Histology--Leukemia: How is "T-Cell prolymphocytic leukemia, cerebriform (Sezary cell-like) variant" coded when the pathology report COMMENT states: The cerebriform (Sezary cell-like) variant accounts for about 5% of cases of T-cell prolymphocytic leukemia?
For cases diagnosed prior to 1/1/2010:
9834/3 [Prolymphocytic leukemia, T-cell type]. According to the WHO Classification of Haematopeietic and Lymphoid Tissue Tumours, cerebriform or Sezary cell-like is a variant form of T-cell prolymphocytic leukemia.
For cases diagnosed 2010 forward, refer to the Hematopoietic and Lymphoid Neoplasm Case Reportability and Coding Manual and the Hematopoietic Database (Hematopoietic DB) provided by SEER on its website to research your question. If those resources do not adequately address your issue, submit a new question to SINQ.
Multiple primaries/Histology--Heme & Lymphoid Neoplasms: How many primaries should be reported for a bone marrow biopsy diagnosis of "lymphoproliferative disorder, small cell lymphocytic lymphoma/small cell lymphocytic leukemia consistent with marginal zone lymphoma"?
According to our hematopoietic/lymphoid neoplasm physician expert, abstract one primary with the histology code 9699/3 [marginal zone lymphoma]. The pathologist is using the expression "small lymphocytic lymphoma" in a descriptive manner (marginal zone lymphoma is comprised of small lymphocytes) rather than in a "diagnostic" manner.
Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned when a patient was diagnosed in 2003 with malignant lymphoma, mixed cell type, follicular in the inguinal lymph nodes and was recently diagnosed with follicular lymphoma (by a neck lymph node biopsy) involving the neck and mediastinal lymph nodes?
This case should be accessioned as a single primary: malignant lymphoma, mixed cell type, follicular [9691/3] diagnosed in 2003. The following describes how this determination was made.
This case is one in which the terminology for follicular lymphoma has changed over time. In 2003, follicular lymphoma was classified as small cleaved cell, large cell, or mixed cell (both small cleaved and large cell). Those designations are no longer used. This disease process is currently classified as follicular lymphoma NOS, grade 1, grade 2 or grade 3. The change was simply a change in classification/terminology.
Appendix A, Table A3 (Obsolete Terms as Defined in ICD-O-3, Lymphoid Neoplasm Obsolete Terms) should be used to determine the current term when an obsolete term is known/given. Per the Table, "Mixed cell type follicular lymphoma" is currently known as "Follicular lymphoma, grade 2" and the correct histology code is 9691/3. This is the correct histology for the 2003 primary.
Per Rule M15, the histologies must be check in the Multiple Primaries Calculator to determine the number of primaries. Enter [follicular lymphoma, grade 2 (malignant lymphoma, mixed cell type, follicular)] for Histology Code 1 and [follicular lymphoma, NOS] for Histology Code 2. The result is "Same Primary." As a result, accession a single 2003 diagnosed primary with the histology follicular lymphoma, grade 2 [9691/3] when the patient is subsequently diagnosed with follicular lymphoma, NOS.
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.
EOD-Extension--Mycosis Fungoides: Explain the difference between extension codes 25 [% of body surface not stated, no tumors] and 30 [skin involvement, NOS; extent not stated, no tumors. Localized, NOS]?
For cases diagnosed 1998-2003:
For mycosis fungoides: Use code 25 when skin involvement is present but only a general location/site is mentioned (i.e., face, legs, torso, arms).
Use code 30 when there is skin involvement but there is no mention of location/site.
Reportability/MP/H Rules/Histology: Is malignant perivascular epithelioid cell tumor (PEComa) reportable, and if so, what is the histology code?
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).
Surgery of Primary Site--Skin: Explain the difference between code 30 and code 45.
Code 30 [Biopsy of primary tumor followed by a gross excision of the lesion]
Code 45 [Wide excision or re-excision of lesion or minor (local) amputation with margins more than 1 cm, NOS. Margins MUST be microscopically negative.]
For cases diagnosed 1/1/2003 and after: Code 30 represents a biopsy or excision in which the margins of excision are less than 1 cm or the margins are unknown. Code 45 represents a wide excision in which it is known that the margins of excision are greater than 1 cm.
Reportability/Primary Site--Head & Neck: If a wedge resection/shield resection is performed on the lower lip for SCCA and the path report refers to "lip, NOS" with no mention of vermilion border, is this case reportable?
Review the operative and pathology reports, and the physical exam for mention of "mucosal surface" (reportable) or "skin" (not reportable). If neither are mentioned, lip, NOS is reportable per the ICD-O-3 code of C009.
Scope of Regional Lymph Node Surgery: Should this field be coded to "unknown or not applicable" for all hematopoietic morphologies, brain primaries and unknown primaries?
For cases diagnosed 1/1/2003 and after: Code the Scope of Regional Lymph Node Surgery field to 9 [Unknown or not applicable] for all hematopoietic morphologies, brain primaries and unknown primaries. .
Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are to be accessioned for a patient with a history of CLL undergoing chemotherapy who is subsequently diagnosed on a liver biopsy with diffuse large B-cell lymphoma (Richter transformation)?
Abstract the diffuse large B-cell lymphoma (Richter transformation) as a second primary per Rule M10. Rule M10 states to abstract as multiple primaries when a neoplasm is originally diagnosed as a chronic neoplasm (CLL) AND there is a second diagnosis of an acute neoplasm (the diffuse large B-cell lymphoma (Richter transformation)) more than 21 days after the chronic diagnosis.
"Richter transformation," also known as "Richter syndrome," is a term that indicates CLL has transformed to DLBCL. Richter syndrome is listed under the Alternate Names section in the Heme DB for DLBCL (9680/3).
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.