xRead - Nasal Obstruction (September 2024) Full Articles

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have been included in the Prognostic Index of Natural Killer lymphoma (PINK) and include age > 60 years, stage III or IV disease, distant lymph node involvement, and nonnasal disease. 1856 The role of immunotherapy in ENKTL has not been well described to date, and the primary indication for immunotherapy has been for refractory or other wise untreatable disease. Current recommendations favor enrollment in a clinical trial to allow for data collec tion, but pembrolizumab and nivolumab have been used when immunotherapy is desired. Future research into outcomes and appropriate patient selection is needed. Table XXVI.3 summarizes evidence surrounding sinonasal ENKTL treatment. Role of chemotherapy: Extranodal NK/T-cell lymphoma

Value

Patients should be counseled on the risks of rituximab treatment as well as the potential benefits including improved OS.

judgments

Policy level Recommendation. Intervention Rituximab should be added to CHOP for the treatment of sinonasal BCL given survival benefits.

F Extranodal natural killer/T-cell sinonasal lymphoma treatment Chemotherapy and RT are the mainstays of treatment for ENKTL. The sequence of treatment is dependent on dis ease stage and site. In patients with stage I or II disease, curative therapy is determined based on the patient’s func tional status. In patients who cannot tolerate chemother apy, RT alone may be offered. There have been several studies that suggest that RT improves LRC. 1766,1839,1845 In patients who can tolerate chemotherapy, combined modal ity therapy with both chemotherapy and RT is generally recommended. Lehrich et al. found that CRT appeared to confer a survival benefit when compared to chemother apy or RT alone, for both early- and late-stage ENKTL patients. 1846 The preferred regimen for concurrent treat ment is RT and three courses of DeVIC (dexamethasone, etoposide, ifosfamide, and carboplatin). Alternatively, RT and cisplatin followed by three cycles of VIPD (etopo side, ifosfamide, cisplatin, and dexamethasone) could be considered. Other options if concurrent treatment is not pursued are the “sandwich” regimen (pegaspargase, gem citabine, oxaliplatin [P-GEMOX] × two cycles followed by RT, and then another two to three rounds of P-GEMOX) or sequential regimen (for stage I and II, modified steroid [dexamethasone], methotrexate, ifosfamide, pegaspargase, and etoposide [SMILE] two to four cycles followed by RT). 1787,1847 There are no robust studies for guidance on differential survival benefits for a particular patient and no recommendation can be made on differential survival by treatment regimen. For stage III/IV ENKTL, outcomes are generally poor regardless of treatment method used. Clinical trials, combined modality therapy (identical to those listed for stage I/II), and asparaginase-containing regimens with or without RT (modified SMILE four to six cycles—steroid [dexamethasone], DDGP [dexametha sone, cisplatin, gemcitabine, pegaspargase]) are options for patients with advanced disease. 1848 There have been several studies that suggest anthracy cline regimens (e.g., CHOP) are insufficient for disease control, resulting in the current recommendations for treatment. 1773,1777,1849–1854 In general, it has been suggested that stage I and II diseases do not need CNS prophy laxis, while stages III and IV should be offered CNS prophylaxis. 1855 Several known negative prognostic factors

Aggregate grade of evidence

C (Level 4: 24 studies)

Benefit

Chemotherapy is a cornerstone to ENKTL treatment, and current evidence suggests a survival benefit with treatment. Chemotherapeutics are known to be toxic with common side effects including hematologic disturbances (e.g., pancytopenia), which can be severe and life threatening. Cost of treatment is significant, especially if several cycles of therapy are required for effect. Preponderance of benefits over harms. In patients with severe comorbidities, RT alone or enrollment in clinical trials can be considered.

Harm

Cost

Benefits–harm assessment

Value

judgments

Policy level Recommendation. Intervention Chemotherapy, as the first-line treatment, should be offered to patients with ENKTL if they are able to tolerate treatment, despite its known toxicities. Role of radiation therapy: Extranodal NK/T-cell lymphoma Aggregate grade of evidence C (Level 4: 24 studies) Benefit RT has been demonstrated to improve LRC and recommended for almost all treatment paradigms outside clinical trials.

Harm

RT has significant potential morbidity in terms of damage to adjacent tissue, including risks of vision loss and brain necrosis in extreme cases.

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