Lymphangiectatic Kaposi's sarcoma in a patient with AIDS

Kaposi''s sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi''s sarcoma. In non-HIV-associated Kaposi''s sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi''s sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis''s sarcoma.     

Recurrent Classical Type of Kaposi's Sarcoma Treated by Interferon-alpha

Kaposi''s sarcoma (KS) is a lympho-angioproliferative disease, with four variants; one of which is classical KS (CKS). Classical KS is clinically characterized by single or multiple pea-sized bluish-red macules on the distal portions of the lower extremities. A 60-year-old man presented with an asymptomatic, solitary patch on the left sole. He had been successfully treated for the classical type of Kaposi sarcoma on the right sole with interferon-alpha 3 years before. The patient was treated with six million units of interferon-alpha three times per week for 6 months. The lesion showed complete resolution and there has been no report of recurrence for 1 year after treatment.     

Cutaneous cryptococcosis and Kaposi's sarcoma occurring in the same lesions in a patient with the acquired immunodeficiency syndrome

A 34-year-old woman presented with a history of fever, malaise and skin lesions. A diagnosis of Kaposi's sarcoma and acquired immunodeficiency syndrome (AIDS) was established, and in addition, the skin lesion which was biopsied also demonstrated cryptococcal infection. Disseminated cryptococcosis was later confirmed and the disease ran a florid course. The co-existence of different diseases within the same lesion is a feature of human immunodeficiency virus (HIV) infection, this being the third documented case of simultaneous Kaposi's sarcoma and cutaneous cryptococcosis occurring at the same site in a patient with AIDS. The nature of this co-existence is discussed with reference to the pathogenesis of Kaposi's sarcoma.     

The development of Kaposi's sarcoma during immunosuppressive therapy for temporal arteritis

Background The appearance of Kaposi's sarcoma during iatrogenic immnunosuppression for renal transplantation is well known. A growing number of patients undergoing immnunosuppressive therapy for conditions other than organ transplanation have also developed this neoplasm, and temporal arteritis is emerging as one of the more common of those conditions. Case: We report a case of Kaposi's sarcoma complicating corticosteroid therapy for temporal arteritis, summarize the previously reported cases and consider the possible association between these two disorders. Conclusion: Although inconclusive from the available data, patients with temporal arteritis may be predisposed to iatrogenic Kaposi's sarcoma due to a shared antigen specific immune response.     

Kaposi's sarcoma associated with immunosuppression for bullous pemphigoid

Kaposi's sarcoma may occur in transplant recipients on immunosuppressive regimens, but is not well recognized in association with treatment for dermatological disease. We report two cases where multifocal Kaposi's sarcoma developed following iatrogenic immunosuppression with prednisolone and azathioprine for bullous pemphigoid. Both patients were HIV negative and, in one case, lesions regressed both clinically and histologically when immunosuppressive therapy was withdrawn.     

Kaposi's sarcoma and immunosuppressive therapy: an appraisal

Kaposi's sarcoma can probably be added to the list of tumours (lymphomas and squamous cell epitheliomas) that are especially prone to arise as a result of immunosuppressive therapy. Sixteen examples of this complication have been described in the literature and we report three further instances. These three patients had been on treatment with prolonged steroid therapy, but many of the previosly reported patients had been on aggressive immunosuppressive regimes to prevent rejection of kidney transplants. Kaposi's sarcoma associated with steroid and immunosuppressive therapy may show enhanced incidence in women (7 females as against 12 males) remembering that the spontaneous disease occurs ten times more frequently in men than women. We discuss the implications of these findings against the background of the high incidence of Kaposi's sarcoma in parts of Africa where immunological derangement consequent on malaria may be significant.     

Molecular diagnosis of deep nodular bacillary angiomatosis and monitoring of therapeutic success

A 51-year-old human immunodeficiency virus (HIV)-positive male patient (CDC stage 3C) had had a painful nodule on his left external ankle joint for 10 months. A biopsy suggested bacillary angiomatosis, but Kaposi's sarcoma could not be excluded. Rods were detectable in lesional skin by a Warthin-Starry stain. A 298 base pair (bp) gene fragment specific for Bartonella species was amplified from lesional skin and direct nucleotide sequence analysis of the amplification product clearly identified Bartonella quintana. Kaposi's sarcoma-associated herpes virus specific DNA was not amplifiable by polymerase chain reaction (PCR) in our patient, suggesting that the lesion represented bacillary angiomatosis alone, despite clinical and histopathological features which suggested the coexistence of bacillary angiomatosis and Kaposi's sarcoma. The lesion regressed after erythromycin was prescribed. However, 4 and 9 weeks after initiation of therapy, PCR still yielded a positive result in material obtained by a swab. After complete healing, following 12 weeks of antibiotic therapy. PCR became consistently negative. The optimal length of antibiotic treatment in HIV-positive patients with bacillary angiomatosis is not yet known and inadequate therapy may be followed by disseminated disease and a fatal outcome. PCR-based monitoring of the success of treatment is valuable for determining the duration of treatment resulting in a cure.     

Loss of Wnt-5α is associated with an invasive phenotype of extramammary Paget's disease

Background: The Wnt (wingless‐type MMTV integration site) gene family encodes secretory signaling molecules that play a diverse biological role in the regulation of normal and pathological processes, including cell growth, differentiation and oncogenesis. However, the role of Wnt genes in the development of extramammary Paget's disease remains unknown. Objective: To investigate the expression of Wnt‐1, Wnt‐5α and their downstream genes, β‐catenin and c‐Myc, in extramammary Paget's disease. Methods: Paraffin‐embedded specimens of extramammary Paget's disease (33 specimens from 22 patients), including 7 specimens with dermal invasion and 4 with lymph node metastasis, were examined immunohistochemically for Wnt‐1, Wnt‐5α, β‐catenin and c‐Myc. Seven normal genital skin specimens served as controls. Results: The expression levels of Wnt‐1 and β‐catenin in extramammary Paget's disease were significantly correlated with each other; however, their expression levels in the invasive extramammary Paget's disease were similar to those of wholly intraepithelial extramammary Paget's disease. Nuclear expression of c‐Myc was significantly higher in the invasive extramammary Paget's disease in comparison with intraepithelial extramammary Paget's disease. Interestingly, the expression of Wnt‐5α in invasive extramammary Paget's disease was significantly downregulated compared to wholly intraepithelial extramammary Paget's disease. Conclusion: The Wnt‐1/β‐catenin pathway may not play an important role in the progression of extramammary Paget's disease. The loss of Wnt‐5α, however, may play a role in the invasiveness of extramammary Paget's disease. Xie L, Hayashida S, Furue M. Loss of Wnt‐5α is associated with an invasive phenotype of extramammary Paget's disease.     

The infra-red coagulator in the treatment of AIDS-related Kaposi's sarcoma and a comparison with radiotherapy

Experience with 1 s pulses of the infra-red coagulator is reported for the treatment of 10 cutaneous AIDS-related Kaposi's sarcoma lesions in seven patients. The infra-red coagulator may be a useful addition in the palliative cosmetic treatment of Kaposi's sarcoma, producing an acceptable result in small (less than 2 cm in diameter) Kaposi's sarcoma lesions of the arms and trunk, but not in those situated on the legs.     

Umbilical Paget's disease and prostatic carcinoma

We report a case of umbilical Paget's disease occurring in a patient with a prostatic carcinoma. No other malignancy was found, and the patient was treated by surgical excision of the lesion. To our knowledge, the occurrence of extramammary Paget's disease of the umbilicus has not been reported previously. The clinical presentation, its association with prostatic carcinoma, and the possible pathogenesis are discussed.     

The theràpeutic effect of intralesional interferon in classical Kaposi's sarcoma

Interferon alpha-2a, which has antineoplastic, antiviral, immunomodulatory, and antiangiogenic effects, was evaluated in a prospective study, treating 10 lesions of patients with classical Kaposi's sarcoma. Ten patients received injections of interferon alpha-2a intralesionally in a dose of 3 million units three times weekly for 4 weeks, and in a variable dose for 4 more weeks. Two of the patients had a complete response, and in one of these, distant lesions also responded. Seven had a partial response, and one did not respond. The treatment was generally well tolerated. The results of our study support the use of interferon in the therapy of classical Kaposi's sarcoma, although it would appear that to achieve maximum efficacy, a longer period of treatment is needed.     

Acute myelocytic leukaemia associated with Kaposi's sarcoma in a patient without serum antibodies to human immunodeficiency virus type 1

We report a 35-year-old man with acute myelocytic leukaemia and Kaposi's sarcoma, whose serum was non-reactive for antibodies to human immunodeficiency virus type-1 by enzyme-linked immunosorbent assay. Western blot and immunofluorescence. Complete remission of both the acute leukaemia and the Kaposi's sarcoma followed treatment with mitoxantrone and cytosine arabinoside. We speculate that the rapid regression of the lesions of Kaposi's sarcoma might be related to mitoxantrone and to the return to normal of his peripheral helper and suppressor T-cell counts.     

Kaposi's sarcoma and rheumatoid arthritis: a possible immunogenic and drug interaction

Background In the literature, Kaposi's sarcoma-rheumatoid arthritis association is reported and it has been suggested that genetic predisposition may play an important role. Numerous studies indicate that immunological deficiency may also have an important role in the pathogenesis of Kaposi's sarcoma, along with a series of others factors. Patients and methods We present two cases of Sardinian women with rheumathoid arthritis and Kaposi's sarcoma where the relationship between the drugs used for the treatment of the former (particularly gold salts) and Kaposi's sarcoma is highlighted. In addition, their genetic background is delineated. Conclusion Our findings suggest that genetic background associated with drugs plays a role in the pathogenesis of Kaposi's sarcoma in rheumatoid arthritis patients.     

Immunohistochemical detection of the latent nuclear antigen-1 of the human herpesvirus type 8 to differentiate cutaneous epidemic Kaposi sarcoma and its histological simulators

Kaposi''s sarcoma is the most common neoplasia diagnosed in AIDS patients and the expression of the human herpesvirus-8 (HHV-8) latent nuclear antigen-1 has been useful for its histological diagnosis. The aim of this study is to confirm that immunohistochemistry is a valuable tool for differentiating KS from its simulators in skin biopsies of HIV patients. Immunohistochemical and histological analyses were performed in 49 Kaposi''s sarcoma skin biopsies and 60 of its histological simulators. Positivity was present in the 49 Kaposi''s sarcoma skin biopsies and no staining was observed in the 60 simulators analyzed, resulting in sensibility and specificity of 100%. HHV-8 immunohistochemical detection is an effective tool for diagnosing Kaposi''s sarcoma, especially in early lesions in which neoplastic features are not evident. It also contributes to its histological differential diagnosis.     

A Case of Pigmented Mammary Paget's Disease

Pigmented mammary Paget''s disease is a uncommon clinicopathologic variant of mammary Paget''s disease, and this mimics malignant melanoma both clinically and histopathologically. Herein, we report on a rare case of pigmented mammary Paget''s disease. An 81-year-old woman presented with 2.5×1 cm sized, red and brown, eczematous plaque on her right areola, and she''d had this lesion for 3 years. Histopathology showed large, atypical cells with large nuclei and abundant pale cytoplasm throughout the epidermis. Dispersed melanocytes were noted in the epidermis and some of the Paget''s cells contained melanin within their cytoplasm. Immunohistochemical studies demonstrated that the intraepidermal pagetoid cells were positive for cytokeratin 7; in contrast, they were negative for S-100, Periodic-acid Schiff (PAS), Alcian blue at PH 2.5, HMB-45 and carninoembryonic antigen (CEA). We recommend that pigmented mammary Paget''s disease should be included in the differential diagnosis of pigmented lesions on the nipple.     

Disseminated Kaposi's Sarcoma with the Involvement of Penis in the Setting of HIV Infection

Kaposi''s sarcoma (KS) is a malignant proliferation of the endothelial cells. It typically presents with several vascular nodules on the skin and other organs. The penile localization of KS, particularly on the shaft area, is exceptional. We report an HIV-positive 34-year-old man who had multiple purplish-black plaques on his extremities and several small violaceous macules on the glans and shaft of the penis. Kaposi''s sarcoma was diagnosed by histopathology.     

The development of pseudo-Kaposi's sarcoma after placement of a vascular access graft

Pseudo-Kaposi's sarcoma developed on the skin overlying a vascular access graft in a haemodialysis patient. After spontaneous thrombosis within the graft, the cutaneous lesions promptly resolved. Light and electron microscopic study of the lesions failed to distinguish them from those of true Kaposi's sarcoma.     

Taponamiento cardíaco por sarcoma de Kaposi

—Kaposi's sarcoma is a systemic angiomatous tumor that generally affects individuals over 50 years of age; it is mainly observed in male patients. Few cases of visceral involvement have been described in its classic form. In this work, we describe a case of classic Kaposi's sarcoma with disseminated skin lesions, involvement of multiple internal organs and fatal evolution. The cause of death was a cardiac tamponade caused by the Kaposi's sarcoma lesions that developed in the epicardium and the pericardium.     

Kaposi肉瘤治疗的研究进展

Kaposi肉瘤的治疗应依据不同临床类型,制定个体化方案.常用治疗方法有电化学疗法、放疗、物理治疗(如硝酸银、液氮冷冻、弹力长筒袜)和皮损内注射等,适用于经典型Kaposi肉瘤及其他类型Kaposi肉瘤的皮肤损害.常用治疗药物有足叶乙甙、紫杉醇、阿霉素、雷帕霉素、吉西他滨等,适用于各型Kaposi肉瘤.Kaposi肉瘤的治疗尚缺乏标准化指南,以上治疗远期疗效欠佳.目前,新治疗靶标的研究主要集中在阻止成瘤过程、炎症调控、阻止病毒复制、细胞周期的调控等方面.     

Acquired progressive lymphangioma: Case report with partial response to imiquimod 5% cream

Acquired progressive lymphangioma (APL), or benign lymphangioendothelioma, is an unusual entity derived from vascular structures. Clinically and histopathologically it may resemble Kaposi's sarcoma and well‐differentiated angiosarcoma, causing a diagnostic problem. We report an individual with APL initially diagnosed with Kaposi's sarcoma who underwent unnecessary laboratory testing. Imiquimod 5% cream stopped the progression of the lesion. Awareness of this rare entity may prevent patients from undergoing excessive testing. Imiquimod may be used as a safe, effective treatment option.