Abstract
Histiocytic and dendritic neoplasms are rare neoplasms that comprise several distinct entities. Histiocytic sarcoma, Langerhans cell histiocytosis (LCH) and follicular dendritic cell sarcoma are discussed in more details. Histiocytic sarcoma is a rare malignant neoplasm derived from histiocytes that commonly involves extranodal sites (skin, gastrointestinal tract), but can also involve lymph nodes. Histiocytic sarcoma is generally an aggressive neoplasm with poor prognosis; some localized cases may have a better prognosis. Langerhans cell histiocytosisis a neoplastic process of Langerhans-type cells. It most commonly occurs in children under the age of 5 years with male predominance. Clinically, LCH can occur in one site (most commonly bone and soft tissue, lung, etc.), multiple sites within the same organ system (usually bone), or it can involve multiple systems (disseminated form). Follicular dendritic cell (FDC) sarcoma is a rare malignant neoplasm that originates from follicular dendritic cells. This tumor occurs in young to middle-aged adults who usually present with slowly enlarging lymph nodes. Follicular dendritic cell sarcoma will recur in up to 50% of patients and subset of patients will have metastatic disease.
Published Version
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