Indolent T-lymphoblastic proliferation: report of a case with a 16-year course without cytotoxic therapy

• Published in: The American journal of surgical pathology Vol. 23; no. 8; p. 977
• Main Authors: Velankar, M M, Nathwani, B N, Schlutz, M J, Bain, L A, Arber, D A, Slovak, M L, Weiss, L M
• Format: Journal Article
• Language: English
• Published: United States 01.08.1999
• Subjects: Adult; DNA, Neoplasm - analysis; Flow Cytometry; Gene Rearrangement; Humans; Immunohistochemistry; Immunophenotyping; Lymphoma, Non-Hodgkin - genetics; Lymphoma, Non-Hodgkin - immunology; Lymphoma, Non-Hodgkin - pathology; Male; Precursor Cell Lymphoblastic Leukemia-Lymphoma - genetics; Precursor Cell Lymphoblastic Leukemia-Lymphoma - immunology; Precursor Cell Lymphoblastic Leukemia-Lymphoma - pathology; Receptors, Antigen - genetics; T-Lymphocytes - metabolism; T-Lymphocytes - pathology
• ISSN: 0147-5185
• DOI: 10.1097/00000478-199908000-00017

T-lymphoblastic lymphoma is a high-grade malignant lymphoma. Clinically indolent T-lymphoblastic proliferations have not been described. We present a case report of an indolent T-cell lymphoblastic proliferation studied by histopathology, immunohistochemistry, flow cytometry, antigen receptor gene rearrangement studies, and cytogenetics. The patient had recurrent masses in the upper aerodigestive tract over a 16-year period, was treated by multiple surgical excisions, and never received either chemotherapy or radiotherapy. A proliferation of lymphoblasts was present histologically. The cells were positive for terminal deoxynucleotidyl transferase, CD1, and CD3, and coexpressed CD4 and CD8. No clonal rearrangements of the T-cell receptor beta or gamma chain genes were identified. Cytogenetic studies revealed a questionable inversion of the short arm of chromosome 9, affecting the 9p21-22 region. Although ectopic thymic tissue was considered, the case was considered to be an indolent lymphoblastic proliferation. It should be recognized that rare lymphoblastic proliferations may not behave in a high grade fashion as typically seen in T-lymphoblastic lymphoma.