Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the first case of acquired immunodeficiency syndrome (AIDS)-related primary hepatic leiomyosarcoma in a 9-year-old girl. The pathologic diagnosis was made on a partial hepatectomy specimen and was confirmed by immunohistochemistry and electron microscopy. No human immunodeficiency virus-related nucleic acid was identified in tumor cells by in situ hybridization. Review of the AIDS-related literature reveals a rising incidence of tumors of smooth muscle origin in human immunodeficiency virus-infected patients. This case study details the eighth pediatric AIDS patient with a tumor of smooth muscle origin and represents the 20th and the youngest patient with primary hepatic leiomyosarcoma to be reported in the world literature.
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PMID:Primary hepatic leiomyosarcoma in a child with the acquired immunodeficiency syndrome. 154 73

We report a rare primary adrenal leiomyosarcoma in a 30-year-old, human immunodeficiency virus--positive man. This is, we believe, the third documented case in the English literature of this tumor in this site, and the first to be reported in an adult male with acquired immunodeficiency syndrome. The smooth muscle origin of this tumor was apparent by routine microscopy and confirmed by positive immunostaining for smooth muscle actin. The patient is presently well and shows no evidence of recurrence 20 months after surgery. The present findings are discussed with reference to the reported rising incidence of smooth muscle tumors in human immunodeficiency virus--infected patients and the associated etiologic role of Epstein-Barr virus in the pathogenesis of these tumors.
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PMID:Primary adrenal leiomyosarcoma in a man with acquired immunodeficiency syndrome (AIDS). Further evidence for an increase in smooth muscle tumors related to Epstein-Barr infection in AIDS. 914 Feb 80

HIV infection is known to increase the incidence of Kaposi's sarcoma and non-Hodgkin's lymphoma. Kaposi's sarcoma preferentially affects homosexual men and risk varies by geographic area, suggesting there is an environmental cofactor for Kaposi's sarcoma in addition to HIV. Despite intensive investigation, the responsible cofactor has not been conclusively identified. HIV-associated non-Hodgkin's lymphoma affects all HIV transmission groups, and non-Hodgkin's lymphoma risk increases with duration of HIV infection and age. Epstein-Barr virus has been implicated in the pathogenesis of this tumor, but the precise mechanisms have not been worked out. Cervical cancer and anal cancer have a less certain association with HIV infection and immunodeficiency, although epithelial dysplasia at these sites does seem to be HIV-related. Children with HIV infection are additionally affected by increased incidence of leiomyosarcoma and benign leiomyoma, whereas adults with HIV infection do not seem particularly susceptible to this tumor, perhaps because of hormonal or growth-promoting factors. Apart from these specific disease associations, HIV infection and related immunodeficiency do not result in a generalized tumor diathesis. Prevention and management of HIV-associated cancers are becoming increasingly important as the HIV epidemic continues to grow.
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PMID:Epidemiology of AIDS-related malignancies. 782 52

In a review of 8724 de novo malignancies that occurred in 8191 organ allograft recipients sarcomas were 7.4% of cancers. Kaposi's sarcoma (KS) made up 5.7%, and other sarcomas (OS) 1.7% a much higher proportion than in the general population. KS was most common in Arab, black, Italian, Jewish, or Greek patients. In 60% of patients with KS the lesions were confined to the skin and/or oropharynx while 40% involved internal organs and/or lymph nodes. Complete remissions following various treatments occurred in 53% of the former group and 27% of the latter. In both groups 32% and 60% of remissions, respectively, occurred when the only treatment was reduction or cessation of immunosuppressive therapy. However, this treatment caused impaired function or allograft loss from rejection in 22 of 34 kidney recipients. Recurrent KS occurred in 5% of patients in remission when immunosuppressive therapy was resumed. Nine of 114 patients (8%) tested for human immunodeficiency virus were positive. Most OS arose in internal organs or soft tissues. The major types were fibrous histiocytoma (20 patients), leiomyosarcoma (15), fibrosarcoma (12), rhabdomyosarcoma (9), hemangiosarcoma (8), undifferentiated sarcoma (7) and mesothelioma (6). Several unusual features were noted. Remarkably, 10 of 105 (10%) sarcomas occurred adjacent to or in a renal (6) or hepatic (4) allograft. Leiomyosarcomas are rare in children, yet 5 of 15 (33%) occurred in pediatric patients. Three hemangiosarcomas occurred in forearms at sites of arteriovenous fistulas used for pretransplant hemodialysis access. One leiomyosarcoma and one fibrosarcoma occurred in previously irradiated areas. One patient with mesothelioma had a history of asbestos exposure and two others had possible exposure.
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PMID:Sarcomas in organ allograft recipients. 854 79

A 35-year-old man infected with human immunodeficiency virus presented with cervical myelopathy of 2 months duration. Clinical and radiographic evaluation revealed a discrete, subdural mass at C-6. At surgery, the mass proved to have a dural attachment and thus clinically, radiographically, and grossly, it resembled meningioma. Histopathological analysis revealed a leiomyosarcoma that stained diffusely for muscle-specific actin. Electron microscopy revealed basal lamina surrounding the tumor cells and intracytoplasmic bundles of myofilaments. Epstein-Barr virus (EBV) was demonstrated within tumor cell nuclei by in situ hybridization for EBER1 messenger RNA and immunohistochemical staining for EBNA2 protein. Epstein-Barr virus latent membrane protein (LMP1) was not detected. This is the first documentation of an EBV-associated smooth-muscle tumor of the dura, and the first demonstration that tumors in this location contain EBV in an unusual form of latency not seen in lymphoid cell lines. With increasing numbers of individuals being afflicted with long-term immunosuppression, EBV-associated dural leiomyoma and leiomyosarcoma may be encountered more frequently in the future.
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PMID:Epstein-Barr virus-associated dural leiomyosarcoma in a man infected with human immunodeficiency virus. Case report. 912 7

A 14-year-old girl with common variable immunodeficiency syndrome was found to have a low-grade malignant neoplasm arising in the left temporal lobe of the brain. Ultrastructural and immunohistochemical studies established a diagnosis of leiomyosarcoma, despite the rarity of this tumor in children. In situ hybridization with the EBER probe revealed essentially all of the neoplastic cells to be infected with Epstein-Barr virus (EBV). Children with the acquired immunodeficiency syndrome (AIDS) are known to exhibit an increased incidence of smooth muscle tumors associated with EBV. Similar tumors have been reported in EBV-infected patients undergoing therapeutic immunosuppression. This appears to be the first reported case of childhood leiomyosarcoma where the cause of the underlying immunodeficiency was a genetic rather than acquired disorder. The authors conclude that electron microscopy, immunohistochemistry, and other ancillary techniques are essential in the evaluation of unusual tumors in immunocompromised children, whether the cause is hereditary or acquired.
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PMID:Primary leiomyosarcoma of brain in an adolescent with common variable immunodeficiency syndrome. 918 31

Dural and skull-base mesenchymal neoplasms other than meningiomas are rare. We report four such tumors, some of which are uncommon even in nonintracranial sites, in three adults and one child. The adult tumors consisted of a synovial sarcoma of the third ventricle region in a 19-year-old woman, a leiomyoma of the suprasellar region in a 57-year-old woman, and an Epstein-Barr virus (EBV)-associated smooth muscle tumor of the cavernous sinus in a 35-year-old woman with acquired immunodeficiency syndrome (AIDS). The pediatric tumor was an EBV-associated leiomyosarcoma of the left dural transverse sinus in a 14-year-old girl with common variable immunodeficiency syndrome. All tumors were thought to be primary in their dural or skull-base locations. The two EBV-associated smooth muscle tumors in immunocompromised patients expand the locations for EBV-associated smooth muscle tumors to dural and skull-base sites, the synovial sarcoma is unique to the intracranial space, and the sellar leiomyoma represents the third reported sellar smooth muscle tumor.
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PMID:Unusual dural and skull-based mesenchymal neoplasms: a report of four cases. 949 26

The Epstein-Barr virus (EBV) was isolated 40 years ago from cultures of Burkitt lymphoma cells (BL). The tumor was encountered in Africa and exhibited characteristical geographical, clinical and pathological features. Serological studies revealed that the virus is ubiquitous in humans. The primary infection is often accompanied by the syndrome of acute infectious mononucleosis (IM). It can induce malignant proliferation of B lymphocytes in conditions of immunodeficiency. EBV can immortalize B lymphocytes in culture. These cells carry the virus as episomes and express 9 virally encoded proteins. Their immunological recognition constitutes the surveillance which is responsible for the healthy virus carrier state. The main virus reservoir is represented by a low number of resting B lymphocyte which contain the viral genome but do not express its transformation proteins. The viral genome is detectable in all African BLs, in variable proportions of nasopharyngeal carcinoma, Hodgkin's disease, T cell lymphoma, lymphoepithelial like carcinoma, gastric carcinoma and leiomyosarcoma cases. The role of EBV in the genesis of these tumors is unknown.
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PMID:The complexity of the Epstein-Barr virus infection in humans. 955 13

Kaposi's sarcoma and non-Hodgkin's lymphoma were among the earliest recognized manifestations of the acquired immunodeficiency syndrome (AIDS) epidemic. Excluding these two tumors, the overall risk of all other cancers in human immunodeficiency virus (HIV)-infected individuals is similar to that of the general population. However, varying levels of evidence link several additional neoplasms to HIV infection. The evidence is strongest for an association with Hodgkin's disease, with lower relative and absolute risks than for non-Hodgkin's lymphoma. Anogenital intraepithelial neoplasia also appears to be HIV associated, but increases of invasive disease are still uncertain for both cervical and anal cancers. Various studies have suggested associations with testicular seminoma, multiple myeloma, oral cancer, and melanoma, but the data are inconsistent. Leiomyosarcoma and benign leiomyomas have increased in incidence in HIV-infected children but are unusual in HIV-infected adults. Conjunctival carcinoma is seen in HIV-infected individuals in sub-Saharan Africa but it is uncommon in Western countries. Most other cancers do not seem to have increased incidences in HIV infection. The etiologic mechanisms of HIV-related cancer likely differ among these diverse cancers and do not globally increase cancer risk.
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PMID:Association of non-acquired immunodeficiency syndrome-defining cancers with human immunodeficiency virus infection. 970 98

We report an intracranial leiomyosarcoma in the pontine cistern of a 34-year-old woman infected with the human immunodeficiency virus (HIV). The clinical, radiological and pathological data are reviewed. The tumor was Epstein-Barr virus (EBV) positive by in situ hybridization. This case emphasizes that smooth muscle neoplasms arising in the setting of immunocompromise can occur intracranially, and corroborates a hypothesis that EBV coinfection may have a role in development of these tumors.
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PMID:Intracranial leiomyosarcoma in a patient with AIDS. 998 66


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