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Table 1 Differential diagnosis of uterine and testicular T-cell lymphoblastic lymphoma

From: Unusual presentation of primary T-cell lymphoblastic lymphoma: description of two cases

Neoplasm

Clinical features

Morphology

Immunophenotype

Molecular features

B-LBL

median age 20 years, M > F, 90% of cases present as B-ALL

convoluted or round nuclear contours; immature blastic chromatin; numerous mitotic figures

CD19 +, PAX-5 +, CD20 +/−, TdT +, CD10 +/−, CD79 + (in 10% of cases), T-cell antigens -

monoclonal IgH gene rearrangement; t (9; 22) (q34; q11.2); t (12;21) (p13; q22); t (v; 11q23); t (1; 19) (q23; p13.3); t (5; 14) (q31; 32)

BL

childhood; head and neck and ileocecal region frequently involved

prominent “starry sky” pattern; monotonous, medium-sized cells with 2–5 prominent nucleoli and distinct cytoplasmic rim; very high mitotic and apoptotic rates

CD10 +, CD19 +, CD20 +, CD79a +, Bcl2 -, Bcl6 +, TdT -, Ki-67 > 99%, T-cell antigens -

monoclonal IgH gene rearrangement; t (8; 14) (q24; q32) or t (2; 8) (p11; q24) or t (8; 22) (q24; q11) involving MYC

DLBCL

adult; frequent nodal involvement

large or medium-sized cells with prominent nucleoli; centroblastic or immunoblastic appearance

CD19+, CD20+, CD79a+, PAX-5+, CD10 +/−, Bcl6 +/−, IRF4/MUM1 +/−, Bcl2 +/−, TdT -, T-cell antigens -

t (14; 18) (q32; q21); Bcl6 (3q27) rearrangement; MYC (8q24) rearrangement

Myeloid sarcoma

median age; frequent history of previous or concomitant AML, MDS, MPN, MDS/MPN

myelocytes with more distinct and eosinophilic cytoplasm

CD43 +, CD68PGM1 +, CD68 +, CD117 +, MPO +, CD10 -, T-cell antigens -

no evidence of monoclonal TCR gene rearrangements, monosomy 7, trisomy 8

Blastoid variant of MCL

median age 68 years, male predominance; frequent extranodal involvement

lymphoblastoid cells with immature chromatin and high mitotic rate

CD19 +, CD20 +, Cyclin-D1 +, CD5 +, CD10 -, TdT -, CD2 -, CD3 -, CD7 -

monoclonal IgH gene rearrangement; no evidence of monoclonal TCR gene rearrangements; t (11; 14) (q13; q32)

ES/ PNET

median age <20 years, M > F; frequent bone involvement

cohesive growth pattern, frequently pseudorosettes formation; small blue monomorphic round cells with fine nuclear chromatin, round nuclei and scanty clear cytoplasm

CD99 +, vimentin +, WT-1 +, lymphoid markers -

no evidence of monoclonal TCR gene rearrangement; t (11; 22) (q24; q12) or t (21; 22) (q22; q12) or t (1; 16) (q11; q11)

Alveolar rhabdomyosarcoma

adolescents and young adults; extremities and paraspinal region frequently involved

nests of round cells separated by fibrous septa, with some giant cells and occasional clear cells

vimentin, desmin, smooth muscle actin, HHF-35, MyoD1, myogenin +, cytokeratins +/−, S100 +/−, CD20 +/−, T-cell antigens -, TdT-

t (2; 13) (q35; q14) or t (1;13) (p36; q14)

Small (oat) cell carcinoma

middle age; frequent pulmonary invlvement

small ovoid, round to spindled cells with markedly increased nuclear-to-cytoplasmic ratio, hypercromatic nuclei and inconspicuous nucleoli

low molecular weight keratins +, chromogranin +/−, synaptophysin +/−, CD3 -, CD5 -, TdT -

 

Seminoma

young adults; testicular enlargement and hydrocele

round, polygonal cells with large and vesicular nuclei, prominent nucleoli, clear and eosinophilic cytoplasm and frequent mitoses, lymphoid infiltrate in the backgournd

CD117 +, D2-40 +, OCT4 +, SALL4 +, cytokeratins -, CD30 -, EMA -, T-cell antigens -, TdT -

 
  1. B-LBL: B lymphoblastic lymphoma; BL: Burkitt lymphoma; DLBCL: diffuse large B-cell lymphoma; AML: acute myeloid leukemia, MDS: myelodysplastic syndrome; MPN: myeloproliferative neoplasm; MDS/MPN: myelodysplastic/myeloproliferative syndrome; MCL: mantle cell lymphoma; ES: Ewing sarcoma; PNET: peripheral neuroectodermal tumour; +: positive; −: negative; IgH: immunoglobulin heavy chain gene; TCR: T-cell receptor gene.