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Case report: Classic Kaposi’s sarcomaClassic KS usually appears between ages 50 and 70 years and has a relatively benign, indolent course for 15 years or longer. Treatment usually controls the disease quite well.Ellen Joffe, PA-C; Louis D. Dubilier, MDEllen Joffe works in a surgical practice in Louisville, Ky. Louis Dubilier is Clinical Assistant Professor of Pathology at the University of Kentucky, Lexington, and a pathologist in private practice. The authors have indicated no relationships to disclose relating to the content of this article.
A surgeon considered the possibility of a vascular problem, but a venogram showed no problems with the veins of the legs. A nodule that developed on the medial aspect of the left knee was thought to be a lymph node enlarging because of lymph drainage from the leg. This nodule was spongy and changed in size with pressure; after a biopsy, it was diagnosed as a lymph node containing a vascular proliferation compatible with angioma. The surgeon recommended gradient compression stockings but had no explanation for the ecchymosis or edema. By the time 2 years had passed since the dropping of the paint can, the lesions had progressed significantly, and the surgeon advised the patient to see a dermatologist. The dermatologist made a tentative diagnosis of classic Kaposi’s sarcoma (KS) on clinical grounds, and this diagnosis was supported by biopsy. The legs were treated from the knees downward with radiation administered over a 4-week period. To ensure treatment of all the lesions, which included those between the toes, the patient’s legs were submerged to midthigh in water in a fish tank, and the entire tank of water was irradiated. The lesions responded quite well to this treatment; within several weeks, they had essentially disappeared, except for discolored, residual old hemorrhage in the former purplish areas. The swelling also decreased significantly, although it did not disappear completely. Then about 8 years after the patient’s last treatment, new, small, nonblanching purplish areas, accompanied by some nodularity, appeared on the lateral aspect of his left foot. A biopsy again showed changes consistent with KS. The lesions were spot treated with additional radiation therapy using the same protocol as before. As happened the first time, the lesions regressed somewhat but not completely. Some residual nodularity and tenderness remained. Since then, however, the lesions have not progressed. Kaposi’s sarcomaKS has four types: epidemic, immunosuppressive treatment-related, African, and classic.1-4 The epidemic form surfaced in association with the AIDS epidemic and occurs predominantly in homosexual or bisexual men or heterosexual IV drug users. The immunologic deficiencies of AIDS predispose the host to a variety of opportunistic infections and unusual neoplasms, of which KS is one. Epidemic KS is characterized by multifocal, widespread lesions at the onset of the illness. Eventually in almost all patients disseminated disease develops, with progression proceeding from a few localized cutaneous or mucocutaneous lesions to more widespread skin lesions and involvement of the lymph nodes, GI tract, and other organs. Immunosuppressive treatment-related KS occurs in recipients of organ transplants who take prednisone and azathioprine (Imuran) to prevent rejection. Lesions of this type of KS often remain localized to the skin. Widespread dissemination with mucocutaneous or visceral organ involvement is uncommon. Some of these KS tumors have regressed after reduction or changes in immunosuppressive therapy. African KS is endemic in the native population in equatorial Africa and comprises approximately 9% of all cancers seen in Ugandan males.5 This form can be either indolent and identical to the classic form or aggressive with deep invasion. Patients with African KS are significantly younger than those with classic KS. A lymphadenopathic form of this disease occurs primarily in prepubescent children and is frequently associated with visceral organ involvement. The prognosis in this form of the disease is very poor, with nearly all patients dying within 3 years.5 Classic KS is usually seen in older, heterosexual, HIV-negative patients of Jewish, Mediterranean, or Italian descent. The predominance of the disease is overwhelmingly male, with a male to female ratio of approximately 10 to 15:1. Classic KS was first described in 1872 by Moritz Kaposi, a Hungarian dermatologist. Until the AIDS epidemic, KS had remained a relatively rare disease, but in Kaposi’s description of his original patients, their disease looked more like the KS seen in AIDS than the KS expected in elderly men of Jewish, Italian, and Mediterranean lineage. In this latter group, the disease is usually indolent, but Kaposi’s patients died within 2 to 3 years of diagnosis. The usual onset of classic KS is at 50 to 70 years of age. It most often has a relatively benign, indolent course for 15 or more years, with slow enlargement of the original tumors and gradual development of additional lesions. Patients usually present with one or more asymptomatic red, purple, or brown patches and plaques or nodular skin lesions localized to one or both lower extremities and especially involving ankles and soles. The most common complications are venous stasis and lymphedema of the involved lower extremity. In patients with longstanding disease, lesions can develop in the GI tract, lymph nodes, and other organs, but generally these are asymptomatic and not discovered until autopsy. Occasionally, patients may have GI bleeding. Up to one third of patients with classic KS develop a secondary primary malignancy, most often non-Hodgkin’s lymphoma.6 Patients with classic KS are expected to die with the disease rather than of the disease. Pathology and etiologyKS is characterized microscopically by an excessive proliferation of spindled cells, generally without identifiable vascular channels but with slits containing RBCs and hemosiderin between the cells. The spindled cells are thought to be of endothelial origin, probably from the lining of lymphatic tissue. Human herpes virus 8 has been identified in more than 90% of all types of KS lesions, suggesting that this virus has a causative role.7 Differential diagnosisThe differential diagnosis of KS includes ecchymoses secondary to hemorrhagic disorders, melanocytic nevi, nodular melanoma, pyogenic granuloma,8 hemangiomas, and malignant melanoma. An important characteristic of KS lesions is that they do not blanch with pressure, which is useful in distinguishing them from hemangiomas. Ecchymoses will change color and usually disappear with time. A biopsy is required to make the definitive diagnosis. TreatmentThere are several treatment options for classic KS. For solitary lesions, surgical excision is possible. Cryotherapy may be used for cosmetic clearance in patients with small superficial lesions, but local recurrence is likely. In radiation therapy, usually superficial radiation beams are used, but here also disease recurrence in adjacent untreated skin is said to be common. Cure rates are better when extended-field radiation is used.9,10 The recommended treatment is electron beam therapy, 400 cGy once weekly for 6 to 8 consecutive weeks, using four to six MeV electron beams with ports that cover the entire skin surface 15 cm above the lesion. For widespread skin disease, modest doses are effective in controlling the disease. The choice of photon or electron radiation and the fields used must be tailored to suit the distribution of the disease in the individual patient. Chemotherapy using various agents has been used infrequently in patients who were initially treated with radiation therapy.11,12 Almost all the patients had a good to excellent response to chemotherapy, but they required prolonged courses lasting up to several years to maintain a partial response. The prognosis for the patient with recurring, progressing, or relapsing disease is highly variable. Decisions about further treatment depend on many factors, including the clinical setting and the overall health and concerns of the patient. REFERENCES
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The patient was a 58-year-old white Jewish man. Ten years previously, he dropped a can of paint onto his right foot proximal to the toes and subsequently noted the development of ecchymosis in that area. The ecchymotic area did not heal. Swelling then developed below both knees and was associated with the appearance of additional purplish areas, first on the feet and then progressing, over a period of 18 months, to the ankles and legs. Some of these purplish areas became confluent into large flat patches on the soles (see the photographs above). The lesions were not painful and did not blanch. The man reported no other pertinent historical, physical, or laboratory findings. His HIV antibody test result was negative.