. 2022 Jun 8;16(4):1242–1250. doi: 10.1007/s12105-022-01461-6

Table 2.

Clinical, pathologic and imaging features

Case no	Age/gender	Clinical features	Imaging	Histopathologic features	Immunohistochemistry	Cytogenetics	Treatment	Recurrence	Follow-up
1	57/M	Swelling hard palate for 2 years; LDH = 150U/l	MRI: 3.8 × 3.6 × 3.3 cm soft tissue sub-mucosal mass in the left maxilla involving pterygoid fossa and extending into infratemporal fossa; no regional lymph nodes Ann Arbor stage: 1AE	Monocytoid lymphoid cells admixed with scattered large cells	Positive for CD20, Bcl2, MNDA (focal), CD21 highlighted residual germinal centres; Negative for CD10, EBV-LMP1, Mib-1 labelling index ~ 10%; Few plasma cell noted with no light chain restriction	No rearrangement of IGH, IGH/BCL2 and BIRC3/MALT1 One copy of BCL2 and MALT1 was observed in 21% cells	EBRT to left infratemporal fossa to a dose of 36 Gray over 1 month	No	Disease free for 1 year
2	40/F	Swelling in both sides of GBS for 5 months; LDH = 179 U/l	PET scan showed FDG avid soft tissue thickening involving bilateral GBS (largest extent 8 cm) with regional sub-centimetric lymph nodes; Ann Arbor stage: IAEX	Nodular monomorphic infiltrate of centrocyte like lymphoid cells. Scattered large or transformed cells were seen	Positive for CD20, Bcl2, MNDA (strong and diffuse). Negative for CD5, Mib-1 labelling index ~ 10%. Few plasma cell noted, which were polyclonal on kappa & lambda immunohistochemistry	No rearrangement of IGH, IGH/BCL2 and BIRC3/MALT1. (Fig. 5B)	Chemotherapy-6 cycles of Bendamustine & Rituximab	No	Disease free for 6 months
3	54/M	Swelling in the right cheek for 2 months HBc antigen was positive Serum LDH levels = 250.8 U/l	PET-CT: 4.2 × 4 cm soft tissue lesion in the right buccal space involving right maxilla and pterygopalatine fossa with sub-centimetric regional lymph nodes; revised Ann Arbor stage: IAE	Diffuse sheets of centrocyte-like cells with interspersed transformed cells	Positive for CD20, bcl2, IgG, MNDA (focal) Negative for CD10, Tdt, Cyclin D1, LMO2, GCET, CD138, CMYC Mib-1 labelling ~ 20%. A few plasma cell noted, which showed no light chain restriction by immuno histochemistry	No rearrangement of IGH, IGH/BCL2 and BIRC3/MALT1 3–4 copies of IGH, BCL2 and MALT1 were observed in 18% cells (tetrasomy)	6 cycles of R-CHOP chemotherapy followed by IFRT to a dose of 45 Gray over one month	After 4 years of disease-free interval	Re-RT at the dose of 20 cycles each of 36 Gy over 4 weeks Disease free for 2 months

Case no

Age/gender

Clinical features

Imaging

Histopathologic features

Immunohistochemistry

Cytogenetics

Treatment

Recurrence

Follow-up

57/M

Swelling hard palate for 2 years; LDH = 150U/l

MRI: 3.8 × 3.6 × 3.3 cm soft tissue sub-mucosal mass in the left maxilla involving pterygoid fossa and extending into infratemporal fossa; no regional lymph nodes

Ann Arbor stage: 1AE

Monocytoid lymphoid cells admixed with scattered large cells

Positive for CD20, Bcl2, MNDA (focal), CD21 highlighted residual germinal centres; Negative for CD10, EBV-LMP1, Mib-1 labelling index ~ 10%; Few plasma cell noted with no light chain restriction

No rearrangement of IGH, IGH/BCL2 and BIRC3/MALT1

One copy of BCL2 and MALT1 was observed in 21% cells

EBRT to left infratemporal fossa to a dose of 36 Gray over 1 month

Disease free for 1 year

40/F

Swelling in both sides of GBS for 5 months; LDH = 179 U/l

PET scan showed FDG avid soft tissue thickening involving bilateral GBS (largest extent 8 cm) with regional sub-centimetric lymph nodes; Ann Arbor stage: IAEX

Nodular monomorphic infiltrate of centrocyte like lymphoid cells. Scattered large or transformed cells were seen

Positive for CD20, Bcl2, MNDA (strong and diffuse). Negative for CD5, Mib-1 labelling index ~ 10%. Few plasma cell noted, which were polyclonal on kappa & lambda immunohistochemistry

No rearrangement of IGH, IGH/BCL2 and BIRC3/MALT1. (Fig. 5B)

Chemotherapy-6 cycles of Bendamustine & Rituximab

Disease free for 6 months

54/M

Swelling in the right cheek for 2 months

HBc antigen was positive

Serum LDH levels = 250.8 U/l

PET-CT: 4.2 × 4 cm soft tissue lesion in the right buccal space involving right maxilla and pterygopalatine fossa with sub-centimetric regional lymph nodes; revised Ann Arbor stage: IAE

Diffuse sheets of centrocyte-like cells with interspersed transformed cells

Positive for CD20, bcl2, IgG, MNDA (focal)

Negative for CD10, Tdt, Cyclin D1, LMO2, GCET, CD138, CMYC

Mib-1 labelling ~ 20%. A few plasma cell noted, which showed no light chain restriction by immuno histochemistry

No rearrangement of IGH, IGH/BCL2 and BIRC3/MALT1

3–4 copies of IGH, BCL2 and MALT1 were observed in 18% cells (tetrasomy)

6 cycles of R-CHOP chemotherapy followed by IFRT to a dose of 45 Gray over one month

After 4 years of disease-free interval

Re-RT at the dose of 20 cycles each of 36 Gy over 4 weeks

Disease free for 2 months

M male, F female, MRI magnetic resonance imaging, PET scan positron emission tomography scan, FGD fluorodeoxyglucose, EBRT external beam radiation therapy, GBS gingivo-buccal sulcus, HBc hepatitis B core antigen, LDH lactate dehydrogenase, PAX5 paired box protein 5, Bcl2 B-cell lymphoma 2, MNDA myeloid cell nuclear differentiation antigen, EBV-LMP1 Epstein-Barr virus latent membrane protein 1, FISH fluorescence in-situ hybridization, IgH immunoglobulin heavy chain, MALT1 mucosa associated lymphoid tissue lymphoma 1, BIRC3 baculoviral IAP repeat containing 3, API2 apoptosis inhibitor 2, Tdt terminal deoxynucleotidyl transferase, LMO2 LIM domain only 2, GCET germinal centre expressed transcript, R-CHOP rituximab, cyclophosphamide, hydroxydaunorubicin hydrochloride, Oncovin, prednisone, IFRT involved field radiation therapy, RT radiotherapy