Histiocytic malignanices are very rare cancers that arise from histiocytes, dendritic cells, and Langerhans
cells. These tumors are highly malignant and rapidly progressive. Because the cancers
are so rare, their therapy is not standardized, and tends to be only minimally to
moderately effective. The tumors most commonly present with lesions in soft tissue
and skin but rapidly advance to involve lymph nodes and organ tissues. These cancers
present in middle-aged and older adults and are very rarely seen in children.
Types of histiocytic malignancies include:
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Langerhans cell sarcomas (LCS);
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Interdigitating dendritic cell sarcomas (IDCS);
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Histiocytic sarcomas (the most common form of histiocytic malignancy); and
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Follicular dendritic cell sarcoma (FDCS).
LCS is an extremely aggressive high-grade tumor that can occur without a known cause.
It can also progress from Langerhans Cell Histiocytosis. These tumors usually involve
the skin and underlying soft tissue. Although rare, it may also involve the lungs,
liver, spleen, and bone. LCS can occur at any age but is most commonly diagnosed in
adults over 45 years of age.
IDCS arises from antigen presenting cells. It mostly affects the lymph nodes. Although
rare, it can also occur in skin and soft tissue. IDCS can occur at any age but is
most commonly diagnosed in adult males over 60 years of age.
Histiocytic sarcoma is an aggressive cancer with an unknown cause. Tumors commonly
occur in the intestinal tract, skin, and soft tissue. Histiocytic sarcoma can occur
at any age but are most common in adults over 45 years old.
FDCS is cancer with painless, slow-growing tumors that most commonly occur in the
lymph nodes (most often cervical), extranodal sites (such as tonsils, gastrointestinal
tract, soft tissue, mediastinum, or lung, among others), or both. FDCS can occur at
any age and is most common in adults aged 50 years and older.
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