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Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology
Bilateral macronodular adrenocortical disease as a part of McCune Albright Syndrome (MAS) is the most common cause of endogenous Cushing’s syndrome (CS) in infancy. Adrenocortical tumors causing CS in infancy are extremely rare. We report the case of a girl with CS who presented at age 4 months with...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Galenos Publishing
2012
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3459167/ https://www.ncbi.nlm.nih.gov/pubmed/22985617 http://dx.doi.org/10.4274/Jcrpe.727 |
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author | Dutta, Deep Jain, Rajesh Maisnam, Indira Mishra, Prafulla Kumar Ghosh, Sujoy Mukhopadhyay1, Satinath Chowdhury, Subhankar |
author_facet | Dutta, Deep Jain, Rajesh Maisnam, Indira Mishra, Prafulla Kumar Ghosh, Sujoy Mukhopadhyay1, Satinath Chowdhury, Subhankar |
author_sort | Dutta, Deep |
collection | PubMed |
description | Bilateral macronodular adrenocortical disease as a part of McCune Albright Syndrome (MAS) is the most common cause of endogenous Cushing’s syndrome (CS) in infancy. Adrenocortical tumors causing CS in infancy are extremely rare. We report the case of a girl with CS who presented at age 4 months with obesity and growth retardation. Her 8 am paired cortisol and adrenocorticotropic hormone levels were 49.3 μg/dL and <1 pg/mL, respectively with non-suppressed serum cortisol (41 μg/dL) on high-dose dexamethasone suppression test. Abdominal computed tomography scan demonstrated a 5.3x4.8x3.7 cm homogenous left adrenal mass with distinct borders. Laparotomy following pre-operative stabilization with ketoconazole 200 mg/day, revealed a 7.5x5x4 cm lobulated left adrenal mass with intact capsule and weighing 115 grams. Histopathology showed small round adrenal tumor cells with increased nucleo-cytoplasmic ratio and prominent nucleoli. The cells were separated by fibrous septae without any evidence of vascular or capsular invasion– findings consistent with adrenal adenoma. On the 8(th) post-operative day, after withholding hydrocortisone supplementation, the 8 am cortisol level was <1 μg/dL, suggestive of biochemical remission of CS. The patient improved clinically with a 7.5 kg weight loss over the next 3.5 months. This is perhaps the youngest ever reported infant with CS due to adrenal adenoma. Lack of clinical and biochemical evidence of hyperandrogenism as well as the benign histology in spite of the large tumor size (>7 cm diameter; 115 g) are some of the unique features of our patient. Conflict of interest:None declared. |
format | Online Article Text |
id | pubmed-3459167 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Galenos Publishing |
record_format | MEDLINE/PubMed |
spelling | pubmed-34591672012-10-09 Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology Dutta, Deep Jain, Rajesh Maisnam, Indira Mishra, Prafulla Kumar Ghosh, Sujoy Mukhopadhyay1, Satinath Chowdhury, Subhankar J Clin Res Pediatr Endocrinol Case Report Bilateral macronodular adrenocortical disease as a part of McCune Albright Syndrome (MAS) is the most common cause of endogenous Cushing’s syndrome (CS) in infancy. Adrenocortical tumors causing CS in infancy are extremely rare. We report the case of a girl with CS who presented at age 4 months with obesity and growth retardation. Her 8 am paired cortisol and adrenocorticotropic hormone levels were 49.3 μg/dL and <1 pg/mL, respectively with non-suppressed serum cortisol (41 μg/dL) on high-dose dexamethasone suppression test. Abdominal computed tomography scan demonstrated a 5.3x4.8x3.7 cm homogenous left adrenal mass with distinct borders. Laparotomy following pre-operative stabilization with ketoconazole 200 mg/day, revealed a 7.5x5x4 cm lobulated left adrenal mass with intact capsule and weighing 115 grams. Histopathology showed small round adrenal tumor cells with increased nucleo-cytoplasmic ratio and prominent nucleoli. The cells were separated by fibrous septae without any evidence of vascular or capsular invasion– findings consistent with adrenal adenoma. On the 8(th) post-operative day, after withholding hydrocortisone supplementation, the 8 am cortisol level was <1 μg/dL, suggestive of biochemical remission of CS. The patient improved clinically with a 7.5 kg weight loss over the next 3.5 months. This is perhaps the youngest ever reported infant with CS due to adrenal adenoma. Lack of clinical and biochemical evidence of hyperandrogenism as well as the benign histology in spite of the large tumor size (>7 cm diameter; 115 g) are some of the unique features of our patient. Conflict of interest:None declared. Galenos Publishing 2012-09 2012-09-11 /pmc/articles/PMC3459167/ /pubmed/22985617 http://dx.doi.org/10.4274/Jcrpe.727 Text en © Journal of Clinical Research in Pediatric Endocrinology, Published by Galenos Publishing. http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Dutta, Deep Jain, Rajesh Maisnam, Indira Mishra, Prafulla Kumar Ghosh, Sujoy Mukhopadhyay1, Satinath Chowdhury, Subhankar Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology |
title | Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology |
title_full | Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology |
title_fullStr | Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology |
title_full_unstemmed | Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology |
title_short | Isolated Cushing’s Syndrome in Early Infancy Due to Left Adrenal Adenoma: An Unusual Aetiology |
title_sort | isolated cushing’s syndrome in early infancy due to left adrenal adenoma: an unusual aetiology |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3459167/ https://www.ncbi.nlm.nih.gov/pubmed/22985617 http://dx.doi.org/10.4274/Jcrpe.727 |
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