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Burkitt -like l ymphoma (probably diffuse blastoid B-cell lymphoma)

Detailed case study of a 74-year-old male with Burkitt-like lymphoma involving skin and small intestine, highlighting clinical, imaging, histologic, and cytogenetic features.

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Burkitt -like l ymphoma (probably diffuse blastoid B-cell lymphoma)

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  1. Burkitt-like lymphoma(probably diffuse blastoid B-cell lymphoma) Harleen K. Sidhu MD1 Jagmohan S. Sidhu MD2 • Department of Dermatopathology, ClearPath Diagnostics, Syracuse, NY, USA • Department of Pathology and Laboratory Medicine, UHS Hospitals, Johnson City, NY, USA

  2. Clinical Data • 74-year-old Caucasian male presented in March 2013 with a 4-month history of skin lesions on multiple sites of his body. • No fever, chills, night sweats, or weight loss. • Biopsy of a left flank skin lesion was performed.

  3. H&E X100 H&E X400

  4. CD10 X400 CD20 X400

  5. BCL2 X400 BCL6 X400 MUM1 X400 Ki67 X400

  6. Other Negative Stains • CD2 • CD3 • CD4 • CD5 • CD7 • CD8

  7. Diagnosis • Malignant lymphoma with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (Burkitt-like lymphoma) • The possibility of this lymphoma being a diffuse blastoid B-cell lymphoma [probably a histologically aggressive variant of t(14;18)-negative diffuse follicular lymphoma] was raised after a jejunal mass, which was detected in this patient by PET/CT, was worked-up more extensively.

  8. F18-FDG PET/CT Scan • Increased uptake in the multiple nodular areas of skin and subcutaneous tissue and in 3 small bowel sites • Range of maximum SUV in skin/subcutis: 10-16.8 • Range of maximum SUV in small bowel: 10.5-17.2 • Maximum SUV (17.2) in the small bowel is in jejunum and is associated with 1 cm of small bowel wall thickening.

  9. Surgical Resection and Gross Description • About 5.5 cm long segment of jejunum was resected to avoid possible perforation during chemotherapy. • A 5.5 cm small bowel segment with attached mesenteric tissue was received fresh. A 5 cm x 2 cm x 2cm “fish-flesh” like tumor was infiltrating the wall, protruding into the lumen and infiltrating the mesenteric tissue. Multiple small lymph nodes (0.3 cm -1.0 cm) were found in the mesentery. • Touch imprints of the jejunal mass were made and a piece of tumor was submitted for flow cytometric analysis. Rest of the specimen was fixed in 10% neutral buffered formalin.

  10. Touch Imprint of Jejunal Mass DiffQuik Stain X1000

  11. Scan of the Slides of Jejunal Mass CD20 BCL2 CD10 BCL6 MUM1 CD43 CD57 Ki67 HGAL LMO2 c-MYC p53 CD21 CD23 TdT BCL1 CD2 CD3 CD4 CD5 CD7 CD8 10 Recuts and 15 Unstained Sections of this block and 10 Recuts and 15 Unstained Sections of a skin block were submitted to the lymphoma workshop panel.

  12. H&E Stain X200 H&E Stain X400

  13. CD20 X400 CD10 X400

  14. BCL2 X400 BCL6 X400 LMO2 X400 HGAL X400

  15. MUM1 X400 CD57 X400

  16. CD43 X400 Ki67 X400 MYC X400

  17. Other Negative Stains • CD2 • CD3 • CD4 • CD5 • CD7 • CD8 • CD21 • CD23 • P53 • BCL1 • TdT

  18. Flow Cytometric Analysis of Jejunal Mass • CD19+, CD20+, CD22+ (dim), CD38+, CD43+, CD57+, surface kappa+, • CD5-, CD10-, CD23-, CD79b-, CD200-, FMC7-, CD11c-, CD16-, CD56-

  19. FISH of Jejunal MassDual Fusion Probes Dual-Color Dual Fusion Probe Tri-Color Dual Fusion Probe Normal IgH-BCL2 IgH: Green BCL2: Orange IgH/MYC Translocation in 66.50% nuclei IgH: Green MYC: Orange CEP8: Aqua Abnormal IgH-BCL2 IgH: Green BCL2: Orange

  20. FISH of Jejunal MassBCL6 Dual Color Break-apart Probe Normal BCL6 3` BCL6: Green 5` BCL6: Orange BCL6 Rearrangement (an overhanging 5` BCL6 DNA sequence in 77% nuclei) 3` BCL6: Green 5` BCL6: Orange

  21. FISH of Jejunal MassDual Color Probes No deletion/ monosomy of chromosome 7 7D7S522, CEP7 (7q31 - 7p11.1-q11.1) 7p11.1-q11.1 : Green 7q31 : Orange No deletion/ monosomy of chromosome 13 D13S319, LAMP1/13q (13q14.3-13q34) 13q34 : Green 13q14.3 : Orange

  22. Cytogenetic Analysis of Jejunal Mass

  23. CD57 on the Previous Skin/Subcutis Specimen CD57 X400

  24. HGAL and LMO2 on Previous Skin/Subcutis Specimen LMO2 X400 HGAL X400

  25. Diagnosis • High grade B-cell lymphoma, Burkitt-like (most probably a diffuse blastoid B-cell Lymphoma)involving skin and small intestine

  26. Differential Diagnosis of Aggressive Diffuse B-cell LymphomaMorphologic Features MORPHOLOGY Our case has features highlighted in the red. Morphologically our case is not a diffuse large B-cell lymphoma, but it can be any other entity in the differential. DBBCL: Diffuse blastoid B-cell lymphoma DLBCL: Diffuse large B-cell lymphoma BL: Burkitt lymphoma DLBCL-BL: Intermediate between DLBCL and BL DBMCL: Diffuse blastoid mantle cell lymphoma B-LBL: B-lymphoblastic lymphoma

  27. Differential Diagnosis of Aggressive Diffuse B-cell LymphomaImmunophenotypic Features IMMUNOPHENOTYPE Our case has features highlighted in the red. Immunophenotypically our case is not a DLBCL, DBMCL or B-LBL, but it can be DBBCL, BL or DLBCL-BL.

  28. Differential Diagnosis of Aggressive Diffuse B-cell LymphomaCytogenetic Features NK: Not known CYTOGENETIC FEATURES Our case shows absence of t(14;18), presence of MYC translocation and presence of BCL6 rearrangement and, therefore, it is not a classic Burkitt lymphoma, can be called a Burkitt-like lymphoma or a Burkitt lymphoma with atypical features and is probably a diffuse blastoid B-cell lymphoma (DBBCL) . Other entities in the differential diagnosis have already been excluded by morphology and immunophenotyping.

  29. Why is Our Case Not a Classic Burkitt Lymphoma (BL)? • Our case has a CD10+/BCL2+/BCL6+/MUM1+/CD57+ immunophenotype by immunohistochemistry • When a Burkitt lymphoma shows BCL2 expression, it can be called Burkitt lymphoma only if it has IgH/MYC translocation without BCL2 translocation and without BCL 6 translocation. • Our case shows BCL6 rearrangement, IgH/MYC translocation and absence of BCL2 translocation by FISH and conventional cytogenetic analysis.

  30. Why did we raise the posssibility of a Diffuse blastoid B-cell lymphoma (DBBCL)? • Because it does not fit in any other entity in the differential diagnosis of aggressive B-cell lymphoma. • Because it shows many features of diffuse blastoid B-cell lymphoma that has been recently described and suggested as a histologically aggressive variant of t(14;18)-negative diffuse follicular lymphoma*. *Chiu A et al. Mod Pathol 2009;22:1507-1517

  31. Treatment and Follow-Up • R-CHOP was initiated with almost 90% resolution of skin lesions after 1 cycle. • Chemotherapy was completed without any major complications. • Follow-up visit in December 2013: No lesions and no symptoms; normal PET/CT scan

  32. Interesting Feature of Our Case Our case has a CD20+/CD10+/BCL2+/BCL6+/MUM1+/CD57+ immunophenotype with almost 100% Ki67 labeling index, shows BCL6 rearrangement and IgH/MYC translocation and shows absence of BCL2 translocation by FISH and conventional cytogenetic analysis. These features are those of a Burkitt-like lymphoma (Burkitt Lymphoma with atypical features) and are suggestive of diffuse blastoid B-cell lymphoma that has been described and suggested as a histologically aggressive variant of t(14;18)-negative diffuse follicular lymphoma in the literature (Chiu A et al. Mod Pathol 2009;22:1507-1517).

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