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SEER Inquiry System - Report
Produced: 04/03/2025 10:05 PM
Question 20210058
Inquiry Details
References:
#1: Expert consultant
#2: WHO Class Hem & Lymph Tumors
Question:
Multiple Primaries/Histology--Lymphoma: What is the histology code and how many primaries are there based on a gastrohepatic lymph node biopsy that shows: Nodular lymphocyte-predominant Hodgkin lymphoma with T-cell/histiocyte rich diffuse large B-cell lymphoma (DLBCL)-like transformation. If two primaries, what is the diagnosis date for each primary? See Discussion.
Discussion:
4/28/21 PET: There is extensive widespread/multifocal hypermetabolic uptake within lymph nodes, skeleton, and spleen, compatible with malignancy. Differential diagnosis includes lymphoma and metastatic disease of indeterminate primary, with lymphoma favored.
4/28/21 Right retroperitoneal lymph node, needle core biopsy: Large B-cell lymphoma. See comment. Comment: The differential includes T-cell/histiocyte-rich large B-cell lymphoma and diffuse variant of nodular lymphocyte predominant Hodgkin lymphoma. It is challenging to distinguish these two on the needle core biopsy. An excisional biopsy is recommended for a definite diagnosis if clinically appropriate. ADDENDUM: B-Cell Lymphoma, FISH: negative. No rearrangement of MYC, BCL2 and BCL6 and no fusion of MYC and IGH.
5/14/21 Gastrohepatic lymph node, biopsy: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) with T-cell/histiocyte rich diffuse large B-cell lymphoma-like transformation. Focal in situ follicular neoplasia.
6/3/21 Medical Oncologist: Biopsy confirms that patient has a nodular lymphocytic Hodgkin lymphoma which has transformed into a T-cell rich DLBCL. This variant of Hodgkin disease is a good prognostic histology which generally behaves indolently, like a low grade lymphoma.
Answer:
We consulted with our expert hematopathologist who advised this is a single primary, Hodgkin lymphoma (9659/3).
The diagnosis from 5/14/2021 states NLPHL. It also states there is T-cell histiocyte rich large B-cell lymphoma-like transformation. The WHO Classification of Hematopoietic and Lymphoid Tissues demonstrates six different patterns to NLPHL, which are: A) 'classical' nodular, B) serpiginous/interconnected nodular, C) nodular with prominent extra-nodular LP cells, D) T-cell-rich nodular, E) diffuse with a T-cell-rich background, and F) diffuse, B-cell-rich pattern.
In this case, they are describing a NLPHL type E (diffuse with a T-cell rich background). The term used is "T-cell histiocyte rich large B-cell lymphoma-LIKE transformation. "Like" as used here means that it is like a transformation; if it was NLPHL transforming to T-cell histiocyte rich large B-cell lymphoma, it would not have the word "like" in the diagnosis. This is a variant of NLPHL and not an actual transformation to another lymphoma. Even though NLPHL can transform to T-cell histiocyte rich large B-cell lymphoma, it is not the case here since the word "like" appears in the diagnosis.
We will update the histology in the Hematopoietic and Lymphoid Neoplasm Database to include these additional patterns.
