Abstract

Exceptional malignancies of the lymph nodes or soft tissue comprising of < 1% of the tumour incidence are the Histiocytic and Dendritic cell neoplasm. A definitive appearance/biology/ Hematology/ histopathology / exclusive therapeutic options describe the condition. Morphology and Immune reactive appraisal may be mandated to distinguish the neoplasm. Preface: Histiocytic and Dendritic neoplasms are infrequent disorders which incriminate the accessory immune system or mesenchymal cells. Subject to the origin of the neoplasm, from the bone marrow precursors or the mesenchye, the lesions may be categorized as. i) The Histiocytic sarcoma (HS), Langerhans cell histiocytosis(LCH) and the Inter-digitizing dendritic cell sarcoma (IDCS) may commence from the bone marrow precursors and ii) The Follicular dendritic cell sarcoma (FDCS), Intermediate dendritic cell sarcoma (INDCS), Fibroblastic reticular cell tumour (FRCT) and Disseminated juvenile xanthogranuloma(DJX) may emanate from the Stromal or the mesenchymal cell. Divergent differentiation of the bone marrow precursors is a usual manifestation, though hybrid or trans-differentiating malignant lymphoid clones may also materialize in Inter-digitizing dendritic cell sarcoma (IDCS), Histolytic sarcoma (HS) and Follicular dendritic cell sarcoma (FDCS). Together, Histolytic and Dendritic cell tumors constitute < 1% of the lymph node or soft tissue neoplasm. The tumors may be misinterpreted as Non Hodgkin’s Lymphoma or adjunct Lymph-Proliferative disorders. The rare malignancies may be a challenge to discern and medicate. Debatable Histolytic and Dendritic neoplasm mandate a referral. The disorder may be ascertained by the combined analysis on Histology and Immunehistochemistry

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