Biomedical subjects
Publications and source records attributed to E Alessi.
We report an exceptional case of smooth muscle tumor arising in an aberrant areola. In spite of the malignant histopathologic features of the tumor, the patient is healthy 6 years after complete surgical removal of his lesion.
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The Italian Cooperative Group on AIDS & Tumors (GICAT) is collecting data on the epidemiological and clinical features of Kaposi's sarcoma (KS) in Italy, a country where intravenous drug users (IVDUs) comprise the largest group affected by AIDS. As of December 31st 1989, among 5317 cases of CDC-defined AIDS reported to the National Registry of AIDS, there were 419 (8%) cases of KS. Twenty-five percent of all of the homosexual men with CDC-defined AIDS had KS, while only 3% of the IVDU patients with CDC-defined AIDS had KS. The first case of KS was observed in 1982, 2 cases in 1983, 7 cases in 1984, 17 cases in 1985, 55 cases in 1986, 89 cases in 1987, 120 cases in 1988 and 128 cases in 1989. Of the 226 cases of KS reported to GICAT, 60% were in homosexual men, 21% in IVDUs, and 9% in heterosexuals. An increased number of KS has been observed since 1982, although the number of new cases in the past year seems to have been stable, possibly due to the usual delay in notification. There is, however, an actual decrease of the percentage of KS among AIDS patients, from 18% in 1984 to 6% in 1989. Almost half of the patients had had a diagnosis of AIDS prior to the appearance of KS, but in 25% of the patients KS was the first AIDS manifestation. Almost all patients had skin involvement either alone or in association with other sites, whereas very few patients had only lymph node involvement. Only treatment with alpha-2 interferon was able to obtain complete remission in 9% of evaluable patients.(ABSTRACT TRUNCATED AT 250 WORDS)
The case of a patient who developed multiple soft fibroma-like lesions on his lower extremities affected by lymphedema and Kaposi's sarcoma is reported. To the best of our knowledge, the coexistence of these three pathologic processes is unusual and has never been described before in the literature.
Pleomorphic peripheral T-cell lymphomas (PPTL) arising primarily in the skin are uncommon. The observation of pleomorphic T-cell lymphoma composed of 100% CD30+ cells is rare. We report the case of a woman in whom such a tumor manifested itself with a single cutaneous lesion. It was composed of medium to large cells expressing markers of helper/inducer T-lymphocytes, as well as activation (CD30) and proliferation antigens (Ki-67), but not the CD25 activation antigen. The lesion was excised and the patient is alive and disease free 2 years after the diagnosis. This case confirms previous reports about the favorable course of some cutaneous pleomorphic lymphomas, and shows that the activation antigen expression and proliferation antigen (Ki-67) expression do not seem to worsen the prognosis.
A selected population of 41 homosexual/bisexual asymptomatic HIV-positive subjects were administered neurophysiological tests to assess language, memory, attention, logic faculties and visuo-motor functions. HIV-positive subjects differed from individually matched control subjects only in certain measures of verbal memory. Longitudinal evaluation performed after 1.5 years, however, did not indicate any further development of this mild amnesic deficit. Despite the small number studied in our sample, there seems to be a trend for older subjects to be at greater risk of developing AIDS and cognitive abnormalities than younger subjects, while differences in immunological status play a significant role in disease progression.
The first case of primary gamma/delta cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated pagetoid reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the gamma/delta variant of the T-cell receptor (CD3+, TCR-delta-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but beta F1-, Ti gamma a-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a-, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included gamma/delta T-cell receptor analysis, suggests that some reported cases of pagetoid reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the gamma/delta T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2- subset of normal human peripheral blood gamma/delta T lymphocytes.
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Oral hairy leukoplakia was first described in homosexual men infected with the human immunodeficiency virus. It is thought to be caused by infection with both the Epstein-Barr virus and human papillomavirus. We report 59 cases of oral hairy leukoplakia. The disease was diagnosed in patients in all risk groups and was categorized in all classes of the Walter Reed classification without significant differences in prevalence. Epstein-Barr virus could be demonstrated in all tissue samples examined; human papillomavirus was found in only a few specimens. In our series oral hairy leukoplakia had a chronic course, although temporary spontaneous healing occurred in some cases. Its appearance was a poor prognostic sign because acquired immunodeficiency syndrome developed in a significant proportion of patients within a few months of onset.
Pityriasis lichenoides is usually classified into an acute and a chronic form. From a review of 89 cases of the disease seen since 1974 it seems that a more realistic classification into three main groups, according to the distribution of pityriasis lichenoides lesions, could be made, namely, a diffuse, a central, and a peripheral form, each characterized by a different clinical course. Conversely, no correlations were detected in our series between the severity of skin lesions and their distribution or the overall course of the disease. None of our cases suggests the possible evolution of pityriasis lichenoides into lymphomatoid papulosis. Although no infectious causative agent has been identified, a viral origin seems likely in some cases. Most patients responded favorably to UVB irradiation. Our conclusions are (1) that pityriasis lichenoides is probably a clinical disorder with a diverse etiology and (2) that its classification by distribution seems more useful than its subdivision into an acute and a chronic form.
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The Italian Cooperative Group on AIDS-related tumors has collected 435 cases of HIV-associated tumors since December 1986. The following conclusions can be drawn from this IVDA-based series: (1) at least 15% of AIDS cases are associated with tumors; (2) the number of malignant lymphomas (high-grade non-Hodgkin's lymphoma [NHL], Hodgkin's disease [HD] is comparable to that of Kaposi's sarcoma (KS) (188 vs. 198); (3) KS among AIDS patients is less common than in countries where homosexual men are the main group affected by AIDS. However, KS also affects intravenous drug abusers (IVDA) almost exclusively males, with characteristics similar to those observed among homosexual men; (4) HD is associated with an aggressive course; (5) anal and oral primary tumors as well as oral and anal involvement of NHL are very rare; (6) testicular cancers occur in patients mainly with early HIV infection, without adversely affecting the dosage of radiotherapy and chemotherapy; (7) cervical cancer successfully treated with conization suggests that PAP test screening in young IVDA women is warranted; (8) lung cancer occurs in a young age group with rapid progression and death.
Fourteen cases of primary cutaneous B-cell lymphomas were investigated at the immunohistochemical and molecular level to further characterize this newly defined entity. Neoplastic cells from all cases, phenotyped with a panel of monoclonal antibodies, were positive for HLA-DR, for the B-cell markers CD19, CD22, but not CD23 (except one case), and negative for the T-cell marker CD2. Monoclonal immunoglobulin light chains were demonstrated in six cases. The reactivity with the Ki-67 monoclonal antibody indicated that the neoplastic cells are proliferating. In five biopsies the presence of dendritic cells infiltrating the neoplastic areas was revealed using the monoclonal antibody Kim4b. By Southern blot analysis, clonal rearrangement of the immunoglobulin heavy chain gene (involving one or both alleles) was shown in 12 of 14 cases and of the light chain genes in 13 cases. The bcl-2 oncogene, normally involved in nodal follicular lymphomas, was in germ-line configuration. The c-myc and the beta and gamma chain genes of the T-cell receptor were also in the germ-line configuration. None of the cases presented Epstein-Barr virus sequences. These data indicate that primary cutaneous lymphomas of B-cell origin share morphological and phenotypic similarities with the nodal B-cell lymphomas of follicular histotype, are proliferating, and express in 45% of cases clear monoclonal immunoglobulin light chain; the molecular analysis confirms the B-cell derivation and the monoclonal nature of this neoplasia; it also shows that neither bcl-2 nor c-myc oncogenes are involved and that no inappropriate rearrangements of the T-cell receptor genes are found in this lymphoma.
Anaplastic large cell lymphoma is a subtype of nodal and extranodal lymphoproliferative disorder characterized by peculiar histopathological features and the positivity of lymphoid proliferating cells for Ki-1/Ber-H2 monoclonal antibody. A case, misdiagnosed as cutaneous metastasis and treated by surgery, in a woman with a history of previous neoplasms, is reported. In spite of the histopathological malignancy of the tumor, the patient is alive and free from recurrent or metastatic disease after 3 years of follow-up.
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