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Objective:To analyze the diagnostic and prognostic value of routine bone marrow examination in patients with extranodal NK/T-cell lymphoma (ENKTCL) based on PET-CT staging.Methods:Clinical data of 186 patients who received bone marrow biopsy and bone marrow aspiration in Fujian Medical University Union Hospital from 2013 to 2021 were retrospectively analyzed. All patients were divided into bone marrow biopsy + bone marrow aspiration group ( n=186) and PET-CT + bone marrow biopsy group ( n=139). The sensitivity, specificity, positive and negative predictive values were compared between two groups. The data were analyzed and plotted. Survival analysis was performed using Kaplan-Meier method and log-rank test. Results:In the whole cohort, 45 patients were positive for bone marrow biopsy, and 30 of them were positive for bone marrow aspiration. A total of 141 patients who were negative for bone marrow biopsy also achieved negative results for bone marrow aspiration. A total of 139 patients completed PET-CT staging and bone marrow biopsy. And 30 patients were diagnosed with positive bone marrow by PET-CT, in which 22 of them were confirmed positive by bone marrow biopsy. Among 109 patients diagnosed with negative bone marrow by PET-CT, 5 of them were confirmed positive by bone marrow biopsy. All these cases were classified as stage Ⅳ due to distant metastases. PET-CT had a diagnostic sensitivity of 81.5%, a specificity of 92.9%, a positive predictive value of 73.3%, and a negative predictive value of 95.4%. Among early stage (Ⅰ-Ⅱ stage) patients diagnosed with PET-CT, all of them were negative for bone marrow biopsy (the negative predictive value was 100%). In stage Ⅳ patients ( n=55), the 1-year overall survival of patients with bone marrow involvement by bone marrow biopsy or PET-CT ( n=35) compared with their counterparts with the involvement of other organs ( n=20) was 28.7% vs.42.0% ( P=0.13), and 1-year progression free survival rates was 23.2% vs. 23.3% in ( P=0.94). Conclusions:Routine bone marrow biopsy does not change the original staging of patients with early stage ENKTCL based on PET-CT staging. Advanced stage patients with positive bone marrow biopsy tend to obtain worse prognosis, indicating that bone marrow biopsy still has certain value.
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Extranodal NK/T cell lymphoma, nasal type（ENKTL） is a highly aggressive malignant tumor derived from NK cells. This article reports a case of ENKTL invading the larynx and digestive tract. The clinical clinical manifestations include hoarseness and intranasal masses.
Subject(s)
Humans , Lymphoma, Extranodal NK-T-Cell/pathology , Nose/pathology , Nose Neoplasms/pathology , Larynx/pathology , Gastrointestinal Tract/pathologyABSTRACT
Introdução: O linfoma não-Hodgkin é dividido em linfomas de células B e linfomas de células T, e o linfoma extranodal de células T / NK do tipo nasal está dentro do último grupo.Relato de caso: Paciente do sexo masculino de 30 anos, relata que há 6 meses, de forma progressiva e de início insidioso, apresenta tumor cervical à direita de crescimento progressivo, pelo qual foi encaminhado ao ambulatório de cabeça e pescoço onde apresentou seus principais sinais e sintomas adenopatia cervical direita, sintoma B e tumoração ao nível da nasofaringe, envolvendo o teto, parede posterior e face lateral; se movimenta com auxílio, com extenso conglomerado linfonodal supraclavicular direito, eritematoso, com calor local, além de áreas de ulceração e secreção serosa.Conclusão: O diagnóstico e tratamento precoces desta doença são as únicas ferramentas para melhorar o mau prognóstico e o grave impacto na qualidade de vida dos pacientes que a padecem
Introduction: Non-Hodgkin's lymphomas are divided into B-cell lymphomas and T-cell lymphomas, and extranodal NK/T-cell lymphoma, nasal type, is in the latter group.Case report: A 30-year-old male patient, for six months, progressively and with an insidious onset, has had a right-sided cervical tumor with progressive growth. He came to a head and neck outpatient clinic where the main signs and symptoms detected were right cervical lymphadenopathy, B-symptoms, and a tumor in the nasopharynx affecting the roof, posterior wall, and lateral wall. The patient moves with assistance and has an enlarged, erythematous warm right supraclavicular lymph node conglomerate. In addition, he has some ulcerated areas with serous drainage.Conclusion: Early diagnosis and treatment of this disease are the only tools to improve these patients' poor prognosis and severely deteriorated quality of life.
Introducción:El linfoma no Hodgkin se divide en linfomas de células B y linfomas de células T; y en este último grupo se encuentra el linfoma extraganglionar de células T / NK de tipo nasal.Caso clínico: Un paciente masculino de 30 años refiere que durante 6 meses de forma progresiva, y con un início insidioso, presenta una tumoración cervical en el lado derecho de crecimiento progresivo, por lo que acude a la consulta externa de cabeza y cuello, donde los signos y síntomas principales fueron adenopatía cervical derecha, síntoma B, y una tumoración a nivel de nasofaringe, que afecta el techo, la pared posterior y la cara lateral. Se moviliza con ayuda, con un extenso conglomerado ganglionar supraclavicular derecho, eritematoso, con calor local. Además, también muestra algunas áreas de ulceración y secreción serosa. Conclusión: El diagnóstico y tratamiento precoz de esta enfermedad son las únicas herramientas para mejorar el mal pronóstico y el deterioro severo en la calidad de vida de los pacientes que la padecen
Subject(s)
Immunohistochemistry , Herpesvirus 4, Human , Nasal CavityABSTRACT
Objective:To investigate the quality of life in patients with early-stage extra-nodal NK/T cell lymphoma of nasal type (ENKTL) arising from the upper aerodigestive tract, who had remained progression-free survival (PFS) for over 3 months after definitive intensity-modulated radiation therapy (IMRT), and to analyze the factors related to main adverse symptoms.Methods:276 patients who received IMRT from March, 2012 to June, 2021 were included. There were 201 males and 75 females with a median age of 41.5 years (range: 13-81 years) upon diagnosis. Consistent target delineation schemes and similar dose gradients were adopted for IMRT, with a median prescribed dose of 54.6 Gy/26F. Cross-sectional investigation was performed with a modified EORTC QLQ-H&N35 questionnaire, the incidence and severity of adverse symptoms, severity of disease and their influencing factors at each time-point during their survival were statistically analyzed.Results:The median age of patients at the investigation was 46.2 years, and the median PFS after IMRT was 47.2 months (range: 3.1-115.7 months). The most common adverse symptoms included nasal symptoms (incidence rate 63.8%), dry mouth (50%), tooth diseases (47.1%), smell and taste alteration, and sexual apathy, etc. Most symptoms were mild (the average standardized score was 5.50, the full score of 100 indicating the most severe), and could be relieved remarkably over survival time, but some symptoms, such as tooth diseases and sexual apathy, were more obvious and recurred for several years. Age and anti-PD-1 immune therapy influenced the symptom scores, and tooth diseases were closely correlated with dry mouth. Conclusion:The quality of life in patients with early-stage ENKTL after definitive IMRT is high, and the most significant symptoms include nasal symptoms, tooth diseases, and sexual apathy, etc. , which need to be mitigated with more studies.
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Objective:To compare the efficacy of concurrent and asynchronous radiochemotheray for early extranodal nasal natural killer/T-cell lymphoma (NKTCL).Methods:From 2007 to 2020, 278 patients with early NKTCL treated with comprehensive treatment in the Affiliated Tumor Hospital of Guizhou Medical University were recruited. According to the adjusted Nomogram-revised risk index (NRI) prognostic model, there were 49 cases in the good prognostic group without adverse prognostic factors (age>60 years old, increased serum lactate dehydrogenase (LDH), ECOG score ≥2, primary tumor invasion (PTI), Ann Arbor stage Ⅱ, and 229 cases in the poor prognostic group with any adverse prognostic factors. 145 of these cases were treated with concurrent radiochemotherapy, and 133 of them were treated with asynchronous radiochemotherapy.Results:The 5-year overall survival (OS) rate of the whole group was 71.0%, and the progression-free survival (PFS) rate was 67.6%. The 5-year OS rate in the good prognostic group was 95.6%, and 65.4% in the poor prognostic group ( P<0.001). In the poor prognostic group, the 5-year OS rates of patients with NRI=1(low-and moderate-risk group), NRI=2(moderate-and high-risk group), NRI≥3(high-risk group) were 72.1%, 61.1% and 47.7%, respectively ( P=0.007). There was no significant difference in curative effect between the concurrent and asynchronous radiochemotherapy groups. The 5-year OS rates were 70.6% and 69.8%( P=0.783), and the 5-year PFS rates were 67.6% and 65.2%( P=0.631). Further stratified analysis showed that the 5-year OS rates of patients with NRI=1 receiving concurrent and asynchronous radiochemotherapy were 73.1% and 76.5%( P=0.576), 62.6% and 69.3%( P=0.427) for those with NRI=2, and 58.1% and 42.3% for those with NRI≥3( P=0.954). Conclusions:Comprehensive treatment can significantly improve the prognosis of early NKTCL in the poor prognostic group. In the sequence of radiotherapy and chemotherapy, there is no significant difference in 5-year OS and PFS rates between concurrent and asynchronous radiochemotherapy. Sequential treatment with better tolerance can be adopted for early NKTCL with poor prognosis.
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Objective To explore the correlation of pretreatment EBV DNA load, EBV EA-IgA and VCA-IgA antibodies levels with the clinical characteristics, curative effect and prognosis of the patients with early-stage extranodal nasal-type NK/T-cell lymphoma (ENKTCL). Methods We analyzed the clinical features and prognostic factors of 78 ENKTCL patients. Results Positive rates of pretreatment EBV DNA, VCA-IgA and EA-IgA were 43.6%, 20.5% and 14.1%, respectively. EBV DNA was significantly associated with Ann Arbor stage, primary site, PTI and non-CR (all P < 0.05). VCA-IgA and EA-IgA were related to positive EBV DNA and non-CR (all P < 0.05). Multivariate analysis showed that age, EBV DNA and non-CR were independent prognostic factors for OS (all P < 0.05); age, EBV DNA, primary site and non-CR were independent prognostic factors for PFS (all P < 0.05). Conclusion The pretreatment positive EBV DNA is related to advanced Ann Arbor stage, PTI, primary extra-nasal subtypes of upper aerodigestive tract and poor response to treatment. The elevated levels of EA-IgA and VCA-IgA are related to positive EBV DNA and poor response to treatment. Pretreatment EBV DNA could be used for risk stratification and prognosis prediction of ENKTCL, while EA-IgA and VCA-IgA play limited role in guiding the prognosis of ENKTCL.
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Objective:To assess the efficacy and toxicity of chemoradiotherapy in the treatment of early stage extranodal nasal-type NK/T-cell lymphoma (ENKTCL).Methods:Retrospective review was conducted for 174 patients with pathological proved early stage ENKTCL who were treated in the Department of Radiation Oncology, Peking University Cancer Hospital & Institute. The Kaplan-Meier survival analysis was adopted to calculate the local-regional control (LRC), overall survival (OS), and progression free survival (PFS), and the Log-rank test COX regression model were applied to univariate and multivariate analyses.Results:The patients in this study included 102 and 72 patients diagnosed with Ann Arbor stage-Ⅰ and stage-Ⅱ, respectively. Among them, two patients received radiotherapy alone and 172 patients were treated with combined chemoradiotherapy. The overall response rate of all the patients was 94.2%, with a complete response (CR) rate of 87.9% (153). Furthermore, the rates of 5-year OS, PFS, and LRC were 87.3%, 83.1%, and 91.9%, respectively. The most common toxicities during the chemotherapy and radiotherapy included myelosuppression and oral mucositis, with grade ≥ 3 myelosuppression and grade ≥ 3 oral mucositis accounting for 62.1% and 10.9% of all patients, respectively. As shown by multivariate analysis, the adverse prognostic factors for OS included age > 60, B symptoms, and stage Ⅱ, while the adverse prognostic factors for PFS included age > 60 and stage Ⅱ. Meanwhile, the PFS rate was significantly improved by increasing the radiation dose (≥ 50 Gy vs.<50 Gy), and the 5-year PFS rates of the two groups were 83.5% and 76.5%, respectively [hazard ratio ( HR) 0.374; 95% CI, 0.169-0.826; P=0.015]. Conclusions:A good therapeutic effect can be achieved for early stage NK/T-cell lymphoma and the toxicities after combined chemoradiotherapy can be tolerated.
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Extranodal natural killer(NK)/T cell lymphoma,nasal type (ENKTL) is a type of highly aggressive non-Hodgkin lymphoma,closely associated with Epstein-Barr virus (EBV) infection in terms of carcinogenesis,and is prevalent in East Asia and South America.Clinicopathologically,ENKTL is characterized by diffuse tumor cell infiltration,angiocentral destructive growth pattern,and prominent tissue necrosis.Typically,ENKTL presents with extranodal involvements,particularly the upper aerodigestive tract,such as nasal cavity,nasopharynx,paranasal sinuses.Great advances has been made in the last decade regarding the molecular pathogenesis,refined tumor staging and risk assignment,and treatment strategies including local radiotherapy,concurrent chemoradiotherapy and sandwich therapy.Accordingly,response rate and long-term prognosis increase remarkably for stage Ⅰ and stage Ⅱ tumors,though clinical outcomes remains relatively poor for advanced ENKTL.Novel immunotherapy has been proving to be an a promising treatment modality for relapsed or refractory ENKTL.These relevant advances are reviewed in the present paper.
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Objective To investigate the clinicopathologic features of inert natural killer/T (NK/T)-cell lymphoma with CD30-positive large cell transformation. Methods The pathological data of one patient diagnosed as inert NK/T-cell lymphoma with CD30-positive large cell transformation in the Third Hospital Affiliated to Soochow University from April 2006 to March 2017 were collected. The histopathological features and immunohistochemical phenotype of the patient were observed and followed up. Results The patient received biopsy twice in April 2006 and March 2017. The first tumorectomy in sacral canal showed that diffuse and small lymphoid cell hyperplasia, irregular nucleus, granular chromatin, unobvious nucleolus, focal necrosis. Immunohistochemistry showed CD20(-), CD3(-), ALK(-), CD30(-), CD43(+), CD2(+), CD56(+), TIA-1(+), GrB(+), CD4(-), CD8(-). Finally, the patient was diagnosed as extranodal NK/T-cell lymphoma, nasal type. The microscopic examinations after the second left cervical lymph node biopsy showed large cells infiltrated into the background of small lymphocytes, plentiful cytoplasm, large nuclei, irregular nucleus, part of nuclear folding, obvious nucleolus, mitotic figures visible, visible intravascular tumor suppository, consistent small lymphocytes. Blood vessel invasion could be seen, as wells as consistent small lymphocytes, regular form, less cytoplasm, rare nuclear fission. Immunohistochemistry showed AE1/AE3(-), CD20(-), CD3(+), ALK (-), CD30(+), CD43(+), CD2(+), EMA(-), Ki-67(70%+), CD56(+), TIA-1(+), GrB(+), CD4(-), CD8(-). Finally, the patient was diagnosed as NK/T-cell lymphoma, nasal, recurrence. In situ hybridization showed EB virus encoded RNA (EBER) was positive for two biopsies. Conclusions Inert extranodal NK/T-cell lymphoma is very rare. The pathogenesis is closely related to EB virus infection. Its diagnosis must rely on histopathology and immunohistochemistry.
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Extranodal natural killer(NK)/T cell lymphoma, nasal type (ENKTL) is a type of highly aggressive non-Hodgkin lymphoma, closely associated with Epstein-Barr virus (EBV) infection in terms of carcinogenesis, and is prevalent in East Asia and South America.Clinicopathologically, ENKTL is characterized by diffuse tumor cell infiltration, angiocentral destructive growth pattern, and prominent tissue necrosis.Typically, ENKTL presents with extranodal involvements, particularly the upper aerodigestive tract, such as nasal cavity, nasopharynx, paranasal sinuses.Great advances has been made in the last decade regarding the molecular pathogenesis, refined tumor staging and risk assignment, and treatment strategies including local radiotherapy, concurrent chemoradiotherapy and sandwich therapy.Accordingly, response rate and long-term prognosis increase remarkably for stage Ⅰ and stage Ⅱ tumors, though clinical outcomes remains relatively poor for advanced ENKTL.Novel immunotherapy has been proving to be an a promising treatment modality for relapsed or refractory ENKTL.These relevant advances are reviewed in the present paper.
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INTRODUCCIÓN: El linfoma extranodal natural killer/célula T (NK/T) de tipo nasal, es una neoplasia poco frecuente, con una alta letalidad, caracterizada por destrucción ósea alrededor de los senos paranasales, el septum nasal u obstrucción de la vía aérea. Puede presentar compromiso primario de la piel, vía aérea y otros órganos. OBJETIVO: Presentar un caso ilustrativo de una afección poco frecuente y de curso agresivo en población pediátrica, para facilitar la sospecha diagnóstica y el rápido reconocimiento por parte de los especialistas. CASO CLÍNICO: Adolescente de 14 años, que consultó por lesiones solevantadas en brazos y piernas, no dolorosas, sugerentes de paniculitis subcutánea, las cuales evolucionaron a máculas violáceas ulceradas. La biopsia de las lesiones fue compatible con linfoma NK/T de tipo nasal. Fue derivada a oncología pediátrica, donde recibió tratamiento quimioterápico. Pese a los esfuerzos médicos, la paciente falleció a los 8 meses producto de una infección pulmonar grave secundaria a inmunosupresión. CONCLUSIONES: El linfoma extranodal NK/T, tipo nasal es una neoplasia poco frecuente, que se comporta de forma agresiva, con una alta mortalidad sin tratamiento. Por lo que su reconocimiento es de gran relevancia para el diagnóstico precoz y rápida derivación a Hemato-Oncología.
INTRODUCTION: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum or obstruction of the airway. Also, may be primary skin involvement, airway and other organs. OBJECTIVE: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists. CASE REPORT: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae. Skin biopsy showed lesion compatible with NK/T lymphoma, nasal type. She was referred to pediatric oncology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression. CONCLUSIONS: Extranodal NK/T-cell lymphoma, nasal type, is a rare neoplasm that behaves aggressively, with high mortality without treatment, therefore, its recognition has a high importance for early diagnosis and prompt referral to Hematology-Oncology.
Subject(s)
Humans , Female , Adolescent , Skin Neoplasms/diagnosis , Lymphoma, Extranodal NK-T-Cell/diagnosis , Fatal OutcomeABSTRACT
Objective Early diagnosis of extra-nodal nasal type NK /T cell lymphoma (ENKTCL) is difficult and it is often misdiagnosed.This article was to analyze the CT and MRI findings of ENKTCL originating in the nasal cavity or sinus and investigate the values of CT and MRI in the diagnosis of the disease . Methods We retrospectively analyzed the clinical data and CT and MRI findings about 13 cases of ENKTCL originating in the nasal cavity or sinus examined in our hospital from January 2012 to December 2017. Results Non-contrast CT scanning displayed soft tissue density shadow in the nasal cavity or sinus , with homogeneous density in the tumor and soft tissue mass with bony destruction ( mostly infiltrative absorption) in the midline nasal cavity and sinal area , and the area of soft tissue lesion was obviously larger than that of bony de -struction.Ten cases of contrast-enhanced CT of the tumor manifested mild to moderate heterogeneous density ; 10 cases of plain MRI pres-ented isointensity of T1WI and slightly increased intensity of T 2WI, while enhanced MRI showed mild to moderate heterogeneous density . Low-intensity T1WI, high-intensity T2WI and edge-enhancement were observed in the 10 cases accompanied by obstructive sinusitis . Conclusion ENKTCL originating in the nasal cavity or sinus has its own specific CT or /and MRI manifestations.Conventional CT or /and MRI combined with clinical signs and symptoms can accurately determine the site of lesion and scope of invasion and is therefore of important clinical significance for the early diagnosis and prognosis of the disease.
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Objective To analyze the clinical features and prognosis of extra-nodal nasal NK/T cell lymphoma originated from the larynx. Methods Clinical data of 15 cases of extra-nodal nasal NK/T-cell lymphoma originated from the larynx were retrospectively analyzed. The overall survival ( OS ) and progression-free survival ( PFS) were calculated by Kaplan-Meier survival analysis. The effect of different clinical factors on the clinical prognosis was assessed by univariate analysis. Results Among 15 patients,13 cases were male and 2 female. The median age of onset was 40 years. In 8 cases,the lesions were confined to the larynx,and only 4 cases suffered from cervical lymph node involvement. According to Ann Abor staging, 11 cases were classified as grade I,3 as gradeⅡand 1 as gradeⅢ.The median OS was 28. 0 months and the 5-year OS was 32. 0％.The median PFS was 24. 7 months and the 5-year PFS was 33. 3％.Among 14 patients with stage Ⅰ/Ⅱ,the clinical prognosis of patients receiving combined chemo-radiotherapy was significantly better than those of their counterparts undergoing radiotherapy or chemotherapy alone ( median OS:37. 2 vs. 11. 2 vs.3. 7 months,P=0. 004) . Conclusion Extra-nodal nasal NK/T cell lymphoma originated from the larynx is extremely rare, predominantly in middle-aged male patients. The general condition is relatively favorable. Patients present with multiple lesions in the early stage and relatively poor prognosis. The clinical efficacy of chemotherapy combined with radiotherapy is probably higher compared with that of radiotherapy or chemotherapy alone.
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Objective To evaluate the prognostic value of anemia in patients diagnosed with extranodal nasal-type natural killer (NK)/T cell lymphoma (NKTCL).Methods Clinical data of 1 225 NKTCL patients receiving the first course of treatment from 10 medical institutions in China were retrospectively analyzed.According to the diagnostic criteria in China,anemia was defined as the hemoglobin (Hb) level＜ 120 g/L for the male and＜ 110 g/L for the female from the sea-level area.The severity of anemia was classified into the extremely severe anemia (Hb ≤ 30 g/L),severe anemia (31-60 g/L),moderate anemia (61-90 g/L) and mild anemia (＞90 g/L).Results Among 1 225 patients,199(16.2％) were complicated with anemia,who had more adverse prognostic factors compared with their counterparts without anemia.Among NKTCL patients with anemia,the proportion of patients with stage Ⅱ-ⅣV,a median age＞ anemia,Eastern Cooperative Oncology Group (ECOG) score of 2-4 and NK/T-cell lymphoma prognostic index (NKTCLPI) ≥ 2 was relatively high.Patients with anemia obtained worse clinical prognosis than those without anemia.The 5-year overall survival (OS) and progression-free survival (PFS) in NKTCL patients with anemia were calculated as 49.4％ and 35.4％,significantly lower compared with 63.3％ and 56.0％ in their counterparts without anemia (both P＜0.01).Single factor analysis demonstrated that anemia,age,ECOG score,group B symptom,lactate dehydrogenase,primary tumor site,primary tumor invasion and staging were the prognostic factors of OS and PFS.Multivariate analysis revealed that anemia was still the independent prognostic factor.Conclusions Anemia is not common in patients with NKTCL and these patients obtain poor clinical prognosis.Anemia is an independent prognostic factor for patients with NKTCL.
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El linfoma T/NK extraganglionar tipo nasal es un linfoma extraganglionar, habitualmente expresa el fenotipo NK y VEB positivo. Cursa ocasionando necrosis y angioinvasión afectando de manera preferente estructuras mediofaciales. Característicamente es muy agresivo. Presentamos un caso con una sobrevida de siete meses a partir de los primeros síntomas y realizamos revisión de la literatura.
Extranodal NK/T-cell lymphoma nasal type, is an extranodal lymphoma, usually with an NK-cell phenotype and EBV possitive. It causes necrosis and angioinvasion, and it is most commonly presenting in the midfacial area. Characteristically it is very aggressive. A case with survival of seven months from the first symptoms is reported and a review of the literature is made.
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Humans , Male , Middle Aged , Nose Neoplasms/pathology , Lymphoma, Extranodal NK-T-Cell/pathology , Nose Neoplasms/diagnostic imaging , Fatal Outcome , Epstein-Barr Virus Infections/complications , Lymphoma, Extranodal NK-T-Cell/diagnostic imagingABSTRACT
Objective To investigate the effect of pretreatment serum hemoglobin (Hb) level on the prognosis of early-stage extranodal nasal-type NK/T-cell lymphoma.Methods A retrospective analysis was performed on the clinical data of 175 patients with stage Ⅰ or Ⅱ extranodal nasal-type NK/T-cell lymphoma who were admitted to The Tumor Hospital Affiliated to Shanxi Medical University from 2000 to 2015.The inclusion criteria included Ann Arbor Ⅰ/Ⅱ stage, the primary tumor located in the upper aerodigestive tract, without other malignant diseases, and complete clinical information and follow-up data.Of the 175 patients, 67 received chemotherapy alone, 8 received radiotherapy alone,100 received radiotherapy and chemotherapyed.The survival rate was calculated using the Kaplan-Meier method.The log-rank test was used for univariate prognostic analysis.The Cox regression model was used for multivariate prognostic analysis.Results The univariate analysis showed that pretreatment serum Hb level (≥120 g/L), lactate dehydrogenase (LDH) level (normal), Eastern Cooperative Oncology Group (ECOG) score (0-1), Ann Arbor stage (IE), and radiotherapy were associated with significantly improved progression-free survival (PFS) and overall survival (OS)(P=0.000-0.046).The multivariate analysis showed that pretreatment serum Hb level, LDH level, ECOG score, and Ann Arbor stage were independent prognostic factors for PFS and OS (P=0.000-0.040).Conclusion Patients with a high pretreatment serum Hb level (≥120 g/L) have a better prognosis than those with a low pretreatment serum Hb level (<120 g/L).
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Objective This study aimed to evaluate the prognostic value of regional lymph node spread in patients with stage Ⅱ nasal-type NK/T-cell lymphoma of the upper aerodigestive tract (UADT-NKTCL).Methods From 1987 to 2013,a total of 97 patients with newly-diagnosed stage Ⅱ UADT-NKTCL were retrospectively reviewed.Primary tumors were located in the nasal cavity (n=52) or extranasal UADT sites (n=45).The majority of patients were treated with primary radiotherapy.Sixty-five patients were treated with combined modality therapy (CMT),and 32 patients were treated with radiotherapy alone (n=27) or chemotherapy alone (n=5).The Kaplan-Meier method was used to calculate the survival rate,and the log-rank test was used for survival difference analysis and monovariate prognostic analysis.The Cox regression model was used to multivariate prognostic analysis.Results The 5-year overall survival (OS) and progression-free survival (PFS) rates for all stage Ⅱ patients were 57% and 49%,respectively.The presence of a lower neck lymph node (defined as extension below the caudal border of the cricoid cartilage) was significantly associated with poor outcomes on univariate analysis and maintained significance on multivariate analysis.The median survival for patients with lower neck lymph node was 19.3 months and the 2-,5-years OS rates were 28% and 11%(P=0.000).For stage Ⅱ patients,CMT significantly improved survival.The 5-year OS and PFS rates were 64% and 52% for CMT,compared with 40.4%(P=0.006) and 42% for single modality therapy (P=0.088).Conclusion The level of regional lymph node is a powerful prognostic factor for stage Ⅱ UADT-NKTCL.The involvement of low neck lymph node is significantly associated with poorer survival outcomes.
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Purpose To explore the prognostic value of deletion of the TNFAIP3 gene in natural killer/T-cell lymphoma,nasal type detected with fluorescence immunophenotyping and interphase cytogenetics as a tool for the investigation of neoplasm (FICTION).Methods FICTION was performed to detect the abnormalities of TNFAIP3 gene in 109 cases of natural killer/T-cell lymphoma,nasal type.Results TNFAIP3 deletion was found in 25/79 detectable cases.The deletion of TNFAIP3 was positively correlated with the International Prognosis Index (IPI) (P =0.019).Conclusion Frequent deletion of TNFAIP3 was associated with IPI in natural killer/T-cell lymphoma,nasal type,suggesting the important prognostic value of TNFAIP3 in the natural killer/T-cell lymphoma,nasal type.
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Purpose To improve the knowledge of the clinicopathologic features of pulmonary extranodal NK/T-cell lymphoma (ENKTL).Methods Three cases of pulmonary ENKTL were analyzed retrospectively,medical recordings,imaging manifestations,pathological features,immunophenotypes,EBER in situ hybridizations and other related literatures were reviewed.Results The group was composed of 1 man and 2 women with ages ranging from 52 to 68 years.The clinical symptoms and computed tomography (CT) manifestations were nonspecific.Histological observation showed that atypical lymphocytes in bronchi and parenchyma of the lung were invasively distributed.The neoplasms presented angiocentric and angiodestructive growth patterns with different degrees of inflammatory response and necrosis.For the immunophenotypes,all cases were positive for CD3,CD56 and cytotoxic granule (TIA-1,Perforin,GranB).The positive ratios of CD5 and CD30 were both 2/3.All 3cases showed positive in situ hybridization for Epstein-Barr virusencoded small RNA (EBER).Conclusion Pulmonary extranodal NK/T-cell lymphoma is very rare with poor prognosis and difficult to diagnose.The immunohistochemical staining and molecular pathological.technology may be helpful for the diagnosis and differential diagnosis of the tumors.
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Objective To investgate the pathogenesis of PTEN/PI3K/AKT/mTOR signaling pathways in nasal type extranodal NK/T cell lymphoma(ENKTCL-N).Methods The expression of PTEN,p-AKT,p-4EBP1 and p-mTOR in 51 patients with ENKTCL-N and 20 patients with reactive proliferative lymph nodes was detected by immunohistochemistry EnVision.The clinical data were collected and the clinical features,curative effect and prognosis were analyzed retrospectively.Survival analysis was performed by Kaplan-Meier method.Results The positive rate of PTEN in ENKTCL-N patients was lower than that in patients with reactive hyperplastic lymph nodes,and the positive rates of p-AKT and p-4EBP1 were higher than those in patients with reactive hyperplastic lymph nodes,and the difference was statistically significant(P＜0.05);The positive rate of p-mTOR in ENKTCL-N patients was higher than that in patients with reactive hyperplastic lymph nodes,but the difference was not statistically significant (P＞0.05).The survival rate of PTEN-positive patients was higher than that of PTEN-negative patients,and the difference was statistically significant(P＜0.05).Conclusion PTEN/PI3K/AKT/mTOR signaling pathway is abnormally active in ENKTCL-N,which may be related to its pathogenesis;PTEN-positive patients may have better prognosis.