Categorizing the rare mature T/NK-cell lymphoid neoplasms previously reported in the pediatric population by prognosis and relative rarity
| Potentially >50% EFS with conventional chemotherapy . | |
|---|---|
| Zebras . | Unicorns . |
| PTCL-NOS | |
| ALK-negative ALCL | |
| Typically <30% EFS without upfront HSCT | |
| Zebras | Unicorns |
| Hepatosplenic TCL | Enteropathy-associated TCL |
| Primary cutaneous γ/δ TCL | Primary cutaneous CD8+ aggressive epidermotropic cytotoxic TCL |
| Nodal TFH cell lymphoma, AITL | |
| Indolent and potentially treatable without cytotoxic chemotherapy | |
| Zebras | Unicorns |
| Subcutaneous panniculitis-like TCL | Indolent T-cell lymphoma of the GI tract |
| Mycosis fungoides | Primary cutaneous acral CD8+ LPD |
| Lymphomatoid papulosis | T-large granular lymphocytic leukemia |
| Primary cutaneous ALCL | |
| Primary cutaneous CD4+ small/medium T-cell LPD | |
| Virus-associated T/NK-cell lymphoid neoplasms | |
| Zebras | Unicorns |
| Limited-stage ENKTL (EBV) | Aggressive NK-cell leukemia (EBV) |
| Advanced-stage ENKTL (EBV) | Adult T-cell leukemia/lymphoma (HTLV-1) |
| Systemic EBV+ TCL of childhood | Severe mosquito bite allergy (EBV) |
| Systemic chronic active EBV disease | |
| Hydroa vacciniforme LPD (EBV) | |
| Potentially >50% EFS with conventional chemotherapy . | |
|---|---|
| Zebras . | Unicorns . |
| PTCL-NOS | |
| ALK-negative ALCL | |
| Typically <30% EFS without upfront HSCT | |
| Zebras | Unicorns |
| Hepatosplenic TCL | Enteropathy-associated TCL |
| Primary cutaneous γ/δ TCL | Primary cutaneous CD8+ aggressive epidermotropic cytotoxic TCL |
| Nodal TFH cell lymphoma, AITL | |
| Indolent and potentially treatable without cytotoxic chemotherapy | |
| Zebras | Unicorns |
| Subcutaneous panniculitis-like TCL | Indolent T-cell lymphoma of the GI tract |
| Mycosis fungoides | Primary cutaneous acral CD8+ LPD |
| Lymphomatoid papulosis | T-large granular lymphocytic leukemia |
| Primary cutaneous ALCL | |
| Primary cutaneous CD4+ small/medium T-cell LPD | |
| Virus-associated T/NK-cell lymphoid neoplasms | |
| Zebras | Unicorns |
| Limited-stage ENKTL (EBV) | Aggressive NK-cell leukemia (EBV) |
| Advanced-stage ENKTL (EBV) | Adult T-cell leukemia/lymphoma (HTLV-1) |
| Systemic EBV+ TCL of childhood | Severe mosquito bite allergy (EBV) |
| Systemic chronic active EBV disease | |
| Hydroa vacciniforme LPD (EBV) | |
“Zebras” reflect diagnoses that are rare but have been reported in large cohorts of children with rare lymphomas or repeatedly reported in case series/reports. “Unicorns” reflect diagnoses that have only been reported infrequently in children in case reports.
EBV, Epstein-Barr virus; EFS, event-free survival; ENKTL, extranodal NK/T-cell lymphoma; GI, gastrointestinal; HTLV-1, human T- lymphotropic virus type 1; LPD, lymphoproliferative disorder; TCL, T-cell lymphoma.