Kaposi sarcoma in unusual locations

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Abstract

Kaposi sarcoma (KS) is a multifocal, vascular lesion of low-grade malignant potential that presents most frequently in mucocutaneous sites. KS also commonly involves lymph nodes and visceral organs. This article deals with the manifestation of KS in unusual anatomic regions. Unusual locations of KS involvement include the musculoskeletal system, central and peripheral nervous system, larynx, eye, major salivary glands, endocrine organs, heart, thoracic duct, urinary system and breast. The development of KS within wounds and blood clots is also presented. KS in these atypical sites may prove difficult to diagnose, resulting in patient mismanagement. Theories to explain the rarity and development of KS in these unusual sites are discussed.

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Adrenal pathology in the acquired immune deficiency syndrome.

J. Anders, H Glasgow, Kenneth D Steinsapir … (1985)

Adrenal pathology was examined in 41 autopsied patients with the acquired immune deficiency syndrome. This represents the largest series and the first study with quantitation of adrenal cortical necrosis. In 32 cases clinical data were analyzed for features of adrenal insufficiency. Common clinical findings included vomiting, diarrhea, fever, hypotension, and hyponatremia. None of the 32 patients showed characteristic skin hyperpigmentation. Two patients were suspected premortem to have adrenal insufficiency. In one of these patients, adrenocorticotrophic hormone (ACTH) stimulation resulted in an adequate rise in plasma cortisol values. In the other patient, the baseline plasma cortisol value was elevated and failed to rise significantly after ACTH stimulation. Pathologic findings included widespread lipid depletion, infection by cryptococcus, and acid-fast organisms consistent with Mycobacterium avium-intracellulare, involvement by Kaposi's sarcoma, and necrotizing adrenalitis due to cytomegalovirus (CMV). A point-counting method was used to quantitate adrenal cortical and medullary necrosis. Necrosis due to CMV was greater in the medulla than the cortex. The maximum amount of adrenal cortical necrosis in any case was 70%. The degree of cortical necrosis was less than that usually associated with adrenal insufficiency.

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Imaging manifestations of Kaposi sarcoma.

Carlos Restrepo, Santiago Madrigal Martínez, Julio Lemos-Espinal … (2016)

Kaposi sarcoma (KS) is a low-grade vascular tumor that typically manifests as one of four variants: classic KS, endemic (African) KS, iatrogenic (organ transplant-related) KS, or acquired immunodeficiency syndrome (AIDS)-related KS. Several clinical and epidemiologic differences have been noted among these variants. Classic KS and endemic KS rarely require radiologic evaluation due to their usually chronic course and stability of skin compromise. However, iatrogenic KS and AIDS-related KS, the most common forms of the disease, are frequently disseminated or symptomatic and may thus require imaging studies for both diagnosis and staging. KS is the most common tumor among AIDS patients, affecting a high percentage of these individuals, and is considered to be an AIDS-defining illness. Multiple organs can be involved by AIDS-related KS. KS has been linked with human herpes virus type 8 infection and other cofactors. Although pulmonary, gastrointestinal, and skin involvement by KS has previously been described, this tumor can affect multiple organs, generating a wide spectrum of imaging findings and pathologic correlates. It is important for the radiologist to be familiar with this spectrum of imaging manifestations and corresponding pathologic findings. Copyright RSNA, 2006

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Impact of highly active antiretroviral therapy on the presenting features and outcome of patients with acquired immunodeficiency syndrome-related Kaposi sarcoma.

Giovanni Di Perri, Marco Fasan, … (2003)

The objective of the current study was to evaluate the impact of highly active antiretroviral therapy (HAART) on clinical characteristics of presentation and the natural history of Kaposi sarcoma (KS) in patients already receiving HAART at the time of KS diagnosis. The authors conducted a retrospective cohort study comparing epidemiologic, clinical, and outcome data for 160 patients who were naive to HAART at the time of KS diagnosis (KS-naive) with the corresponding data for 51 patients already receiving HAART at the time of KS diagnosis (KS-HAART). The analysis included all patients with a diagnosis of KS since January 1996 within two Italian cohorts of patients with human immunodeficiency virus. Immunologic and virologic status at the time of KS diagnosis were significantly more favorable in the KS-HAART group than in the KS-naive group. The frequency of cutaneous involvement was similar in both groups, but cutaneous disease was more indolent among KS-HAART patients, with 1 anatomic site of involvement in 9 patients (21%) and less than 10 lesions in 26 patients (60%), compared with 16 patients (12%; P = 0.06) and 47 patients (34%; P = 0.01), respectively, in the KS-naive group. A smaller proportion of KS-HAART patients presented with visceral disease (24% vs. 39%; P = 0.06); in particular, gastrointestinal tract involvement was significantly less frequent among KS-HAART patients (14%) compared with KS-naive patients (28%; P = 0.05). Median survival was not reached in either group, and the 3-year survival rates of KS-HAART patients (64%) and KS-naive patients (78%) were not significantly different. The data from the current study indicate that KS exhibits a less aggressive presentation in patients already receiving HAART compared with patients who are naive to HAART at KS diagnosis. Natural history and outcome do not appear to be influenced by the initiation of HAART before development of KS. Copyright 2003 American Cancer Society.

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Journal

Journal ID (nlm-ta): BMC Cancer

Title: BMC Cancer

Publisher: BioMed Central

ISSN (Electronic): 1471-2407

Publication date Collection: 2008

Publication date (Electronic): 7 July 2008

Volume: 8

Page: 190

Affiliations

[1 ]Department of Pathology, Baystate Medical Center, Tufts University School of Medicine, Springfield, MA, USA

[2 ]Department of Medicine, Division of Hematology Oncology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

Article

Publisher ID: 1471-2407-8-190

DOI: 10.1186/1471-2407-8-190

PMC ID: 2464778

PubMed ID: 18605999

SO-VID: de5ebe50-ad43-4c40-8794-90ceee53f042

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Kaposi sarcoma in unusual locations

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Karger: Oncology

Most cited references 168

Adrenal pathology in the acquired immune deficiency syndrome.

Imaging manifestations of Kaposi sarcoma.

Impact of highly active antiretroviral therapy on the presenting features and outcome of patients with acquired immunodeficiency syndrome-related Kaposi sarcoma.

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