| Objective:To investigate the clinical and imaging features, pathological characteristics,treatment outcome and prognostic factors of primary diffuse large B cell lymphoma(DLBCL)originated from nasal cavity and paranasal sinuses and nasopharynx in order to provide basis for the early diagnosis, treatment and prognosis of the disease.Methods:The data is collected from the First Affiliated Hospital of Jilin University and the Second Affiliated Hospital of Jilin University where patients were diagnosed of DLBCL originated from nasal cavity and paranasal sinuses and nasopharynx during October 2007 to October 2015 and followed the patients up. We also use the reference from both domestic and foreign literatures to analysis and summarize the diagnosis and treatment strategy and prognosis of the disease. The univariate survival analysis were calculated by Kaplan-Meier method. The Cox model was applied to the multivariate survival analysis.Results:1.clinical and imaging features Retrospective analysis 2400 cases who were diagnosed of DLBCL and treated in the First Affiliated Hospital of Jilin University and the Second Affiliated Hospital of Jilin University during October 2007 to October 2015. Of the 2400 cases, 18 cases including 10 cases of male and 8 cases of female were found originated from nasal cavity and paranasal sinuses and nasopharynx, and all of them consult to the department of ENT because of nasal symptoms, the ratio of male to female is 5:4. The onset age range is 42 ~ 87 years old with the median age is 62 years old. 14 cases occurred in the nasal cavity and paranasal sinuses, 4 cases occurred in nasopharynx. The main clinical symptoms are stuffy nose, runny nose, stuff with blood and headache.The number of patients with Ann Arbor staging ⅠE + ⅡE is 11 cases, 7 cases of patients with staging Ⅲ E + Ⅳ, 2 cases of patients with elevated serum lactate dehydrogenase(LDH), 2 cases of patients with B symptoms(fever, night sweats, and weight loss), 3 cases of patients with lymph node metastasis.12 cases with international prognostic index(IPI) score of 0 ~ 2 points and 6 cases with 3 ~ 4 points.2.Imaging examination16 patients received chest CT, abdominal CT or Color Doppler ultrasound, nasal sinus CT or MRI,nasopharynx CT or MRI and neck Color Doppler ultrasound and 2cases of patients received PET-CT examination before and after treatment. Nasal sinus CT examination showed: mass with soft tissue density in nasal cavity and paranasal sinuses, enhanced CT scanning of the nasal sinus showed a mild to moderate enhancement of the mass. T1 WI of Sinus MRI examination showed low or equal signal while T2 WI with long signal of the lesions, long T1 and long T2 signal of the lesions were visible in the enhanced MRI examination. PET-CT examination showed that the lesion and the affected lymph nodes were abnormal radioactive concentration.3.Pathological characteristics The structure of the lesions were completely destroyed, immunoblast, centroblasts and RS like giant cells scattered or diffuse infiltration in inflammatory background and map like necrosis in the case of EBER positive lesions was visible under the microscope.The results of immunohistochemistry showed that CD79 a and CD20 were positive, 1 case with germinal center derived was CD10 positive, Bcl-6 negative and Mum-1 positive, the other 17 cases were non-germinal center derived, CD10 were all negative and 16 cases were Mum-1 positive,1 case was Mum-1 negative,14 cases were Bcl-6 positive, 3 cases were Bcl-6 negative. EBER staining was positive in 1 case, 1case with scattered positive(EBER positive cells scattered in the proportion of the number of <5%). The median value of index of tumor cell proliferation Ki-67 was80%.4.Treatment and response All of 18 patients received chemotherapy with CHOP, among which 6 cases were combined with rituximab, 4 cases combined with local radiotherapy. 12 cases reached complete remission(CR), 3 cases achieved partial remission(PR), 1 cases were in stable disease(SD), 2 cases were in progress disease(PD). The ORR was 83.3% and the3 year overall survive was 77.8%. 6 patients died by the end of the observation.Conclusion:1.The primary DLBCL originated from nasal cavity and paranasal sinuses is a rare and aggressive malignant tumor in clinic.2.The initial symptoms of the disease are mainly stuffy nose, runny nose, stuff with blood and headache, lymph node enlargement and B symptoms were rare, the clinical manifestations lack of specificity, and may lead to misdiagnosis.3. The median age of the patients is high, but mostly LDH level was normal, IPI was more of 0-2.4. The diagnosis of this disease mainly depends on the histopathology and immunohistochemistry, mostly are non germinal center derived.5. R-CHOP chemotherapy is the main treatment method, combine with local radiation therapy should be considered for patients with tumor diameter greater or equal to 6 cm.6. IPI score and Ann Arbor stage associated with the 3 year overall survival of the patients(P<0.05).7. Prognosis of elderly patients with EB virus positive DLBCL are poor. |
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