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© 2002 American Society for Clinical Oncology
Skin Lesions in Melanoma and Kaposi’s SarcomaCase 3. Familial Classic Mediterranean Kaposi's SarcomaGeisinger Medical Center, Danville, PA An 82-year-old white man of Syrian descent presented with purplish raised skin lesions involving both feet. The skin lesions on the left foot preceded the occurrence of lesions on the right foot by 4 months and were associated with severe edema of the foot (Fig 1). The lesions progressed gradually and spread to the trunk, involving the right flank and left lateral chest wall. His 35-year-old son had similar purple-blue skin lesions localized to the face that were histologically proven to be Kaposi’s sarcoma. The son’s serum human immunodeficiency virus (HIV) antibodies were negative. The facial skin lesions had a complete response to intralesional interferon therapy, and this patient remains in complete remission after 3 years.
Examination of our elderly patient showed that the skin lesions had irregular borders, were slightly raised, and were nontender to palpation. The rest of the physical examination showed left inguinal lymphadenopathy and edema of the left leg. The biopsy of one of the skin lesions revealed irregular vascular spaces lined by endothelial cells with slightly enlarged and hyperchromatic nuclei (Fig 2). Between the vascular spaces, there were aggregates of spindle cells, extravasated erythrocytes, and hemosiderin pigment (Fig 2, arrows; hematoxylin and eosin, x 100). These features are characteristic of Kaposi’s sarcoma. Serum antibodies to HIV-1, HIV-2, and human T-cell leukemia virus-1 were not present. Computed tomography scans of the chest, abdomen, and pelvis did not reveal visceral metastases. The disease was extensive and not amenable to local treatment with surgery or radiation or to topical treatment with cryotherapy, liquid nitrogen, or intralesional vinblastine. This prompted the initiation of paclitaxel as an antiangiogenic agent at 60 mg/m2 administered for 3 consecutive weeks followed by 1 week of rest. After 12 weeks of therapy, the lesions showed significant improvement, with flattening of surface, decrease in size, and fading of color. The edema of the left leg also showed significant improvement (Fig 3). The lesions continue to improve after 5 months of paclitaxel therapy (Fig 4).
The classic form of Kaposi’s sarcoma is rare and is seen primarily in older men of Eastern European and Mediterranean origin. The lesions typically appear initially on the hands and feet and gradually progress up the arms and legs. Lymphedema usually follows the development of skin lesions, but it may precede them. If untreated, the disease can spread to involve the viscera or mucosa. Familial classic Kaposi’s sarcoma is exceedingly rare. Our case adds to the few documented cases reported in the English literature (Table 1).1-6
Single lesions can be treated with excisional biopsy, and patients with a few lesions in a limited area can be treated with radiation therapy. Patients with extensive disease can be treated with chemotherapy including vinblastine, doxorubicin, and dacarbazine alone or in combination, but it has limited efficacy.7 Paclitaxel has antiangiogenic and apoptotic effects.8 Its antitumor activity has been reported in AIDS-associated Kaposi’s sarcoma,9,10 but there are limited data describing efficacy in classic Kaposi’s sarcoma, an unusual form of hemangiosarcoma. We have previously reported paclitaxel to be an active agent in the treatment of angiosarcoma of the scalp and face, a hemangiosarcoma similar to Kaposi’s sarcoma.11 This case reveals that paclitaxel should be considered an appropriate therapy with good palliative effects in the treatment of classic Kaposi’s sarcoma. REFERENCES 1. McGinn JT, Ricca JJ, Currin JF: Kaposi’s sarcoma following allergic angiitis. Ann Intern Med 42: 921-927, 1955 2. Zeligman I: Kaposi’s sarcoma in a father and son. Bull Johns Hopkins Hosp 107: 208-212, 1960[Medline] 3. Epstein E: Kaposi’s sarcoma and parapsoriasis en plaque in brothers. JAMA 219: 1477-1478, 1972 (letter) 4. Brownstein MH, Shapiro L, Skolnik P: Kaposi’s sarcoma in community practice. Arch Dermatol 107: 137-138, 1973 (letter) 5. Digiovanna J, Safai B: Kaposi’s sarcoma: Retrospective study of 90 cases with particular emphasis on the familial occurrence, ethnic background and prevalence of other diseases. Am J Med 71: 779-783, 1981[CrossRef][Medline] 6. Perniciaro C, Gross DJ, White JW, et al: Familial Kaposi’s sarcoma. Cutis 57: 220-2222, 1996[Medline]
7.
Antman K, Yuan C: Medical progress: Kaposi’s sarcoma. N Engl J Med 342: 1027-1038, 2000 8. Belotti N, Vergani V, Drudis T: The microtubule-affecting drug paclitaxel has anti-angiogenic activity. Clin Cancer Res 2: 1843-1849, 1996[Abstract] 9. Welles L, Saville W, Lietzau J, et al: Phase II trial with dose titration of paclitaxel for the therapy of human immunodeficiency virus-associated Kaposi’s sarcoma. J Clin Oncol 16: 1112-1121, 1998[Abstract]
10.
Gill P, Tulpule A, Byron E, et al: Paclitaxel is safe and effective in the treatment of advanced AIDS-related Kaposi’s sarcoma. J Clin Oncol 17: 1876-1883, 1999 11. Fata F, O’Reilly E, Ilson D, et al: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 86: 2034-2037, 1999[CrossRef][Medline]
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Copyright © 2002 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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