Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases

Histopathology - Tập 41 Số 1 - Trang 1-29 - 2002
Stefano Pileri1, Thomas M. Grogan2, Nancy L. Harris3, Peter M. Banks4, Elı́as Campo5, John K. Chan6, Riccardo Dalla Favera7, Georges Delsol8, Christiane De Wolf‐Peeters9, Brunangelo Falini10, R. D. Gascoyne11, Philippe Gaulard12, Kevin C. Gatter13, Peter G. Isaacson14, Elaine S. Jaffe15, Philip M. Kluin16, Daniel M. Knowles17, Miguel Á. Piris13, Shunsuke Mori18, Hans Konrad Müller‐Hermelink19, Elisabeth Ralfkiær20, Harald Stein21, Ih‐Jen Su22, Roger A. Warnke23, Lawrence M. Weiss24
1Service of Pathologic Anatomy and Hematopathology, Bologna University, Italy,
2Department of Pathology, University of Arizona, Tucson, AZ USA
3Department of Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA USA
4Hematopathology, Carolinas Medical Center, Charlotte, NC, USA,
5Service of Pathologic Anatomy, Clinic Hospital, Barcelona, Spain,
6Department of Pathology, Queen Elizabeth Hospital, Hong Kong, S. A. R. China.
7Department of Pathology, Columbia University, New York, USA
8Central Laboratory of Pathologic Anatomy, Toulouse University, Toulouse, France,
9Department of Pathology, Leuven University, Leuven, Belgium
10Institute of Haematology, University of Perugia, Perugia, Italy
11British Columbia Cancer Agency, Vancouver, Canada
12Department of Pathology Henri Mondor Hospital, Creteil, France
13Department of Cellular Science, Oxford University, Oxford, UK,
14Department of Histopathology, University College London, London, UK
15National Cancer Institute, Bethesda, MD USA
16Stem Cell Aging Leukemia and Lymphoma (SALL)
17Department of Pathology, Cornell University, New York, USA
18Department of Pathology, Tokyo University, Tokyo, Japan
19Department of Pathology, Würzburg University, Würzburg, Germany,
20Department of Pathology, Copenhagen University, Herlev, Denmark,
21Institute of Pathology, Benjamin Franklin University, Berlin, Germany,
22Department of Pathology, National Cheng Kung University Hospital, Taiwan, ROC.
23Department of Pathology, Stanford University, Stanford, USA
24Division of Pathology, City of Hope National Medical Center, Duarte, CA, USA

Tóm tắt

Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases

Neoplasms of histiocytes and dendritic cells are rare, and their phenotypic and biological definition is incomplete. Seeking to identify antigens detectable in paraffin‐embedded sections that might allow a more complete, rational immunophenotypic classification of histiocytic/dendritic cell neoplasms, the International Lymphoma Study Group (ILSG) stained 61 tumours of suspected histiocytic/dendritic cell type with a panel of 15 antibodies including those reactive with histiocytes (CD68, lysozyme (LYS)), Langerhans cells (CD1a), follicular dendritic cells (FDC: CD21, CD35) and S100 protein. This analysis revealed that 57 cases (93%) fit into four major immunophenotypic groups (one histiocytic and three dendritic cell types) utilizing six markers: CD68, LYS, CD1a, S100, CD21, and CD35. The four (7%) unclassified cases were further classifiable into the above four groups using additional morphological and ultrastructural features. The four groups then included: (i) histiocytic sarcoma (n=18) with the following phenotype: CD68 (100%), LYS (94%), CD1a (0%), S100 (33%), CD21/35 (0%). The median age was 46 years. Presentation was predominantly extranodal (72%) with high mortality (58% dead of disease (DOD)). Three had systemic involvement consistent with `malignant histiocytosis'; (ii) Langerhans cell tumour (LCT) (n=26) which expressed: CD68 (96%), LYS (42%), CD1a (100%), S100 (100%), CD21/35 (0%). There were two morphological variants: cytologically typical (n=17) designated LCT; and cytologically malignant (n=9) designated Langerhans cell sarcoma (LCS). The LCS were often not easily recognized morphologically as LC‐derived, but were diagnosed based on CD1a staining. LCT and LCS differed in median age (33 versus 41 years), male:female ratio (3.7:1 versus 1:2), and death rate (31% versus 50% DOD). Four LCT patients had systemic involvement typical of Letterer–Siwe disease; (iii) follicular dendritic cell tumour/sarcoma (FDCT) (n=13) which expressed: CD68 (54%), LYS (8%), CD1a (0%), S100 (16%), FDC markers CD21/35 (100%), EMA (40%). These patients were adults (median age 65 years) with predominantly localized nodal disease (75%) and low mortality (9% DOD); (iv) interdigitating dendritic cell tumour/sarcoma (IDCT) (n=4) which expressed: CD68 (50%), LYS (25%), CD1a (0%), S100 (100%), CD21/35 (0%). The patients were adults (median 71 years) with localized nodal disease (75%) without mortality (0% DOD). In conclusion, definitive immunophenotypic classification of histiocytic and accessory cell neoplasms into four categories was possible in 93% of the cases using six antigens detected in paraffin‐embedded sections. Exceptional cases (7%) were resolvable when added morphological and ultrastructural features were considered. We propose a classification combining immunophenotype and morphology with five categories, including Langerhans cell sarcoma. This simplified scheme is practical for everyday diagnostic use and should provide a framework for additional investigation of these unusual neoplasms.

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