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

Department of Pathology, University of Southern California School of Medicine, USA.
American Journal of Surgical Pathology (Impact Factor: 5.15). 09/1999; 23(8):977-81. DOI: 10.1097/00000478-199908000-00017
Source: PubMed


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.

6 Reads
  • Source
    • "Indolent proliferations of benign T-lymphoblasts have been described in various conditions and body sites [16], [17], [18], [19], [20]. These proliferations often involve the oropharynx [19], [20], the salivary glands [16] and tissues known to harbor hemolymphopoiesis during fetal development (liver, mesentery) [18], [21]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Adenoidal tissue (also known as nasopharyngeal tonsils) of 58% of humans in the pediatric age group contains immature T-lymphoid cells with the phenotype of thymocytes (TdT+,CD1abc+, cytoplasmic CD3+, coexpressing CD4 and CD8, lacking an Intraepithelial Lymphocyte-associated phenotype). The notable difference in comparison to palatine tonsils is the clustering in groups and sheets, comprising hundreds or thousands of cells (1.7%±0.2 of total T cells). The thymic epithelium is morphologically and phenotypically absent. Adenoids share with tonsils and lymph nodes the presence of immature B cell precursors (TdT+, CD1a-, Pax5+, Surrogate light chain±), however in these latter the presence of TdT+, CD1a+, Pax5- precursors is absent or limited to individual cells. Human adenoids are distinct among the Waldeyer's ring lymphoid tissue because of the known embryogenic derivation from the third pharyngeal pouch, from which the thymus develops; in addition, they may display phenotypic incomplete features of a vestigial thymus.
    PLoS ONE 05/2014; 9(5):e98222. DOI:10.1371/journal.pone.0098222 · 3.23 Impact Factor
  • Source
    • "They suggested that their patient had indolent T-lymphoblastic proliferation combined with hepatocellular carcinoma (15). Indolent T-lymphoblastic proliferation was first described by Velankar et al. in 1999 in the upper aerodigestive tract (16). Two years later Strauchen reported a similar phenomenon in the oropharynx in a myasthenia gravis patient (17). "
    [Show abstract] [Hide abstract]
    ABSTRACT: Indolent T-lymphoblastic proliferation has been rarely reported in the upper aerodigestive tract. The lymphoid cells associated with this condition have the morphological and phenotypical features of immature thymocytes. However, their pathogenesis and biology are unknown. We present an unusual type of tumor infiltrating lymphocytes in a case with hepatocellular carcinoma, presumed to be a T-lymphoblastic proliferation. A 58-yr-old female patient presented with indigestion and a palpable epigastric mass. The abdominal computed tomography revealed a mass in the S6 region of the liver. A hepatic segmentectomy was performed. Microscopic examination showed dense isolated nests of monomorphic lymphoid cells within the tumor. Immunohistochemically, the lymphoid cells were positive for CD3, terminal deoxymucleotide transferase (TdT) and CD1a. In addition, they showed dual expression of CD4 and CD8. The polymerase chain reaction used to examine the T-cell antigen receptor gamma gene rearrangement showed polyclonal T-cell proliferation. This is the second case of hepatocellular carcinoma combined with indolent T-lymphoblastic proliferation identified by an unusual tumor infiltrating lymphocytes.
    Journal of Korean medical science 02/2010; 25(2):309-12. DOI:10.3346/jkms.2010.25.2.309 · 1.27 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: T-lymphoblastic lymphoma is a high-grade malignant lymphoma with frequent occurrence in young males, mediastinal involvement, and systemic dissemination. Indolent T-lymphoblastic proliferations have rarely been recognized. In the present case, we report on an indolent T-lymphoblastic proliferation involving the oropharynx in a patient with myasthenia gravis with multiple local recurrences over an 11-year period without evidence of systemic dissemination. The T-lymphoblasts were consistently positive for terminal deoxynucleotidyl transferase (TdT), CD1, CD3, CD4, and CD8, corresponding to an intermediate thymocyte stage of differentiation. No cytokeratin-positive thymic epithelial cells were identified, ruling out an ectopic thymus or thymoma. T-receptor gene rearrangement studies by Southern blot revealed no monoclonal CT-beta rearrangement. Indolent T-lymphoblastic proliferations of undetermined clonality may rarely occur; predilection for involvement of oropharynx and possible association with myasthenia gravis are suggested.
    American Journal of Surgical Pathology 03/2001; 25(3):411-415. DOI:10.1097/00000478-200103000-00018 · 5.15 Impact Factor
Show more