Kaposi sarcoma (Part 2) : Classic Kaposi sarcoma / Chronic kaposi sarcoma / European kaposi sarcoma

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Kaposi sarcoma (KS) is an angioproliferative disorder that requires infection with human herpes virus 8 (HHV-8), also known as Kaposi sarcoma-associated herpes virus (KSHV), for its development . KS is classified into four types based on the clinical circumstances in which it develops: classic (the type originally described by Kaposi, which typically presents in middle or old age), endemic (several forms described in sub-Saharan indigenous Africans prior to the acquired immunodeficiency syndrome [AIDS] epidemic), iatrogenic (a type associated with immunosuppressive drug therapy, typically seen in renal allograft recipients), and AIDS associated (epidemic KS).

Classic KS occurs most often in older men of Mediterranean or Central/Eastern European ancestry, in whom the lesions usually occur on the distal extremities, particularly the lower legs and feet

Initial lesions may occur in unusual cutaneous locations, however, in the absence of distal extremity involvement

Skin lesions — Classic KS is characterized by the appearance of purplish, reddish blue, or dark brown/black macules, plaques, and nodules on the skin

Extracutaneous involvement — During the course of the disease (rarely initially), mucous membranes of the mouth and gastrointestinal tract, and regional lymph nodes may be affected. Gastrointestinal tract involvement is usually asymptomatic, but bleeding, diarrhea, protein-losing enteropathy, intussusception, and perforation have been reported. In general, gastrointestinal tract/oral mucosal involvement is less common than with acquired immunodeficiency syndrome (AIDS)-related KS, affecting ≤10 percent of patients.

Biopsy — Biopsy is required for definitive diagnosis. In addition to observing typical histologic features on standard microscopy, polymerase chain reaction can be performed on the skin lesions to detect amplified human herpes virus 8 (HHV-8) DNA sequences, and immunohistochemical staining of biopsy specimens can also be performed to detect the presence of HHV-8 latency-associated nuclear antigen (LANA-1) within the spindle cells, thus confirming the diagnosis

Radiographic evaluation — Asymptomatic patients with classic KS rarely require radiographic evaluation of the affected extremity because of the chronic, usually indolent course. Screening for distant organ involvement is not needed due to the low frequency of radiographically evident metastatic disease. When present, mucosal involvement of the gastrointestinal tract is not generally visible on radiographic studies. As noted above, patients with symptoms referable to the gastrointestinal tract should be considered for endoscopy to evaluate for visceral mucosal involvement. In the absence of symptoms, we do not routinely work patients up for visceral disease based on the status of the cutaneous disease.

Staging

In contrast to acquired immunodeficiency syndrome (AIDS)-related KS, there is no commonly used or universally agreed upon staging system for classic KS.
The most recent American Joint Committee on Cancer (AJCC) tumor, node, metastasis (TNM) staging system for soft tissue sarcomas of the extremity and trunk specifically excludes both classic and AIDS-related KS [36,37]. Given the variable natural history of classic KS, one group of investigators used their experience with 300 classic KS patients to derive a proposed staging system based on disease distribution and the clinical pace of progression .

• Stage I (maculonodular stage) – Small macules and nodules primarily confined to the lower extremities.
• Stage II (infiltrative stage) – Plaques mainly involving the lower extremities, sometimes associated with a few nodules.
• Stage III (florid stage) – Multiple angiomatous plaques and nodules involving the lower extremities that are often ulcerated.
• Stage IV (disseminated stage) – Multiple angiomatous nodules and plaques extending beyond the lower extremities.

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