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Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma

CONTEXT: Classic Kaposi sarcoma (KS), also known as Mediterranean KS, affects immunocompetent patients and is usually limited to the skin, without profound organ involvement. We report an exceptional case of a primary adrenal classic KS. CASE DESCRIPTION: A left adrenal incidentaloma was fortuitousl...

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Autores principales: de Risi-Pugliese, Tullia, Genc, Seray, Bertherat, Jérôme, Larousserie, Frédérique, Bollet, Marc, Bassi, Clotilde, Dupin, Nicolas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5686574/
https://www.ncbi.nlm.nih.gov/pubmed/29264526
http://dx.doi.org/10.1210/js.2017-00149
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author de Risi-Pugliese, Tullia
Genc, Seray
Bertherat, Jérôme
Larousserie, Frédérique
Bollet, Marc
Bassi, Clotilde
Dupin, Nicolas
author_facet de Risi-Pugliese, Tullia
Genc, Seray
Bertherat, Jérôme
Larousserie, Frédérique
Bollet, Marc
Bassi, Clotilde
Dupin, Nicolas
author_sort de Risi-Pugliese, Tullia
collection PubMed
description CONTEXT: Classic Kaposi sarcoma (KS), also known as Mediterranean KS, affects immunocompetent patients and is usually limited to the skin, without profound organ involvement. We report an exceptional case of a primary adrenal classic KS. CASE DESCRIPTION: A left adrenal incidentaloma was fortuitously discovered on a computed tomography scan performed for chest pain in a 60-year-old man. Magnetic resonance imaging showed a heterogeneous left adrenal nodule enhanced by gadolinium injection. Adrenalectomy revealed a massive spindle cell infiltrate of the adrenal gland that was positive for CD31, CD34, and herpes virus 8 (HHV8) on immunohistochemistry, allowing for the diagnosis of KS. The explorations revealed no immunodeficiency or other involvement of KS. Four months later, another nodular lesion appeared on the right adrenal gland, and 2.5 years later, two nodular angiomatous KS lesions had appeared on the right foot. The evolution was indolent, and no complementary treatment of KS was required at 3 years after the diagnosis. CONCLUSIONS: Adrenal involvement of KS is rare, eventually observed in AIDS-KS. The present case is, to the best of our knowledge, the first report of primary isolated adrenal classic KS. KS should be considered in the etiology of adrenal incidentaloma, especially if the patient has epidemiological risk factors for HHV8 infection, mainly, but not exclusively, in the context of immunodeficiency.
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spelling pubmed-56865742017-12-20 Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma de Risi-Pugliese, Tullia Genc, Seray Bertherat, Jérôme Larousserie, Frédérique Bollet, Marc Bassi, Clotilde Dupin, Nicolas J Endocr Soc Case Reports CONTEXT: Classic Kaposi sarcoma (KS), also known as Mediterranean KS, affects immunocompetent patients and is usually limited to the skin, without profound organ involvement. We report an exceptional case of a primary adrenal classic KS. CASE DESCRIPTION: A left adrenal incidentaloma was fortuitously discovered on a computed tomography scan performed for chest pain in a 60-year-old man. Magnetic resonance imaging showed a heterogeneous left adrenal nodule enhanced by gadolinium injection. Adrenalectomy revealed a massive spindle cell infiltrate of the adrenal gland that was positive for CD31, CD34, and herpes virus 8 (HHV8) on immunohistochemistry, allowing for the diagnosis of KS. The explorations revealed no immunodeficiency or other involvement of KS. Four months later, another nodular lesion appeared on the right adrenal gland, and 2.5 years later, two nodular angiomatous KS lesions had appeared on the right foot. The evolution was indolent, and no complementary treatment of KS was required at 3 years after the diagnosis. CONCLUSIONS: Adrenal involvement of KS is rare, eventually observed in AIDS-KS. The present case is, to the best of our knowledge, the first report of primary isolated adrenal classic KS. KS should be considered in the etiology of adrenal incidentaloma, especially if the patient has epidemiological risk factors for HHV8 infection, mainly, but not exclusively, in the context of immunodeficiency. Endocrine Society 2017-05-05 /pmc/articles/PMC5686574/ /pubmed/29264526 http://dx.doi.org/10.1210/js.2017-00149 Text en Copyright © 2017 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Reports
de Risi-Pugliese, Tullia
Genc, Seray
Bertherat, Jérôme
Larousserie, Frédérique
Bollet, Marc
Bassi, Clotilde
Dupin, Nicolas
Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma
title Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma
title_full Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma
title_fullStr Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma
title_full_unstemmed Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma
title_short Classic Kaposi Sarcoma: An Exceptional Cause of Adrenal Incidentaloma
title_sort classic kaposi sarcoma: an exceptional cause of adrenal incidentaloma
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5686574/
https://www.ncbi.nlm.nih.gov/pubmed/29264526
http://dx.doi.org/10.1210/js.2017-00149
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