Article

ALK-Positive Anaplastic Large Cell Lymphoma Mimicking Nodular Sclerosis Hodgkin??s Lymphoma

University of Barcelona, Barcino, Catalonia, Spain
American Journal of Surgical Pathology (Impact Factor: 5.15). 03/2006; 30(2):223-9. DOI: 10.1097/01.pas.0000179123.66748.c2
Source: PubMed

ABSTRACT

Anaplastic large cell lymphoma (ALCL) and Hodgkin lymphoma (HL) are recognized as biologically distinct entities. However, occasionally, these two entities may share some morphologic features responsible for diagnostic difficulties. In the last 10 years, we have collected 380 cases of ALK-positive ALCL of which 10 cases were originally diagnosed as nodular sclerosis classic HL (NSHL) on conventional histopathological examination. After immunostaining, these cases proved to be ALK-positive ALCL mimicking HL (so-called Hodgkin-like ALCL). These cases account for 2.6% of our cases of ALK-positive ALCL (10 of 380 cases). Median age was 11 years (3-92 years) with a female predominance (male/female ratio, 3:7). Characteristically, these lesions showed thick nodular fibrosing bands highly suggestive of NSHL. Neoplastic cells were scarce in 6 cases, whereas in the 4 remaining cases, sheets of tumor cells were also present. A perivascular and a sinusoidal growth pattern was observed in various degrees in all cases. Few binucleated Reed-Sternberg-like cells were present in every case in a background of small lymphocytes. Inflammatory cells (ie, granulocytes, eosinophils, and histiocytes) were rare. Neoplastic cells were positive for CD30 (10 of 10 cases), ALK protein (10 of 10 cases), epithelial membrane antigen (EMA) (9 of 9 cases), CD43 (6 of 9 cases), and perforin (8 of 8 cases), but negative for CD15 (10 of 10 cases), CD20 (10 of 10 cases), Pax5/BSAP (6 of 6 cases), and EBV (8 of 8 cases). In addition, in 7 cases, neoplastic cells were of T-phenotype, whereas the 3 remaining cases were considered to be of null/undetermined phenotype. Although rare, Hodgkin-like ALCL may mimic NSHL, and it is advisable to include EMA in the first line panel and to ask for ALK staining in EMA-positive, CD15-negative lesions with morphologic features suggestive of NSHL.

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    • "Finally, the so-called ALCL of the Hodgkinlike type deserves special attention [24]. It was originally described as a form of the tumor, presenting in young people with a bulky mediastinal mass and consisting of anaplastic cells arranged in nodules surrounded by sclerotic bands, as seen in nodular sclerosing Hodgkin's lymphoma (NSHL) [24]. Following the introduction of the REAL Classification [5], which regarded it as a provisional entity, such diagnosis was by no means also applied to cases of aggressive HL that could not be easily differentiated from ALCL, both on morphologic and on phenotypic grounds [5]. "
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    ABSTRACT: The authors revise the concept of anaplastic large cell lymphoma (ALCL) in the light of the recently updated WHO classification of Tumors of Hematopoietic and Lymphoid Tissues both on biological and clinical grounds. The main histological findings are illustrated with special reference to the cytological spectrum that is indeed characteristic of the tumor. The phenotype is reported in detail: the expression of the ALK protein as well as the chromosomal abnormalities is discussed with their potential pathogenetic implications. The clinical features of ALCL are presented by underlining the difference in terms of response to therapy and survival between the ALK-positive and ALK-negative forms. Finally, the biological rationale for potential innovative targeted therapies is presented.
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    ABSTRACT: Rezumat: Limfoamele maligne nonhodgkin (LMNH) reprezintă un grup heterogen de tumori monoclonale al celulelor sistemului imunitar (limfocite şi histiocite). LMNH se împart din punct de vedere fenotipic în limfoame de tip T şi de tip B. LMNH de tip T sunt recunoscute doar de 3 decenii. Au o incidenţă de 12-15% şi reprezintă un grup heterogen din punct de vedere clinic, evolutiv şi histofenotipic ceea ce ridică numeroase probleme de diagnostic şi de conduită terapeutică. Abstract: Nonhodgkin malignant lymphomas (NHL) represent a heterogeneous group of immune system cells-monoclonal tumors (lymphocytes and histiocytes). NHL can be divided phenotypically in T cell lymphomas and B cell lymphomas. T cell NHL are recognized only by three decades. They have an incidence of 12-15% and represents a heterogeneous group from clinical, evolutional and hystophenotypical point of view, which raises many problems of diagnosis and course of treatment.
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    ABSTRACT: Nonhodgkin malignant lymphomas (NHL) represent a heterogeneous group of immune system cells-monoclonal tumors (lymphocytes and histiocytes). NHL can be divided phenotypically in T cell lymphomas and B cell lymphomas. T cell NHL are recognized only by three decades. They have an incidence of 12-15% and represent a heterogeneous group from clinical, evolutional and hystophenotypical point of view, which raises many problems of diagnosis and therapy. Rezumat: Limfoamele maligne nonhodgkin (LMNH) reprezintă un grup heterogen de tumori monoclonale al celulelor sistemului imunitar (limfocite şi histiocite). LMNH se împart din punct de vedere fenotipic în limfoame de tip T şi de tip B. LMNH de tip T sunt recunoscute doar de 3 decenii. Au o incidenţă de 12-15% şi reprezintă un grup heterogen din punct de vedere clinic, evolutiv şi histofenotipic ceea ce ridică numeroase probleme de diagnostic şi de conduită terapeutică.
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