is reportable. However, reportability in the United States is based on ICD-O-3. The histology code for "Carcinoid of appendix" is 8240/1; the histology code for a carcinoids of all other primary sites is 8240/3. Until the United States adopts the proposed changes for ICD-O-3, reportability of appendix cases is as follows:

• Carcinoid

• Well-differentiated neuroendocrine tumor

• Grade 1 neuroendocrine tumor

• Well-differentiated neuroendocrine tumor/carcinoid (Pathologist uses both terms in reporting the diagnosis and does not want to choose one diagnosis over the other.)

For cases diagnosed prior to 1/1/2022

A low-grade appendiceal mucinous neoplasm (LAMN) is not reportable. The WHO classification designates LAMN with the behavior code /1 [uncertain whether benign or malignant].

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

Code the primary site to C421 [bone marrow] per Rule PH5. Code the primary site to the bone marrow when the peripheral blood is involved, even if no bone marrow biopsy is performed.

According to the notes for Rule PH5, CLL always has peripheral blood involvement (PH5 Note 1). CLL/SLL may also have involvement of lymph node regions in later stages (PH5, Note 2). For this patient a bone marrow biopsy was not performed but he had extensive lymph node and peripheral blood involvement. Therefore, the primary site is coded to C421. In addition, the physician's documentation specifies this patient has Stage 0 disease which indicates this disease process is being classified as leukemia (CLL).

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

The histology should be coded as diffuse large B-cell lymphoma [9680/3]. The biopsy pathology report definitively diagnosed DLBCL. The physician's diagnosis cannot be used because it is an ambiguous diagnosis only, "favored anaplastic large cell lymphoma." "Favor" is an ambiguous term.

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.

Glomus tympanicum tumors of the middle ear are not reportable. The 2005 WHO Classification of Head and Neck Tumors classified these tumors as a borderline [/1] behavior and recorded them in the ICD-O-3 with histology code 8690 [glomus jugulare tumor, NOS].

According to WHO, "the distinction between jugular and tympanic paragangliomas can easily be made in the patient by modern imaging methods ... the jugular neoplasm is identified as arising from the jugular bulb region ... while the tympanic neoplasm is confined to the middle ear." Benign and borderline neoplasms of the middle ear [C301] are not reportable. The middle ear is not a reportable CNS site for benign and borderline tumors.

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

Code the primary site to soft tissue of the back, NOS [C496] per Rule PH24 and the Abstractor Notes in the Heme DB for B-cell lymphoma intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma.

Code the primary site to the organ when lymphoma is present only in an organ. The lesion is described as epidural (tissue surrounding the dura) and involving paraspinal muscle, NOS. Both are connective or other soft tissues of the trunk, NOS [C496].

B-cell lymphoma intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma is a synonym for DLBCL 9680/3. When there is no primary site listed in the Heme DB, go to the Abstractor Notes. In the Abstractor Notes section it states that patients present with lymphadenopathy OR mass lesions in extranodal sites.

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

Under the Alternate Names section of the Heme DB, systemic mastocytosis with Associated Clonal Hematological Non-Mast Cell Lineage Disease (SM-AHNMD) is a synonym for systemic mastocytosis. Per Rule M2, this is one primary. Abstract a single primary when there is a single histology. Code the histology to 9741/3 [systemic mastocytosis].

Per the pathology report, the two diagnoses of systemic mastocytosis and mantle cell lymphoma meet the criteria for SM-AHNMD. The B-cell lymphoma is a symptom/marker of the AHNMD. In systemic mastocytosis with AHNMD, a myeloid or lymphatic malignancy is diagnosed with the SM. The prognosis is usually dominated by the non-mast cell malignancy.

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.

Rule PH21 applies to this situation which states to code the primary site to multiple lymph node regions, NOS (C778) when multiple lymph node regions, as defined by ICD-O-3, are involved and it is not possible to identify the lymph node region where the lymphoma originated. Axillary nodes are coded to C773 and inguinal nodes are coded to C774. There are two lymph node regions involved. Code the primary site to C778 [multiple lymph nodes].

If this patient had only bilateral axillary OR only bilateral inguinal nodes are involved, then PH20 would have applied and you would code to the specific lymph node region mentioned.