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Question: 20130035 Status

Source 1:   Heme & Lymph Manual & DB
Source 2:  

Multiple primaries--Heme & Lymphoid Neoplasms: How many primaries are accessioned and what rule applies when a subsequent diagnosis of diffuse large B-cell lymphoma (95%) and follicular lymphoma, grade 3 (5%) is made following an original diagnosis of low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL) ? See Discussion.

In 2011, patient presented with a large mesenteric mass, numerous other smaller mesenteric lymph nodes, moderate retroperitoneal and extensive iliac chain adenopathy greater on right; small inguinal nodes are also present mostly on right side and splenomegaly per the CT scan. Abdominal pelvic mass needle biopsies showed low grade CD-10 positive B-cell lymphoma, most consistent with low grade follicular lymphoma (FL). The patient was treated with R-CVP with unknown response.

In June 2012, patient presented again for laparoscopy and lymph node biopsy for stated recurrence of lymphoma found on CT scan. A large mass was seen in mesentery of bowel. Abdominal mass biopsy showed diffuse large B-cell lymphoma (DLBCL). Abdominal mass #2 excisional biopsy showed diffuse large B-cell lymphoma, 95%, and follicular lymphoma grade 3, 5%. The majority of the tumor is now DLBCL.

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This case should be accessioned as a single primary, diffuse large B-cell lymphoma diagnosed in 2011 per Rule M7. Note 4 for Rule M7 states to change the histology code on the original abstract to the more specific histology, diffuse large B-cell lymphoma in this case. There is no time restriction for rule M7. Apply rule PH11 and code the histology as 9680/3 [DLBCL] when both DLBCL and follicular lymphoma are present in the same lymph node(s).

Ambiguous terminology is not used to code a more specific histologic type per the Heme Manual. The information submitted states only that this low grade B-cell lymphoma was “most consistent with follicular lymphoma.” The term “consistent with” is an ambiguous term per SEER and cannot be used to code the histology of the 2011 neoplasm as follicular lymphoma. There was no subsequent clinical statement that this patient was diagnosed with follicular lymphoma in 2011.

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Note: Although the ambiguous terminology on the pathology report is not used to code the histology to follicular lymphoma, had there been a subsequent clinical statement that this patient had follicular lymphoma, the histology would be coded to follicular lymphoma [9690/3]. A diagnosis of follicular lymphoma followed by a diagnosis of DLBCL more than 21 days later is considered a new primary per rule M12.

The DLBCL is a new primary.

For this case, use the Hematopoietic Database since the second diagnosis is in 2012. First, go the database and look up follicular. In the transformations section, DLBCL is listed. This is a case with a chronic neoplasm (FL) and an acute neoplasm (DLBCL). Rules M8-M13 apply to these types of cases.

This scenario fits rule M10: chronic (follicular) followed by an acute (DLBCL) greater than 21 days.

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