Cutaneous T-cell lymphoma and human immunodeficiency virus. The spectrum broadens.
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Biomedical subjects
Publications and source records attributed to P L Myskowski.
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The immunodeficient state that evolves in persons infected with the human immunodeficiency virus (HIV) appears to increase their risk of certain types of cancer. Among these are primary lymphoma of the central nervous system, undifferentiated non-Hodgkin's lymphoma, squamous cell carcinoma, anorectal carcinoma, and cutaneous malignancies. These malignancies are similar in incidence to those seen in other immunodeficient patients. Lymphoma, in particular, is associated with a more aggressive disease state. In HIV-infected patients, the disease is usually diagnosed at a more advanced stage, frequently has extranodal involvement, and usually responds poorly to chemotherapy. Viruses, such as Epstein-Barr virus and papillomavirus, have been implicated in the pathogenesis of lymphoma and other malignancies in immunosuppressed patients, including those with HIV infection.
The original cutaneous biopsy specimens of 93 patients who presented themselves to the Memorial Sloan-Kettering Cancer Center with acquired immunodeficiency syndrome (AIDS)-related Kaposi's sarcoma (KS) were systematically reviewed for 23 histologic variables. KS was the initial manifestation of AIDS in all of the patients. The vast majority of patients presented with plaque histology of KS. Early lesions of KS were characterized by the presence of dilated vascular spaces haphazardly arranged in the biopsy specimen, a sparse inflammatory cell infiltrate composed of lymphocytes (usually without plasma cells), and aggregates of cuboidal cells with the appearance of epithelioid cells. Individually necrotic tumor cells were present in nearly every case. Spindle cells arranged in fascicles or nodules were seen in a minority of cases. These data provide an overview of the different histologic patterns seen in initial lesions of AIDS-associated KS and may lead to better understanding of the pathogenesis of this tumor.
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Two patients had regression of basal cell carcinoma while receiving systemic chemotherapy for carcinoma of the lung. The chemotherapeutic agents with some efficacy in basal cell carcinoma are reviewed. Based on our patients and data from the literature, cisplatin appears to be a drug of potential usefulness in some patients with this neoplasm.
The records of 187 consecutive patients with acquired immunodeficiency syndrome (AIDS)-associated Kaposi's sarcoma were analyzed retrospectively for a number of prognostic variables. In a multivariate analysis, the initial site of disease was found to be related to survival. Initial lesions on the skin of the lower extremities or in the lymph nodes were associated with longer survival (p = 0.005 and p = 0.01, respectively). Higher helper/suppressor T cell ratios were strongly associated with longer survival (p less than 0.0001). Age and serum IgG antibody levels to cytomegalovirus did not appear to correlate. These results suggest that there are different subgroups of patients with AIDS-associated Kaposi's sarcoma, and that the initial site of disease, as well as immunologic parameters, may be useful in prognosis.
The relationship between basal cell carcinoma and lymphoma was investigated in 63 patients with both diagnoses who were seen at Memorial Sloan-Kettering Cancer Center between 1949 and 1984. The majority of patients were diagnosed with lymphoma before the onset of their first basal cell carcinoma. Multiple basal cell carcinomas developed in most patients. The overall recurrence rate of basal cell carcinoma was high (17%), and metastatic disease developed in one patient. These data support a more aggressive behavior of basal cell carcinomas in patients with lymphoma than in otherwise healthy individuals.
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Pulmonary Kaposi's sarcoma related to the acquired immune deficiency syndrome (AIDS) has not been well characterized. To define the clinical, radiographic, and pathologic features of this entity, 11 autopsy-proved cases of pulmonary Kaposi's sarcoma were reviewed. The most common clinical symptoms were dyspnea and cough, but hemoptysis and stridor were also found. Nodular infiltrates and pleural effusions were the most commonly found radiographic abnormalities. Pulmonary function tests were sensitive in detecting the pulmonary abnormalities due to Kaposi's sarcoma. A low diffusion capacity, lack of arterial desaturation with exercise, and obstruction to airflow were suggestive of pulmonary involvement with this malignancy. Although endobronchial Kaposi's sarcoma was visualized at bronchoscopy as cherry-red, slightly raised lesions, bronchial biopsy specimens always showed no abnormalities. Transbronchial brushings and biopsy specimens and analysis of pleural fluid were also not helpful in establishing a diagnosis. In the seven subjects with extensive parenchymal Kaposi's sarcoma at autopsy, the pleura was always involved. Eight subjects had involvement of the tracheobronchial tree. In all of the subjects, pulmonary Kaposi's sarcoma was a significant cause of morbidity, and in three of 11 subjects (27 percent) it was the direct cause of death.
Basal cell carcinoma is the most common form of skin cancer and is one in which both host and environmental factors are thought to play a role in its pathogenesis. For an investigation of the role of human leukocyte antigen (HLA)-associated variations in genetic susceptibility, thirty-one patients with multiple basal cell carcinomas were typed for HLA-A, B, C, and DR antigens. Patients were compared with both local and appropriate ethnic group controls. No statistically significant association with HLA-A, B, or C antigens was noted in any group. However, a significant increase in HLA-DR1 was noted in non-Irish, non-Ashkenazi patients. A tendency toward a decrease in HLA-DR3 was also noted among patients of Irish or Ashkenazi Jewish descent. The role of HLA-associated genetic factors in this form of skin cancer needs further investigation.
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A 69-year-old white Italian woman was first seen at Memorial Sloan-Kettering Cancer Center in 1981 concerning several skin growths on her back. The patient had had several basal cell carcinomas surgically removed from her back during the preceding 5 years. There was no history of arsenic ingestion or prolonged sun exposure and her family history was negative for skin cancer. The patient had developed pulmonary tuberculosis in 1938 and was treated with pneumothorax therapy. She had had more than 50 fluoroscopic examinations of the chest following this therapy, as well as multiple diagnostic x-ray films since that time. She recalled that she had faced the fluoroscopy beam during the procedure. In 1959, she had a transabdominal hysterectomy for fibroid tumors. In 1980 she underwent a right modified radical mastectomy for adenoid cystic carcinoma of the breast; biopsies of lymph nodes were negative. Physical examination revealed a thin, white woman with a right mastectomy scar. On the back, clustered in the interscapular region, were multiple scars and nine erythematous nodules with pearly borders, telangiectasia, and translucent surfaces. Within several nodules there were areas of light and dark brown pigmentation. There were no other suspicious lesions on the head, chest, or extremities, nor did the patient show any evidence of the basal cell nevus syndrome. Biopsy of all lesions revealed basal cell carcinoma, some of which were pigmented, without evidence of chronic radiodermatitis. All lesions were treated with curettage and electrodesiccation three times with good cosmetic results (Fig. 1).
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Kaposi's sarcoma is a multifocal systemic neoplasm histologically characterized by proliferating fibroblastic and microvascular elements. Initial signs include macules, papules, or nodules on the skin or mucosal surface. Lesions are frequently found on the trunk, arms, and head and neck. In general, sites of involvement and tumor load do not correlate with prognosis. A general decrease in the functional capacities of T and B cells is found in most patients. Kaposi's sarcoma is reported as the initial manifestation of the acquired immunodeficiency syndrome (AIDS) in approximately 30% of cases. Most cases are in men, although it has been reported in all risk groups. Kaposi's sarcoma in AIDS is more frequent among whites and homosexuals than blacks and intravenous drug abusers. Overall mortality is approximately 41%, with over 60% of patients alive at 1 year and 50% at 22 months. Overall survival is 18 months; however, some patients who have had the disease for 3 to 4 years are still doing well.
Kaposi's sarcoma (KS) is seen with increased frequency in the course of the epidemic of acquired immune deficiency syndrome. In this population, KS has manifested in an aggressive and more disseminated fashion as compared to the classical type. As the epidemic of acquired immune deficiency syndrome continues to spread and more cases of KS are evaluated, a distinct diversity in the clinical presentation and in the course of the disease as well as in variation in the prognosis and response to therapy is being observed. A preliminary description of the spectrum of KS in the epidemic of acquired immune deficiency syndrome is presented here.
A case of sarcoidal granuloma in tattoos, occurring in the setting of systemic sarcoidosis, is presented. The differential diagnosis of granulomatous reactions in tattoos is reported.
Preleukemia is a well-defined syndrome of hematopoietic dysfunction that may antedate the development of acute myelogenous leukemia. Granulocytic sarcoma refers to neoplastic infiltration in the skin, composed of immature cells of the granulocyte series. We report two cases of granulocytic sarcoma in the setting of preleukemia. The clinical importance of these cases, as well as the cutaneous manifestations of leukemia and the clinical spectrum of granulocytic sarcoma, are presented.
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