KAPOSI'S SARCOMA 

Pietro Sollena, Ketty Peris

Institute of Dermatology, Catholic University, Rome, Italy

A 83-year-old man presented with asymptomatic nodules on the upper extremities. Physical examination showed multiple red to purple plaques and nodules located on the right hand and forearm (Panels A-C).

Panel A

Panel B

Panel C

The patient reported that oedema and erythema located on the right hand occurred approximately 8 months earlier, followed by the development of itchy and firm plaques and nodules. He had no HIV infection, no contact with animals, aquariums or plants and he was taking no medications. Clinical differential diagnosis included cutaneous lymphoma, Kaposi’s sarcoma, bacillary angiomatosis, epithelioid sarcoma, cutaneous metastasis. An excisional biopsy was performed on a nodule of the forearm. Examination of biopsy specimen showed proliferation of both spindle cells and vessels, with sparse chronic inflammatory cell and extra-vascated red blood cells (Panel D).

Panel D

Treatment with interferon (IFN) alfa, at a dose of 3 million IU, once daily, was administered. After three months of treatment, a partial regression of cutaneous lesions could be observed. The patient is still under IFN treatment, and no adverse events have occurred so far.

Kaposi’s sarcoma (KS) is a rare proliferative disease of the vascular endothelium. Its pathogenesis is correlated to Kaposi sarcoma herpes virus (previously known as Human Herpes Virus type 8), identified in the majority of tumor lesions. On the basis of epidemiological and clinical data, KS is usually classified into four subtypes: Classic, African (endemic), AIDS-associated (epidemic), and Iatrogenic KS. The typical clinical features consist of red to purple/red patches, plaques and nodules, most commonly located on the lower extremities (classic and iatrogenic KS), mucous membranes or visceral organs (endemic and AIDS-associated form). The treatment may vary depending on the KS subtype as well as patient’s characteristics and include surgical excision, radiation therapy, chemotherapy, new target therapy.

References

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Andrei G, Snoeck R. Kaposi's sarcoma-associated herpesvirus: the role of lytic replication in targeted therapy.Curr Opin Infect Dis. 2015 Dec;28(6):611-24. doi: 10.1097/QCO.0000000000000213.

Bhutani M, Polizzotto MN, Uldrick TS, Yarchoan R. Kaposi sarcoma-associated herpesvirus-associated malignancies: epidemiology, pathogenesis, and advances in treatment. Semin Oncol. 2015 Apr;42(2):223-46. doi: 10.1053/j.seminoncol.2014.12.027. Epub 2014 Dec 31.