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Malignant perivascular epithelioid cell tumor (PEComa) is reportable because it is malignant. Assign 8005/3 to malignant PEComa.

We consulted an ICD-O-3 expert who explained that some PEComas such as angiomyolipoma and lymphangiomyomatosis have specific ICD-O codes and their malignant counterparts may be coded to 8860/3 and 9174/3 respectively. There are no separate ICD-O codes for other specific PEComas, e.g., clear cell “sugar” tumor of lung, clear cell myomelanocytic tumor of the falciform ligament and some “unusual” clear cell tumors occurring in other organs—or for PEComa, NOS. These PEComas may therefore be coded to 8005 as clear cell tumors NOS; in other words as clear cell tumors that are not clear cell variants of carcinomas, sarcomas, or other specific tumor type.

Please note, PEComa is non-specific as to behavior. Unless the pathologist states that it is malignant, (as was the case for this question), the default code is 8005/1 (non-reportable).

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

Per PH27, code the primary site to C809 [unknown]. Rule PH27 states one is to code the primary site to unknown [C809] when there is no evidence of lymphoma in lymph nodes AND the physician documents in the medical record that he/she suspects that the lymphoma originates in an organ(s) OR there is multiple organ involvement without any nodal involvement.

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 1998-2003:

Code the EOD-Extension field to 10 [localized disease, solitary plasmacytoma only] for all cases of solitary plasmacytoma.

Clobetasol is not cancer-directed treatment at this time.

Note: Question originally submitted in 2010. During 2014 review, this was checked and Clobetasol is still not cancer directed treatment for Mycosis Fungoides.

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

Code the histology to 9837/3 [T lymphoblastic leukemia/lymphoma].

To determine the primary site for leukemia/lymphoma histologies, first go to Module 4. Per Rule PH8, code the primary site to the site of origin when lymph nodes, tissue or organs are involved. To determine a more specific histology, go to Module 7, rules for coding primary site for lymphomas. Per Rule PH20, code the lymph node region when multiple lymph node chains within the same region are involved. Mediastinal and hilar lymph nodes are intrathoracic lymph nodes. The substernal mass is also intrathoracic and is presumed to be a lymph node mass which involved the pericardium. For this case, code the primary site to C771 [Intrathoracic lymph nodes].

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.

Code grade to 6 [B-cell] for the histology malignant non-Hodgkin lymphoma, large B-cell type, with features consistent with T-cell rich variant [9680/3]. Under the Definition section for histology code 9680/3 it states there are morphologic variants of the disease: centroblastic, immunoblastic, plasmablastic, T-cell/histiocyte-rich, anaplastic.

Rule G3 in the Heme Manual confirms the grade listed in the Heme DB under its Grade section for the histology 9680/3. While the patient presented with a variant of DLBCL that is T-cell/histiocyte rich, it is still a B-cell phenotype. The grade is coded accordingly.

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 1998-2003:

Per AJCC, code spleen involvement which is demonstrated by:

1. Unequivocal palpable splenomegaly alone.

2. Equivocal palpable splenomegaly with radiologic confirmation (ultrasound or CT).

3. Enlargement or multiple focal defects that are neither cystic nor vascular (radiologic enlargement alone is inadequate).

If the spleen is proven to be involved, code extension for this case as 20 [Involvement of two or more lymph node regions on the same side of the diaphragm; Stage II].

If the spleen is not proven to be involved, code extension as 10 [Involvement of a single lymph node region; Stage I].

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

Per PH Rule22, code the primary site to C779 [lymph nodes, NOS].

Rule PH22 is a default rule for lymphomas that is used when there is no other information regarding the primary site and the Heme DB does not indicate a primary site under its Primary Site(s) section. Rule PH27, code the primary site to unknown [C809], does not apply. Only use C809 [unknown] as the primary site when there is no evidence of lymphoma in lymph nodes AND the physician documents that the lymphoma originates in an organ(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 cases diagnosed 2007 or later:

There are two primaries. Primary 1: The two tumors described on the pathology report from the lumpectomy are a single primary using rule M13. Primary 2: Disregard the foci of residual DCIS. Paget disease of the nipple is a separate primary using rule M12.

Primary 1: invasive mucinous adenocarcinoma and extensive ductal carcinoma in situ: Code the histology as 8480/3 [mucinous adenocarcinoma] using rule H27.

Primary 2: Paget disease of nipple: Code the histology as 8540/3 [Paget disease] using rule H14.