Neurological complications of Kaposi's sarcomat

We analyzed the neurological complications in 25 patients with Kaposi's sarcoma, 5 encountered at Memorial Sloan-Kettering Cancer Center and 20 culled from the literature. Patients with all clinical forms of Kaposi's sarcoma [14 classical cases, 2 African cases, 5 cases associated with immunosuppressive therapy and 4 cases associated with acquired immunodeficiency syndrome (AIDS)] suffered neurological dysfunction which included neoplastic involvement of the nervous system (Kaposi's sarcoma or another primary), autoimmune disorders and opportunistic infections. Neoplastic involvement was recorded most frequently in patients with classical and African Kaposi's sarcoma and was favored in a setting of extensive tumor dissemination. Opportunistic infections (cryptococcal meningitis and cerebral toxoplasmosis) were observed in all forms of Kaposi's sarcoma but were most frequent in AIDS cases and correlated with the degree of immune dysfunction. Our data suggest that more diverse opportunistic central nervous system infections, neurological disorders of an autoimmune nature, and neoplastic involvement of the nervous system are to be anticipated in AIDS.     

Autopsy findings in AIDS-related lymphoma

Autopsies in 20 patients with the acquired immune deficiency syndrome (AIDS)-related lymphoma were studied retrospectively to ascertain the precise cause of death and the extent of lymphomatous disease. Eight patients had primary central nervous system (CNS) lymphoma: two of them were diagnosed antemortem; CNS lymphoma was suspected in three others by computerized tomographic (CT) scan and was confirmed at autopsy. The remaining three were diagnosed incidentally at autopsy. All had concurrent infections at autopsy, including opportunistic infection in six and pyogenic infections in two. Opportunistic infections at autopsy in these patients outnumbered those diagnosed clinically. Twelve patients had systemic lymphoma. Three were diagnosed only at autopsy, two of whom had extensive Kaposi's sarcoma (KS) as well. Eight patients were treated with chemotherapy, but died with disseminated disease. Opportunistic infections were found at autopsy in five patients. Secondary involvement of the CNS by lymphoma was frequent (66%) and was not related to previous bone marrow involvement. The authors conclude that the incidence of lymphoma occurring in AIDS may be more frequent than diagnosed clinically. Similarly, multiple opportunistic infections occur in these patients which are not diagnosed premortem and may contribute to early death.     

Kaposi's Sarcoma and Autoimmune Thrombocytopenia

Outbreaks of Kaposi's sarcoma, opportunistic infections, and autoimmune thrombocytopenia among homosexual men have recently been described. We report here a case with the combination of Kaposi's sarcoma and autoimmune thrombocytopenia. Our patient presented with Kaposi's sarcoma limited to the integument and autoimmune thrombocytopenia diagnosed by elevated platelet bound IgG. Characteristics of immunosuppression included lymphocytopenia and a reversed blood helper: suppressor T lymphocyte ratio. Immunohistochemical evaluation of skin biopsy specimens revealed tumor cells to contain Factor VIII-related antigen (a vascular endothelial cell marker). In addition some tumor cells stained positively with a monoclonal antibody directed against a cytomegalovirus antigen.     

Treatment of advanced Kaposi's sarcoma using a combination of bleomycin and vincristine.

Eighteen patients with disseminated AIDS-related Kaposi's sarcoma (KS) and compromised bone marrow function were treated with a relatively non-myelosuppressive regimen of bleomycin and vincristine (BV). At study entry, the patients presented with the following median laboratory values: hemoglobin of 9.5 g/dl, granulocyte counts of 1,173/mm3, platelet counts of 218,000/mm3, and CD4 lymphocyte counts of 58/mm3. All patients had extensive Kaposi's sarcoma. Nine patients had visceral involvement: four with pulmonary involvement, two with gastrointestinal involvement, and three with both. Following a median number of seven cycles of biweekly chemotherapy, complete or partial tumor responses were achieved in 13 patients (72%). Two patients experienced bleomycin-induced skin toxicities, whereas 10 others (55%) experienced peripheral sensory neuropathy requiring vincristine dose reductions. Opportunistic infections had occurred in 11 patients prior to initiation of chemotherapy and in 16 after initiation of chemotherapy. Despite the frequent development of opportunistic infections, BV chemotherapy was relatively well tolerated and resulted in a high response rate in this patient population that presented with suboptimal marrow function and extremely low CD4 lymphocyte counts.     

Kaposi's sarcoma and the acquired immunodeficiency syndrome: treatment with high and low doses of recombinant leukocyte A interferon

The efficacy of recombinant leukocyte A interferon (rIFN-alpha A [Roferon-A, Hoffman-La Roche, Nutley, NJ]) treatment of Kaposi's sarcoma in patients with acquired immunodeficiency syndrome was evaluated in sequential trials using high doses (36 X 10(6) units) and low doses (3 X 10(6) units) of interferon. A major response was seen in 38% of patients treated at the high dose, with a median response duration of 18 months. At the low dose, the major response rate was 3%; dose escalation to 36 X 10(6) units resulted in an additional major response rate of 17% in low-dose nonresponders, with a median response duration of 10 months. Four of 11 patients who achieved a complete response remain free of disease, whereas all partial responders have shown disease progression. Unacceptable toxicity occurred in 27% of patients initially treated at the high dose and only in 10% of those who had progressive dose escalation up to 36 X 10(6) units. Prior opportunistic infections correlated negatively with therapeutic response, whereas large tumor burden and gastrointestinal involvement did not. Responding patients showed a significantly longer survival and a lower incidence of subsequent opportunistic infections than nonresponders. However, from our study we cannot determine whether rIFN-alpha A has an effect on the natural history of Kaposi's sarcoma in patients with the acquired immunodeficiency syndrome.     

Mortality associated with mode of presentation in the acquired immune deficiency syndrome

A population-based survival study was done for all cases of the acquired immune deficiency syndrome diagnosed in the city of San Francisco through May 1983. Follow-up was obtained for 165 of 173 diagnosed cases. Median survival among 75 patients presenting with Kaposi's sarcoma (KS) alone was 21 months. Median survival among 90 patients presenting with opportunistic infections, primarily Pneumocystis carinii pneumonia, was 9 months; survival at 21 months was zero. Survival among patients presenting with both KS and opportunistic infections was not statistically different from survival among patients presenting with opportunistic infections only. When cases were divided into those diagnosed before and after May 1982, there was no significant improvement in survival from diagnosis in the more recently diagnosed cohort.     

Treatment of epidemic Kaposi's sarcoma with etoposide or a combination of doxorubicin, bleomycin, and vinblastine

An epidemic of disseminated Kaposi's sarcoma in male homosexuals has recently been described. Forty-one evaluable patients with epidemic Kaposi's sarcoma were treated with etoposide. The majority of these patients had early stage disease, no prior opportunistic infections, and no prior therapy. Twelve patients (30%) achieved complete remission, 19 (46%) partial remission, and ten (24%) no response. With follow-up time to 31 months, the median response duration is nine months. The median survival of patients with complete and partial remissions has not been reached. A combination of doxorubicin (Adriamycin, Adria Laboratories, Columbus, Ohio), bleomycin, and vinblastine (ABV) was used in 31 evaluable patients with epidemic Kaposi's sarcoma. The majority of these patients had late stage disease, prior opportunistic infections, or had failed prior treatment. Seven patients (23%) achieved complete remission, 19 (61%) partial remission, and five (61%) no response. With follow-up time to 24 months, the median response duration is eight months. The projected median survival for all patients treated with ABV is nine months. Both regimens were well tolerated, with an overall response rate of 76% for etoposide and 84% for ABV. However, while successfully treating the Kaposi's sarcoma, the underlying immune deficiency in these patients has persisted. Future treatments of Kaposi's sarcoma will need to focus on reversing the underlying immune incompetence as well as controlling the malignant manifestations of Kaposi's sarcoma arising in relation to the acquired immune deficiency syndrome.     

Pulmonary Involvement as the Major Manifestation of Chronic Lymphocytic Leukemia

Respiratory symptoms and abnormal findings on chest X-ray are frequently noted in patients with chronic lympllocytic leukemia (CLL). However, most of these represent pulmonary infections or mediastinal lymphadenopathy, and leukemic involvement of the lung is seldom diagnosed during life. In this report we describe three patients with non-progressive, responsive CLL who developed biopsy proven pulmonary infiltration with CLL. In one case, pulmonary involvement was the sole manifestation of recurrent disease and a second case had little disease elsewhere with minimal CLL in the blood at the time pulmonary involvement appeared. In all three cases, transbronchial biopsy and bronchoalveolar lavage performed during fibreoptic bronchoscopy provided adequate tissue for diagnosis. We conclude that CLL may involve the lung even in the presence of a low peripheral white blood cell count with responsive disease elsewhere, and can readily be diagnosed by transbronchial biopsy and bronchoalveolar lavage.     

Role of diagnostic lymphnode biopsies in 56 anti-HIV positive patients

The authors have examined two separate series of samples from 33 and 23 anti-HIV positive patients with persistent generalized lymphadenopathy (PGL) undergoing lymphnode biopsy. For the first group of patients, the aim was to evaluate the diagnostic and prognostic correlation between clinical and laboratory features and histologic findings. For the second group, an assessment was made of the indications for lymphnode biopsy as determined by the rate of positive histopathologic findings. Histologic alterations were classified into four types according to Ràcz, without knowledge of clinical and laboratory data. At the time of biopsy, the first sample (n = 33) included 21 lymphadenopathy associated syndrome (LAS) (64%), 6 ARC (18%), and 6 AIDS (18%) patients; a significant prevalence of type 1 histologic features was evident in LAS/ARC patients, while type 4 was exclusively present in the AIDS group. These preliminary results suggested that only symptomatic anti-HIV positive patients (ARC/AIDS) should have been selected for lymphnode biopsy, because of the expected high rate of opportunistic infections, malignant lymphoma, or Kaposi's sarcoma (KS) in this specific subset of patients. Actually, in the second sample of 23 patients (ARC = 8: AIDS = 15) the detection rate of AIDS-related neoplasms included ten Kaposi's sarcoma and three Hodgkin's disease. These findings suggest that diagnostic lymphnode biopsies should be limited to selected anti-HIV positive patients with evidence of AIDS-related neoplasms or treatable opportunistic infections, or for the purpose of detecting histopathologic features of prognostic significance.     

支气管镜介入治疗肺肉瘤

背景与目的肺肉瘤为发生于肺部少见的软组织恶性肿瘤,常规治疗首选手术。本研究旨在探讨采用支气管镜介入治疗的效果。方法回顾性分析2008年11月-2014年7月我院收治的16例肺肉瘤患者,平均年龄（53.1±5.4）岁。所有患者首次治疗均选用全凭静脉麻醉下硬质气管镜检查,发现肿瘤后行支气管镜介入治疗。再次检查时行电子支气管镜检查。结果16例患者中肉瘤样癌10例,纤维肉瘤和肉瘤各2例,纤维粘液性肉瘤和梭性细胞型滑膜肉瘤各1例。周围型11例（占68.8%）,主要位于右上叶和左下叶肺内;中央型5例（31.2%）。位于肺内者以混合型多见（9/11,82%）,原发性较多（9/11,82%）;而位于大气道者以管内型多见（9/16,56%）,转移性较多（11/16,69%）。支气管镜介入治疗后4例全肺不张均缓解,7例肺叶不张3例完全缓解,部分缓解和无效各2例。患者气道阻塞、卡氏体力状态（Karnofsky performance status, KPS）和气促评分均有明显改善。结论支气管镜介入治疗能快速、有效地清除气道内肿瘤,缓解气道梗阻,改善症状。     

Clinical aspects of Kaposi's sarcoma.

Kaposi's sarcoma is the most common tumor found in patients with the acquired immunodeficiency syndrome. This opportunistic neoplasm has characteristics of a sexually transmitted disease. Growth factors, cytokines, immune suppression, and interaction with infectious organisms all appear to play a role in the pathogenesis of this enigmatic disorder. The manifestations of Kaposi's sarcoma are protean, and lesions may appear at any time in the course of human immunodeficiency virus disease, remain localized and asymptomatic, or spread aggressively and cause morbidity. Treatment, which must be individualized, ranges from observation, local therapy with cosmetic makeup, and cryotherapy with liquid nitrogen or local intralesional injection of agents, to radiotherapy and systemic therapy with interferon-alpha and cytotoxic chemotherapy.     

Atrial metastasis detected by two-dimensional echocardiography

With recent improvements in therapeutic procedures and prolongation of survival, the incidence of cardiac metastasis is likely to increase. The aim of the current study was retrospectively to analyze cases of atrial metastases detected by echocardiography at our center. We report on a series of 10 patients with various solid malignancies and secondary atrial involvement detected by echocardiography. Transesophageal and transthoracic echocardiographic studies were performed using an Acuson 128XP, Aspen or Sequoia (Mountain View CA). Seven patients had cardiac symptoms at the time of diagnosis of atrial metastasis. After diagnosis, therapy included surgery, chemotherapy or radiotherapy. Echocardiography was also used in monitoring response to therapy. One patient with Wilms' tumor has remained free of disease for 50+ months and a patient with Kaposi's sarcoma is alive with disease 20+ months after diagnosis. The remaining eight patients died with disease 3-24 months after being diagnosed with atrial metastasis. Atrial metastases can be diagnosed in patients without cardiac symptoms, and is probably more prevalent than was previously recognized. Echocardiography is a useful diagnostic tool in detecting secondary atrial tumors.     

Classic Kaposi's sarcoma in Arabs--widening ethnic involvement

Kaposi's sarcoma is a tumor caused by human herpes virus 8 also known as Kaposi sarcoma-associated herpes virus. Originally described by Kaposi in 1872, this tumor is recognized as an AIDS-defining illness. Classic Kaposi's sarcoma (CKS) is a relatively indolent disease affecting elderly men from the Mediterranean region or of eastern European descent, besides Jews in whom it is the most common. It has been also reported in the Arab population living in Israel. Kaposi's sarcoma has been reported in Arabs after kidney transplantation; however, there are no reports of CKS occurring in non-Israeli Arabs. This is first such article reporting two Arab patients who presented with CKS thus widening the ethnic and geographic area of involvement with this condition.     

Endobronchial metastatic disease: Analysis of 32 cases

Endobronchial metastasis (EM) from nonpulmonary tumors is uncommon. A 9-year retrospective study at the University Hospital Vall d'Hebron (Barcelona, Spain) identified 32 patients with EM. All but four cases were diagnosed by fiberoptic bronchoscopy with bronchial biopsy. Primary tumors included the following types: breast cancer (20), colorectal cancer (3), melanoma (2), gastric cancer (1), neuroblastoma of the olfactory nerve (1), abdominal leiomyosarcoma (1), hypernephroma (1), endometrial carcinoma (1), papillary thyroid cancer (1), and hepatocarcinoma (1). Median age at diagnosis of EM was 58.7 years and median interval from the diagnosis of the primary tumor to the diagnosis of EM was 50.4 months. Seventeen patients (53%) had evidence of other metastatic sites at endobronchial relapse. The more common clinical manifestations included cough (37.5%), haemoptysis (28%), dyspnea (18.7%), and recurrent pulmonary infections (6.2%). Eight patients (25%) had no symptoms. There appears to be a predilection for metastatic involvement of the right and left upper lobe bronchus. Treatment was instituted in 20 patients, and their median survival was 11 months, in comparison with the 3 months found in 12 patients who received only palliative therapy because of advanced disseminated disease. Breast cancer is the most common tumor causing EM. The prognosis of patients with EM depends on the type of the primary tumor and the presence of other metastatic sites. Treatment must be individualized. © 1996 Wiley-Liss, Inc.     

A prospective comparison of fiberoptic transbronchial needle aspiration and bronchial biopsy for bronchoscopically visible lung carcinoma.

BACKGROUND: Fiberoptic bronchoscopy is the most common modality used to diagnose endobronchial carcinoma. The authors prospectively compared the sensitivity of endobronchial needle aspiration (EBNA) and immediate cytologic assessment with bronchial biopsy and bronchial washing in the diagnosis of endobronchial malignancy. METHODS: A prospective trial comparing the sensitivity of EBNA, bronchial biopsy, and bronchial washings during fiberoptic bronchoscopy for endobronchially visible lung tumor was conducted. The authors enrolled 65 consecutive patients with endobronchial abnormalities identified during bronchoscopy. All patients in the study underwent fiberoptic bronchoscopy that included EBNA, bronchial biopsy, and bronchial wash. The sensitivities of the individual techniques were compared. The sensitivities of bronchoscopy were also prospectively compared when multiple sampling techniques were employed. RESULTS: Malignancy was present in 57 of 65 study patients. Cancer was diagnosed in 47 patients by EBNA, 42 patients by bronchial biopsy, and 36 patients by bronchial washing. The sensitivity of a strategy employing bronchial biopsy and bronchial washings was 0.82 (95% CI, 0.70-0.90). The addition of EBNA to bronchial biopsy and bronchial washings significantly increased the sensitivity to 0.95 (95% CI, 0.85-0.98; McNemar P = 0.02). Subset analysis revealed that this strategy was especially useful in cases in which lesions were submucosal or causing extrinsic compression. CONCLUSIONS: There is a modest increase in the sensitivity of fiberoptic bronchoscopy in diagnosing endobronchial cancer with the addition of EBNA to bronchial biopsy and bronchial washings, especially for patients with submucosal abnormalities. Collection of EBNA, followed by biopsy and washings only if immediate interpretation of EBNA is negative or inadequate, may be the most effective bronchoscopy strategy for evaluating visible endobronchial abnormalities.     

Kaposi's sarcoma in heterosexual intravenous drug users

Kaposi's sarcoma is a feature of the acquired immunodeficiency syndrome (AIDS) in male homosexuals and Haitians. In this report the authors describe six heterosexual intravenous drug users who developed Kaposi's sarcoma. They had other characteristics of AIDS including opportunistic infections (oral candidiasis, six patients; Pneumocystis carinii pneumonia, two patients; genital herpes simplex, two patients; disseminated cytomegalovirus, one patient; Mycobacterium avium, one patient) and inversion of the normal ratio of helper-suppressor T-cell subsets in peripheral blood lymphocytes in the two patients in whom it was determined. These observations indicate that intravenous drug users as well as Haitians and homosexual men are at risk for developing Kaposi's sarcoma as part of the acquired immunodeficiency syndrome.     

A surgical oncology perspective on AIDS

During the summer of 1981 reports of unusual opportunistic infections among male homosexuals first began to appear in the literature and by December 1983, 3000 cases has been reported to the Center for Disease Control in Atlanta, Georgia. Homosexual and bisexual men comprise 71% of the reported cases, while 17% are known intravenous drug users, and the remaining 12% made up of persons with other risk factors. Approximately 80% of AIDS patients with Kaposi's sarcoma have identifiable gastrointestinal lesions. Specific risk factors for Kaposi's sarcoma in AIDS patients have not been exclusively identified, but it is thought that repeated sexual involvement with multiple partners exposes the victim to the immunosuppressive effect of cytomegalovirus and allogenic sperm, which may in turn impair lymphoid cells. The immunological abnormalities associated with AIDS have included striking changes in T-cell ratios, with more profound changes in the Kaposi-AIDS groups. Increases in IgG, decreased antibody production by B-cells to challenge antigen, decreased B-cell proliferative responses to B-cell mitogens, abnormalities in macrophage function, and increased serum thymosin levels have been found. Various opportunistic infections afflict the patient with AIDS. Antimicrobial therapy, various interferons, interleukin-2, and immunomodulation with thymosin have all been used without much success. The distribution and transmission of AIDS parallels hepatitis B virus infection which is transmitted sexually and parenterally. On the whole the same precautions as for hepatitis B should be taken for AIDS. Health care personnel treating patients with AIDS should also wear masks and eye protection against the splatter of body fluids and secretions.(ABSTRACT TRUNCATED AT 250 WORDS)     

Angioimmunoblastic lymphadenopathy with dysproteinemia complicated by Kaposi's sarcoma.

A case of clinically and morphologically typical angioimmunoblastic lymphadenopathy (AILD) in a 68-year-old man during a prolonged antibiotic treatment for urinary infection is presented. Lymph node biopsy at first showed findings suggestive of an exhaustion of the germinal center immunological activity (like those characterizing angiofollicular lymph any clear transition into malignant lymphoma. The course of the disease was characterized by the occurrence of opportunistic infections (toxoplasmosis, herpes zoster), and finally by the onset of a cutaneous Kaposi's sarcoma. The possible relation of AILD to Kaposi's sarcoma is discussed, and the main clinical and morphological data of the case of AILD (about 200) reported in the literature are reviewed.     

Indolent lymphadenopathic Kaposi's sarcoma

Kaposi's sarcoma is usually an indolent skin neoplasm. Diffuse lymph node and visceral involvement have been described in young African patients and in patients who are immunosuppressed; in such patients the disease is usually rapidly progressive. We describe the case of a 64-year-old man of Italian ancestry with diffuse lymph node involvement by Kaposi's sarcoma in the absence of any skin lesions. The patient had no evidence of immunosuppression. The course has been indolent, with no disease progression over 2 years of follow-up on no therapy.