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Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare malignant neoplasm primarily arising in the nasal cavity, paranasal sinuses, orbits, and upper airway. It predominantly affects males in the fifth decade of life. Advanced disease stages are associated with poor prognosis and may involve dissemination beyond the upper aerodigestive tract to other organs.
No HPO-linked phenotype data are recorded for extranodal nasal NK/T cell lymphoma in this packet. Based on the disease definition, documented clinical presentations include nasal mass, nasal bleeding, nasal obstruction, midline perforation of the hard palate, and mid-facial or upper airway destructive lesions. A subset of cases has been described as presenting primarily in the lymph nodes.
No causative genes or hereditary predisposition data are recorded for NKTCL in this packet. No inheritance pattern is documented.
No specific diagnostic methods or biomarkers for NKTCL are recorded in this packet beyond the disease definition and classification as a malignant neoplasm of the nasal and upper airway region.
No approved treatments or foundational therapies for NKTCL are recorded in this packet. Active clinical trial investigation across drug therapy, biologic, procedural, and other intervention categories is ongoing, with both academic and industry sponsorship represented.
29 trials found
According to the disease definition in this packet, advanced disease stages of NKTCL are associated with poor prognosis. No additional quantitative prognosis data are provided in this packet.
Active clinical trials with specific scope for extranodal NK/T cell lymphoma include: NCT07502768 ("Tislelizumab Plus Zeprumetostat for Relapsed or Refractory NK/T-Cell Lymphoma"; Phase 1; sponsor: Rong Tao; status: recruiting; start: April 2026; completion: December 2028); NCT05700448 ("Sugemalimab or Placebo Plus PGemOx Regimen in Extranodal NK/T-Cell Lymphoma"; Phase 3; CStone Pharmaceuticals; not yet recruiting; projected start: June 2027; completion: December 2030); NCT07574528 ("Sintilimab, Pegaspargase and Selinexor Followed by Radiotherapy in Stage I/II Extranodal NK/T-Cell Lymphoma"; Phase 2; Sun Yat-sen University; not yet recruiting; projected start: May 2026; completion: May 2031); NCT07554482 ("Selinexor Combined With Reduced-Dose Radiotherapy for Early-Stage Extranodal NK/T-Cell Lymphoma"; Phase 2; Beijing Tongren Hospital; not yet recruiting; projected start: May 2026; completion: April 2029). The classified publication landscape encompasses 162 publications (43 reviews, 37 case reports), with biomarker and gene therapy publications documented.
Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 1:01 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
AI-curated news mentioning extranodal nasal NK/T cell lymphoma
Updated Jul 21, 2026
A single-center study in Vietnam evaluates real-world outcomes of frontline multimodal treatment strategies for localized extranodal NK/T-cell lymphoma, nasal type. The findings contribute to understanding treatment efficacy in this rare lymphoma subtype.
A case report highlights the occurrence of synchronous rectal cancer and extranodal NK/T-cell lymphoma, nasal type. This unique presentation may provide insights into the interplay between these two malignancies.
New research identifies DNA methylation signatures that differentiate extranodal NK/T-cell lymphoma from EBV-positive nodal T/NK-cell lymphoma. This discovery may help in identifying prognostic subgroups, enhancing patient stratification and treatment approaches.