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Int J Clin Exp Pathol 2010;3(4):437-442
Case Report
A rare pediatric case of a thymic cytotoxic and lymphoblastic T/NK cell lymphoma
Timo Gaiser, Wolfgang Haedicke, Maria R. Becker
Section of Cancer Genomics, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA; Institute of Pathology, IFP-Spandau,
Berlin, Germany; Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
Received February 17, 2010, accepted March 17, 2010, available online: March 20, 2010
Abstract: Attempts to establish a concise classification of lymphoblastic lymphomas (LBLs) have gained momentum in recent years, mainly
due to the expanding possibilities of immunohistochemical and genetic characterization of different disease entities. Thus, cases of immature
lymphoid malignancies with unusual immunopathological features have been reported during the last years, suggesting the need for new LBL
classification concepts. To further characterize and demonstrate the extended spectrum of LBL, we present an unusual pediatric case of LBL
that could not be categorized into one of the subgroups and exhibited a benign course after surgical treatment and subsequent chemotherapy.
A mediastinal tumor of a 6-year-old Caucasian boy was examined by clinical staging, light microscopy, immunohistochemistry and PCR
assays. The tumor cells reacted with TdT and had a positive cytoplasmic immunoreactivity for CD3. Further T cell markers CD1a, CD4, CD8,
CD10, and CD56 reacted positively, but CD57, CD16 and CD 30 (Ber H2) were all negative. B cell markers (CD20, CD22, Cd79a and IgM) were
all negative. No clonal B cell Ig or T cell gamma chain rearrangements were detectable. Epstein Barr virus and other Herpes Virus DNA were
not detected using a sensitive PCR assay. The applied chemotherapy was tolerated well and a complete remission of the tumor was achieved
(observation period three years after the initial diagnosis). Localization, morphology, and the expressions markers made the tumor a typical
member of the LBL group. However, our case represents a rare pediatric lymphoma derived from a thymic precursor committed to T/NK-cell
differentiation and a favourable outcome after chemotherapy. (IJCEP1002006).
Key words: Lymphoblastic lymphomas, T/NK cell lymphoma, pediatric lymphoma
Full text PDF
Address all correspondence to:
Timo Gaiser, MD
Section of Cancer Genomics
National Cancer Institute, National Institutes of Health
Bethesda, MD, USA.
Tel: 001-301-496-6501; Fax: 001-310-435-4428
Email: timo_gaiser@web.de
Case Report
A rare pediatric case of a thymic cytotoxic and lymphoblastic T/NK cell lymphoma
Timo Gaiser, Wolfgang Haedicke, Maria R. Becker
Section of Cancer Genomics, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA; Institute of Pathology, IFP-Spandau,
Berlin, Germany; Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
Received February 17, 2010, accepted March 17, 2010, available online: March 20, 2010
Abstract: Attempts to establish a concise classification of lymphoblastic lymphomas (LBLs) have gained momentum in recent years, mainly
due to the expanding possibilities of immunohistochemical and genetic characterization of different disease entities. Thus, cases of immature
lymphoid malignancies with unusual immunopathological features have been reported during the last years, suggesting the need for new LBL
classification concepts. To further characterize and demonstrate the extended spectrum of LBL, we present an unusual pediatric case of LBL
that could not be categorized into one of the subgroups and exhibited a benign course after surgical treatment and subsequent chemotherapy.
A mediastinal tumor of a 6-year-old Caucasian boy was examined by clinical staging, light microscopy, immunohistochemistry and PCR
assays. The tumor cells reacted with TdT and had a positive cytoplasmic immunoreactivity for CD3. Further T cell markers CD1a, CD4, CD8,
CD10, and CD56 reacted positively, but CD57, CD16 and CD 30 (Ber H2) were all negative. B cell markers (CD20, CD22, Cd79a and IgM) were
all negative. No clonal B cell Ig or T cell gamma chain rearrangements were detectable. Epstein Barr virus and other Herpes Virus DNA were
not detected using a sensitive PCR assay. The applied chemotherapy was tolerated well and a complete remission of the tumor was achieved
(observation period three years after the initial diagnosis). Localization, morphology, and the expressions markers made the tumor a typical
member of the LBL group. However, our case represents a rare pediatric lymphoma derived from a thymic precursor committed to T/NK-cell
differentiation and a favourable outcome after chemotherapy. (IJCEP1002006).
Key words: Lymphoblastic lymphomas, T/NK cell lymphoma, pediatric lymphoma
Full text PDF
Address all correspondence to:
Timo Gaiser, MD
Section of Cancer Genomics
National Cancer Institute, National Institutes of Health
Bethesda, MD, USA.
Tel: 001-301-496-6501; Fax: 001-310-435-4428
Email: timo_gaiser@web.de
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