Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias
Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micron...
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| Format: | Article |
| Language: | English |
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Korean Society of Pediatric Endocrinology
2016-03-01
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| Series: | Annals of Pediatric Endocrinology & Metabolism |
| Subjects: | |
| Online Access: | http://e-apem.org/upload/pdf/apem-21-47.pdf |
| _version_ | 1818413669027938304 |
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| author | Hyeoh Won Yu Won Im Cho Hye Rim Chung Keun Hee Choi Sumi Yun Hwan Seong Cho Choong Ho Shin Sei Won Yang |
| author_facet | Hyeoh Won Yu Won Im Cho Hye Rim Chung Keun Hee Choi Sumi Yun Hwan Seong Cho Choong Ho Shin Sei Won Yang |
| author_sort | Hyeoh Won Yu |
| collection | DOAJ |
| description | Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. |
| first_indexed | 2024-12-14T11:06:52Z |
| format | Article |
| id | doaj.art-b482fccf2199439eb1d06b429cc16f73 |
| institution | Directory Open Access Journal |
| issn | 2287-1012 2287-1292 |
| language | English |
| last_indexed | 2024-12-14T11:06:52Z |
| publishDate | 2016-03-01 |
| publisher | Korean Society of Pediatric Endocrinology |
| record_format | Article |
| series | Annals of Pediatric Endocrinology & Metabolism |
| spelling | doaj.art-b482fccf2199439eb1d06b429cc16f732022-12-21T23:04:29ZengKorean Society of Pediatric EndocrinologyAnnals of Pediatric Endocrinology & Metabolism2287-10122287-12922016-03-01211475010.6065/apem.2016.21.1.47644Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasiasHyeoh Won Yu0Won Im Cho1Hye Rim Chung2Keun Hee Choi3Sumi Yun4Hwan Seong Cho5Choong Ho Shin6Sei Won Yang7Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Korea.Department of Orthopaedic Surgery, Seoul National University Bundang Hospital, Seongnam, Korea.Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.http://e-apem.org/upload/pdf/apem-21-47.pdfCushing syndromeMicronodular adrenal diseaseOsteoblastoma |
| spellingShingle | Hyeoh Won Yu Won Im Cho Hye Rim Chung Keun Hee Choi Sumi Yun Hwan Seong Cho Choong Ho Shin Sei Won Yang Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias Annals of Pediatric Endocrinology & Metabolism Cushing syndrome Micronodular adrenal disease Osteoblastoma |
| title | Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias |
| title_full | Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias |
| title_fullStr | Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias |
| title_full_unstemmed | Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias |
| title_short | Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias |
| title_sort | multiple osteoblastomas in a child with cushing syndrome due to bilateral adrenal micronodular hyperplasias |
| topic | Cushing syndrome Micronodular adrenal disease Osteoblastoma |
| url | http://e-apem.org/upload/pdf/apem-21-47.pdf |
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