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Histiocytic and dendritic cell neoplasms are rare hematological malignancies derived from histiocytes (macrophages) and dendritic cells. These neoplasms are part of the mononuclear phagocyte system and exhibit diverse clinical presentations and biological behaviors. Here's an overview: Types of Neoplasms Histiocytic Neoplasms: Histiocytic Sarcoma: A malignant tumor of histiocytes, often presenting in lymph nodes, skin, or other organs. Hemophagocytic Lymphohistiocytosis (HLH): A severe inflammatory condition involving histiocytes and T-cells. Dendritic Cell Neoplasms: Langerhans Cell Histiocytosis (LCH): Characterized by abnormal proliferation of Langerhans cells, affecting bones, skin, and other organs. Follicular Dendritic Cell Sarcoma: A rare tumor arising from follicular dendritic cells, typically found in lymph nodes. Interdigitating Dendritic Cell Sarcoma: A rare malignancy of interdigitating dendritic cells. Clinical Features Symptoms vary depending on the type and location of the neoplasm and may include: Swelling of lymph nodes. Skin lesions. Bone pain or fractures. Systemic symptoms like fever, weight loss, and fatigue. Diagnosis Histopathology: Examination of tissue samples to identify cellular morphology. Immunohistochemistry: Identifies specific markers to differentiate histiocytic and dendritic cell neoplasms. Molecular Studies: Detect genetic mutations or abnormalities. Treatment Localized Disease: Surgical resection is often the first-line treatment. Disseminated Disease: Chemotherapy, radiation therapy, or targeted therapies may be used. Novel Therapies: Immunotherapy and molecular-targeted treatments are being explored. #Neoplasm