Adrenocorticotropic Hormone-Independent Bilateral Adrenocortical Macronodular Hyperplasia: A Case Report and Immunohistochemical Studies
A 55-year-old woman developed Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia. Plasma ACTH was undetectable, and was not stimulated by administration of metyrapone, CRH, or insulin. Hypercortisolism was not suppressed by a high dose of dexamethasone,...
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| Veröffentlicht in: | Endocrine Journal 1994, Vol.41(4), pp.429-435 |
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| container_title | Endocrine Journal |
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| creator | KOIZUMI, SHIGEKI BENIKO, MATSUO IKOTA, AKEMI MIZUMOTO, HIROAKI MATSUYA, KUMIKO MATSUDA, AKIRA SAKUMA, SATOSHI MASHIO, YASUO KUNITA, HARUHIKO OKAMOTO, KENZO SASANO, HIRONOBU |
| description | A 55-year-old woman developed Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia. Plasma ACTH was undetectable, and was not stimulated by administration of metyrapone, CRH, or insulin. Hypercortisolism was not suppressed by a high dose of dexamethasone, but was responsive to ACTH. Both adrenal glands were enlarged with a total weight of 200g, and contained multiple nodules composed of two cell types (large clear cells and small compact cells). In immunohistochemical studies, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while that of 3βHSD was observed exclusively in large clear cortical cells. This pattern of expression of steroidogenic enzymes as well as histological and clinical features is considered to be unique to ACTH-independent bilateral adrenocortical macronodular hyperplasia. |
| doi_str_mv | 10.1507/endocrj.41.429 |
| format | Article |
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Plasma ACTH was undetectable, and was not stimulated by administration of metyrapone, CRH, or insulin. Hypercortisolism was not suppressed by a high dose of dexamethasone, but was responsive to ACTH. Both adrenal glands were enlarged with a total weight of 200g, and contained multiple nodules composed of two cell types (large clear cells and small compact cells). In immunohistochemical studies, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while that of 3βHSD was observed exclusively in large clear cortical cells. 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Plasma ACTH was undetectable, and was not stimulated by administration of metyrapone, CRH, or insulin. Hypercortisolism was not suppressed by a high dose of dexamethasone, but was responsive to ACTH. Both adrenal glands were enlarged with a total weight of 200g, and contained multiple nodules composed of two cell types (large clear cells and small compact cells). In immunohistochemical studies, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while that of 3βHSD was observed exclusively in large clear cortical cells. This pattern of expression of steroidogenic enzymes as well as histological and clinical features is considered to be unique to ACTH-independent bilateral adrenocortical macronodular hyperplasia.</description><subject>ACTH-independent bilateral adrenocortical macronodular hyperplasia</subject><subject>Adrenal Cortex - metabolism</subject><subject>Adrenal Cortex - pathology</subject><subject>Adrenocorticotropic Hormone - metabolism</subject><subject>Cushing Syndrome - metabolism</subject><subject>Cushing Syndrome - pathology</subject><subject>Cushing's syndrome</subject><subject>Female</subject><subject>Humans</subject><subject>Hydrocortisone - biosynthesis</subject><subject>Hyperplasia - metabolism</subject><subject>Immunohistochemistry</subject><subject>Middle Aged</subject><subject>Steroidogenic enzymes</subject><issn>0918-8959</issn><issn>1348-4540</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1994</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpNkMuO0zAUhi0EGkphyw7JK3YpdmzHNrtSwbTSICQu68ixT2mqxA62s5g3mMfGMw0Vm2Md_Zcjfwi9pWRDBZEfwLtg43nD6YbX-hlaUcZVxQUnz9GKaKoqpYV-iV6ldCaEMcHZDbpRolZM6BV62LoIPtgQc29DjmHqLd6HOAYP1cE7mMoB8Bl_6geTIZoB_58o61djY_DBzYOJeH8_QZwGk3rzEW_xziTA32EqXmy8w4dxnH049SkHe4LxKf8jz66H9Bq9OJohwZvlXaNfXz7_3O2ru2-3h932rrKCylwp0znecSGJkKppaqg7xjtbN5YpxWTXECYNZ41wxKnOiGMtqeyEULS2WknH1uj9pXeK4c8MKbdjnywMg_EQ5tRKSSmVhc4abS7G8r2UIhzbKfajifctJe0j-nZB33LaFvQl8G5pnrsR3NW-sC767UU_p2x-w1U3jyAH-FdHtWZPlcuo9dVhTyYWG_sLr5GdKg</recordid><startdate>1994</startdate><enddate>1994</enddate><creator>KOIZUMI, SHIGEKI</creator><creator>BENIKO, MATSUO</creator><creator>IKOTA, AKEMI</creator><creator>MIZUMOTO, HIROAKI</creator><creator>MATSUYA, KUMIKO</creator><creator>MATSUDA, AKIRA</creator><creator>SAKUMA, SATOSHI</creator><creator>MASHIO, YASUO</creator><creator>KUNITA, HARUHIKO</creator><creator>OKAMOTO, KENZO</creator><creator>SASANO, HIRONOBU</creator><general>The Japan Endocrine Society</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>1994</creationdate><title>Adrenocorticotropic Hormone-Independent Bilateral Adrenocortical Macronodular Hyperplasia: A Case Report and Immunohistochemical Studies</title><author>KOIZUMI, SHIGEKI ; BENIKO, MATSUO ; IKOTA, AKEMI ; MIZUMOTO, HIROAKI ; MATSUYA, KUMIKO ; MATSUDA, AKIRA ; SAKUMA, SATOSHI ; MASHIO, YASUO ; KUNITA, HARUHIKO ; OKAMOTO, KENZO ; SASANO, HIRONOBU</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c517t-8abd4b4570578662e2b34bc26c38837b6037a4365d0d8ba5f2717b55812c987d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1994</creationdate><topic>ACTH-independent bilateral adrenocortical macronodular hyperplasia</topic><topic>Adrenal Cortex - metabolism</topic><topic>Adrenal Cortex - pathology</topic><topic>Adrenocorticotropic Hormone - metabolism</topic><topic>Cushing Syndrome - metabolism</topic><topic>Cushing Syndrome - pathology</topic><topic>Cushing's syndrome</topic><topic>Female</topic><topic>Humans</topic><topic>Hydrocortisone - biosynthesis</topic><topic>Hyperplasia - metabolism</topic><topic>Immunohistochemistry</topic><topic>Middle Aged</topic><topic>Steroidogenic enzymes</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>KOIZUMI, SHIGEKI</creatorcontrib><creatorcontrib>BENIKO, MATSUO</creatorcontrib><creatorcontrib>IKOTA, AKEMI</creatorcontrib><creatorcontrib>MIZUMOTO, HIROAKI</creatorcontrib><creatorcontrib>MATSUYA, KUMIKO</creatorcontrib><creatorcontrib>MATSUDA, AKIRA</creatorcontrib><creatorcontrib>SAKUMA, SATOSHI</creatorcontrib><creatorcontrib>MASHIO, YASUO</creatorcontrib><creatorcontrib>KUNITA, HARUHIKO</creatorcontrib><creatorcontrib>OKAMOTO, KENZO</creatorcontrib><creatorcontrib>SASANO, HIRONOBU</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Endocrine Journal</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>KOIZUMI, SHIGEKI</au><au>BENIKO, MATSUO</au><au>IKOTA, AKEMI</au><au>MIZUMOTO, HIROAKI</au><au>MATSUYA, KUMIKO</au><au>MATSUDA, AKIRA</au><au>SAKUMA, SATOSHI</au><au>MASHIO, YASUO</au><au>KUNITA, HARUHIKO</au><au>OKAMOTO, KENZO</au><au>SASANO, HIRONOBU</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Adrenocorticotropic Hormone-Independent Bilateral Adrenocortical Macronodular Hyperplasia: A Case Report and Immunohistochemical Studies</atitle><jtitle>Endocrine Journal</jtitle><addtitle>Endocr J</addtitle><date>1994</date><risdate>1994</risdate><volume>41</volume><issue>4</issue><spage>429</spage><epage>435</epage><pages>429-435</pages><issn>0918-8959</issn><eissn>1348-4540</eissn><abstract>A 55-year-old woman developed Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia. 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| subjects | ACTH-independent bilateral adrenocortical macronodular hyperplasia Adrenal Cortex - metabolism Adrenal Cortex - pathology Adrenocorticotropic Hormone - metabolism Cushing Syndrome - metabolism Cushing Syndrome - pathology Cushing's syndrome Female Humans Hydrocortisone - biosynthesis Hyperplasia - metabolism Immunohistochemistry Middle Aged Steroidogenic enzymes |
| title | Adrenocorticotropic Hormone-Independent Bilateral Adrenocortical Macronodular Hyperplasia: A Case Report and Immunohistochemical Studies |
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