NK/T-cell lymphoma diagnosis and prognosis

  NK/T-cell lymphoma originating in the nasal cavity or pharyngeal lymphatic ring is common in the East Asian population, and NK/T-cell lymphoma originating in extra-nodal organs other than the nasal cavity is called nasal NK/T-cell lymphoma.  NHL originating in the nasal cavity is common in China, accounting for 6-10% of all non-Hodgkin’s lymphomas, most of which are of NK/T-cell origin, accounting for more than 90%.  The pathology is characterized by a vascular-centered lesion with tumor cells surrounding or infiltrating within the vessel wall and destroying the vessel wall, leading to inflammation and tumor coagulation necrosis.  Tumor cell morphology is pleomorphic, variable in size, with complex or distorted nuclei, and rarely large cells, immunoblasts, or large intercellular morphology. The tumor cells can be diffuse, and the background may show more reactive acute and chronic inflammatory cells.  The immunological features are: CD2, CD3 plasma, CD56, CD57 NK cell antigen positive; surface CD3 negative, cytoplasmic CD3 positive, perforin, GramB, TIA and other NK cell antibody positive; often no TCR gene recombination. Nasal NK/T-cell lymphoma EBV positivity rate is almost 100%, and EBER virus can be detected by in situ hybridization.  Clinical prevalence is male, male:female = 2-4:1; median age of onset is 44 years; nasal masses, easily invading the ipsilateral maxillary sinus, septal sinus and nasopharynx; stage I accounts for 67-84% of cases, while stages II-IV are rare. The most common symptom is nasal congestion. When the local lesion is extensively invaded, signs and symptoms such as protruding eyes, facial swelling, hard palate perforation, cranial nerve palsy, foul odor and fever may appear.  The tumor is often confined to the nasal cavity and its adjacent structures. Invasion of adjacent organs or structures is most common in the ipsilateral maxillary sinus, followed by the ipsilateral septal sinus, nasopharynx, local skin, hard palate, soft palate, eye and oropharynx in that order. 42% of patients have multisite invasion. The overall five-year survival rate of patients with tumors is 30%-50%, and the prognosis is related to age, clinical stage, and responsiveness to first treatment. In early stage cases, complete remission (CR) rate after radiotherapy is 80-90%, and 5-year survival rate reaches 40-92%.  Radiotherapy alone is recommended for limited stage I nasal NK/T-cell lymphoma, consolidation chemotherapy after radiotherapy is recommended for super nasal stages I and II, and stage III/IV should be dominated by chemotherapy and supplemented by radiotherapy at the primary site. Due to the poor efficacy of chemotherapy in stage IIE III/IVE and tumor resistance to chemotherapy, stronger or new effective chemotherapy regimens need to be considered.