The subtypes of<unk>  reflect the number of lymph node regions with enlarged lymph nodes and known causes of the<unk> . In unicentric<unk> , one or more enlarged lymph nodes are present in a single region of lymph nodes. In multicentric<unk> , enlarged lymph nodes are present in multiple regions of lymph nodes. The only known cause of<unk>  is uncontrolled infection with human herpesvirus 8, which has only been reported in cases of multicentric<unk> . There are three established subtypes of<unk> . Unicentric<unk>  In unicentric<unk>  (UCD) one or more enlarged lymph nodes are present in a single region of lymph nodes. The cause of UCD is unknown. It is the most common subtype of<unk>  and compared to HHV-8-associated MCD and iMCD, symptoms are typically milder and relate to the lymph node affected, and organ dysfunction is uncommon. Surgical removal of enlarged lymph nodes is the treatment of choice, but in cases that resection is not an option, treatment is similar to iMCD cases. Idiopathic multicentric<unk>  In idiopathic multicentric<unk>  (iMCD), enlarged lymph nodes are present in multiple lymph node regions and no known cause for the<unk>  is identified. It is less common than unicentric<unk>  (UCD) and compared to UCD, symptoms are typically more severe, laboratory abnormalities must be present for diagnosis, and medications are used for treatment as surgery is ineffective. Symptoms include systemic inflammatory and organ dysfunction. Medications used for treatment include antivirals, cytotoxic chemotherapy and rituximab. Two great challenges in the diagnosis and treatment of iMCD are the spectrum of nonspecific symptoms and the unclear etiology of the<unk> . Human herpesvirus 8 associated multicentric<unk>  In human herpesvirus 8 associated multicentric Castelman<unk>  (HHV-8-associated MCD), enlarged lymph nodes are present in multiple lymph node regions and infection with human herpesvirus 8 is present. It is less common than unicentric<unk>  and diagnosed most frequently in patients infected with human immunodeficiency virus (HIV). Compared to UCD and iMCD, HHV-8-associated MCD presents with similar symptoms and clinical findings to iMCD. While UCD is readily treatable with surgery, HHV-8-associated MCD, like iMCD, is treated with medications as surgery is ineffective. TAFRO Syndrome: a constellation of clinical symptoms reported in some iMCD and HHV-8 associated MCD patients. The clinical symptoms are Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, and Organomegaly. These patients respond well to immunosuppressive therapy and steroids.
Castleman disease
Kaposi's sarcoma
Ewing's sarcoma
Histiocytoma
Familial myxovascular fibromas
Bartholin's cyst
Angiomatoid fibrous histiocytoma
Progressive nodular histiocytoma