Nasal-type Natural Killer Research Articles

Characteristics and prognosis of distant metastasis after primary treatment for early-stage extranodal nasal-type natural killer/T-cell lymphoma from the China Lymphoma Collaborative Group database.

This study aimed to investigate the characteristics and prognosis of distant metastasis (DM) after primary treatment for early-stage extranodal nasal-type natural killer (NK)/T-cell lymphoma (ENKTCL). A total of 1619 patients from the China Lymphoma Collaborative Group database were retrospectively reviewed. The cumulative incidence of DM was assessed using Fine and Gray's competing risk analysis. The correlation between DM sites was evaluated using phi coefficients, while DM sites were classified using hierarchical clustering. Regression analysis was used to assess the linear correlation between DM-free survival (DMFS) and overall survival (OS). The 5-year cumulative DM rate was 26.2%, with the highest annual hazard rate being in the first year (14.9%). The most frequent DM sites were the skin and soft tissues (SSTs, 32.4%) and distant lymph nodes (LNs, 31.3%). DM sites were categorized into four subgroups of distinct prognosis - distant LN, SST, extracutaneous site, and lymphoma-associated hemophagocytic lymphohistiocytosis. SST or distant LN, solitary metastasis, and late-onset DM demonstrated a relatively favorable prognosis. Contemporary chemotherapy significantly decreased DM rates and improved DMFS. Decreased DM rates were further associated with increased OS probabilities. Our findings improve the understanding of the variable clinical behaviors of early-stage ENKTCL based on four distinct DM sites and thus provide guidance for future therapeutic decisions, metastatic surveillance, and translational trial design.

Analysis of latent T-cell epitopes in Epstein-Barr virus isolated from extranodal nasal-type natural killer/T-cell lymphoma in Taiwanese population

Extranodal nasal-type natural killer (NK)/T-cell lymphoma (NKTCL) is an aggressive lymphoma that is prevalent among East Asian and South American populations. Although Epstein-Barr virus (EBV) is commonly detected in NKTCL, there are limited studies that have analyzed the EBV genomic variations in NKTCL. In this study, 8 EBV latent genes were analyzed using targeted gene sequencing in 23 formalin-fixed paraffin-embedded tissues derived from 18 patients with NKTCL. Five cases with paired samples were comparatively analyzed. The consistency of EBV sequencing data between tissue samples was high (96.3%–98.7%), whereas that of variant calling among the tissue samples and plasma samples (74.3%–79.2%) was low. The highest densities of non-synonymous variants were detected in the EBNA3B gene. Among the 74 known T-cell epitopes, 363 non-synonymous variants were identified in 32 (43.2%) epitopes. Additionally, the AVFDRKSDAK (A1S/P and V2F/M/L) and YHLIVDTDSL (I4L and L10R/V/G/H) epitopes were associated with 5 patterns of amino acid changes in EBNA3B and EBNA-2, respectively. The frequency of variation in the human leukocyte antigen (HLA)-restricted epitopes with corresponding HLA types common among Taiwanese population was significantly low (P = 0.011), whereas that in anchor residues was significantly high (P = 0.012). In conclusion, this study demonstrated the genomic diversity of EBV in NKTCL and its correlation with the HLA-restricted epitope variations in Taiwanese population. The findings of this study provide useful insights for the development of novel therapeutic strategies for NKTCL.

NASAL TYPE T/NATURAL KILLER LYMPHOMA - CASE REPORT

A 55-year-old white female patient was referred to our oral medicine department for evaluation of a palate lesion. Past medical history included of a diagnosis of nasal type natural killer (NK)/T-cell lymphoma being treated. At the extraoral physical examination there was an increase volume in the upper third of the face involving the whole nose. In the mouth, an exophytic lesion was found, which was hard to palpation and painless with areas of ulceration in the hard palate with approximately 60 days since onset. Incisional biopsy was performed with an inconclusive histopathologic report. Immunohistochemical analysis showed positivity for CD3 and CD56 plus Ki67 positivity in 90% of the cells leading to the diagnosis of nasal T-cell lymphoma/NK type. At the time of diagnosis, chemotherapy was started using cyclophosphamide, doxorubicin (hydroxydaunorubicin), vincristine (Oncovin), prednisone protocols that promoted a significant decrease in tumor volume. The patient is currently being followed up with no signs or symptoms of recurrence.

A neutralized human LMP1-IgG inhibits ENKTL growth by suppressing the JAK3/STAT3 signaling pathway.

Latent membrane protein 1 (LMP1), which is associated with the development of different types of Epstein-Barr virus (EBV) related lymphoma, has been suggested to be an important oncoprotein. In this study, a human anti-LMP1 IgG antibody (LMP1-IgG) was constructed and characterized by ELISA, western blotting (WB), affinity and immunohistochemistry (IHC) analyses. CCK-8, MTT, apoptosis assays, antibody-dependent cell-mediated cytotoxicity (ADCC) and CDC (complement-dependent cytotoxicity) assays were performed to evaluate the inhibitory effects of LMP1-IgG on extranodal nasal-type natural killer (NK)/T-cell lymphoma (ENKTL). Then, the influence of LMP1-IgG on the JAK/STAT signaling pathway was investigated. The results showed that the successfully constructed LMP1-IgG inhibited proliferation, induced apoptosis, and activated ADCC and CDC of ENKTL in a concentration- and time- dependent manner. Moreover, phosphorylation of JAK3 and STAT3 was inhibited by LMP1-IgG. Our data indicate that LMP1-IgG may provide a novel and promising therapeutic strategy for the treatment of LMP1-positive ENKTL.

Primary natural killer/T cell lymphoma of the cervix: Case report and clinicopathological analysis

ObjectiveTo present pathological and molecular characterizations of a rare case that was diagnosed as nasal-type natural killer (NK)/T cell lymphoma primarily arising in the cervix. Case reportAn Asian woman was admitted to hospital with a hysteromyoma, and laparotomy was performed. A large tumor of the uterus was found, which was limited to the cervix. Pathological examination showed NK/T cell lymphoma, which was supported by histological and immunohistochemical studies and was confirmed by evidence of Epstein–Barr virus infection. Less commonly, this case concerned a cytotoxic T cell phenotype, as molecular studies showed evidence of a clonal T cell receptor γ chain gene rearrangement. Microscopically, prominent and extensive necrosis was the distinctive feature of this case, which reminded us of considering it as a tumor. ConclusionPrimary NK/T lymphoma of the cervix is rare. Our experience in this case provided variable information on both pathological and molecular studies. This case may be of value in the differential diagnosis of lymphoid lesions and other small cell tumors of the cervix.

Early radiotherapy has an essential role for improving survival in patients with stage I-II nasal-type of NK/T cell lymphoma treated with L-asparaginase-containing chemotherapy--a single institution experience.

The purpose of this study was to investigate the role of early radiotherapy in patients with localized-stage nasal-type natural killer (NK)/T cell lymphoma treated with L-asparaginase-containing chemotherapy. Sixty-four patients with stage I-II nasal-type NK/T cell lymphoma were enrolled in this study. All patients received an L-asparaginase-containing regimen. Thirty-four patients received late radiotherapy (RT), which was defined as receiving 6 cycles of prior chemotherapy (CT) followed by RT, and 30 patients received early RT, which was defined as receiving no more than 3 cycles of CT followed by early RT. With a median follow-up of 35 months (range, 12-49 months), 19 patients (29.7 %) died from lymphoma-related causes, and 22 patients (34.4 %) developed local and/or distant relapse. The 3-year overall survival (OS) and progression-free survival (PFS) were 84.2 and 74.3 % for early RT and 57.6 and 55.9 % for late RT, respectively, and these differences were significant (OS, p = 0.027; PFS, p = 0.034). After 2 cycles of initial CT, 58 patients achieved treatment response (complete response and partial response). For the 58 patients, there were still significant differences for 3-year OS and PFS when early RT was compared with late RT (3-year OS 94.4 vs. 58 %, P = 0.005; 3-year PFS 82.9 vs. 56.3 %, P = 0.01). Early RT has an essential role in improving survival for localized-stage nasal-type NK/T cell lymphoma (TCL) patients even when used in combination with L-asparaginase-containing CT. Prospective, randomized studies should to be performed to confirm these results.

Efficacy and tolerance of pegaspargase-based chemotherapy in patients with nasal-type extranodal NK/T-cell lymphoma: a pilot study.

Nasal-type extranodal natural killer (NK)/T-cell lymphoma (ENKL) is a highly invasive cancer with a poor prognosis. More effective and safer treatment regimens for ENKL are needed. Pegaspargase (PEG-Asp) has a similar mechanism of action to L-asparaginase (L-Asp), but presents lower antigenicity. The aim of the present research was to evaluate the safety profile and the latent efficacy of a PEG-Asp-based treatment regimen in patients with ENKL. Data collected from 20 patients with histologically confirmed ENKL, admitted to the Third Affiliated Hospital of Sun Yat-Sen University from January 2009 to August 2013, were included in the study. All patients received 2500 IU/m2/IM PEG-Asp on day 1 of every 21-day treatment cycle. Patients received combination chemotherapy with CHOP (n=5), EPOCH (n=7), GEMOX (n=7) or CHOP with bleomycin (n=1). After 2-5 treatment cycles (median, 4 cycles) of PEG-Asp-based chemotherapy, five patients (25%) showed a complete response (CR), and the overall response rate (ORR) was 60%. Grade 3/4 neutropenia occurred in fourteen patients (70%). Grade 3 alanine aminotransferase (ALT) elevation was observed in two. Grade 1-2 non-hematological toxicity consisted of activated partial thromboplastin time (APTT) elongation (n=9), hypofibrinogenemia (n=6), hypoproteinemia (n=17), hyperglycemia (n=3), and nausea (n=6). No allergic reactions were detected. No treatment related death was reported. Our results suggested that PEG-Asp-based chemotherapy presented an acceptable tolerance and a potential short-term outcome in patients with nasal-type ENKL.

Tumor testicular bilateral, tipo linfoma no Hodgkin de células T/NK extranasal

Lymphoma is a malignant neoplasia characterized by the proliferation of cells native to the lymphatic system. It makes up 1 % to 9 % of testicular neoplasia. Bilateral testicular tumor presentation was first described by Aberhouse in 1955. Extranodal nasal-type natural killer (NK) lymphoma is the most frequent variant of non-Hodgkin's tumors and presents with extensive vascular destruction and necrosis; the most common extranodal site is the upper respiratory system. It is characterized by poor outcome and systemic dissemination and does not respond well to current treatments.A 45-year-old man presented with bilateral increased testicular volume of 6-month progression, having received multiple treatments. Testicular ultrasound and abdominal computed tomography scan revealed increased testicular volume, retroperitoneal metastasis, and metastasis to the subcutaneous cell tissue. Tumor markers were negative. Right radical orchiectomy and biopsy of the left testis were performed. The definitive histopathologic diagnosis was extranasal- type NK/T-cell non-Hodgkin's lymphoma. The patient was referred to the Hematology Service and chemotherapy and hormone replacement treatment were begun.This tumor represents 1 % of all non-Hodgkin's lymphomas and 5 % of testicular masses. Mean presentation age is 50 years and there is a higher incidence in Asia and South America. Twenty percent of the cases affect areas outside of the nasal cavity. There is no staging classification for this disease. Five-year survival is 60 % and 17 % for disseminated disease. None of the current treatments (orchiectomy, chemotherapy and/or chemo-radiotherapy) are considered the criterion standard.

Clinical Disparity and Favorable Prognoses for Patients With Waldeyer Ring Extranodal Nasal-type NK/T-cell Lymphoma and Diffuse Large B-cell Lymphoma

This study aimed to compare the clinical characteristics and prognosis of Waldeyer ring extranodal nasal-type natural killer (NK)/T-cell lymphoma (WR-NKTCL) and Waldeyer ring diffuse large B-cell lymphoma (WR-DLBCL). Consecutive diagnoses of 122 WR-DLBCL and 44 WR-NKTCL patients, receiving mainly primary radiotherapy in early-stage WR-NKTCL and primary chemotherapy in early-stage WR-DLBCL, were reviewed. WR-NKTCL occurred predominately in young males, as nasopharyngeal stage I disease with B-symptoms, extranodal dissemination, and involving adjacent structures. WR-DLBCL was mainly stage II tonsillar disease with regional lymph node involvement. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 74% and 67% in WR-DLBCL, respectively, and 68% (P=0.468) and 59% (P=0.303) in WR-NKTCL. In stages I and II disease, WR-DLBCL 5-year OS and PFS were 79% and 76% compared with 72% (P=0.273) and 62% (P=0.117) in WR-NKTCL. In stage I disease, WR-DLBCL 5-year OS and PFS were 81% and 81%, compared with 76% (P=0.394) and 63% (P=0.236) in WR-NKTCL. In addition, the prognostic factors and failure patterns in WR-DLBCL and WR-NKTCL differed substantially. These results indicate that remarkable clinical disparities exist between WR-DLBCL and WR-NKTCL; however, different treatment strategies for each can result in similarly favorable prognoses.

JAK3 deregulation by activating mutations confers invasive growth advantage in extranodal nasal-type natural killer cell lymphoma

Extranodal, nasal-type natural killer (NK)/T-cell lymphoma (NKCL) is an aggressive malignancy with poor prognosis in which, usually, signal transducer and activator of transcription 3 (STAT3) is constitutively activated and oncogenic. Here, we demonstrate that STAT3 activation mostly results from constitutive Janus kinase (JAK)3 phosphorylation on tyrosine 980, as observed in three of the four tested NKCL cell lines and in 20 of the 23 NKCL tumor samples under study. In one of the cell lines and in 4 of 19 (21%) NKCL primary tumor samples, constitutive JAK3 activation was related to an acquired mutation (A573V or V722I) in the JAK3 pseudokinase domain. We then show that constitutive activation of the JAK3/STAT3 pathway has a major role in NKCL cell growth and survival and in the invasive phenotype. Indeed, NKCL cell growth was slowed down in vitro by targeting JAK3 with chemical inhibitors or small-interfering RNAs. In a human NKCL xenograft mouse model, tumor growth was significantly delayed by the JAK3 inhibitor CP-690550. Altogether, the constitutive activation of JAK3, which can result from JAK3-activating mutations, is a frequent feature of NKCL that deserves to be tested as a therapeutic target.

The Role of18F-FDG PET/CT for Initial Staging of Nasal Type Natural Killer/T-Cell Lymphoma: A Comparison with Conventional Staging Methods

The utility of (18)F-FDG PET/CT in patients with nasal-type natural killer (NK)/T-cell lymphoma has not been established. Therefore, we evaluated the role of (18)F-FDG PET/CT for determining cancer staging by comparing its results to those of conventional staging methods (CSMs) (physical examination, CT with intravenous contrast, biopsies from primary sites, and bone marrow examinations) in patients with nasal-type NK/T-cell lymphoma. In this study, 52 consecutive patients (34 men, 18 women; mean age, 49.4 y) with newly diagnosed nasal-type NK/T-cell lymphoma were studied. Anatomic regions (n = 1,300; 16 nodal and 9 extranodal regions per patient) were assessed with an (18)F-FDG PET/CT scan and with CSMs, and each anatomic region was classified as positive or negative for malignancy. Biopsy and clinical follow-up, including additional imaging studies, were used as the gold standard for diagnosis. Of the 59 nodal and 71 extranodal anatomic regions that were truly positive for malignancy, (18)F-FDG PET/CT detected 58 nodal and 69 extranodal. CSMs, however, detected only 44 of the nodal and 61 of the extranodal anatomic regions that were positive for malignancy (nodal comparison of PET/CT vs. CSMs, P < 0.001; extranodal comparison of PET/CT vs. CSMs, P = 0.008). PET/CT scans exhibited a significantly better sensitivity (97.7% vs. 80.7%, P < 0.001) than CSMs for the detection of malignant lesions. PET/CT findings altered the original staging category for 12 patients (21.2%) and affected treatment planning in 23 cases (44.2%). Our study demonstrated that (18)F-FDG PET/CT scanning is a valuable modality for staging and treatment planning in patients with nasal-type NK/T-cell lymphoma.

Flow cytometric immunophenotyping is of great value to diagnosis of natural killer cell neoplasms involving bone marrow and peripheral blood

Natural killer (NK) cell neoplasms are unusual disorders. In this study we compared results of flow cytometric immunophenotype (FCI) with cytomorphology, histopathology and clinical findings in a series of patients with NK cell neoplasms with peripheral blood and/or bone marrow involvement, and the FCI of neoplastic and normal NK cells were compared. Retrospective data and specimens (bone marrow aspiration or peripheral blood) from 71 cases of NK cell neoplasms were obtained. All patients have been demonstrated laboratory and clinical features consistent with NK cell neoplasms, and the subtypes were determined by integrated clinical estimation. Routine 4-color flow cytometry (FCM) using a NK/T cell related antibody panels was performed. NK cell neoplasms were divided into two major subtypes by FCI, namely malignant NK cell lymphoma, including extranodal nasal type NK cell lymphoma (ENKL, 11 cases) and aggressive NK cell lymphoma/leukemia (ANKL, 43 cases), and relative indolent chronic lymphoproliferative disorder of NK cell (CLPD-NK, 17 cases). The former exhibited stronger CD56-expressing, larger forward scatter (FSC) and more usually CD7- and CD16-missing. FCI of CLPD-NK was similar to normal NK cells, but CD56-expressing was abnormal, which was negative in five cases and partially or dimly expressed in eight cases. Cytomorphologic abnormal cells were found on bone marrow slides of 4 cases of ENKL and 30 cases of ANKL. Eight cases of ENKL were positive in bone marrow biopsies, and other three cases were negative. In 32 cases of ANKL which bone marrow biopsies were applied, 21 cases were positive in the first biopsies. Lymphocytosis was found only in six cases of CLPD-NK by cytomorphology, and biopsy pathology was not much useful for diagnosing CLPD-NK. These results suggest that FCM analysis of bone marrow and peripheral blood was superior to cytomorphology, bone marrow biopsy, and immunohistochemistry in sensitivity and early diagnosis for ANKL, stage III/IV ENKL and CLPD-NK. FCI could not only define abnormal NK cells but also determine the malignant classification. It is beneficial for clinical management and further study of NK cell neoplasms.

Nasal Natural Killer/T-cell Lymphoma with Skin, Eye, and Peroneal Nerve Involvement

Nasal-type natural killer (NK)/T-cell lymphoma (NKTL) is a rare disease strongly associated with Epstein-Barr virus and is often localized to the upper aerodigestive tract at presentation. Extranodal NKTL may involve any extranodal site and disease beyond the nasal cavity is highly aggressive, with short survival time and poor response to therapy. Herein we present a 57-year-old male that had been treated with systemic chemotherapy and cranial radiotherapy for nasaltype NKTL in the palate with skin, right eye, and right peroneal nerve involvement. He was given salvage chemotherapy consisting of 3 cycles of ICE and his response to the therapy was satisfactory, except for persistent right drop foot. About 6 weeks later, the patient presented with bilateral total loss of vision and proptosis; therefore, DHAP chemotherapy was started. Unfortunately, after 1 cycle of the second salvage chemotherapy, he died due to severe fungal infection of the hard palate. Despite the fact that involvement of any extranodal site is possible, concurrent involvement of many systems in NKTL patients is unusual. Nasal-type NKTL has a poor prognosis, despite local radiotherapy and systemic chemotherapy. Physicians should be aware of this rare disorder than can only be diagnosed after extensive immunohistochemical studies.Conflict of interest:None declared.

Extranodal nasal–type NK/T-cell lymphoma of the palate and paranasal sinuses

SummaryBackground:Extranodal nasal-type natural killer (NK)/T-cell lymphoma represents a rare entity, typically originating in the nasal cavity, palate or midfacial region. Signs and symptoms include non-specific rhinitis and/or sinusitis, nasal obstruction, epistaxis, facial swelling and development of deep necrotic ulceration in the midline of the palate, causing an oronasal defect. Differential diagnosis includes fungal infections, Wegener’s granulomatosis, tertiary syphilis, other non-Hodgkin’s lymphomas and malignant epithelial midline tumors.Case Report:We present a case of a 40-year-old man complaining of headache, facial pain, nasal congestion and fever. Examination revealed a large deep necrotic ulcer in the middle of the palate, presenting as an oronasal defect. Endoscopic rhinoscopy revealed crusts in the nasal cavities, moderate perforation of the nasal septum cartilage and contraction of the middle and inferior conchae. Computer tomography showed occupation of the maxillary sinuses, ethmoidal cells and sphenoidal sinus by a hyperdense soft tissue mass. Laboratory investigation revealed increased erythrocyte sedimentation rate. A wide excision of the lesion was performed. Histopathological and immunohistochemical evaluation established the diagnosis of extranodal nasal-type NK/T-cell lymphoma. The patient was treated with CHOP chemotherapy, involved-field radiotherapy and autologous bone marrow transplantation. A removable partial denture with obturator was fabricated and inserted to relieve problems caused by the oronasal defect.Conclusions:Extranodal nasal-type NK/T-cell lymphoma is a very aggressive, rapidly progressing malignant neoplasm with a poor prognosis, which can be improved by early diagnosis and combined treatment.

Diagnosis of a multicentric extranodal nasal-type natural killer T-cell lymphoma made with an anterior chamber tap.

To report the case of an unusual multicentric Epstein-Barr virus (EBV)-associated extranodal nasal-type natural killer T-cell lymphoma masquerading as unilateral panuveitis, diagnosed with an anterior chamber tap. The clinical history and the morphological, immunohistochemical, and molecular features of a 51-year-old white man with left severe panuveitis were retrospectively evaluated. The patient initially presented with a 3-month history of recalcitrant sinusitis and new onset of unilateral loss of vision. Clinical examination revealed right peripheral facial nerve palsy, severe panuveitis of the left eye, and nasopharyngeal obstruction. Anterior chamber aspirates were examined. The combination of the presence of small- to intermediate-size lymphocyte proliferation, moderate elevation of the interleukin-10 level on cytokine profiling, and slightly positive polymerase chain reaction for EBV in the aqueous humor indicated an EBV-induced nasal-type natural killer T-cell lymphoma. Transnasal biopsy revealed the presence of numerous irregular lymphoma cells with positive staining for CD3, CD56, EBV-encoded RNA in situ hybridization, and negative staining for CD4, CD8, and CD1a. Lumbar puncture, cerebral magnetic resonance imaging, thoracoabdominal computed tomography, and upper digestive tract endoscopy revealed meningeal, renal, adrenal, and digestive involvement. Massive hemorrhage of the upper digestive tract caused rapid death. Extranodal nasal-type natural killer T-cell lymphoma is a very uncommon disease that may present acutely, sometimes as pseudouveitis. Simple investigations such as anterior chamber aspirates for cytological examination, reinforced by cytokine profiling and viral polymerase chain reaction looking for EBV, may provide a rapid diagnosis, necessary given the poor prognosis of the disease. To our knowledge, and after extensive review of the literature, we did not find another case report diagnosing this entity by anterior chamber paracentesis.

Unfavorable prognosis of elderly patients with early-stage extranodal nasal-type NK/T-cell lymphoma

BackgroundExtranodal nasal-type NK (natural killer)/T-cell lymphoma in elderly patients is rare, and its prognosis is unclear. This study aims to investigate the clinical characteristics and prognosis of this lymphoma. Patients and methodsWe analyzed 24 patients (age, >60 years old) with early-stage extranodal nasal-type NK/T-cell lymphoma. Among these patients, 23 received radiotherapy alone (n = 15) or radiotherapy and chemotherapy (n = 8) and 1 received chemotherapy alone. ResultsElderly patients with early-stage extranodal nasal-type NK/T-cell lymphoma were characterized by male predominance, good performance, large proportion of localized disease, more comorbidities, low-risk international prognostic index, and unfavorable prognosis. The 5-year cancer-specific survival (CSS), overall survival (OS), and progression-free survival (PFS) rates for all patients were 54.3%, 42.0%, and 40.2%, respectively. The 5-year CSS, OS, and PFS rates were 43.5%, 36.6%, and 34.1% in patients who received radiotherapy alone, and 50%, 50%, and 50% in patients who received radiotherapy and chemotherapy, respectively (P = 0.852 for CSS, P = 0.801 for OS, and P = 0.694 for PFS). Four patients died of treatment-related mortality. ConclusionElderly patients with early-stage extranodal nasal-type NK/T-cell lymphoma exhibit poor prognosis and need special attention because of high treatment-related mortality.

Extranodal nasal-type NK/T-cell lymphoma: Computed tomography findings of head and neck involvement

Extranodal nasal-type natural killer (NK)/T-cell lymphoma (NKTL) is a rare, but distinct clinical entity in Asian populations. However, it is not uncommon for NKTL to be belatedly or postoperatively diagnosed because radiologists are not familiar with its radiological features. To investigate the computed tomography (CT) findings in patients with extranodal nasal-type NKTL involving the head and neck. Twenty-nine patients (18 male, 11 female; mean age 51.5 years) with pathologically proven extranodal nasal-type NKTL were enrolled in this study. Two head-and-neck radiologists retrospectively evaluated the CT scans with special attention to the primary site, the extent of involvement, and patterns of the lesions (i.e., morphologic features, ulceration or necrosis, and bone destruction). The cases of nasal-type NKTL consisted of primary tumors arising from the sinonasal cavity in 13 patients (45%), the naso/oropharynx in three patients (10%), and simultaneous involvement in the nasal cavity and naso/oropharynx in 13 patients (45%). Fourteen patients (48%) showed tumor extension into adjacent structures. Of the 26 patients with involvement of the nasal cavity, 18 (69%) presented with an infiltrative pattern, seven (27%) with a polypoid pattern, and one (4%) with a combined pattern. Areas of necrosis within the tumor were observed in six patients (21%), bone destruction in five (17%), and regional lymphadenopathies in two (7%). Extranodal NKTL may be initially suggested by CT findings in the head and neck. Clinicians should consider these findings indicative of the possibility of NKTL, and should perform a histopathologic confirmation to establish a diagnosis.

Orbital Apex Syndrome with Nasal Type Natural Killer(NK)/T-cell Lymphoma of Sphenoid and Ethmoid Sinus

Purpose: To report a case of nasal-type NK/T cell lymphoma occurring in the sphenoid and ethmoid sinuses of an orbital apex syndrome patient. Case summary: A 61-year-old male patient visited our hospital for the impairment of vision in the right eye for the previous month, and for right-side blepharoptosis, pain around the eyeball, and limitation of extraocular movement in the right eye for three days earlier. In MRI (magnetic resonance image) and CT (computed tomography), shades with vague boundaries were observed in the right sphenoid and ethmoid sinuses, and open biopsy and decompression were performed for the lesions. After the surgery, the vision of the right eye increased, and improvement was observed in the right blepharoptosis, the pain around the eyeball, and in the limitation of motility of the extraocular muscle in the right eye. The patient was diagnosed with nasal type NK/T cell lymphoma in biopsy, and radiotherapy and chemotherapy were performed.

Extranodal NK/T cell Lymphoma, nasal type: clinical, radiological, histological features for early diagnosis

Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin’s lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.

Primary radiotherapy showed favorable outcome in treating extranodal nasal-type NK/T-cell lymphoma in children and adolescents

AbstractExtranodal nasal-type natural killer (NK)/T-cell lymphoma is rarely observed in children and adolescents. We aim to investigate the clinical features, prognosis, and treatment outcomes in these patients. Thirty-seven patients were reviewed. There were 19, 14, 2, and 2 patients with stage I, stage II, stage III, and stage IV diseases, respectively. Among the patients with stage I and II disease, 19 patients received initial radiotherapy with or without chemotherapy, and 14 patients received chemotherapy followed by radiotherapy. The 4 patients with stage III and IV disease received primary chemotherapy and radiation of the primary tumor. Children and adolescents with extranodal nasal-type NK/T-cell lymphoma usually presented with early-stage disease, high frequency of B symptoms, good performance, low-risk age-adjusted international prognostic index, and chemoresistance. The complete response rate after initial radiotherapy was 73.7%, which was significantly higher than the response rate after initial chemotherapy (16.7%; P = .002). The 5-year overall survival (OS) and progression-free survival (PFS) rates for all the patients were 77.0% and 68.5%, respectively. The corresponding OS and PFS rates for patients with stage I and II disease were 77.6% and 72.3%, respectively. Children and adolescents with early-stage extranodal nasal-type NK/T-cell lymphoma treated with primary radiotherapy had a favorable prognosis.