Clinicopathological Study of Primary Pediatric Gastrointestinal Lymphomas

J Indian Assoc Pediatr Surg. 2021 Sep-Oct;26(5):327-333. doi: 10.4103/jiaps.JIAPS_201_20. Epub 2021 Sep 16.

Abstract

Background: Primary gastrointestinal lymphomas (PGIL) are uncommon in children and account for <5% of all pediatric malignancies. The objective of our study was to analyze the prognostic factors of pediatric PGILs with reference to its histological subtypes, stage, and outcomes using immunohistochemistry.

Materials and methods: Twenty cases were studied over 11 years. Clinicopathological profiles, histological details, and immunohistochemical (IHC) profiles were analyzed.

Results: The mean age at the presentation was 6 years. Using IHC stains (CD CD10, CD19, CD20, CD3, terminal deoxynucleotidyl transferase, BCL 2 and 6, PAX 5, and MUM1), diffuse large B-cell lymphoma (DLBCL) was most prevalent (45%), followed by Burkitt lymphoma (35%) and lymphoblastic lymphoma (20%). DLBCLs (9) were classified using the Han's algorithm. Six were activated B-cell and 3 were germinal center B-cell subtypes. The cases of lymphoblastic lymphoma and those in Stage I disease had the best prognosis.

Conclusion: Pediatric PGILs have variable presentation, outcomes, and diverse treatment modalities depending on the histological subtypes. A panel of IHC stains can be a useful adjunct for the categorization and prognosis of pediatric PGILs.

Keywords: Burkitt lymphoma; diffuse large B-cell lymphoma; lymphoblastic lymphoma; pediatric gastrointestinal lymphomas.