title: Long-term Tumor-free Survival With Untreated Primary Diffuse Large B-cell Lymphoma of the Tonsil: A Report of 9 Cases.
abstract: In some cases, long-term tumor-free survival might be possible for untreated primary diffuse large B-cell lymphoma (DLBCL) of the tonsil. Here, we report of 9 untreated patients who had primary tonsil DLBCL with long-term tumor-free survival. All these patients were children or young adults (4 male and 5 female individuals; ages: 6 to 38 y, median age: 25 y) with clinically evident swollen tonsils or papillary neoplasms. Tonsillectomy and biopsies indicated partial structural destruction of the tonsils with diffuse infiltration of large lymphoid cells. The large cells expressed CD20 with a Ki-67 proliferative index >50%. All samples were negative for CD5, Epstein-Barr virus-encoded RNA, and t(14;18) translocation. Except for cases 2 and 4, all samples showed monoclonal immunoglobulin gene rearrangements. Although chemotherapy and radiotherapy had not been administered after tonsillectomy (for various reasons), periodic imaging and clinical evaluation showed that none of the 9 patients developed or died of lymphoma (median follow-up: 40 mo). In conclusion, primary tonsil DLBCL does not always behave in a malignant manner, and some patients can achieve long-term tumor-free survival without chemotherapy or radiotherapy. On the basis of this case series, we concluded that close follow-up and observation might be possible for pediatric and young-adult patients who have undergone complete tonsillectomy for primary tonsil DLBCL with the following features: short disease course, morphologically early lesions, negativity for Epstein-Barr virus-encoded RNA and t(14;18) translocation, and no involvement of any other sites, after careful clinical evaluation.
authors.affiliation: *Department of Pathology, Beijing Friendship Hospital, Capital Medical University (Lymphoma Diagnostic Center, Beijing Institute of Clinical Medicine) †Department of Lymphoma, Beijing Cancer Hospital, Peking University, Beijing ‡Department of Pathology, The First Affiliated Hospital of Xiamen University, Xiamen §Department of Pathology, Shenzhen Second People's Hospital, Shenzhen, China.
authors.forename: Xiaojing
authors.initials: X
authors.lastname: Zhang
authors.forename: Yuanyuan
authors.initials: Y
authors.lastname: Zheng
authors.forename: Jianlan
authors.initials: J
authors.lastname: Xie
authors.forename: Jun
authors.initials: J
authors.lastname: Zhu
authors.forename: Yuqin
authors.initials: Y
authors.lastname: Song
authors.forename: Xiaojing
authors.initials: X
authors.lastname: Teng
authors.forename: Wei
authors.initials: W
authors.lastname: Liu
authors.forename: Yi
authors.initials: Y
authors.lastname: Ding
authors.forename: Yuhua
authors.initials: Y
authors.lastname: Huang
authors.forename: Xiaoge
authors.initials: X
authors.lastname: Zhou
mesh_terms: D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001706:Biopsy; D002648:Child; D002883:Chromosomes, Human, Pair 14; D002887:Chromosomes, Human, Pair 18; D018572:Disease-Free Survival; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000601:surgery / Q000821:virology; D008297:Male; D025202:Molecular Diagnostic Techniques; D064847:Multimodal Imaging; D011237:Predictive Value of Tests; D012367:RNA, Viral / Q000235:genetics; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D017741:Survivors; D013997:Time Factors; D014067:Tonsillar Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology* / Q000601:surgery / Q000821:virology; D014068:Tonsillectomy; D014178:Translocation, Genetic; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D012367:RNA, Viral